Comprehensive Textbook of Echocardiography Volume 2 PDF

Comprehensive Vol.
Textbook of Echocardiography
Comprehensive Vol.
Textbook of Echocardiography
Editor
Navin C Nanda MD
Distinguished Professor of Medicine and Cardiovascular Disease and
Director, Heart Station/Echocardiography Laboratories
University of Alabama at Birmingham and the University of
Alabama Health Services Foundation
The Kirklin Clinic, Birmingham, Alabama, USA
President, International Society of Cardiovascular Ultrasound
Under the Aegis of

The International Society of Cardiovascular Ultrasound
and
The Indian Academy of Echocardiography
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Comprehensive Textbook of Echocardiography (Vol. 2)

First Edition: 2014
ISBN 978-93-5090-634-7
Printed at:
Dedicated to
My late parents
Balwant Rai Nanda MD and Mrs Maya Vati Nanda
My wife
Kanta Nanda MD
Our children
Nitin Nanda, Anita Nanda Wasan MD and Anil Nanda MD
Their spouses Sanjeev Wasan MD and Seema Tailor Nanda, and
our grandchildren Vinay and Rajesh Wasan, and Nayna and Ria Nanda
Contributors
Masood Ahmad M
D FRCP (C) FACP FACC Manreet Basra MBBS Monodeep Biswas MBBS MD
FAHA FASE Professor of Medicine Division of Cardiology
Division of Cardiology University at Buffalo School of Medicine Geisinger-Community Medical
Department of Internal Medicine and Biological Sciences Center, and The Wright Center for
University of Texas Medical Branch New York, USA Graduate Medical Education
Galveston Texas, USA Scranton, Pennsylvania, USA
Charles E Beale MD
Dheeraj Arora DNB PDCC MNAMS Department of Medicine Steven Bleich MD
Institute of Critical Care and Anesthesia Division of Cardiovascular Diseases Department of Medicine
Stony Brook University Medical Center Division of Internal Medicine
Medanta The Medicity
Stony Brook, New York, USA University of Alabama at Birmingham
Gurgaon, Haryana, India
Birmingham, Alabama, USA
Roy Beigel MD
Mohammad Al-Admawi MD
The Heart Institute, Cedars Sinai Medical O Julian Booker MD
King Faisal Specialist Hospital and
Center, Los Angeles, California, USA Division of Cardiovascular Disease
Research Center The Leviev Heart Center Sheba Medical University of Alabama at Birmingham
Heart Center Center, Affiliated to the Sackler Birmingham, Alabama, USA
Riyadh, Saudi Arabia School of Medicine
Tel Aviv University, Tel Aviv, Israel Eduardo Bossone MD PhD FCCP FESC FACC
Bader Almahdi MD Via Principe Amedeo Lauro (AV), Italy
King Faisal Specialist Hospital and Ricardo Benenstein MD Heart Department, University of
Research Center Echocardiography and Vascular Lab Salerno, “Scuola Medica Salernitana”
Heart Center Assistant Professor of Medicine Salerno, Italy
Riyadh, Saudi Arabia New York University School of Medicine Department of Cardiac Surgery IRCCS
New York, New York, USA Policlinico San Donato, Milan, Italy
Ahmed Almomani MBBS
Division of Cardiology Kunal Bhagatwala MBBS Luis Bowen MD
Department of Medicine Division of Cardiovascular Disease Department of Medicine
University of Texas Medical Branch University of Alabama at Birmingham University of Alabama at Birmingham
Galveston, Texas, USA Birmingham, Alabama, USA Birmingham, Alabama, USA
Neeraj Awasthy FNB Aditya Bharadwaj MD Gerald Buckberg MD

Fortis Escorts Heart Institute Department of Cardiology Department of Cardiothoracic Surgery
Loma Linda University and David Geffen School of Medicine
New Delhi, India
VA Medical Centers University of California-Los Angeles
Loma Linda, California, USA Los Angeles, California, USA
Rula Balluz MD MPH
Heart Center, St Christopher’s Hospital Michael J Campbell MD
Aarti H Bhat MBBS
for Children and Department of Pediatrics
Assistant Professor
Section of Cardiology Division of Pediatric Cardiology
Division of Pediatric Cardiology
Department of Pediatrics, Drexel Seattle Children’s Hospital and Duke University Medical Center
University College of Medicine University of Washington Durham, North Carolina, USA
Philadelphia, Pennsylvania, USA Seattle, Washington, USA
Premindra PAN Chandraratna MD
Piers Barker MD Nicole Bhave MD FRCP FACC
Department of Pediatrics University Health Network Professor Emeritus
Division of Pediatric Cardiology Toronto General Hospital Division of Cardiology
Duke University Medical Center University of Toronto UC-Irvine School of Medicine
Durham, North Carolina, USA Toronto, Ontario, Canada Irvine, California, USA
viii Comprehensive Textbook of Echocardiography
Leon H Charney Michele D’ Alto MD PhD Daniel Forsha MD

Division of Cardiology Department of Cardiology Department of Pediatrics
New York University Medical Center Second University of Naples: Division of Pediatric Cardiology
New York, New York, USA Monaldi Hospital, Naples, Italy Duke University Medical Center
Durham, North Carolina, USA
Farooq A Chaudhry M
D FACP FACC David Daly MD
FASE FAHA Department of Medicine Gary P Foster MD
Professor of Medicine University of Alabama at Birmingham Department of Cardiology
Director, Echocardiography Laboratories Birmingham, Alabama, USA Loma Linda University and
Associate Director, Mount Sinai Heart VA Medical Centers, Loma Linda
Network, Icahn School of Medicine at Hisham Dokainish M
D FRCPC California, USA
Mount Sinai, Zena and Michael A Wiener Associate Professor of Medicine
Cardiovascular Institute and McMaster University Leon J Frazin MD
Marie-Josée and Henry R Kravis Center Director of Echocardiography and
for Cardiovascular Health University of Illinois Hospital & Health
Medical Diagnostic Units Science System
New York, New York, USA Hamilton Health Sciences Jesse Brown VA Medical Center
Hamilton, Ontario, Canada Chicago, Illinois, USA
Preeti Chaurasia MD
Division of Cardiovascular Disease Maximiliano German
University of Alabama at Birmingham Naveen Garg MBBS Dip. Cardiology
Amado Escañuela MD
Birmingham, Alabama Fellow, Noninvasive Cardiac Lab
Division of Cardiovascular Disease
Indraprastha Apollo Hospitals
University of Alabama at Birmingham
Reema Chugh MD FACC Birmingham, Alabama, USA
New Delhi, India
Consultant in Cardiology/Specialist in
Adult Congenital Heart Disease and Luna Gargani MD
Bahaa M Fadel MD
Heart Disease in Pregnancy Institute of Clinical Physiology
Kaiser Permanente Medical Center King Faisal Specialist Hospital and
National Research Council
Panorama City, California, USA Research Center
Pisa, Italy
Heart Center
Krishnaswamy Chandrasekaran MD Riyadh, Saudi Arabia
Eleonora Gashi DO MPhil
Mayo Clinic, Scottsdale, Arizona, USA
Abid Ali Fakhri MD Senior Cardiology Fellow
Rochester, Minnesota, USA
Fellow, Division of Cardiology Lenox Hill Hospital
Allegheny General Hospital Non-Invasive Cardiology
Michael Chen MD
Pittsburgh, Pennsylvania, USA New York, New York, USA
University of Washington
Seattle, Washington DC, USA
Francesco Faletra MD Robert P Gatewood Jr MD FACC
HK Chopra MD Division of Cardiology Chief of Cardiac Services
Moolchand City Hospital Fondazione Cardiocentro Ticino Kaleida Heath; Clinical Associate
New Delhi, India Lugano, Switzerland Professor of Medicine
University at Buffalo School of
Cecil Coghlan MD Francesco Ferrara MD Medicine and Biological Sciences
Division of Cardiovascular Disease Heart Department, University of Salerno Buffalo Cardiology and Pulmonary
University of Alabama at Birmingham “Scuola Medica Salernitana” Associates, Main Street Williamsville
Birmingham, Alabama, USA Salerno, Italy New York, USA
Department of Internal Medicine and
Neil L Coplan MD FACC Cardiovascular Sciences Shuping Ge MD FAAP FACC FASE
Director, Division of Clinical Cardiology University “Federico II” of Naples Chief, Section of Cardiology
Program Director, Cardiovascular Naples, Italy St Christopher’s Hospital for Children
Fellowship, Lenox Hill Hospital Associate Professor of Pediatrics
New York, USA Brandon Fornwalt MD PhD Drexel University College of Medicine
Assistant Professor of Pediatrics Philadelphia, Pennsylvania, USA
David Cosgrove MD Department of Pediatrics Acting Chair, Pediatric Cardiology
Honorary Consultant University of Kentucky Deborah Heart and Lung Center
Imperial and King's Colleges, London, UK Lexington, Kentucky, USA Browns Mills, New Jersey, USA
Contributors ix
Gopal Ghimire MD DM MRCP Donald Hagler MD Rachel Hughes-Doichev MD FASE

Division of Cardiovascular Diseases Mayo Clinic Temple University School of Medicine
University of Alabama at Birmingham Rochester, Minnesota, USA Pittsburgh, Pennsylvania, USA
Birmingham, Alabama, USA
Stephanie El-Hajj MD Arzu Ilercil MD
Nina Ghosh MD Department of Internal Medicine Associate Professor of Medicine
Division of Cardiovascular Medicine Louisiana State University Health Department of Cardiovascular Sciences
Sciences Center University of South Florida
Brigham and Women’s Hospital
Baton Rouge, Louisiana, USA Tampa, Florida, USA
Harvard Medical School
Francis Street
Kamran Haleem MD Trevor Jenkins MD
Boston, Massachusetts, USA
Yale University Harrington Heart and
New Haven, Connecticut, USA Vascular Institute
Edward Gill MD University Hospital Case
Professor of Medicine and Medical Center, Cleveland
Dan G Halpern MD
Cardiology, University of Washington Ohio, USA
St Luke’s-Roosevelt Hospital Center
Seattle, Washington DC, USA
Columbia University, College of
Physicians and Surgeons
Madhavi Kadiyala MD
Rohit Gokhale MBBS New York, New York, USA Saint Francis Hospital, Roslyn
University at Buffalo New York, USA
Buffalo, New York, USA Rachel Harris MD MPH
Arshad Kamel MD
Morehouse School of Medicine
Aasha S Gopal MS MD FACC FAHA FASE Department of Medicine
Section of Cardiology
University of Alabama at Huntsville
Associate Professor of Medicine Assistant Professor
Huntsville, Alabama, USA
Stony Brook University Echo Lab Co-Director
Stony Brook, New York, USA Grady Memorial Hospital
Atlanta, Georgia, USA Abdallah Kamouh MD
Director, Advanced Echocardiography
University of Buffalo
St Francis Hospital, Washington Blvd
Buffalo, New York, USA
Roslyn, New York, USA Christine Henri MD
Department of Cardiology
Poonam Malhotra Kapoor MD
Willem Gorissen Heart Valve Disease Clinic
CHU Sart Tilman, University of All India Institute of
Clinical Market Manager Medical Sciences
Toshiba Medical Systems Europe Liège, Belgium
New Delhi, India
Zoetermeer, The Netherlands
Julien IE Hoffman MD
Kanwal K Kapur MD
Department of Pediatrics
Luis Gruberg MD FACC DM Cardiology, Sr Consultant and Chief
University of California
Department of Medicine Noninvasive Cardiology
San Francisco, California, USA
Division of Cardiovascular Diseases Indraprastha Apollo Hospitals
Stony Brook University Medical Center New Delhi, India
Brian D Hoit MD Department of Noninvasive Cardiology
Stony Brook, New York, USA
Director of Echocardiography Indraprastha Apollo Hospitals
Harrington Heart & Vascular Center New Delhi, India
Rakesh Gupta MD University Hospitals of Cleveland
JROP Healthcare Texas, USA Nidhi M Karia MBBS
New Delhi, India
Steven J Horn MD FACC FASE FASNC University of Alabama at Birmingham
Fadi G Hage MD SUNY Buffalo Birmingham, Alabama, USA
Division of Cardiovascular Disease Buffalo, New York, USA
University of Alabama at Birmingham Jarosław D Kasprzak MD
Birmingham, Alabama, USA Ming Chon Hsiung MD Chair and Department of Cardiology
Section of Cardiology, Birmingham Cardiologist Biegański Hospital
Veteran’s, Administration Medical Center Cheng Hsin General Hospital Medical University of Lodz
Birmingham, Alabama, USA Taipei, Taiwan Lodz, Poland
x Comprehensive Textbook of Echocardiography
Martin G Keane MD FACC FAHA FASE Arthur J Labovitz MD Gerald R Marx MD

Professor of Medicine Professor of Medicine Associate Professor
Cardiology Section Chair, Department of Harvard School of Medicine
Director of Echocardiography Cardiovascular Sciences Senior Associate Cardiology
Temple University School of Medicine University of South Florida Boston Children’s Hospital
Parkinson Pavilion, Suite Tampa, Florida, USA Boston, Massachusetts, USA
North Broad Street, Philadelphia
Pennsylvania, USA Jennifer K Lang MD Wilson Mathias Jr MD
University at Buffalo Heart Institute (InCor)
Tuğba Kemaloğlu Öz MD Buffalo, New York, USA The University of São Paulo School of
Division of Cardiovascular Disease Medicine and Fleury Group
University of Alabama at Birmingham Roberto M Lang MD São Paulo, Brazil
Birmingham, Alabama, USA University of Chicago
Medical Center Angele A A Mattoso MD
Anant Kharod MD Chicago, Illinois, USA Heart Institute (InCor)
Department of Medicine The University of São Paulo School of
University of Alabama at Birmingham Fabrice Larrazet MD PhD Medicine, São Paulo, Brazil and
Birmingham, Alabama, USA Department of Cardiology Santa Izabel Hospital, Salvador, Bahia
HÔpital Saint Camille
Jennifer Kiessling MD Bry sur Marne, France Sula Mazimba MD MPH
Division of Cardiovascular Division of Cardiovascular
Diseases, University of Steve W Leung MD Disease University of Alabama at
Alabama at Birmingham Assistant Professor of Medicine Birmingham
Birmingham, Alabama, USA Division of Cardiovascular Medicine Birmingham, Alabama, USA
University of Kentucky
Allan L Klein M
D FRCP(C) FACC Lexington, Kentucky, USA Anjlee M Mehta MD
FAHA FASE Fellow, Division of Cardiology
Director, CV Imaging Research and Sachin Logani MD Dartmouth-Hitchcock Heart and
Pericardial Center Department of Medicine Vascular Center
Professor of Medicine, Cleveland Clinic Division of Cardiovascular Diseases Lebanon, New Hampshire, USA
Heart and Vascular Institute Stony Brook University
Department of Cardiovascular Medicine Medical Center, Stony Brook Kruti Jayesh Mehta MBBS PGDCC
Cleveland, Ohio, USA New York, USA Division of Cardiovascular Disease
Smadar Kort MD FACC FASE Javier López MD PhD Birmingham, Alabama, USA
Professor of Medicine Hospital Clinico Universitario de
State University of New York Valladolid, Spain Yatin Mehta MD MNAMS FRCA FAMS
Stony Brook Director FIACTA FTEE FICCM
Non Inavasive Cardiac Imaging Director Judy R Mangion MD Institute of Critical
Echocardiography Diretor Division of Cardiovascular Medicine Care and Anesthesia
Valve Center, Stony Brook Medicine Brigham and Women’s Hospital Medanta The Medicity
Stony Brook, New York, USA Harvard Medical School Gurgaon, Haryana, India
Francis Street, Boston
Itzhak Kronzon M
D FASE FACC FACP Massachusetts, USA Julien Magne PhD
FESC FAHA Department of Cardiology
Professor of Cardiology CN Manjunath MD DM Heart Valve Disease Clinic
Hofstra University Director, Professor and Head CHU Sart Tilman, University of
North Shore LIJ, School of Medicine Department of Cardiology Liège, Belgium
Chief of Noninvasive Cardiac Imaging Sri Jayadeva Institute of Cardiovascular
Lenox Hill Hospital Sciences and Research Andrew P Miller MD
Noninvasive Cardiology Bannergutta Road Cardiovascular Associates
New York, New York, USA Bengaluru, Karnataka, India Birmingham, Alabama, USA
Contributors xi
Dilbahar S Mohar MD Ryozo Omoto MD Eugenio Picano MD PhD

Division of Cardiology Professor Emeritus, Saitama Medical Institute of Clinical Physiology
UC-Irvine School of Medicine University National Research Council
Irvine, California, USA Honorary Director, Saitama Medical Pisa, Italy
University Hospital
Caroline Morbach MD Moro-Hongou, Moroyama
Luc A Pierard MD PhD
Yale University Iruma-Gun, Saitama, Japan
New Haven, Connecticut, USA
Heart Valve Disease Clinic
Ahmad S Omran MD FACC FESC FASE
CHU Sart Tilman, University of
Hoda Mojazi-Amiri MD Consultant Cardiologist
Liège, Belgium
Loma Linda University Medical Center Head, Non-Invasive Cardiology Lab
Loma Linda, California , USA King Abdulaziz Cardiac Center–Riyadh
Eisenhower Medical Center Health Affairs–Ministry of National Guard Atif N Qasim MD MSCE
Rancho Mirage, California, USA Kingdom of Saudi Arabia Assistant Professor of Medicine
University of California
Nagaraja Moorthy MD DM Jatinder Singh Pabla BSc (Hons) MBBS San Francisco, California, USA
Assistant Professor MRCP
Department of Cardiology Department of Cardiovascular Medicine Anita Radhakrishnan MD
Sri Jayadeva Institute of Cardiovascular Northwick Park Hospital, Harrow, UK Fellow, Division of Cardiology
Sciences and Research Allegheny General Hospital
Bengaluru, Karnataka, India Shyam Padmanabhan MD Pittsburgh, Pennsylvania, USA
Hirohiko Motoki MD University of Alabama at Birmingham Peter S Rahko MD
Cardiovascular Research Birmingham, Alabama, USA
Professor of Medicine
Imaging Fellow, Cleveland Clinic Division of Cardiovascular Medicine
Foundation, Cleveland, Ohio, USA Ramdas G Pai MD
Department of Medicine, University of
Professor of Medicine
Wisconsin School of Medicine and Public
Bernhard Mumm Loma Linda University
Health, Madison, Wisconsin, USA
President and COO Medical Center
TomTec Imaging Systems Loma Linda, California, USA
GmbH, Edisonstr
Rajesh Ramineni MD
Unterschleissheim, Germany Natesa G Pandian MD University of Texas Medical Branch
Professor, Tufts University School of Galveston, Texas, USA
Rachel Myers RDCS Medicine, Director, Heart Valve Center
Allegheny General Hospital Co-Director, Cardiovascular JRTC Roelandt MD
Pittsburgh, Pennsylvania, USA Imaging Center Professor of Cardiology
Director, Cardiovascular Ultrasound Honorary Chairman, Thoraxcentre
Navin C Nanda MD Research, Tufts Medical Center Erasmus University Medical
Boston, Massachusetts, USA
Distinguished Professor of Medicine and Centre, Rotterdam
Cardiovascular Disease and The Netherlands
Director, Heart Station/Echocardiography Satish K Parashar MD
Laboratories, University of Alabama at Metro Heart Institute
Lindsay Rogers MD
Birmingham and the University of New Delhi, India
Heart Center, St Christopher’s
Alabama Health Services Foundation
Hospital for Children and
The Kirklin Clinic, Birmingham David A Parra MD
Section of Cardiology
Alabama, USA, President, International Department of Pediatrics
Division of Pediatric Cardiology Department of Pediatrics
Society of Cardiovascular Ultrasound
Vanderbilt University Medical Center Drexel University, College of Medicine
Nashville, Tennessee, USA Philadelphia, Pennsylvania, USA
Elizabeth Ofili MD MPH FACC
Morehouse School of Medicine
Chief of Section of Cardiology Ashvin K Patel MD Asad Ullah Roomi MD
Associate Dean of Clinical Research University of Wisconsin School of Prince Sultan Cardiac Center
Professor of Medicine Medicine and Public Health Military Hospital Riyadh
Atlanta, Georgia, USA Madison, Wisconsin, USA Riyadh, Kingdom of Saudi Arabia
xii Comprehensive Textbook of Echocardiography
José Alberto San Román MD PhD FESCC Teresa Sevilla MD Robert J Siegel MD
Hospital Clínico, Universitario de Hospital Clínico Universitario de The Heart Institute, Cedars Sinai
Valladolid, Spain Valladolid, Spain Medical Center, Beverly Boulevard
Los Angeles, California, USA
Emanuele Romeo MD James Seward MD
Department of Cardiology Satinder P Singh MD FCCP
Mayo Clinic
Second University of Naples Professor, Radiology and
Rochester, Minnesota, USA
Monaldi Hospital, Naples, Italy Medicine—Cardiovascular Disease
Chief, Cardiopulmonary Radiology
Benoy Nalin Shah BSc

(Hons) MBBS MRCP Chief, 3D Lab, Director, Cardiac CT
Utpal N Sagar MD
Department of Cardiovascular Medicine Director, Combined Cardiopulmonary
Advanced Cardiovascular Imaging Fellow
Northwick Park Hospital and Abdominal Imaging
Heart and Vascular Institute Fellowship Program
Harrow, UK
Department of Cardiovascular Medicine University of Alabama at Birmingham
Cardiovascular Biomedical
Cleveland, Ohio, USA Birmingham, Alabama, USA
Research Unit
Royal Brompton Hospital
Hamid Reza Salehi MD Siddharth Singh MD MS
London, UK
Research Fellow in Echocardiography Division of Cardiovascular Disease
National Heart and Lung Institute
Tufts Medical Center University of Alabama at Birmingham
Imperial College
Boston, Massachusetts, USA Birmingham, Alabama, USA
London, UK
Ivan S Salgo MD MSc Chittur A Sivaram MD
Philips Healthcare
Chetan Shenoy MBBS David Ross Boyd Professor
Andover, Massachusetts, USA Fellow in Cardiovascular Disease Vice Chief of Cardiovascular Section
Tufts Medical Center Associate Dean for Continuing
Giovanni Di Salvo MD Boston, Massachusetts, USA Professional Development
King Faisal Specialist University of Oklahoma
Hospital and Research Center Mark V Sherrid MD Health Sciences Center
Director, Echocardiography Laboratory Oklahoma City, Oklahama, USA
Heart Center
Riyadh, Saudi Arabia Roosevelt Division
Sushilkumar K Sonavane MD
Program Director, Hypertrophic
Assistant Professor
Muhamed Saric MD PhD Cardiomyopathy Program
Cardiopulmonary Radiology
Director, Echocardiography Lab St. Luke's-Roosevelt Hospital Center
Associate Professor of Medicine Professor, Clinical Medicine
Department Radiology
New York University Columbia University, College of Birmingham, Alabama, USA
Langone Medical Center Physicians and Surgeons
New York, New York, USA New York, New York, USA Vincent L Sorrell MD
Anthony N DeMaria Professor of
Nelson B Schiller MD Savitri Shrivastava MD DM FACC FAMS Medicine, Assistant Chief
Professor of Medicine Director Pediatric and Congenital Heart Division of Cardiovascular Medicine
University of California Diseases Fortis Escorts Heart Institute University of Kentucky
San Francisco New Delhi, India Lexington, Kentucky, USA
UCSF Division of Cardiology
Parnassus Avenue Jonathan H Soslow MD
Peter Sidarous MD
San Francisco, California, USA Department of Pediatrics
Research Associate
Division of Pediatric Cardiology
UC-Irvine School of Medicine
Roxy Senior MD DM FRCP FESC FACC Vanderbilt University Medical Center
Irvine, California, USA
Cardiovascular Biomedical Nashville, Tennessee, USA
Research Unit, Royal
Brompton Hospital, London, UK Khadija Siddiqui DO Anna Agnese Stanziola MD
National Heart and Lung Institute Division of Cardiology Clinical and Surgery Department
Imperial College, London, UK Department of Medicine Division of Respiratory Medicine
Department of Cardiovascular Medicine University of Texas Medical Branch University “Federico II”of Naples
Northwick Park Hospital, Harrow, UK Galveston, Texas, USA Naples, Italy
Contributors xiii
Sharath Subramanian MD George Thomas MD Isidre Vilacosta MD PhD FESCC

Medical College of Wisconsin Department of Cardiology Hospital Clínico
Milwaukee, Wisconsin, USA Saraf Hospital, Kochi, Kerala, India San Carlos
Lissa Sugeng MD Madrid, Spain
Wendy Tsang MD
Associate Professor
University Health Network, Toronto Victor Vacanti MD
Director of Yale Echo Lab and
YRCG Echo Corelab General Hospital, University of Toronto
University of Buffalo
Section of Cardiovascular Medicine Toronto, Ontario, Canada
Buffalo, New York, USA
Division of Medicine
Yale University School of Medicine Jeane M Tsutsui MD
New Haven, Connecticut, USA Heart Institute (InCor), The University of Leon Varjabedian MD
São Paulo School of Medicine and Fleury University of Buffalo
Jie Sun MD PhD Group, São Paulo, Brazil Buffalo, New York, USA
Heart Center, St Christopher’s Hospital
for Children and Section of Cardiology Teena Tulaba RDCS Karina Wierzbowska-Drabik MD
Department of Pediatrics Allegheny General Hospital
Drexel University College of Medicine Chair and Department of
Pittsburgh, Pennsylvania, USA Cardiology
Philadelphia, Pennsylvania, USA
Biegański Hospital
Aylin Sungur MD Padmini Varadarajan MD
Medical University of Lodz
Division of Cardiovascular Disease Department of Cardiology
Lodz, Poland
University of Alabama at Birmingham Loma Linda University and VA Medical
Birmingham, Alabama, USA Centers, Loma Linda, California, USA
Timothy D Woods MD
Azhar Supariwala MD Mahdi Veillet-Chowdhury MD Associate Professor of
Division of Cardiology Stony Brook University Medical Center Medicine and Radiology
St Luke’s-Roosevelt Hospital Center Health Sciences Center Medical College of Wisconsin
New York, New York, USA
Stony Brook, New York, USA Cardiology Division
Pooja Swamy MD Milwaukee, Wisconsin, USA
Colette Veyrat MD
Loma Linda University and VA Centre National de la Recherche Siu-Sun Yao MD FACC
Medical Centers Scientifique Honorary Researcher
Division of Cardiology
Loma Linda, California, USA Department of Cardiovascular Medicine
Valley Health System
L’Institut Mutualiste de Montsouris
Kiyoshi Tamura PhD Ridgewood
Boulevard Jourdan, Paris Cedex, France
Hitachi Aloka Medical, Ltd. New Jersey, USA
Imai, Ome-Shi, Tokyo, Japan IB Vijayalakshmi MD DM (Card) FICC
FIAMS FIAE FICP FCSI FAMS DSc Elisa Zaragoza-Macias MD MPH
Rohit Tandon MBBS MD
Professor of Pediatric Cardiology Cardiovascular
Dayanand Medical College and
Hospital Unit, Hero DMC Sri Jayadeva Institute of Cardiovascular Diseases Fellow
Heart Institute Sciences and Research University of Washington
Ludhiana, Punjab, India Bengaluru, Karnataka, India Seattle, Washington, USA
Preface
Monumental strides have occurred in the evolution of echocardiography since its first introduction in the 1950s.
It began with A-mode and M-mode echocardiography which progressed to real time two-dimensional echocardiography
in the 1970s after a hiatus of several years. This development completely revolutionized the field of noninvasive cardiac
imaging; and within a few years of its introduction, there were hardly any cardiology divisions in any hospital anywhere in
the world which did not own an ultrasound machine. The next few years saw the development of continuous and pulsed
wave Doppler and color Doppler flow imaging which provided assessment of cardiac hemodynamics to supplement
the structural information obtained using two-dimensional echocardiography. Other advances rapidly followed
or occurred concurrently. These included stress echocardiography, transesophageal echocardiography, contrast
echocardiography and tissue Doppler and velocity vector imaging. More recently, further innovations were introduced
such as live/real time three-dimensional echocardiography and both two-and three-dimensional speckle tracking
echocardiography which have obviated some of the limitations of the previous techniques and have further enhanced
the clinical usefulness of echocardiography. To this day, echocardiography represents the most useful and most cost-
effective noninvasive modality available for the assessment of various cardiac disease entities. The development of
allied noninvasive technologies like magnetic resonance imaging and computed tomography has further added to
the information provided by echocardiography and are useful and important additions to the armamentarium of the
cardiologists and other patient care providers in the comprehensive assessment and management of cardiac disease.
The aim of the current book is to provide an overview of the subject of clinical echocardiography as it is practiced
to-day. Given the many advances that have not only been recently introduced but are also ongoing in this field it would
be very difficult for anyone to realistically come up with a comprehensive book on echocardiography but an attempt has
been made to cover as many topics as possible in this book. In addition, the supplementary information provided by
magnetic resonance imaging and computed tomography is also included in this book.
The book consists of a total of 85 chapters organized into seven sections. The first section deals with the basics of
ultrasound, Doppler, speckle tracking, three-dimensional echocardiography and instrumentation. A short history
of echocardiography and Doppler are also included in this section. The second section consists of various aspects
of echocardiography and ultrasound examination. M-mode and two- and three-dimensional transthoracic and
transesophageal examination, nonstandard planes, various aspects of Doppler assessment including tissue Doppler,
velocity vector and speckle tracking imaging, assessment of endothelial function, contrast echocardiography for
evaluation of left ventricular endocardial border opacification and myocardial perfusion, transpharyngeal echo,
epiaortic echocardiography and both intracardiac and intravascular ultrasound are dealt with in this section. In
addition, examination with a small hand-held ultrasound system, peripheral ultrasound, echocardiographic artifacts,
quantification techniques in echocardiography and echocardiography training form a part of this section. Valvular heart
disease is covered in the next section. It deals with evaluation of mitral valve disease, mitral regurgitation, aortic stenosis
including assessment of low gradient stenosis with preserved left ventricular function, aortic regurgitation, aortic disease,
tricuspid and pulmonary valves, pulmonary hypertension, infective endocarditis and prosthetic valves. Rheumatic heart
disease is also included in this section. Section 4 deals with two- and three-dimensional echocardiographic assessment
of systolic and diastolic function of both left and right ventricles. Newer aspects of structure and function to assess cardiac
motion, evaluation of left atrial function, ventricular assist devices, pacemakers and intracardiac defibrillators and use
of echocardiography for the assessment of cardiac hemodynamics and guidance of therapy are also included in this
section. The next section contains chapters covering ischemic heart disease, coronary arteries and coronary flow reserve,
xvi Comprehensive Textbook of Echocardiography
different aspects of stress echocardiography including three-dimensional stress echocardiography, obstructive and
non-obstructive cardiomyopathies, differentiation of ischemic and nonischemic cardiomyopathy, pericardial disorders
and tumors and masses. Section 6 deals with congenital heart disease and consists of chapters on fetal cardiac imaging,
M-mode and two- and three-dimensional assessment of pediatric congenital heart disease, ventricular function, adult
congenital heart disease and acquired heart diseases in childhood. The final section in the book, Section 7, covers
systemic diseases, life-threatening conditions, echocardiography in women and the elderly, echocardiography for the
electrophysiologist and lung ultrasound. A separate chapter assesses the future of echocardiography and ultrasound.
Lastly, two chapters cover the allied techniques of magnetic resonance imaging and cardiac computed tomographic
imaging. A very large number of echocardiographic images and other figures illustrate most of the chapters of the book
and six DVDs contain numerous movie clips to supplement the images. These represent a major highlight of the book.
All chapters in this book are written by well-known experts in the field of echocardiography and ultrasound. Because
of the large number of contributors, some overlap of content and chapters do exist in the book. This has been deliberately
not excluded because it provides a different perspective to the reader and also serves to reinforce important concepts
and echocardiographic findings.
Navin C Nanda MD
Acknowledgments
I am most grateful to all the contributors from different countries of the world who have taken valuable time off from
their busy schedule to prepare chapters for this book. I am also grateful to the faculty, clinical and research fellows,
medical residents, and observers, both past and present, from our institution who have directly or indirectly helped
in the preparation of this book. Special mention needs to be made of Kunal Bhagatwala, Nidhi M Karia, Steven Bleich,
Aylin Sungur, Tuğba Kemaloğlu Öz, Kruti Jayesh Mehta, Maximiliano German Amado Escañuela and Ming Hsuing
for their invaluable help. I wish to express my thanks to the International Society of Cardiovascular Ultrasound and
the Indian Academy of Echocardiography for agreeing to have the book under their aegis. Special thanks to all the
members of the Indian Academy of Echocardiography including the current President Dr ST Yavagal as well as Drs
Satish Parashar, HK Chopra and Rakesh Gupta for their unstinting support of this project. I especially appreciate the
constant support and encouragement of Shri Jitendar P Vij (Group Chairman) and Mr Ankit Vij (Managing Director) of
M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India, in helping publish this book and also all their
associates particularly Ms Chetna Malhotra Vohra (Senior Manager–Business Development) and Ms Saima Rashid
(Development Editor) who have been prompt, efficient and most helpful. I also deeply appreciate the help of Lindy
Chapman, Administrative Associate at the University of Alabama at Birmingham, who provided excellent editorial and
secretarial assistance, and Diane Blizzard, Office Associate, for her help. Last but not least, I appreciate the patience,
understanding and support of my wife, Kanta Nanda.
Contents xix
Contents
Volume 1
Section 1: History and Basics

1. History of Echocardiography 3
Fadi G Hage, Anant Kharod, David Daly, Navin C Nanda
• History of Ultrasound 4
• The Development of Clinical Cardiac Ultrasound: A-Mode and M-Mode Echocardiography 4
• Two-Dimensional Echocardiography 8
• Conventional Doppler Ultrasound 9
• Color Doppler Ultrasound 11
• Contrast Echocardiography 11
• Transesophageal Echocardiography 13
• Tissue Doppler and Speckle Tracking Imaging 14
• Three-Dimensional Echocardiography 14
• Perspective 19
2. Early Cardiac Flow Doppler Era: A Key for a New Clinical

Understanding of Cardiology 24
Colette Veyrat
• The Preflow Doppler Era: Paucity of Existing Noninvasive Tools 25
• Explosive Emergence of the “Flow Concept”, an Indispensable Mutation from
Pressure Measurements, which Prepared the Doppler Flow Era 27
• Return to the Doppler Technique in Search of a Noninvasive Tool
Documenting the “Flow Concept” 28
3. Basics of Ultrasound 55
Caroline Morbach, Kamran Haleem, Lissa Sugeng
• General Physics 55
• Imaging by Ultrasound 57
• Image Optimization and Equipment 60
• Artifacts 61
• Doppler Ultrasound 63
4. Doppler Echocardiography—Methodology, Application and Pitfalls 65

George Thomas
• Doppler in Cardiology 65
• Doppler Instrumentation 66
• Continuous Wave Doppler 68
• Pulsed Wave Doppler 69
• Color Doppler 71
xx Comprehensive Textbook of Echocardiography
• Power Doppler 71
• Tissue Doppler 72
• The Doppler Methodology 72
• Information Derived from Doppler 73
5. Basics of 3D Ultrasound 74
Ivan S Salgo, Wendy Tsang, Nicole Bhave, Roberto M Lang
• Evolution of 3D Echocardiography 74
• Transducer Technology 76
• Beam Forming 77
• Rendering 78
• Limitations in 3D Image Quality 80
• 3D Echocardiography Quantification 81
6. Speckle Tracking Acquisition: Basics and Practical Tips 87
Willem Gorissen, Navin C Nanda
• M-Mode (1D Speckle Tracking) 87
• Two-Dimensional Speckle Tracking 88
• R-R Interval 91
• Standard Views 91
• Standardization 91
• Two-Dimensional Speckle Tracking Limitation 92
• Speckle Tracking Versus Tissue Doppler Imaging 92
• Tissue Doppler Imaging Versus Speckle Tracking 92
• Three-Dimensional Acquisition 92
• Multiview Monitoring During Live Acquisition 97
• Multiview Orientation 97
• Gain Setting 97
• Patient Breath-Hold 98
• Arrhythmias 98
7. Instrumentation for Transesophageal Echocardiography Including

New Technology 99
Ryozo Omoto, Kiyoshi Tamura
• Kinds of Transesophageal Echo (TEE) 99
• What Makes Image Quality 104
• Artifacts 107
• Safety Considerations 110
• Current and Future Technologies 112
• In the Future 116
Section 2: Echocardiography/Ultrasound Examination and Training

8. M-Mode Examination 119
Kamran Haleem, Caroline Morbach, Lissa Sugeng
• Historical Perspective 119
• Underlying Concept 119
Contents xxi
• Color M-Mode 120

• Advantages and Disadvantages of M-Mode 120
• Use of M-Mode 121
9. The Complete Transthoracic Echocardiography 132

Rachel Hughes-Doichev, Anita Radhakrishnan, Abid Ali Fakhri
Teena Tulaba, Rachel Myers
• Getting Started 132
• Echocardiographic Imaging Windows and Planes 135
• Imaging Modalities 135
• Parasternal Window 137
• Apical Window 146
• Subcostal Window 155
• Suprasternal Notch Window 159
• Three-Dimensional Echocardiography 159
• Left Ventricle Chamber Quantification and Regional Wall Motion Determination 161
10. The Standard Transthoracic Examination: A Different Perspective 164

Atif N Qasim, Nelson B Schiller
• Set-Up and Patient Positioning 164
• Imaged Planes 166
11. Nonstandard Echocardiographic Examination 188

Navin C Nanda, Aylin Sungur, Kunal Bhagatwala, Nidhi M Karia, Tuğba Kemaloğlu Öz
• Right Parasternal Examination Planes 188
• Right and Left Supraclavicular Examination 189
• Left Parasternal and Apical Planes for Examination of Coronary Arteries 190
• Examination of Left Atrial Appendage 212
• Examination from the Back 216
• Abdominal Examination 220
12. Technique and Applications of Continuous Transthoracic Cardiac Imaging 224

Premindra PAN Chandraratna, Dilbahar S Mohar, Peter Sidarous
• Feasibility of Continuous Cardiac Imaging 224
• Limitations 237
13. The Basics of Performing Three-Dimensional Echocardiography 240

Steven Bleich, Navin C Nanda, Satish K Parashar, HK Chopra, Rakesh Gupta
• 3D Technology 240
• 3D Examination Protocol 241
• Left Parasternal Approach 244
• Apical Approach 244
• Subcostal Approach 244
• Suprasternal Approach 244
• Supraclavicular Approach 244
• Right Parasternal Approach 246
• Color Doppler Imaging 248
• Advantages/Disadvantages of 3D Echocardiography 262
xxii Comprehensive Textbook of Echocardiography
14. How to do Three-Dimensional Transthoracic Echocardiography Examination 268

Fabrice Larrazet, Colette Veyrat
• History 268
• Methods for Data Acquisition 268
• Left Ventricular Assessment 270
• Reproducibility 272
• Regional LV Function 276
• Aortic Regurgitation 280
• Aortic Annulus 280
• Mitral Stenosis 280
• Mitral Regurgitation 282
• Tricuspid Valve Disease 283
• Pulmonic Valve Disease 284
• Advances in Pediatric and Fetal Cardiac Pathologies 285
15. Point-of-Care Diagnosis with Ultrasound Stethoscopy 291

JRTC Roelandt
• Battery-Powered Ultrasound Imagers 291
• The Traditional Physical Examination 292
• The New Physical Examination 293
• Acute Care Environment 294
• Screening 294
• Preparticipation Screening of Athletes 295
• Imaging in Remote Areas and Developing Countries 295
• Training Requirements 295
• Future Directions 296
16. Spectral Doppler of the Hepatic Veins 299

Bahaa M Fadel, Bader Almahdi, Mohammad Al-Admawi, Giovanni Di Salvo
• Imaging of the Hepatic Veins 299
• Physiological and Other Factors that Affect Hepatic Venous Flow 302
• Doppler Pattern of the Hepatic Veins Versus the Superior Vena Cava 304
• Transthoracic Echocardiography 304
• Transesophageal Echocardiography 305
• Technical Considerations 305
• Hepatic Venous Flow in Disease States 305
• Limitations, Technical Pitfalls and Artifacts 319
17. Spectral Doppler of the Pulmonary Veins 325

Bahaa M Fadel, Bader Almahdi, Mohammad Al-Admawi, Giovanni Di Salvo
• Historical Perspective 325
• Imaging of the Pulmonary Veins 325
• Physiological Factors that Affect Pulmonary Venous Flow 329
• Pulmonary Venous Flow in Disease States 331
• Limitations and Technical Pitfalls 342
• Artifacts 343
Contents xxiii
18. Tissue Doppler Imaging 349

Hisham Dokainish
• Technical Considerations 349
• Development of Tissue Doppler Imaging 350
• Current Clinical Uses of TD Imaging 350
19. Speckle Tracking Echocardiography: Clinical Usefulness 360

Shyam Padmanabhan, Siddharth Singh, Navin C Nanda
• Cardiac Muscular Anatomy, Cardiac Mechanics 360
• What is Strain? 362
• Two-Dimensional Speckle Tracking Echocardiography (2D STE) 365
• Image Acquisition and Processing 367
• Clinical Application of 2D STE 367
• Three-Dimensional Speckle Tracking Echocardiography (3D STE) 372
• Clinical Applications of 3D STE 373
• Limitations of Speckle Tracking Echocardiography 374
20. Echocardiographic Assessment of Global and Segmental

Function Using Velocity Vector ImagingTM 380
Michael J Campbell, David A Parra, Daniel Forsha, Piers Barker, Jonathan H Soslow
• Application of Velocity Vector Imaging by Age and Disease Group 390
• Dyssynchrony, Velocity Vector Imaging Analysis 400
• Reproducibility and Correlation Between Vendors 401
• Future Directions 404
21. Contrast Echocardiography 416

Jatinder Singh Pabla, Benoy Nalin Shah, Roxy Senior
• What is Ultrasound Contrast? 416
• How does Ultrasound Contrast Work? 417
• Indications for the Use of Ultrasound Contrast 426
• Why Should I Use Ultrasound Contrast Agents? 428
• Practical Tips 431
• Safety of Ultrasound Contrast Agents 434
• Saline Contrast Echocardiography 435
22. Myocardial Perfusion Echocardiography 441

Angele A A Mattoso, Jeane M Tsutsui, Wilson Mathias Jr
• Acute Coronary Syndromes 443
• Assessment of Myocardial Viability 443
• Chronic Coronary Artery Disease 443
23. Endothelial Dysfunction 449

Naveen Garg, Kanwal K Kapur
• History 450
• Endothelial Functions 450
• Endothelial Dysfunctions 451
xxiv Comprehensive Textbook of Echocardiography
• Role of Acetylcholine 451

• Shear Stress and Flow-Mediated Dilatation 452
• Vasoactive Molecules Involved in Vasoregulation 454
• NO Release 455
• Methodology for Assessing Endothelial Function 455
• Analysis of Shear Stress and Flow-Mediated Dilatation Response 457
• Limitations 458
• Factors Affecting the Flow-Mediated Dilatation 463
• Clinical Utility 465
• Other Noninvasive Methods to Assess Endothelial Function 465
• Assessment of Endothelial Function and Future Directions 471
24. How to do a Two-Dimensional Transesophageal Examination 480

Andrew P Miller, Navin C Nanda
• Patient Selection and Consent 480
• Preparation, Conscious Sedation and Esophageal Intubation 480
• The TEE Examination 481
25. Upper Transesophageal and Transpharyngeal Examination 487

Stephanie El-Hajj, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia, Fadi G Hage
• Technique and Recognition of Vessels 487
• Application 495
26. How to Perform a Three-Dimensional Transesophageal Echocardiogram 507

Elisa Zaragoza-Macias, Michael Chen, Edward Gill
• Three-Dimensional Transesophageal Technology 507
• Performing 3D TEE Evaluation 508
• Specific Uses of 3D TEE 512
• Guidelines and Final Recommendations 514
27. Three-Dimensional Examination to Evaluate Valvular Heart Disease:

The Value of an Added Dimension 515
Nina Ghosh, Judy R Mangion
• Data Acquisition 515
• 3D Echo Image Optimization 516
• 3D Echo of the Mitral Valve 516
• 3D Echo of the Aortic Valve 520
• 3D Echo of the Pulmonic Valve 522
• 3D Echo of the Tricuspid Valve 523
Case Examples of 3D Echo in Valvular Heart Disease 525
• Case Study 1: Paravalvular Leak Mechanical MV 525
• Case Study 2: MV Repair and Aortic Valve Replacement 526
• Case Study 3: S/P Cardiac Transplant with Right Heart Failure, Tricuspid Valve Replacement 526
• Case Study 4: Flail Middle-Scallop, Posterior Leaflet, MV 526
• Case Study 5: Bileaflet MV Prolapse, Moderate to Severe Mitral Insufficiency 527
• Case Study 6: Severe Aortic Stenosis, Evaluate for Possible TAVR 527
• Case Study 7: Rheumatic Mitral Stenosis 527
Contents xxv
• Case Study 8: S/P Balloon Aortic Valvuloplasty 527

• Case Study 9: Mechanism and Severity of Eccentric Mitral Insufficiency 528
• Case Study 10: Question of Carcinoid Involvement of the Pulmonic Valve 528
28. Three-Dimensional Echocardiographic Guidance of Percutaneous Procedures 531

Muhamed Saric, Ricardo Benenstein
• Fluoroscopy Versus Echocardiography in Guiding Percutaneous Interventions 532
• Transseptal Puncture: A Common Element of Many Interventional Procedures 532
• Valvular Disease 533
• Device Closure of Cardiac Shunts 548
• Occlusion of the Left Atrial Appendage 559
• Guidance of Electrophysiology Procedures 566
• Miscellaneous Procedures 569
29. Three-Dimensional Echocardiography in the Operating Room 577

Ahmad S Omran
• Mitral Valve Disease 577
• Aortic Valve Disease 582
• Tricuspid Valve Disease 589
• Native Valve Endocarditis 597
• Prosthetic Valve Dysfunction 605
• Cardiac Masses 617
• Limitations of 3D TEE, Future Directions 628
30. Epiaortic Ultrasonography 638

Dheeraj Arora, Yatin Mehta
• Background for Epiaortic Ultrasonography Examination 638
• Indications 638
• Epiaortic Probe and Preparation 638
• Imaging Views/Planes 639
• Role of Epiaortic Ultrasonography in Aortic Pathology 640
• Advantages of Three-Dimensions over Two-Dimensions in Epiaortic Ultrasonography 641
31. Intracardiac Echocardiography 643

Krishnaswamy Chandrasekaran, Donald Hagler, James Seward
• Equipment and the Catheters 643
• Imaging Specifications 644
• Intracardiac Echocardiography: Clinical Applications 644
• Intracardiac Echocardiography during Electrophysiology (EP) Intervention 644
• Intracardiac Echocardiography during Structural Intervention 648
32. Intravascular Ultrasound Imaging 655

Sachin Logani, Charles E Beale, Luis Gruberg, Smadar Kort
• Principles of Ultrasound Technology 655
• Image Acquisition 655
• Intravascular Ultrasound Examination 656
• Image Interpretation 657
• Utility of Intravascular Ultrasound in Clinical Practice 659
• Safety Considerations 661
• Future Perspectives 661
xxvi Comprehensive Textbook of Echocardiography
33. Peripheral Vascular Ultrasound 663

Ricardo Benenstein, Muhamed Saric
• Ultrasound Diagnosis of Carotid Artery Diseases 663
• Ultrasound Diagnosis of Femoral Access Complications 694
34. Advanced Noninvasive Quantification Techniques in Echocardiography 705

Bernhard Mumm, Navin C Nanda
• Technological Background of the Different Advanced Quantification Tools 706
• Clinical Applications of Advanced Three-Dimensional Echo Quantification Tools 721
• Right Ventricular Quantification 723
• Mitral Valve Assessment 725
• Aortic Valve Assessment 727
• Conclusion and Future Outlook 728
35. Artifacts in Echocardiography 732

Shyam Padmanabhan, Navin C Nanda, Aylin Sungur, Tuğba Kemaloğlu Öz,
Kunal Bhagatwala, Nidhi M Karia, Kruti Jayesh Mehta, Rohit Tandon
• Acoustic Shadowing and Acoustic Enhancement 733
• Reverberation Artifacts 734
• Mirror Image Artifacts 735
• Double Image Artifacts 736
• Side Lobe Artifact 736
• Artifacts Secondary to Use of Electronic Equipment 736
• Aliasing 736
• Range Ambiguity 736
• Artifacts in Three-Dimensional Echocardiography 736
• Techniques to Identify and Eliminate Artifacts 737
36. Echocardiography Training 750

Monodeep Biswas, Steven Bleich, Navin C Nanda
• Training of Noncardiologists 752
• Training for Cardiac Sonographers 753
• Training in Computed Tomography and Magnetic Resonance Imaging 755
• Certification and Maintenance of Proficiency 758
• Appropriate Use Criteria 758
Section 3: Valvular Heart Disease

37. Echocardiography in Acute Rheumatic Fever and Chronic
Rheumatic Heart Disease 765
IB Vijayalakshmi
• Echocardiography in the Diagnosis of Carditis in ARF 765
• Chronic Rheumatic Heart Disease 775
• Mitral Valve Diseases 775
• Mitral Stenosis 776
• Mitral Regurgitation 791
• Aortic Valve Diseases 802
Contents xxvii
• Aortic Stenosis 802

• Tricuspid Valve Diseases 812
• Tricuspid Stenosis 812
• Tricuspid Regurgitation 813
38. Echocardiographic Assessment of Mitral Valve Disease 826

C N Manjunath, Nagaraja Moorthy, Luis Bowen, Navin C Nanda
• Overview 826
• Echocardiographic Assessment of Mitral Stenosis 826
• Echocardiographic Assessment of Mitral Regurgitation 847
• Assessment of Severity of Mitral Regurgitation 863
39. Mitral Regurgitation 880

Luc A Pierard, Christine Henri, Julien Magne
• Etiology 880
• Mechanisms 884
• Severity of Mitral Regurgitation 885
• Mitral Regurgitation Consequences 889
• Sequential Evaluation of Chronic Asymptomatic Mitral Regurgitation 890
• Feasibility of Mitral Valve Repair 892
• Role of Exercise Echocardiography 892
40. Aortic Stenosis 896

Timothy D Woods, Ashvin K Patel, Sharath Subramanian
• Normal Aortic Valve Anatomy 896
• Etiology of Aortic Stenosis 897
• Echocardiography in Aortic Stenosis 898
• Aortic Valve Doppler Examination 904
• Use of Stress Echo and Strain in Evaluation of Aortic Stenosis 912
• Indications and Appropriateness for Echocardiography in Aortic Valve Stenosis 913
41. Low-Gradient, Severe Aortic Stenosis with Depressed and

Preserved Ejection Fraction 919
Eleonora Gashi, Neil L Coplan, Itzhak Kronzon
• Myocardial Response to Chronic Aortic Stenosis 920
• High-Flow, High-Gradient Aortic Stenosis in Setting of Normal Ejection Fraction 920
• Low-Flow, Low-Gradient Aortic Stenosis in Setting of Low Ejection Fraction 920
• Low-Flow, Low-Gradient Aortic Stenosis in Setting of Normal Ejection Fraction 921
• Mechanisms Behind PLFLG-AS 924
• Role of Surgical Aortic Valve Replacement (SAVR) in Aortic Stenosis 926
• SAVR in Low-Flow, Low-Gradient Aortic Stenosis with Low Ejection Fraction 927
• SAVR in Paradoxical Low-Flow, Low-Gradient Aortic Stenosis with Normal Ejection Fraction 927
42. Aortic Regurgitation 930

Arzu Ilercil, Arthur J Labovitz
• AR Etiologies 930
• Quantification of AR Severity 936
• Timing of Aortic Valve Surgery 941
xxviii Comprehensive Textbook of Echocardiography
43. Echocardiographic Evaluation of Aortic Disease 945

Martin G Keane
• Echocardiographic Evaluation of the Aorta 945
• Aortic Aneurysms 951
• Aortic Dissection 954
• Common Genetic Syndromes Affecting the Aorta 958
• Aortic Atheroma 959
• Aortic Trauma and Free Rupture 961
• Coarctation of the Aorta 963
44. Transesophageal Echocardiography in the Diagnosis of Aortic Disease 967

Leon J Frazin
• The Anatomical Relationship of the Aorta and Esophagus 967
• Imaging the Aorta with Transesophageal Echocardiography 967
45. Echocardiographic Examination of the Tricuspid Valve 984

Poonam Malhotra Kapoor, Kunal Bhagatwala, Nidhi M Karia, Navin C Nanda
• The Anatomy of Tricuspid Valve (TV) 984
• M-Mode Echocardiography 984
• Two-Dimensional (2D) Transthoracic Examination 986
• Two-Dimensional Transesophageal Examination 988
• Three-Dimensional Examination 988
• Tricuspid Stenosis 1004
• Tricuspid Valve Prolapse: Flail Tricuspid Valve 1007
46. Echocardiographic Assessment of Pulmonary Valve 1031

Hoda Mojazi-Amiri, Padmini Varadarajan, Ramdas G Pai
• Epidemiology 1031
• Pulmonary Stenosis 1032
• Pulmonary Regurgitation 1036
• Echocardiographic Evaluation 1037
• Ross Procedure 1038
• Postpulmonary Valve Surgery: Monitoring Sequelae 1039
• Other Complementary Techniques for Evaluation of Pulmonary Valves 1040
47. Echocardiography in Infective Endocarditis 1042

Javier López, Teresa Sevilla, José Alberto San Román, Isidre Vilacosta,
Maximiliano German Amado Escanuela, Kunal Bhagatwala, Nidhi M Karia, Navin C Nanda
• Echocardiographic Findings in Infective Endocarditis 1043
• Special Considerations in Patients with Infective Endocarditis 1047
• Role of Echocardiography in the Prognostic Stratification of Infective Endocarditis 1050
• Indications of Echocardiography in Infective Endocarditis 1058
48. The Role of Echocardiography in Pulmonary Hypertension 1063

Michele D' Alto, Francesco Ferrara, Emanuele Romeo, Anna Agnese Stanziola, Eduardo Bossone
• Conventional Echocardiography 1063
• Nonconventional Echocardiography 1070
• Diagnostic Algorithm in Pulmonary Hypertension 1073
Contents xxix
49. Echocardiographic Assessment of Prosthetic Valves 1080

Aditya Bharadwaj, Pooja Swamy, Gary P Foster, Padmini Varadarajan, Ramdas G Pai
• Types of Prosthetic Valves 1080
• Assessment of Prosthetic Valves 1082
• Prosthetic Valve Dysfunction 1087
• Other Complementary Imaging Modalities 1092
50. Three-Dimensional Transthoracic and Transesophageal Echocardiographic

Evaluation of Prosthetic Valves 1094
Steven Bleich, Navin C Nanda
• Three-Dimensional Visualization of Prosthetic Valves 1094
• Three-Dimensional Transthoracic Echocardiographic Assessment of Prosthetic Valves 1095
• Three-Dimensional Transesophageal Echocardiographic Assessment of Prosthetic Valves 1100
Volume 2
Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics

51. M-Mode and Two-Dimensional Echocardiographic Assessment of
Left Ventricular Systolic Function 1115
Anjlee M Mehta, Navin C Nanda
• Visual Estimation of Left Ventricular Systolic Function 1115
• M-Mode and Two-Dimensional Transthoracic Echocardiographic Methods for
Assessment of Left Ventricular Systolic Function 1116
• Doppler Echocardiographic Methods of Assessment of Left Ventricular Function 1119
• Two-Dimensional Speckle Tracking Echocardiography and Velocity Vector Imaging 1120
• Myocardial Performance Index 1120
• Contrast Echocardiography in the Assessment of Left Ventricular Systolic Function 1121
• Arterial–Ventricular Coupling 1121
• Three-Dimensional Transthoracic Echocardiography 1122
52. How to Assess Diastolic Function 1124

Hisham Dokainish
• Integrating Echocardiographic Variables for Accurate Diagnosis of Diastolic Function 1130
• Novel Imaging Techniques and Future Directions 1131
53. Evaluation of the Right Ventricle 1134

Vincent L Sorrell, Steve W Leung, Brandon Fornwalt
• General Overview 1134
• Right Ventricle Morphology 1135
• Echocardiography 1136
• Speckle Tracking 1141
• Hemodynamics 1143
• Other Imaging Modalities 1144
xxx Comprehensive Textbook of Echocardiography
54. Three-Dimensional Echocardiographic Assessment of LV and RV Function 1149

Aasha S Gopal
• 3D Quantitation of the Left Ventricle 1149
• 3D Quantitation of the Right Ventricle 1165
55. Newer Aspects of Structure/Function to Assess Cardiac Motion 1176

Gerald Buckberg, Navin C Nanda, Julien IE Hoffman, Cecil Coghlan
• Basic Heart Function 1177
• State-of-the-Art 1180
• Composite of State-of-the-Art Reports 1181
• Novel Mechanical and Timing Interdependence between Torsion and Untwisting 1184
• The Normal Heart 1185
• The Septum 1194
• The Right Ventricle 1198
• Other Considerations 1198
56. Echocardiography in Assessment of Complications Related to Permanent

Pacemakers and Intracardiac Defibrillators 1210
Ahmed Almomani, Khadija Siddiqui, Masood Ahmad
• Normal Echocardiographic Findings in Permanent Pacemakers/Implantable
Cardioverter-Defibrillators 1210
• Pacemaker and Implantable Cardioverter-Defibrillator-Related Complications 1212
• Masses: Lead Infection and Thrombus 1214
• Myocardial Perforation 1215
• Deleterious Effects of Right Ventricular Apical Pacing on Left Ventricular Function 1217
57. Echocardiographic Evaluation of Ventricular Assist Devices 1222

Peter S Rahko
• Clinical Uses of Ventricular Assist Devices 1224
• Reverse Remodeling 1226
• Types of Devices 1226
• Preoperative Echocardiographic Evaluation 1229
• Immediate Postsurgical Evaluation 1234
• Serial Changes in Cardiac Structure and Function 1234
• Complications of Left Ventricular Assist Devices 1240
• Evidence of Underfilling of the Left Ventricle 1246
• Optimizing Left Ventricular Assist Device Settings 1248
• Explantation 1249
• Percutaneous Continuous Flow Devices 1250
58. Echocardiographic Assessment of Left Atrial Function 1255

Utpal N Sagar, Hirohiko Motoki, Allan L Klein
• Anatomy 1255
• Physiology 1256
• Functional Assessment 1257
• Left Atrial Pathophysiology 1259
Contents xxxi
59. The Use of Echocardiography to Assess Cardiac

Hemodynamics and Guide Therapy 1264
Roy Beigel, Robert J Siegel
• Right Atrial Pressure/Central Venous Pressure 1264
• Pulmonary Artery Hemodynamics 1269
• Left-Sided Filling Pressures 1273
• Additional Parameters for Estimation of Left Atrial Pressure 1279
• Stroke Volume, Stroke Distance, Cardiac Output, and Systemic Pulmonary Shunts (QP/QS) 1280
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders,

Tumors and Masses
60. Echocardiography in Ischemic Heart Disease 1289
Chetan Shenoy, Hamid Reza Salehi, Francesco F Faletra, Natesa G Pandian
• Detection of Ischemia 1289
• Role in Acute Coronary Syndromes 1292
• Mechanical Complications of Myocardial Infarction 1294
• Role of Echocardiography in Chronic Ischemic Cardiomyopathy 1298
• Novel Echocardiography Techniques in Ischemic Heart Disease 1301
61. Stress Echocardiography 1306

Azhar Supariwala, Siu-Sun Yao, Farooq A Chaudhry
• Fundamentals of Stress Echocardiography 1306
• Types of Stress Echocardiography 1307
• Interpretation of Stress Echocardiography 1309
• Stress Echocardiography: Future Directions 1319
62. Squatting Stress Echocardiography 1323

Premindra PAN Chandraratna, Dilbahar S Mohar, Peter Sidarous
• Squatting Echocardiography 1324
63. Three-Dimensional Stress Echocardiography 1328

Rajesh Ramineni, Masood Ahmad
• Two-Dimensional Stress Echocardiography 1328
• Three-Dimensional Transducers 1329
• Advantages of Three-Dimensions in Stress Imaging 1329
• Three-Dimensional Image Acquisition 1330
• Three-Dimensional Stress Protocol 1331
• Postacquisition Analysis 1331
• Review of Studies Comparing Three-Dimensional Stress
Echocardiography to Current Standards 1331
• Differences between 2DSE and 3DSE in Wall Visualization 1334
• Parametric Imaging in Three-Dimensional Stress Echocardiography 1334
• Role of Contraction Front Mapping in RT3DSE 1334
• Contrast in Three-Dimensional Stress Testing 1335
xxxii Comprehensive Textbook of Echocardiography
64. Echocardiographic Assessment of Coronary Arteries—Morphology and

Coronary Flow Reserve 1337
Karina Wierzbowska-Drabik, Jarosław D Kasprzak
• The Assessment of Coronary Morphology and Flow in
Transthoracic and Transesophageal Studies 1337
• Visualization of Coronary Arteries 1337
• Distal Coronary Flow and Coronary Flow Reserve 1340
• Congenital Abnormalities of the Coronary Arteries 1343
65. Echocardiography in Hypertrophic Cardiomyopathy 1348

Dan G Halpern, Mark V Sherrid
• Definitions and Types of Hypertrophy 1349
• Mid-Left Ventricular Hypertrophic Cardiomyopathy 1356
• Differential Diagnosis 1359
• Treatment Strategies in Hypertrophic Cardiomyopathy 1361
66. Echocardiographic Assessment of Nonobstructive Cardiomyopathies 1369

Rohit Gokhale, Manreet Basra, Victor Vacanti, Steven J Horn, Aylin Sungur,
Robert P Gatewood Jr, Navin C Nanda
• Cardiomyopathies 1369
• Dilated Cardiomyopathy (DCM) 1370
• Secondary Findings in Dilated Cardiomyopathy 1372
• The Role of Echocardiography in Optimizing Heart Failure 1376
• Echocardiography in Assessing Ventricular Remodeling 1379
• Findings in Dilated Cardiomyopathy Based on Etiology 1379
• Restrictive Cardiomyopathy 1397
• Other Infiltrative Cardiomyopathies 1405
• Infectious and Metabolic Cardiomyopathies 1405
• Carcinoid Heart Disease 1407
67. Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy:

Comparison with Other Noninvasive Modalities 1418
Sula Mazimba, Arshad Kamel, Navin C Nanda, Maximiliano German Amado Escanuela,
Kunal Bhagatwala, Nidhi M Karia
• Echocardiographic Assessment of Ischemic and Nonischemic Cardiomyopathy 1419
• M-Mode Echocardiography 1419
• Two-Dimensional/Three-Dimensional/Doppler Echocardiography 1421
• Echocardiographic Distinction between Ischemic Cardiomyopathy and
Nonischemic Dilated Cardiomyopathy 1425
• Other Noninvasive Imaging Modalities 1425
68. Pericardial Disease 1435

Trevor Jenkins, Brian D Hoit
• Acute Pericarditis 1436
• Pericardial Effusion 1436
Contents xxxiii
• M-Mode and Two-Dimensional Echocardiography 1437

• Pericardial Tamponade 1438
• Constrictive Pericarditis 1444
• Effusive-Constrictive Pericarditis 1448
• Congenital Anomalies 1448
• Multimodality Imaging of the Pericardium 1450
69. Three-Dimensional Echocardiographic Assessment in Pericardial Disorders 1452

O Julian Booker, Navin C Nanda
• Two-Dimensional Transthoracic Echocardiography Versus Three-Dimensional
Transthoracic Echocardiography in Pericardial Effusion 1453
Transthoracic Echocardiography in Constriction 1456
Transthoracic Echocardiography in Pericardial Masses 1458
70. Echocardiographic Assessment of Cardiac Tumors and Masses 1462

Leon Varjabedian, Jennifer K Lang, Abdallah Kamouh, Steven J Horn, Tuğba Kemaloğlu Öz
Aylin Sungur, Kruti Jayesh Mehta, Kunal Bhagatwala, Nidhi M Karia
Maximiliano German Amado Escañuela, Robert P Gatewood Jr, Navin C Nanda
• Echocardiographic Assessment of Cardiac Tumors and Masses 1462
• Primary Benign Cardiac Tumors 1464
• Malignant Primary Cardiac Tumors 1484
• MICE 1511
Section 6: Congenital Heart Disease

71. Fetal Cardiac Imaging 1527
Aarti H Bhat
• Scope of Fetal Cardiology 1527
• Indications for Fetal Cardiac Evaluation 1528
• Fetal Physiology 1528
• Indications for Fetal Echocardiography 1529
• Extracardiac Reasons and Associations for Fetal Heart Disease 1529
• Fundamentals of Fetal Cardiac Imaging 1530
• Case Studies 1556
72. M-mode and Two-Dimensional Echocardiography in

Congenital Heart Disease 1561
Neeraj Awasthy, Savitri Shrivastava
Part 1: Basics of Imaging and Sequential Segmental Analysis 1562
• Patient Preparation 1562
• Imaging 1563
• Dextrocardia 1570
• Principles of Sequential Chamber Analysis 1575
xxxiv Comprehensive Textbook of Echocardiography
Part 2: Left-to-Right Shunts: Atrial Septal Defect, Ventricular Septal Defect, Patent Ductus Arteriosus, and
Aortopulmonary Window 1582
• General Features: Shunt Lesions 1582
• Atrial Septal Defects 1585
• Ventricular Septal Defect 1591
• Patent Ductus Arteriosus 1599
• Aortopulmonary Window 1602
• Gerbode Defect 1603
Part 3: Atrioventricular Septal Defects 1604
Part 4: Congenital Left Ventricular and Right Ventricular Inflow Anomalies 1610
• Congenital Anomalies of Mitral Valve 1610
• Congenital Abnormalities of Tricuspid Valve 1616
Part 5: Left Ventricular Outflow Tract Obstruction 1618
• Valvular Aortic Stenosis 1618
• Subvalvular Aortic Stenosis 1624
• Supravalvular Aortic Stenosis 1626
• Sinus of Valsalva Aneurysm 1630
• Aortocameral Communications 1632
Part 6: Echocardiographic Anatomy of Tetralogy of Fallot with Pulmonary Stenosis 1633
• Aortic Override 1633
• Double Outlet Right Ventricle 1644
• Truncus Arteriosus 1650
Part 7: Complete Transposition of Great Arteries 1653
• Transposition of Great Vessels (TGA) 1653
Part 8: Atrioventricular and Ventriculoarterial Discordance 1664
Part 9: Pulmonary Veins 1670

• Normal Flow Pattern of Pulmonary Veins 1670
• Anomalies of Pulmonary Veins 1672
• Total Anomalous Pulmonary Venous Connection 1673
• Anomalies of Systemic Veins 1678
Part 10: Imaging of Coronary Anomalies and Pulmonary Arteries 1684
• Coronary Artery Anomalies 1684
• Coronary Arteriovenous Fistula 1688
• Coronary Aneurysms 1688
Part 11: Echocardiographic Evaluation of Aortic Arch and Its Anomalies 1690
• Abnormal Formation of Arch 1690
• Coarctation of Aorta (CoA) 1692
• Interruption of Aortic Arch 1694
• Aortic Aneurysm 1695
Part 12: Univentricular Heart and Heterotomy Syndrome 1696
• Univentricular Atrioventricular Connections 1697
• Tricuspid Atresia 1700
Contents xxxv
• Mitral Atresia and Hypoplastic Left Heart Syndrome 1701

• Heterotaxy Syndrome 1704
73. Real Time 3D Echocardiography for Quantification of Ventricular Volumes,

Mass and Function in Children with Congenital and Acquired Heart Diseases 1721
Shuping Ge, Jie Sun, Lindsay Rogers, Rula Balluz
• Left Ventricular Volumes, Ejection Fraction, and Mass 1722
• Right Ventricular Volumes, Ejection Fraction, and Mass 1723
• Single Ventricular Volumes, Ejection Fraction, and Mass 1725
• Three-Dimensional Analysis of Regional Wall Motion, Synchrony, and Strain 1726
74. Three-Dimensional Echocardiography in Congenital Heart Disease 1733

Steven Bleich, Gerald R Marx, Navin C Nanda, Fadi G Hage
• Shunt Lesions/Septal Defects 1733
• Common Atrium 1747
• Aortopulmonary Window 1751
• Patent Ductus Arteriosus (PDA) 1751
• Conotruncal Anomalies 1754
• Outflow Tract Obstruction 1766
• Aortic Arch Anomalies 1770
• Atrial and Atrioventricular Valve Abnormalities 1773
• Other Abnormalities 1776
• Double Outlet Right Ventricle 1779
• Sinus of Valsalva Aneurysm 1784
75. Echocardiography in the Evaluation of Adults with

Congenital Heart Disease 1791
Reema Chugh
• Key Concepts of Echocardiography in Adults with Congenital Heart Disease 1793
• Simple Congenital Heart Defects in Adults 1798
• Valvular Disease 1813
• Complex Congenital Heart Defects 1826
76. Echocardiographic Evaluation for Acquired

Heart Diseases in Childhood 1856
Jie Sun, Rula Balluz, Lindsay Rogers, Shuping Ge
• Infective Endocarditis 1856
• Modified Duke Criteria for the Diagnosis of Infective Endocarditis 1857
• Echocardiographic Findings 1857
• Complications of Infective Endocarditis 1859
• Rheumatic Heart Disease 1859
• Jones Criteria, Updated 1992 1859
• Kawasaki Disease 1861
• Coronary Ectasia and Aneurysms by Echocardiography 1861
xxxvi Comprehensive Textbook of Echocardiography
Section 7: Miscellaneous and Other Noninvasive Techniques

77. Echocardiography in Systemic Diseases 1867
Mahdi Veillet-Chowdhury, Smadar Kort
• Systemic Lupus Erythematosus 1867
• Rheumatoid Arthritis 1868
• Hypereosinophilic Syndrome 1868
• Systemic Sclerosis 1869
• Renal Disease 1871
• Amyloidosis 1872
• Carcinoid 1874
• Chagas Disease 1875
• Sarcoidosis 1876
• Thyroid Disorders 1879
• Nutritional Deficiency 1880
78. Echocardiography in Women 1886

Jennifer Kiessling, Navin C Nanda, Tuğba Kemaloğlu Öz, Aylin Sungur,
Kunal Bhagatwala, Nidhi M Karia
• Differences in Echocardiographic Measurements and Technical Considerations 1886
• Structural Heart Disease: MVP, Mitral Stenosis, and Mitral Annular Calcification 1888
• Ischemic Heart Disease/Stress Echocardiography/Polycystic Ovarian Syndrome 1889
• Takotsubo Cardiomyopathy 1899
• Congenital Heart Disease 1900
• Echocardiography in Pregnancy, Peripartum Cardiomyopathy, Fetal Echocardiography 1902
79. Echocardiography in the Elderly 1921

Gopal Ghimire, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia
• Aortic Atherosclerosis and Penetrating Aortic Ulcer 1921
• Aortic Valve Sclerosis 1923
• Aortic Stenosis 1924
• Aortic Aneurysm 1934
• Left Ventricular Mass, Dimensions, and Function 1942
• Echocardiography in Stroke Patients: Assessment of Coronary Stenosis 1943
• Mitral Annular Calcification 1946
• Prosthetic Valves 1948
80. How to do Echo for the Electrophysiologist 1957

Chittur A Sivaram
• Echocardiography in Supraventricular Tachycardia 1957
• Left Atrium 1960
• Atrial Septum 1962
• Pulmonary Veins 1963
• Inferior Vena Cava 1964
• Echocardiography in Ventricular Tachycardia 1966
• Echocardiography in Cardiac Implantable Electronic Devices 1967
Contents xxxvii
81. Echocardiography in Life-Threatening Conditions 1969

Rachel Harris, Elizabeth Ofili
• Chest Trauma 1969
• Blunt Chest Trauma 1969
• Penetrating Chest Trauma 1972
• Acute Mitral Regurgitation 1972
• Acute Severe Aortic Regurgitation 1972
• Debakey Classification 1974
• The Stanford Classification 1974
• Pulmonary Thromboembolic Disease 1976
• Air Embolism 1977
• Hypovolemia 1977
• Large Intracardiac Thrombus 1978
82. Lung Ultrasound in Cardiology 1982

Luna Gargani, Eugenio Picano
• Physical and Physiological Basis of Lung Ultrasound 1982
• Methodology 1983
• Pulmonary Interstitial Edema 1984
• Pleural Effusion 1985
• Pulmonary Embolism 1985
• Acute Respiratory Distress Syndrome 1986
• Pneumothorax 1986
• Cardiopulmonary Ultrasound: An Integrated Approach 1987
• Limitations 1987
83. The Future of Echocardiography and Ultrasound 1990

David Cosgrove
• Plane Wave Ultrafast Imaging 1990
• Trends in Scanners 1991
• Doppler 1993
• Microbubbles 1993
• Elastography 1994
• Light and Sound 1995
• Therapeutic Applications of Ultrasound 1996
84. A Primer on Cardiac MRI for the Echocardiographer 1998

Madhavi Kadiyala, Aasha S Gopal
• Quantitative Left and Right Ventricular Assessment 1998
• Strain Assessment 1999
• Left Ventricular Structure 2000
• Myocarditis and Sarcoidosis 2004
• Cardiac Hypertrophy 2006
• Cardiomyopathies 2008
• Velocity Mapping, Flow and Shunt Assessment 2008
• Valvular Heart Disease and Prosthetic Valves 2009
xxxviii Comprehensive Textbook of Echocardiography
• Pericardial Disease 2014

• Normal Variants and Masses 2016
• Limitations of Cardiac MRI and CT 2017
• Glossary of Cardiac MRI Sequences 2020
85. Cardiac CT Imaging 2023

Satinder P Singh, Sushilkumar K Sonavane
• Challenges for Cardiac Computed Tomography 2024
• Radiation Dose 2025
• Patient Selection 2027
• Technique 2027
• Image Postprocessing 2028
• Image Analysis 2032
• Pitfalls and Artifacts 2034
• Diagnostic Accuracy of Coronary Computed Tomography Angiogram 2040
• Coronary Plaque 2041
• Prognostic Information from Coronary Computed Tomography Angiogram 2042
• Cardiac Function 2042
• Myocardial Perfusion 2042
• How to Improve Accuracy of Computed Tomography Angiogram in Determining
Flow Limiting Disease 2044
• Clinical Indications 2044
Index
I-i

SECTION 4
Left and Right Ventricles,

Left Atrium, Hemodynamics
Chapters
Chapter 51 M-Mode and Two-Dimensional Echocardiographic Chapter 57 Echocardiographic Evaluation of Ventricular
Assessment of Left Ventricular Systolic Function Assist Devices
Chapter 52 How to Assess Diastolic Function Chapter 58 Echocardiographic Assessment of Left
Chapter 53 Evaluation of the Right Ventricle Atrial Function
Chapter 54 Three-Dimensional Echocardiographic Chapter 59 The Use of Echocardiography to Assess
Assessment of LV and RV Function Cardiac Hemodynamics and Guide Therapy
Chapter 55 Newer Aspects of Structure/Function to Assess
Cardiac Motion
Chapter 56 Echocardiography in Assessment of Complications
Related to Permanent Pacemakers and Intracardiac
Defibrillators
CHAPTER 51
M-Mode and Two-Dimensional
Echocardiographic Assessment of Left
Ventricular Systolic Function
Anjlee M Mehta, Navin C Nanda
Snapshot
Visual Estimation of Left Ventricular Systolic Function
¾¾ Myocardial Performance Index
¾¾
¾¾M-Mode and Two-Dimensional Transthoracic Echocar- Contrast Echocardiography in the Assessment of Left
¾¾
diographic Methods for Assessment of Left Ventricular Ventricular Systolic Function
Systolic Function Arterial–Ventricular Coupling
¾¾
Doppler Echocardiographic Methods of Assessment of
¾¾ Three-Dimensional Transthoracic Echocardiography
¾¾
Left Ventricular Function
¾¾Two-Dimensional Speckle Tracking Echocardiography
and Velocity Vector Imaging
INTRODUCTION VISUAL ESTIMATION OF LEFT

The evaluation of left ventricular systolic function by VENTRICULAR SYSTOLIC FUNCTION
echocardiography has undergone many recent advance Visual estimation of LV ejection fraction, in the eyes of
ments. Assessment of ejection fraction as a surrogate for an experienced echocardiographer, is a quick method
left ventricular systolic function is one of the primary for determination of systolic function and is widely used
clinical questions for which echocardiograms are obtained. to help make immediate decisions in clinical settings. A
A review of methodologies for determining ejection framework for evaluation involving division of the LV into
fraction and/or left ventricular function by M-mode and 16 segments was proposed by the American Society of
two-dimensional (2D) echocardiography allows for a Echocardiography in 1989. In this model, the LV is divided
better understanding of advantages, disadvantages, and into a basal level, mid (or papillary) level, and apical level.
appropriate indications for echocardiographic evaluation There are six segments at both basal and midventricular
of left ventricle (LV) systolic function. levels, and four segments at the apex. In 2002, the
1116 Section 4: Left and Right Ventricles, Left Atrium, Hemodynamics
Fig. 51.1: Seventeen-segment model with correspondence to coronary artery distribution. (LAD: Left anterior descending artery;
LCX: left circumflex artery; RCA: Right coronary artery).
Source: Reproduced with permission from Pereztol-Valdes O, Candell-Riera J, et al. Correspondence between left ventricular
17 myocardial segments and coronary arteries. Eur Heart J. 2005(26):2637–43.
American Heart Association Writing Group on Myocardial by the right coronary artery (if dominant), and segments
Segmentation and Registration for Cardiac Imaging 5, 6, 11, 12, and 16 by the left circumflex artery. As a
added a 17th segment encompassing the apical cap, the result, if regional wall motion abnormalities are involved,
segment beyond the end of the LV cavity1 (Fig. 51.1). This describing the segments involved can allow one to
model allows for segmental determination of regional wall surmise which epicardial coronary vessel may be involved
motion abnormalities and utilizes a scoring system based (Fig. 51.2).1,2
on the motion and systolic thickening of each segment.
The walls are evaluated in multiple echocardiographic M-MODE AND TWO-DIMENSIONAL
views including a parasternal long-axis ([PLAX], or apical
3-chamber [A3Ch]/apical long-axis [ALA]), parasternal
TRANSTHORACIC ECHOCARDIO-
short-axis (PSAX), apical 4-chamber (A4Ch), and apical GRAPHIC METHODS FOR ASSESS-
2-chamber (A2Ch) view for correlation. Each segment MENT OF LEFT VENTRICULAR
can be scored from 1 to 5 with the following definitions:
1 = normal or hyperkinesis, 2 = hypokinesis, 3 = akinesis
SYSTOLIC FUNCTION
(negligible thickening), 4 = dyskinesis (paradoxical systolic The Teichholz formula using M-mode echocardiography
motion), and 5 = aneurysmal (diastolic deformation). The was one of the earliest methods developed for assessment
score for each segment is added up to give a total score. of left ventricular ejection fraction (LVEF). In a PLAX
This score is then divided by the number of segments to view, inferior and lateral to the mitral valve chordae,
create a wall motion index score. A normal ventricle has a measurements of the left ventricular end-diastolic internal
wall motion score of 1.1,2 diameter and the left ventricular end-systolic internal
The blood supply from a particular coronary artery to diameter are made. These measurements are then used to
each segment can also be defined. In general, segments calculate end-diastolic and end-systolic volumes, and the
1, 2, 7, 8, 13, 14, and 17 are generally supplied by the left difference between these two volumes divided by the end-
anterior descending artery, segments 3, 4, 9, 10, and 15 diastolic volume can be used to calculate the LVEF.3
Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function 1117
Fig. 51.2: Typical distributions of the right coronary artery (RCA), the left anterior descending (LAD), and the circumflex (CX) coronary
arteries. The arterial distribution varies between patients. Some segments have variable coronary perfusion. J Am Soc Echocardiogr.
2005;18(12):1440–63.
M-mode is also used to obtain the E point septal The Baran, Rogal, and Nanda method for quantification
separation (EPSS), an indirect estimation of global of LVEF, in the absence of wall motion abnormalities,
LV function. In the setting of LV dysfunction, there is requires end-systolic and end-diastolic measurements of
increased separation between the E point (peak of mitral the LV minor axis at the midventricular level and the LV
valve opening) and the ventricular septum. With LV major axis from the apex to the base of the LV. These values
chamber enlargement and dysfunction, the mitral valve is are obtained in an A4Ch view (Fig. 51.3). End-diastolic and
shifted further away from the septum and there is reduced end-systolic volumes are then calculated using a modified
transmitral flow (and reduced stroke volume) relative cylinder–ellipse formula in which the LV is assumed to be a
to chamber size. Typically, an EPSS > 1 cm is considered combination of a cylinder and prolate ellipse (Fig. 51.4). If
abnormal.4 wall motion abnormalities are present, then measurement
Using the Quinones method, measurements are of the minor axes at three equidistant points that divide
taken at several minor-axis locations of the LV in three 2D the LV into three regions are obtained. Each region
echocardiographic views (PLAX, A4Ch, and ALA views). contributes one-third to the total ejection fraction and the
The minor-axis measurement locations in the PLAX view chance of including wall motion abnormalities in one of
are at the base and midcavity levels. In the A4Ch and the regions is increased. The total LVEF is an average of the
ALA views, measurements are taken at the upper third, LVEFs obtained from each of the three regions.6
middle third, and lower thirds of the LV in end-systole and Another method for assessing left ventricular systolic
end-diastole. The contribution of the apex to the LVEF is function is to calculate a fractional area change. Measure
made by a qualitative assessment of apical wall motion ments of minor axis dimensions are taken from M-mode
abnormalities.5 echocardiograms obtained with 2D echocardi ographic
Fig. 51.3: Apical four-chamber view of a normal heart at end- Fig. 51.4: Modified cylinder–ellipse formula. A, cross-sectional
systole and end-diastole. In method A, left ventricular (LV) minor area of cylinder (hatched); (D: Diameter of circle A; L: Length of
axis D is measured at end-systole and end-diastole at the mid- entire object; LVV: Left ventricular volume).
ventricular cavity level. The left ventricular major axis is measured Source: Reproduced with permission from Baran AO, et al. Ejec-
from the apex of the left ventricle to the base of the mitral valve. tion fraction determination without planimetry by two-dimen
In method B, measurements of the regional left ventricular minor sional echocardiography: a new method. J Am Coll Cardiol.
axes, D1, D2, and D3 are measured at three equidistance points 1983;1:1471–8.
at the upper, middle, and lower third of the left ventricular cavity at
end-systole and end-diastole of the same cardiac cycle. The major
wall geometry, such as hypertrophied hearts. In addition
axis L is measured as before. Directions I, L, R, and S are inferior,
left, right, and superior, respectively. (LA: Left atrium; RA: Right to the M-mode measurements used in FSendo, the MWFS
atrium; RV: Right ventricular). also uses measurements of septal wall thickness at diastole
Source: Reproduced with permission from Baran AO, et al. (SWTd) and posterior wall thickness (PWTd) at diastole and
Ejection fraction determination without planimetry by two-di-
incorporates a separate equation for the inner shell (inner
mensional echocardiography: a new method. J Am Coll Cardiol.
1983;1:1471–8. wall) with the following formulas.7,8
Inner shell = ([LVIDd + SWTd/2
guidance. The left ventricular internal dimension in + PWTd/2]3 – LVIDd3 + LVIDs3)1/3−LVIDs
diastole (LVIDd) and left ventricular internal dimension MWFS= ([LVIDd ± SWTd/2 ± PWTd/2]
in systole (LVIDs) are used in the formula for fractional –[LVIDs ± inner shell])
shortening, measured at the endocardium (FSendo [%]) (LVIDd + SWTd/2 + PWTd/2) × 100
such that, FSendo = 100 × (LVIDd − LVIDs)/(LVIDd).7 The biplane method of discs, or modified Simpson’s
Unfortunately, fractional shortening at the endoc rule, recommended by the American Society of
ardium is affected by changes in left ventricular geometry Echocardiography is one of the most commonly applied 2D
and loading conditions. Another parameter called midwall techniques for obtaining the left ventricular volumes used
fractional shortening (MWFS) is less influenced by left in calculating an LVEF. The left ventricular endocardial
ventricular geometry and has been shown to be useful border is traced during end-diastole and end-systole in
in detection of early systolic dysfunction in hypertensive orthogonal planes that include the apex (e.g. A4Ch and
patients with concentric left ventricular hypertrophy.7 A2Ch views; Fig. 51.5). The ventricle is then divided, along
MWFS, unlike FSendo, does not assume uniformity of the long axis, into a series of ellipsoid discs of equal height
systolic thickening throughout the myocardium, and is (Fig. 51.6). Computer software then determines the volume
therefore less likely to overestimate contractile function. of each disc (height × disc area). All the volumes are added
In reality, inner wall (subendocardial) and outer wall to obtain the total LV volumes in systole and diastole and
(epicardial) thickening fractions are not equal, and the allow for calculation of the LVEF (EDV − ESV/EDV).
inner wall contributes more to systolic thickening than the Limitations of the biplane method of discs include
outer wall.8 This is more pronounced in conditions with endocardial dropout and apical foreshortening that result
increased relative wall thickness/altered left ventricular in incorrectly small ventricular volumes. It also assumes
Fig. 51.6: Use of Simpson’s rule. Using this rule, the volume of left
ventricle is usually calculated by approximating areas along the
apical axis by circles and employing axial integration.
Source: Reproduced with permission from Ghosh A, Nanda
NC, Maurer G. Three-dimensional reconstruction of echocardio-
graphic images using the rotation method. Ultrasound Med Biol.
1982;8(6);655–61.
DOPPLER ECHOCARDIOGRAPHIC
METHODS OF ASSESSMENT OF LEFT
VENTRICULAR FUNCTION
Fig. 51.5: Two-dimensional measurements for volume calcula-
tions using biplane method of disks (modified Simpson’s rule) in
Tissue Doppler Imaging
apical four-chamber (A4C) and apical two-chamber (A2C) views Tissue Doppler imaging (TDI) is based on the principle
at left ventricular end-diastole (LV EDD) and at left ventricular end-
that myocardial velocities, like blood flow velocities, can
systole (LV ESD). Papillary muscles should be excluded from the
cavity in the tracing. be differentiated based on their different amplitudes and
Source: Reproduced with permission from Lang RM, Bierig M, Doppler frequencies. The peak systolic ejection velocity
Devereux RB, et al. Recommendations for chamber quantifica- represented by the S’-wave on velocity tracings is obtained
tion: a report from the American Society of Echocardiography’s by placing a sample volume 1 cm above (apical) the medial
Guidelines and Standards Committee and the Chamber Quantifi-
cation Writing Group, developed in conjunction with the European (septal) side of the mitral annulus and obtaining the tissue
Association of Echocardiography, a branch of the European Soci- velocity as the LV moves toward the apex in systole.7
ety of Cardiology. J Am SocEchocardiogr. 2005;18(12):1440–63. When the LV is in the early filling phase of diastole and
moving away from the apex, this velocity is represented
by the E’-wave on velocity tracings. The A’-wave velocity is
obtained during late diastole as the atria contract and the
that the ventricle is ellipsoidal, which is not always the LV moves away from the apex.7 These waves represent the
case (e.g. LV aneurysm). Poor acoustic windows, as is longitudinal motion of the LV (base to apex shortening).
commonly seen in obese, ventilated, or severe chronic The contribution of longitudinal fiber shortening
obstructive pulmonary disease (COPD) patients, can also and myocardial contractile velocity to left ventricular
make accurate endocardial tracing very difficult. function forms the basis for analysis of the mitral annular
Calculation of a sphericity index is another method that descent velocity by tissue Doppler echocardiography.
has been used as a marker of left ventricular dysfunction. 2D echocardiography guides placement of the M-mode
The sphericity index is the ratio of the LV long-axis cursor at the mitral annulus at two sites in the A4Ch,
dimension and LV short-axis dimension. With negative LV A2Ch, and ALA views.10 The maximal color-coded velocity
remodeling that occurs in dilated cardiomyopathies, heart toward the transducer during left ventricular ejection is
failure, myocardial infarcts, and valvular regurgitation, the the peak mitral annular descent velocity. This velocity is
LV adapts by becoming more spherical. A normal elliptical independent of endocardial definition, but only looks at
LV has a sphericity index of ≥ 1.5.9 As the heart becomes longitudinal movement of the LV walls during systole and
more spherical, this value decreases and approaches one is influenced by loading conditions and heart rate. Values
signifying a more spherical LV. of < 7 cm/s suggest LV dysfunction.10
torsion of the heart. Rotation is defined as the movement

of the heart in relation to an axis through the middle of the
LV cavity from the apex to the base. Twist is the difference
between the rotation of the apex and the base. Torsion
is defined as the twist normalized to the length of the LV
cavity (i.e. twist divided by the vertical distance between
the apex and base).11 There are many ongoing studies
showing that changes in these parameters are useful in
subclinical detection of systolic dysfunction prior to a
visual or measured reduction in LVEF.11
Velocity vector imaging also uses speckle tracking
and incorporates this tracking into velocity vectors taken
from the LV endocardium and epicardium to follow the
direction of the LV myocardium. Unlike for TDI, where
the myocardial velocities being interrogated must be
from tissue moving parallel to the ultrasound beam (only
movements toward and away from the probe), speckle
tracking and velocity vector imaging are not limited
by the angle at which velocities are obtained and can
Fig. 51.7: Tei’s Index. Time a is the interval between cessation and better account for movements of the myocardium in
onset of mitral inflow. It includes isovolumic contraction time (ICT),
multiple directions.11 A more detailed description of these
ejection time (ET), and isovolumic relaxation time (IRT). Left ven-
tricular ejection time b is the duration of the left ventricular outflow modalities and their use assessing systolic and diastolic
velocity profile left ventricle (LV) outflow. The index of combined left ventricular dysfunction can be found in other chapters
left ventricular systolic and diastolic function (the sum of isovo- of this book.
lumic contraction time and isovolumic relaxation time divided by
ejection time) is calculated as (a − b)/b.
MYOCARDIAL PERFORMANCE INDEX
Myocardial performance index, or the Tei’s Doppler
TWO-DIMENSIONAL SPECKLE
index, is the sum of isovolumic contraction time (ICT)
TRACKING ECHOCARDIOGRAPHY and isovolumic relaxation time (IRT) divided by ejection
AND VELOCITY VECTOR IMAGING time (ET) and, as such, reflects global (combined systolic
In addition to inward and longitudinal motion, the LV also and diastolic) cardiac function.12 It provides a measure of
rotates and twists during the cardiac cycle. To quantify the ventricular function independent of ventricular geometry.
complexity of cardiac motion, a technique called speckle The ICT corresponds to the interval between mitral valve
tracking has been developed. Speckles are small groups closure and aortic valve opening as measured by pulsed
of myocardial pixels created by the interaction between Doppler from the apical position. Physiologically, this
ultrasound beams and the myocardium.11 Many vendors correlates with influx of calcium into the mycoplasma.12
have developed algorithms for tracking these speckles. The IRT is the interval between aortic valve closure and
Speckle tracking measures aspects of strain, or myocardial onset of mitral valve opening and represents the removal
deformation, that occur during the cardiac cycle. Radial of calcium from the myoplasm by calcium-ATPases.12 The
strain (thickening of the myocardium during the inward ejection time is the interval from the onset to the end of the
motion of the ventricle), longitudinal strain (percentage LV outflow velocity pattern13 (Fig. 51.7).
decrease in length of the myocardium during systole as the The presence of arrhythmias including atrial
base moves toward the apex), and circumferential strain fibrillation, frequent atrial and ventricular ectopy, and
(change in length along the circumferential perimeter) can tachycardias limit the application of the Tei-index. Pseudo
be assessed.11 In addition to measuring strain and strain normalization of the index also limits its applicability in
rate, speckle tracking also assesses the rotation, twist, and patients with restrictive filling patterns.14
A B
Figs 51.8A and B: Contrast echocardiography. (A) Precontrast. The left ventricle (LV) cavity shows multiple trabeculations
(arrowhead) in the apex consistent with noncompaction. LV endocardial border is not well visualized in this area; (B) Postcontrast. Fol-
lowing injection of the contrast agent, the LV cavity is completely filled with contrast echoes, resulting in complete delineation of the
endocardium.
CONTRAST ECHOCARDIOGRAPHY ARTERIAL–VENTRICULAR COUPLING

IN THE ASSESSMENT OF LEFT The concept of arterial–ventricular coupling (EA/ELV) looks
VENTRICULAR SYSTOLIC FUNCTION at how properties of the arterial system affect the function
of the LV. Several studies have looked at how effective
Commercially available echo contrast agents are widely
arterial elastance (EA, arterial load) and left ventricular
used to assist in determination of left ventricular systolic
end-systolic elastance (ELV, LV performance) relate and
function and evaluation of cardiac chambers and
affect cardiac performance especially in conditions
myocardial perfusion. In patients with poor acoustic
where the arterial tree becomes thicker and stiffer like
windows, contrast can be given to help enhance detection
aging, heart failure, and hypertension.17 EA is calculated
of the endocardial border and result in more accurate
as end-systolic pressure (ESP) divided by stroke volume
visual estimates of left ventricular systolic function and
measurements of left ventricular volumes. Echo contrast (SV) and serves as an index of the vascular load on the
agents consist of reflective microbubbles. They are injected LV. ESP is estimated as systolic blood pressure times 0.9
intravenously and pass through right heart, the pulmonary and SV is EDV − ESV as obtained from 2D/Doppler echo
circulation, and into the left side of the heart, where they methods. ELV is noninvasively calculated using a modified
opacify the left heart chambers and help delineate the single-beat method to estimate end-systolic elastance
endocardial borders. The technique is similar to the more from arm-cuff pressures (systolic and diastolic BP), echo-
invasive left ventriculogram obtained by injection of Doppler SV, echo-derived ejection fraction, and estimated
contrast during left heart catheterization15,16 (Figs 51.8A normalized ventricular elastance at arterial end-diastole.
and B). Other features that can be adjusted to improve It represents a relatively load-independent measure of LV
contrast opacification include harmonic imaging and low performance. Arterial–ventricular coupling is evaluated as
mechanical index imaging. With these advances, contrast the ratio of these values (EA/ELV) and maximal efficiency is
echocardiography provides improved endocardial border attained when EA/ELV approaches 0.5.17 In states of elevated
imaging, resulting in better detection of wall motion afterload (e.g. aging, heart failure, and hypertension), there
abnormalities, ventricular volume, and ejection fraction. is increased total peripheral resistance, left ventricular
The result is a more accurate estimate of LV systolic concentric remodeling, inefficient arterial–ventricular
function. coupling, and ultimately, impaired LV function.18,19
A community-based study by Redfield et al. speculated method required identification of 3–5 points at the apex and
that an increase in heart failure with preserved ejection mitral annulus in the two- and four-chamber end-diastolic
fraction (HFnlEF) especially amongst elderly women, and end-systolic views. Software using automated border-
may be related to maintenance of optimal arterial– detection created a 3D endocardial shell of the LV from
ventricular coupling.20 In an effort to maintain stroke which a volume was calculated.22 They noted that 3DE also
volume in the setting of higher arterial elastance as a had less intraobserver and interobserver variability when
result of aging, there is an increase in left ventricular compared with 2DE and under-represented true values
systolic stiffness. Unfortunately, increases in these approximately 50% less often. Dorosz et al. did find that
parameters are not without consequence. Redfield et al. compared to cardiac magnetic resonance imaging (CMR),
note that altered LV chamber geometry (e.g. hypertrophy, 3DE underestimated LV volumes and there was significant
concentric remodeling, or fibrosis) to maintain arterial– variability in the LV volumes. This was more pronounced
ventricular coupling and resultant impaired LV relaxation in patients with poor windows or large ventricles due to
(diastolic dysfunction) could contribute to the increase inability to fit the entire ventricle into the sector scan. They
acknowledged that using CMR as a gold standard might
in HFnlEF they observed in elderly women.20 A study by
be problematic due to errors in border detection and
Lam et al also noted an increase in arterial elastance and
controversy surrounding the inclusion of basal LV planes.22
left ventricular end-systolic elastance in hypertensive and
Three-Dimensional transthoracic echocardiography
HFnlEF patients as compared to healthy controls.21
continues to be increasingly used in assessment of ventri
cular volumes, ejection fractions, valvular disorders, cong
THREE-DIMENSIONAL TRANS enital heart disease, and evaluation of cardiac masses.23
THORACIC ECHOCARDIOGRAPHY Further discussion regarding the specific advantages of
this modality can be found in other chapters in this book.
With 2D transthoracic echocardiography (2D TTE), only
one slice of the LV can be obtained at a time. Obtaining
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26(24):2637–43.
hundreds of ultrasound waves through the heart allowing
2. Lang RM, Bierig M, Devereux RB, et al; Chamber
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3D volume can then be cropped using any desired plane Echocardiography’s Guidelines and Standards Committee;
angulation. For example, a single apically acquired 3D European Association of Echocardiography. Recomm
data set potentially allows for display and analysis of all the endations for chamber quantification: a report from the
standard apical 2D views (apical 2-, 3-, 4-, and 5-chamber American Society of Echocardiography’s Guidelines and
Standards Committee and the Chamber Quantification
views). This data set can also be used for analysis of short- Writing Group, developed in conjunction with the
axis views from the apex to the base of the LV. In 2D echo, European Association of Echocardiography, a branch of
many geometric assumptions are made about LV shape.16 the European Society of Cardiology. J Am Soc Echocardiogr.
As mentioned earlier, many of the formulas, including 2005;18(12):1440–63.
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Echocardiography. 1998;15(8 Pt 1):709–12.
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According to a meta-analysis by Dorosz et al. Three- echocardiography. J Am Soc Echocardiogr. 2010;23:240–57.
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7. Otto CM. Textbook of Clinical Echocardiography. Proceedings of the Preconference CME Program of
Philadelphia: Saunders Elsevier; 2009. the XV Annual Conference of the Indian Academy of
8. Palmiero P, Maiello M, Nanda NC. Is echo-determined Echocardiography, February 11–14, 2010, Kochi, India.
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filling and midwall shortening in hypertensive ventricular coupling: mechanistic insights into cardiovascular
hypertrophy? Echocardiography. 2008;25(1):20–6. performance at rest and during exercise. J Appl Physiol.
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18. Fernandes VR, Polak JF, Cheng S, et al. Arterial stiffness is
10. Gulati VK, Katz WE, Follansbee WP, et al. Mitral annular
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descent velocity by tissue Doppler echocardiography as
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11. Biswas M, Sudhakar S, Nanda NC, et al. Two- and three- 19. Saba PS, Ganau A, Devereux RB, Pini R, et al. Impact of
dimensional speckle tracking echocardiography: clinical arterial elastance as a measure of vascular load on left
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Echocardiogr. 2000;13(2):116–23. and ventricular-vascular function in persons with heart
13. Arnlöv J, Ingelsson E, Risérus U, et al. Myocardial perfor failure and preserved ejection fraction from Olmsted
mance index, a Doppler-derived index of global left County, Minnesota. Circulation. 2007;115(15):1982–90.
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22. Dorosz JL, Lezotte DC, Weitzenkamp DA, et al. Perfor
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mance of 3-dimensional echocardiography in measuring
14. Karatzis EN, Giannakopoulou AT, Papadakis JE, et al.
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systolic function assessment by echo doppler examination. 48(6):291–303.
CHAPTER 52
How to Assess Diastolic Function
Hisham Dokainish
Snapshot
Integrating Echocardiographic Variables for Accurate
¾¾ Novel Imaging Techniques and Future Directions
¾¾
Diagnosis of Diastolic Function
INTRODUCTION by using a comprehensive diastolic assessment—incor

porating many 2-dimensional (2D), conventional, and
In patients presenting with dyspnea, accurate assessment tissue Doppler variables—as opposed to relying on any
of left ventricular (LV) systolic and diastolic function is of single, diastolic parameter, which can lead to errors.
utmost importance to establish or exclude heart failure as
a cause or component of dyspnea. Echocardiography with
Doppler readily assesses LV diastolic function; advantages
Two-Dimensional Echocardiography:
include that echocardiography is noninvasive, does not Left Ventricular Mass and Wall Motion,
require radiation, is portable, rapid, readily available, and Left Atrial Size
and in competent hands, it can provide an accurate and
comprehensive assessment of LV systolic and diastolic According to current guidelines, the following three
function. Correct assessment of LV diastolic function is criteria are needed for the diagnosis of diastolic heart
relevant in patients with both depressed and preserved LV failure (DHF): clinical picture consistent with HF, demon
ejection fraction (EF < 50%, and ≥ 50%, respectively). Tissue stration of preserved LVEF, and demonstration of diastolic
Doppler (TD) imaging has been useful in demonstrating dysfunction.1 Clinically, diastolic dysfunction, secondary
impaired LV relaxation in the setting of preserved left to impaired LV relaxation and increased LV stiffness, is
ventricular ejection fraction (LVEF), which, in the setting usually demonstrated by echocardiography and Doppler.2–6
of increased cardiac volume, can result in elevated LV The best correlate of symptoms and survival in DHF is
filling pressures and dyspnea due to diastolic heart failure. elevation of left atrial (or left ventricular filling) pressure,
TD imaging is not always critical in patients with depressed readily estimated using comprehensive echocardiography
LVEF, since such patients by definition have impaired LV with Doppler.1,2 Demonstration of preserved LVEF is
relaxation, and thus significant increases in volume will readily demonstrated with 2D echocardiography.7 It should
result in increases in LV filling pressure due to impaired be noted that DHF is a term used relatively intercha
LV compliance. Thus, in depressed LVEF, transmitral ngeably with “HF with preserved LVEF” and “HF with
flow velocities (E and A, and E/A) and deceleration normal LVEF”.
time, pulmonary venous Doppler, left atrial volume, and In DHF, LVEF is preserved ≥ 50%, yet left atrial
pulmonary artery (PA) pressures suffice for the accurate pressures—synonymous with LV filling pressures in
assessment of LV filling pressures. Overall, diastolic the absence of obstructive mitral valve (MV) disease—
assessment by echo Doppler can be readily achieved in are elevated, causing increased pulmonary venous
Chapter 52: How to Assess Diastolic Function 1125
Fig. 52.1: Left ventricular hypertrophy in a patient with diastolic Fig. 52.2: Presence of left atrial dilation in the patient with left
dysfunction. Chronic hypertension is a common scenario for the ventricular diastolic dysfunction. The same patient as in
development of diastolic dysfunction, and the hypertrophied left Figure 52.1 has severely dilated left atrium (LA) from chronic
ventricle (LV) develops impaired relaxation, and in the right load- elevation in LA pressures in the setting of left ventricular hypertro-
ing conditions, can result in elevated left atrial (LA) pressure. This phy from chronic hypertension. Note that the LA does not appear
patient had concentric LV hypertrophy (LV mass index = 119 g/m2). significantly dilated by anteroposterior diameter in Figure. 52.1; this
is the reason current guidelines recommend the measurement of LA
volume in the apical views. This patient had severe LA enlargement,
with an unindexed LA volume of 137 mL and an indexed volume of
72 mL/m2.
pressures and dyspnea at rest or during exertion.1–6 In since the LA cannot adequately empty in to the LV during
order for left atrium (LA) pressures to be elevated in the diastole in this hemodynamic scenario, LA enlargement
absence of significantly depressed LVEF, LV relaxation (≥ 30 mL/m2) is usually seen.5 Increasing LA size correlates
and compliance generally are depressed, most often with increasing LV filling pressures and worse outcome in
occurring in hypertensive or ischemic heart disease.2–5 patients with diastolic HF.9 In addition, LA size has been
Two-dimensional echocardiography, therefore, identifies called the barometer of LV diastolic dysfunction or LV
LV abnormalities that create the substrate for LV diastolic filling pressures, although certainly other entities, such as
dysfunction: LV hypertrophy and LV wall motion atrial fibrillation or chronic hypertension, can result in LA
abnormalities. Increased LV mass (≥ 90 g/m2 for women enlargement in the absence of significant elevation of LA
and ≥ 115 g/m2 for men; i.e. LV hypertrophy) is common pressure.6 It has therefore been said that LA volume has a
in patients with DHF5 (Figs 52.1 and 52.2). Previous better negative—as opposed to positive—predictive value
studies have correlated increasing degrees of LV mass for significant diastolic dysfunction and heart failure;
with increasing LV diastolic dysfunction and filling that is, a normal or small LA largely excludes significantly
pressures.8 In addition, since LVEF can be preserved even elevated LA pressure, while a large LA volume may occur
in the presence of significant coronary artery disease, in the absence of significant LA dilation.10 Studies have
LV wall motion abnormalities create the substrate for also shown that LA volume is a much better measurement
significant LV diastolic dysfunction even in the patient of LA enlargement than a simple anteroposterior diameter,
with preserved LVEF who may have a diagnosis of DHF. and therefore is the recommended way to measure LA size
Therefore, accurate identification of LV wall motion by echocardiography.6 It is also important to integrate 2D
abnormalities is of great importance in the assessment of echocardiographic variables in the assessment of diastolic
the patient with potential diastolic dysfunction. Since LA function; for instance, in cases of ischemic or infiltrative
pressures are elevated in patients with significant diastolic heart disease, significant LV hypertrophy may be absent,
dysfunction in the presence of increased preload, and yet LA volumes are often enlarged.5
Fig. 52.3: Transmitral diastolic inflow for the assessment of left Fig. 52.4: Restrictive transmitral filling pattern. When left ventricular
ventricular filling pressures. (Left panel): mitral inflow in the nor- (LV) filling pressures are severely elevated due to increased LV
mal heart shows early transmitral diastolic inflow greater than diastolic stiffness, early transmitral diastolic flow (E) has a high
late transmitral diastolic inflow (E > A) due to rapid diastolic func- velocity because there is an initial high gradient between very
tion and normal left ventricular filling pressures (LVFP); (Middle elevated left atrial (LA) pressure and LV diastolic pressure.
panel): in the patient with impaired LV relaxation but normal LVFP, However, due to elevated resting LV diastolic pressures, the LA
E is lower than A, as the LV depends more on atrial kick for LV and LV pressures rapidly equilibrate, resulting in a rapid decelera-
filling; this is also termed Grade I diastolic dysfunction (DD); (Right tion time (DT) of transmitral E. In general, in a patient with car-
panel): in the patient with pseudonormal filling pattern, there is diac disease, restrictive filling occurs when E/A > 2 and DT < 150
impaired LV relaxation but E > A, and is caused by elevated LA milliseconds. A = late transmitral diastolic velocity.
pressures; this is also termed Grade I diastolic dysfunction (DD).
Valsalva maneuver and tissue Doppler imaging can help distin-
guish normal from pseudonormal filling (see Fig. 52.5 and text for
details).
Identification of Diastolic Dysfunction this pattern is termed “pseudonormalization.” In markedly

elevated LV filling pressure in which LV stiffness is high,
and Demonstration of Elevated Left the MV is forced open early due to high LA pressure,
Ventricular Filling Pressures but there is rapid equilibration with the high resting
LV diastolic pressure resulting in a rapid deceleration
Transmitral Doppler time of E. This pattern is termed “restrictive filling”
Pulsed Doppler interrogation of mitral valve diastolic (Fig. 52.4).3–5 The grades of diastolic function, as assessed
flow (“mitral inflow pattern”) is critical for the assessment using comprehensive echo Doppler examination, are
of LV filling pressures. Early mitral filling depends on shown in Figure 52.5.
intrinsic LV relaxation, and the difference between LA and
LV early (or “opening”) diastolic pressure.5 In a healthy, Valsalva Maneuver
young heart with normal, rapid diastolic suction, the LV
literally “sucks” blood into the LV, resulting in rapid LA In the Valsalva maneuver in which the patient forces
emptying. In this scenario, there is a relatively tall E-wave expiration against a closed glottis, there is increased
and a shorter A (late diastolic or “atrial contraction” wave; intrathoracic pressure that results in decrease in right
Fig. 52.3). In an LV with impaired relaxation but normal heart filling which by definition, results in decreased LV
LV filling pressures, there is no rapid LV diastolic suction, filling (decreased preload). Since a pseudonormal filling
thus LA emptying is more gradual and results in a relatively pattern exists in the setting of elevated LA pressure in
low velocity E-wave; LA emptying is therefore dependent the presence of impaired LV relaxation, this decrease in
on LA contraction and results in a relatively high amplitude preload lowers LA pressure, which then “unmasks” the
A-wave. In the setting of impaired LV relaxation and mildly underlying impaired relaxation pattern (i.e. E > A in the
elevated LA pressure, high LA pressure that “drives” open setting of impaired relaxation and with Valsalva maneuver
the MV, resulting in a large E-wave and smaller A-wave; changes the transmitral pattern to E < A; Fig. 52.5). On the
pressure in patients with preserved or depressed LVEF

(Figs 52.6A to D).11–14 It should be mentioned that one
study, performed in the intensive care unit in patients in
decompensated heart failure, questioned the correlation
of /e' and LV filling pressures.15 In patients with normal
hearts, E/e' does not accurately predict LV filling pressure
due to correlation of e' with LV filling pressures in such
subjects, as opposed to lack of such a correlation in patients
with cardiac disease.16 The E/e' ratio has since been demon
strated to be useful in estimating LV filling pressures in
hypertrophic cardiomyopathy,17 sinus tachycardia,18 atrial
fibrillation,19 and postcardiac transplantation.20 The E/A
ratio, mitral deceleration time, and E/e' ratio have all been
shown to be useful echocardiographic indicators of LV
Fig. 52.5: Grades of left ventricular diastolic dysfunction. Left diastolic dysfunction, and more particularly, of elevated
ventricular (LV) diastolic function ranges from normal (Grade 0)
LV filling pressures.5 These variables have also been shown
to impaired relaxation (Grade I), to pseudonormal (Grade II), to
restrictive (Grade III), and irreversibly restrictive (Grade IV). LV to be markers of outcome in patients with HF.9,21
relaxation and left atrial pressures (LAp) increase from Grades 0
to IV, as does LA volume. Mitral valve inflow (MVI), tissue Doppler
imaging, Valsalva maneuver, flow propagation velocity (Vp), and
Flow Propagation Velocity
pulmonary venous flow are all helpful in distinguishing grades of In the apical four-chamber view, color Doppler imaging
LV diastolic function and should be used together for an integrated
approach to the assessment of diastolic function as recommended
can be used and then M-mode applied to semi-quantitate
in current guidelines. (Adapted from ref. 31). blood flow across the mitral valve to the LV apex (Fig. 52.7).
In this way, the early diastolic filling wave by color M-mode,
which appears in red color as blood flow from the mitral
other hand, in the setting of normal diastolic function, the valve level to the LV apex can be identified. The slope of this
decrease in preload resulting from the Valsalva maneuver early diastolic color M-mode wave (Vp) is rapid (vertical)
preserves the E > A pattern, without changing it to E < A. in patients with normal diastolic function due to rapid
Therefore, one of the main uses of the Valsalva maneuver— diastolic suction in which blood quickly flows from MV to
similar to tissue Doppler e'—is to help distinguish normal LV apex. However, in the presence of increasingly impaired
from pseudonormal filling pattern. In the presence relaxation, this slope become flatter and flatter, reflecting
of a restrictive filling pattern, the Valsalva maneuver increasingly impaired LV relaxation. A ratio, E/Vp, similar
will decrease preload and therefore help distinguish to E/e, has therefore been developed and validated, and
irreversible restrictive filling pattern (in which E >> A correlates to mean LA pressure.22 An E/Vp > 15 reasonably
will not change) from reversible restrictive filling, where correlates with PCWP > 15 mm Hg, although there are
decreased preload changes the restrictive filling pattern many hemodynamic, rhythmic, and myocardial motion
to either pseudonormal (E > A) or impaired relaxation variables that can impact this relationship. Furthermore,
pattern (E < A), since the increased intrathoracic pressure some studies have shown that, in comparison to invasive
resulting from Valsalva decreases LA pressure. measurement of LV filling pressures, E/e' appears more
accurate than E/Vp.23
Tissue Doppler Imaging
The best estimate of LV relaxation, which is relatively
Pulmonary Venous Flow
nonload dependent in patients with cardiac disease, is Pulmonary venous flow is also of great importance in the
tissue Doppler early diastolic mitral annular velocity assessment of LV diastolic function. In the normal heart
(e');11–14 the slower the LV relaxation, the lower the e' with rapid ventricular suction, the diastolic pulmonary
velocity. The resulting E/e' ratio, has been validated as a venous wave is augmented due to rapid flow through the
reasonably reliable non-invasive indicator of LV filling pulmonary veins into the LA, through the mitral valve, and
A B
C D
Figs 52.6A to D: Tissue Doppler imaging for the assessment of left ventricular (LV) diastolic function. Figure A shows an apical four-
chamber view with moderate to severe dilation of the left atrium (LA) with an LA volume index of 39 mL/m2; Figure B demonstrates
elevated early transmitral diastolic velocity (E) = 117 cm/s; Figure C shows very depressed mitral lateral annular early diastolic relaxation
velocity (e') = 3.5 cm/s; Figure D shows very depressed septal tissue Doppler (TD) early diastolic velocity (e') = 2.5 cm/s. Therefore,
E/e' septal = 47, and E/e' lateral = 33, indicating severely impaired LV relaxation with elevated LV filling pressures. (PCWP: Pulmonary
capillary wedge pressure).
into the LV. Therefore, in the completely normal heart, the pressure; this results in PV S < D, corresponding to E > A
dominant PV diastolic wave corresponds to the dominant (Grade II diastolic dysfunction). Therefore, PV flow is
transmitral E-wave and is a sign of normal LV lussotropic subject to pseudonormalization in the same way as
function; the completely normal heart therefore has transmitral flow. In restrictive filling, the PV pseudonormal
PV S < D. However, when LV relaxation becomes impaired, pattern becomes more exaggerated, with S << D, with a
PV flow during LV diastole becomes truncated, and rapid deceleration time of D. While the PV S/D ratio reflects
therefore most filling occurs during LV systole, resulting mean LA pressure, the PV atrial reversal wave reflects LV
in S > D, which corroborates to transmitral E < A (Grade end-diastolic pressure, as, if the LA contracts against the
I diastolic dysfunction; Fig. 52.8).24 When LA pressure high diastolic pressure in the LV, blood will preferentially
becomes elevated, PV flow during LV systole decreases, flow backward into the PV, as opposed to transmitrally into
as LA pressure in the setting of a closed MV prevents the LV. Therefore, PV Ar wave is higher and longer than
normal PV flow, and PV flow becomes higher in diastolic transmitral A, rendering PV Ar-A a measure of LV end-
when the mitral valve opens, relieving elevated LA diastolic pressure.
Fig. 52.7: Color Doppler flow propagation velocity (Vp) in the Fig. 52.8: Pulmonary venous Doppler velocities in the assess-
assessment of left ventricular diastolic function. Placing the color ment of left atrial pressures. In normal heart, systolic pulmonary
Doppler sample volume from the mitral annular level to the left venous (PV) flow (S) is lower than diastolic PV flow (D), as rapid
ventricular (LV) apex, the more rapid the LV relaxation, the faster left ventricular (LV) suction during diastole results in elevated PV D
blood travels from the mitral annular level to the LV apex, and velocities; thus, PV S < D. In patients with impaired LV relaxation
hence the more vertical the color Doppler mitral inflow M-mode and elevated LA pressure, when the mitral valve (MV) is closed in
and the more rapid the Vp slope (left panel). On the other hand, ventricular systole, the elevated LA pressure prevents PV S flow,
the more impaired the LV relaxation, the slower it takes for blood and therefore most flow occurs when the MV opens, resulting in
to go from the mitral annular to the LV apex, hence a “flatter” Vp S < D. Therefore, as with mitral inflow velocities, PV velocities
slope (right panel). are also prone to pseudonormal filling. In this example, there is
impaired LV relaxation due to cardiomyopathy, but LA pressure
is not elevated; thus, when the MV is closed (LV systole), there is
Pulmonary Artery Pressure unimpeded flow through the PV, and hence S > D.
In the setting of elevated LA pressure, this pressure is

transmitted back into the pulmonary veins and across mean LA pressure, can be estimated from the pulmonary
the pulmonary venous-capillary bed into the pulmonary regurgitation diastolic wave, with RA pressure then added
arterioles and into the pulmonary arteries; therefore, to it as is done with PASP.26
pulmonary arterial hypertension (PAH) can result.25 In this
way, PA pressure estimated by addition of an estimate of Assessment of Diastolic Function in Nonsinus
RA pressure (by assessing IVC size and response to
respiration) is a good surrogate marker of significant,
Rhythm and Other Special Situations
and often chronic, LA pressure elevation (Figs 52.9A One commonly encountered scenario where it can be
and B). As recommended by guidelines, PASP > 35 mm Hg challenging to accurately assess LV diastolic function
often accompanies advanced or significant LV diastolic by echocardiography is in atrial fibrillation. Owing to
dysfunction with elevated LA pressures.5 However, as elevated heart rate, irregular R–R intervals, and loss of
with LA volume, the presence of significant PAH does atrial contraction, echo Doppler assessment can be
not necessarily mean significant diastolic dysfunction, as difficult. However, mitral DT < 150 milliseconds, lack of
significant elevations in pulmonary vascular resistance variation in E-wave velocity despite varying R–R intervals
(PVR) due to intrinsic lung disease must first be excluded. (as there remains an elevated opening gradient between
Likewise, normal PASP can be helpful in excluding the LA and LV at mitral opening—early diastolic filling
significant and long-standing LA pressure elevation, E-wave—despite longer diastolic filling periods when the
provided a complete TR jet is obtained with correct LA pressure should decrease), IVRT < 65 milliseconds,
Doppler sample volume angulation in respect of the elevation of E/e' (>11), and the presence of pulmonary
direction of TR, and with correct RA pressure estimation. hypertension in the absence of lung disease, are all clues
PA end-diastolic pressure, which in the absence of to the presence of elevated LA pressure in the setting of
significant elevations in PVR can be a good estimate of AF.5,27,28 The E/e' ratio can also be used in patients with AF,
A B
Figs 52.9A and B: Pulmonary artery systolic pressure in the assessment of left ventricular diastolic function. In patients with signifi-
cant left ventricular (LV) diastolic dysfunction with chronically elevated LV filling pressures, back pressure through the left atrium (LA),
into the pulmonary veins, and across the pulmonary venous capillary bed into the pulmonary arterioles and pulmonary arteries (PA),
results in elevation of PA pressure. Thus, PA systolic pressure elevation, in the absence of significant intrinsic lung disease and resultant
elevated pulmonary vascular resistance (PVR) is a reasonable correlate of elevated LA pressures. PA systolic pressure can be estimat-
ed by Doppler using the tricuspid regurgitation peak systolic velocity and adding to it an estimate of right atrial (RA) pressure. This image
shows a TR velocity of 3.64 m/s, equivalent to a TR systolic pressure of 53 mm Hg, which indicates at least moderate PA hypertension
in a patient with chronically elevated LA pressure due to ischemic cardiomyopathy and diastolic dysfunction. (RV: Right ventricular).
although with somewhat lower accuracy than in patients

in sinus rhythm, for the estimation of LV filling pressures,
as long as greater than five cardiac cycles are used and
averaged (which holds for any Doppler parameter
in AF).19 In patients who are in supraventricular
tachycardia, atrial flutter, paced rhythm, or heart block,
LV diastolic assessment can be very difficult, although
the presence of both significant LA enlargement and
pulmonary hypertension in the absence of lung disease
can be an important clue to elevated LA pressures in
these scenarios. Another unclear scenario is the effect of
significant mitral regurgitation (MR) on e' and the E/e'
ratio in estimating LV filling pressures. It has been shown
that in patients with secondary MR (due to LV disease),
E/e' accurately predicted PCWP; however, in patients with
Fig. 52.10: An integrated approach to the assessment of left ven- primary MR (due to a primary mitral valve abnormality),
tricular diastolic function: normal LV ejection fraction. As recom- E/e' was not reliably predictive of PCWP.29
mended in the current guidelines, use of multiple echo Doppler
parameters results in a more accurate assessment of left ven-
tricular (LV) diastolic function than using any single echo Doppler INTEGRATING ECHOCARDIOGRAPHIC
parameter in isolation. In the patient with normal LV ejection frac-
tion (EF), it is reasonable to start with early transmitral diastolic
VARIABLES FOR ACCURATE
velocity/tissue Doppler early diastolic velocity (E/e'), as it can be DIAGNOSIS OF DIASTOLIC FUNCTION
difficult to discern whether a patient with preserved LVEF has im-
paired or normal LV relaxation. Following E/e', other echo Doppler The use of a single diastolic variable (such as E/e' or LA
variables are added to result in an accurate assessment of LV volume in isolation) can lead to significant errors in
diastolic function (from ref. 5).
the assessment of LV diastolic function.5 It is therefore
Fig. 52.11: An integrated approach to the assessment of left Fig. 52.12: Longitudinal strain by speckle echocardiography in
ventricular diastolic function: depressed ejection fraction. As rec- the demonstration of systolic myocardial dysfunction in a patient
ommended in the current guidelines, use of multiple echo Dop- with normal left ventricular ejection fraction. This elderly patient
pler parameters results in a more accurate assessment of left presented with dyspnea and underwent echocardiography with
ventricular (LV) diastolic function than using any single echo speckle imaging. Left ventricular ejection fraction (LVEF) was
Doppler parameter in isolation. In the patient with depressed calculated at 55%, while global longitudinal peak strain (GLPS
LV ejection fraction (EF), it is reasonable to start with early and Avg) was −11.1, consistent with significantly decreased
late transmitral diastolic inflow velocities and deceleration time myocardial systolic function (normal longitudinal systolic strain
(E, A and DT, respectively), as it can assumed that patients with less than −16%). GLPS Avg was obtained by averaging GLPS in
depressed LVEF (< 50%) have, by definition, impaired LV relaxa- the apical long-axis (LAX), four-chamber (A4C), and two-chamber
tion. Following transmitral diastolic flow, other echo Doppler varia- (A2C) views. There are substantial data using tissue Doppler and
bles are added to result in an accurate assessment of LV diastolic speckle imaging demonstrating significant systolic abnormalities
function (from ref. 5). in patients with diastolic dysfunction and heart failure despite
normal LVEF. This patient was subsequently diagnosed with dias-
tolic heart failure and single vessel coronary artery disease.
of great importance to integrate several variables—2D, reached, although in some cases, the diastolic assessment
conventional, and tissue Doppler—in order to arrive at a may remain equivocal. Above all, no single diastolic
correct diastolic assessment. Indeed, current guidelines parameter should be used in isolation to arrive at a
recommend an integrated approach of many diastolic diastolic conclusion in a given patient.5
variables (Figs 52.10 to 52.12), and data have shown that
additional echocardiographic variables, when added to NOVEL IMAGING TECHNIQUES AND
E/e' can result in more accurate diastolic determination,
compared to invasively measured LV filling pressures, than
FUTURE DIRECTIONS
E/e' alone.30 Not infrequently, echo Doppler parameters Non-Doppler-based 2D imaging (“speckle echocardi
appear to conflict: for instance, in a patient with normal ography”) tracks signature grayscale characteristic of
LVEF, E/e' = 13, but LA volume is not enlarged, E < A, and points in the LV myocardium, thus providing information
there are normal pulmonary pressures by Doppler. In such on displacement, velocity, deformation, and deformation
cases, the E/e' ratio should likely be dropped, since all rate (strain and strain rate, respectively), independent
other variables point toward normal LV filling pressures. of angulation and cardiac translational motion.32
Therefore, as a rule, multiple echo Doppler parameters of Such variables have provided detailed information on
LV diastolic function should be assessed in every patient,31 myocardial mechanics in hypertensive heart disease,
and the conclusion to which most parameters point hypertrophic cardiomyopathy, diastolic and systolic LV
should be the overall diastolic assessment, with “outlying” failure, as well as in cases of pulmonary hypertension.33–35
parameters discarded. In most cases of conflicting echo Currently, such speckle-based measures are being studied
Doppler diastolic parameters, a cogent conclusion can be to assess their role in identifying patient outcome in
various heart failure states. One of the most attractive 4. Lester SJ, Tajik AJ, Nishimura RA, et al. Unlocking the
features of speckle tracking is that it can demonstrate the mysteries of diastolic function: deciphering the Rosetta
presence of systolic abnormalities, especially regional Stone 10 years later. J Am Coll Cardiol. 2008;51(7):679–89.
5. Nagueh SF, Appleton CP, Gillebert TC, et al. Recomm
ones, in the presence of preserved LVEF in patients with
endations for the evaluation of left ventricular diastolic
cardiac disease (see Fig. 52.12).36 Speckle tracking can function by echocardiography. J Am Soc Echocardiogr.
also demonstrate systolic and diastolic abnormalities in 2009;22(2):107–33.
multiple vectors (longitudinal, radial, circumferential, 6. Abhayaratna WP, Seward JB, Appleton CP, et al. Left atrial
and rotational) as characteristic myocardial markers are size: physiologic determinants and clinical applications.
tracked throughout the cardiac cycle and in space as the J Am Coll Cardiol. 2006;47(12):2357–63.
7. Lang RM, Bierig M, Devereaux RB, et al. Recommendations
heart translates in the thoracic cavity. In particular, patients
for chamber quantification. J Am Soc Echocardiogr 2005;
with diastolic dysfunction and DHF have been shown to 18:1440–63.
have preserved LV twist (systole) and untwist (diastole) 8. Dokainish H, Sengupta R, Pillai M, et al. Assessment of
but impaired longitudinal strain, whereas patients with HF left ventricular systolic function using echocardiography
with depressed EF have impaired twist/untwist as well as in patients with preserved ejection fraction and elevated
depressed longitudinal and circumferential strain.30–34 diastolic pressures. Am J Cardiol. 2008;101(12):1766–71.
9. Dokainish H, Zoghbi WA, Lakkis NM, et al. Incremental
predictive power of B-type natriuretic peptide and
SUMMARY tissue Doppler echocardiography in the prognosis of
patients with congestive heart failure. J Am Coll Cardiol.
Comprehensive echocardiography with 2D imaging, and
2005;45(8):1223–6.
spectral and color Doppler—as well as newer techniques 10. Dokainish H, Zoghbi WA, Lakkis NM, et al. Optimal
like speckle strain echocardiography—provide a complete noninvasive assessment of left ventricular filling pressures:
assessment of cardiac diastolic function. This assessment, a comparison of tissue Doppler echocardiography and
which includes LV mass and regional wall motion B-type natriuretic peptide in patients with pulmonary
assessment, LA volume, transmitral, pulmonary venous, artery catheters. Circulation. 2004;109(20):2432–9.
11. Sohn DW, Chai IH, Lee DJ, et al. Assessment of mitral
and tissue Doppler as well as estimation of PA systolic and
annulus velocity by Doppler tissue imaging in the
diastolic pressures, can provide accurate assessments of evaluation of left ventricular diastolic function. J Am Coll
diastolic function in the majority of patients. It is important Cardiol. 1997;30(2):474–80.
to note that, as recommended in current guidelines, use 12. Nagueh SF, Middleton KJ, Kopelen HA, et al. Doppler tissue
of any single echo Doppler diastolic variable (e.g. only imaging: a noninvasive technique for evaluation of left
E/e') in isolation, can lead to errors. Therefore, it is of ventricular relaxation and estimation of filling pressures.
J Am Coll Cardiol. 1997;30(6):1527–33.
utmost importance that a comprehensive assessment of
13. Ommen SR, Nishimura RA, Appleton CP, et al. Clinical utility
LV diastolic function include integration of all available 2D of Doppler echocardiography and tissue Doppler imaging
and Doppler, and tissue Doppler variables to arrive at the in the estimation of left ventricular filling pressures: A
most accurate diastolic assessment. comparative simultaneous Doppler-catheterization study.
Circulation. 2000;102(15):1788–94.
14. Kasner M, Westermann D, Steendijk P, et al. Utility of
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diastolic heart failure: a consensus statement on the diag with normal ejection fraction: a comparative Doppler-
nosis of heart failure with normal left ventricular ejection conductance catheterization study. Circulation. 2007;
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Associations of the European Society of Cardiology. Eur 15. Mullens W, Borowski AG, Curtin RJ, et al. Tissue Doppler
Heart J. 2007;28(20):2539–50. imaging in the estimation of intracardiac filling pressure
2. Zile MR, Baicu CF, Gaasch WH. Diastolic heart failure– in decompensated patients with advanced systolic heart
abnormalities in active relaxation and passive stiffness of failure. Circulation. 2009;119(1):62–70.
the left ventricle. N Engl J Med. 2004;350(19):1953–9. 16. Firstenberg MS, Levine BD, Garcia MJ, et al. Relationship
3. Oh JK, Hatle L, Tajik AJ, et al. Diastolic heart failure can be of echocardiographic indices to pulmonary capillary
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echocardiography. J Am Coll Cardiol. 2006;47(3):500–6. 2000;36(5):1664–9.
17. Nagueh SF, Lakkis NM, Middleton KJ, et al. Doppler 27. Nagueh SF, Kopelen HA, Quiñones MA. Assessment of left
estimation of left ventricular filling pressures in patients ventricular filling pressures by Doppler in the presence of
with hypertrophic cardiomyopathy. Circulation. 1999;99(2): atrial fibrillation. Circulation. 1996;94(9):2138–45.
254–61. 28. Al-Omari MA, Finstuen J, Appleton CP, et al. Echocardi
18. Nagueh SF, Mikati I, Kopelen HA, et al. Doppler estimation ographic assessment of left ventricular diastolic function
of left ventricular filling pressure in sinus tachycardia. A and filling pressure in atrial fibrillation. Am J Cardiol.
new application of tissue Doppler imaging. Circulation. 2008;101(12):1759–65.
1998;98(16):1644–50. 29. Bruch C, Stypmann J, Gradaus R, et al. Usefulness of
19. Sohn DW, Song JM, Zo JH, et al. Mitral annulus velocity in
tissue Doppler imaging for estimation of filling pressures
the evaluation of left ventricular diastolic function in atrial
in patients with primary or secondary pure mitral regurgi
fibrillation. J Am Soc Echocardiogr. 1999;12(11):927–31.
tation. Am J Cardiol. 2004;93(3):324–8.
20. Sundereswaran L, Nagueh SF, Vardan S, et al. Estimation
of left and right ventricular filling pressures after heart 30. Dokainish H, Nguyen JS, Sengupta R, et al. Do additional
transplantation by tissue Doppler imaging. Am J Cardiol. echocardiographic variables increase the accuracy of E/e’
1998;82(3):352–7. for predicting left ventricular filling pressure in normal
21. Yu CM, Sanderson JE, Marwick TH, et al. Tissue Doppler ejection fraction? An echocardiographic and invasive
imaging a new prognosticator for cardiovascular diseases. hemodynamic study. J Am Soc Echocardiogr. 2010;23(2):
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22. Garcia MJ, Ares MA, Asher C, et al. An index of early left 31 Ommen SR, Nishimura RA. A clinical approach to the
ventricular filling that combined with pulsed Doppler peak assessment of left ventricular diastolic function by
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ventricular ejection fraction on estimation of left ventri sional strain imaging by echocardiography—from tech
cular filling pressures using tissue Doppler and flow nical considerations to clinical applications. J Am Soc
propagation velocity. Am J Cardiol. 2003;91(6):780–4. Echocardiogr. 2007;20(3):234–43.
24. Appleton CP, Galloway JM, Gonzalez MS, et al. Estimation 33. Wang J, Khoury DS, Yue Y, et al. Preserved left ventricular
of left ventricular filling pressures using two-dimensional
twist and circumferential deformation, but depressed
and Doppler echocardiography in adult patients with
longitudinal and radial deformation in patients with
cardiac disease. Additional value of analyzing left atrial
diastolic heart failure. Eur Heart J. 2008;29(10):1283–9.
size, left atrial ejection fraction and the difference in
34. Wang J, Khoury DS, Yue Y, et al. Left ventricular untwisting
duration of pulmonary venous and mitral flow velocity at
atrial contraction. J Am Coll Cardiol. 1993;22(7):1972–82. rate by speckle tracking echocardiography. Circulation.
25. Neuman Y, Kotliroff A, Bental T, et al. Pulmonary artery 2007;116(22):2580–6.
pressure and diastolic dysfunction in normal left ventricular 35. Dokainish H, Sengupta R, Pillai M, et al. Usefulness of new
systolic function. Int J Cardiol. 2008;127(2):174–8. diastolic strain and strain rate indexes for the estimation of
26. Paraskevaidis IA, Tsiapras DP, Karavolias GK, et al. Doppler- left ventricular filling pressure. Am J Cardiol. 2008;101(10):
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model using the combined analysis of mitral and 36. Nguyen JS, Lakkis NM, Bobek J, et al. Systolic and diastolic
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Am J Cardiol. 2002;90(7):720–4. pressure. J Am Soc Echocardiogr. 2010;23(12):1273–80.
CHAPTER 53
Evaluation of the Right Ventricle
Vincent L Sorrell, Steve W Leung, Brandon Fornwalt
Snapshot
General Overview
¾¾ Two-Dimensional Strain (Speckle Tracking)
¾¾
¾¾Right Ventricle Morphology Three-Dimensional Echocardiography
¾¾
¾¾Echocardiography ¾¾Transesophageal Echocardiography
¾¾M-Mode Echocardiography ¾¾ Hemodynamics
¾¾ Two-Dimensional Echocardiography ¾¾ Other Imaging Modalities
¾¾ Doppler Echocardiography
GENERAL OVERVIEW short axis) is triangular (curved); axial view (echo long axis)
is crescent-shaped; and coronal view (not possible with
The assessment of the right ventricle (RV) is valuable in two-dimensional [2D] echo) is most similar to a teapot
many patients with heart disease. In patients with either (Fig. 53.1). The RV myocardial wall is highly trabeculated
RV volume overload (e.g. repaired tetralogy of Fallot [TOF], and barely 3 mm thin. In summary, there is no convenient
atrial septal defect [ASD], anomalous pulmonary venous geometric model that accurately approximates the normal
return, tricuspid regurgitation [TR] from any cause) or or the diseased RV shape.
RV pressure overload (e.g. pulmonary hypertension from In addition to the variable shape, the regional contr
any cause, pulmonary stenosis), management decisions action pattern is also unique to the RV. The normal RV
increasingly rely on evaluation of the RV size and function. apex is virtually immobile and tethered to the LV apex,
Their trends during serial follow-up examinations and therefore is dominated by the shape and function of
predict heart failure, arrhythmias, and death and must the adjacent left ventricular apex. The global RV systolic
be reliable.1 The noninvasive diagnostic evaluation of RV function is strongly influenced by the normally concave
size and function in normal and pathological conditions interventricular septum and ventricular interdependence.
is daunting due to its complex shape, nonsymmetrical Acute and chronic pathological pressure and volume
regional contraction pattern, and the lack of published overload will greatly impact global and regional RV
literature on normal reference values. Most physicians performance.
practicing echocardiography are comfortable analyzing Finally, global RV performance is influenced by
the relatively simple circular geometry of the left ventricle volume shifts that occur with normal respiration. During
(LV), but the RV is shaped like a “pyramidal banana.” The inspiration, venous return increases, causing an increased
inflow and outflow portions are separated. The normal RV RV preload, with a slight but detectable increase in RV
shape varies depending on orientation: sagittal view (echo stroke volume. Therefore, when quantifying RV volume
Chapter 53: Evaluation of the Right Ventricle 1135
Fig. 53.1: Three dimensional display of complex RV geometry. Fig. 53.2: Left Image: Three-dimensional echocardiography (3DE)
Left column demonstrates a 3D echo and the middle column is volume rendered display with color-coded regions representing
a 3D cardiac MR (CMR) exam of a normal patient. Top row: ”car- the triangle of dysplasia. The arrows in the inflow and outflow re-
diac-aligned” short axis; Middle row: sagittal orientation; Bottom gions reflect the myofiber alignment and direction of contraction.
row: oblique coronal or frontal plane. The two images in the right The larger center arrow represents the ventricular septal motion
column represent conventional long axis orientation (four-cham- into the cavity (toward the left ventricle). Right Image: Posterior-
ber view) of CMR (top) and 2D echo (bottom). anterior orientation of a cadaveric cast from a normal RV. Note the
complex nongeometric shape as well as the extensive trabecula-
tion throughout the RV myocardium, although slightly less obvious
and function with echo, one should consider whether data in the so-called “smooth” inflow region.
Courtesy: Special thanks to Frank Marcus for the image.
were acquired during inspiration, expiration, or apnea
(preferred).
These features unique to the RV result in highly band is a variably prominent muscular extension that
variable interpretations despite highly trained experts in houses the electrical apparatus of the right bundle of His as
echocardiography. Most clinical studies simply use the it travels from the ventricular septum to the anterolateral
“eye-ball” qualitative assessment rather than resorting region of the RV.
to quantitative, or even semiquantitative, estimates of These RV regions are commonly the initial sites
RV size and function. Unfortunately, compared with the involved in pathology and have been termed the “triangle
reference standard (cardiac magnetic resonance imaging of dysplasia.”3 These anatomical regions develop separ
[CMR]), the ability to accurately detect severely dilated RV ately and at distinct embryological time points and are
size or moderate to severe RV dysfunction is low and the consequently independently subjected to congenital
interobserver variability is extremely poor.2 malformations. Each anatomical region has been
This chapter describes the unique characteristics of demonstrated to have unique responses to pathology as
the RV and offers a comprehensive, quantitative echocar well as pharmacological interventions. The right ventri
diographic (and multimodal imaging) approach to the cular outflow tract (RVOT) has been demonstrated to
investigation of the normal and pathological RV. be more reactive than the RV inflow tract to inotropic
stimulation.4 This may be important when evaluating RV
response to treatment with inotropic drugs.
RIGHT VENTRICLE MORPHOLOGY
The global RV systolic function is determined by the
The RV can be considered to comprise three individual following individual RV contraction patterns: (a) move
and separate components—the apex, the inflow, and the ment of the basal free wall toward the apex (the “bellows
outflow (Fig. 53.2). Although there is significant individual effect”); (b) the contraction of the RVOT; and (c) the
normal variability, the anterior and posterior trabecular contribution of the LV (tethering) at the interventricular
muscles, and the smaller medial papillary muscle are insertion sites. The influence of the ventricular septum
within the RV inflow region and connected to the tricuspid (interventricular dependence) is illustrated by impairment
valve leaflets via the chordae tendineae. The moderator of RV function due to the adjacent diseased LV, but not
muscle bundles, because these may alter the symmetric

contraction of the RV free wall.7,8 Moreover, different
regions of the RV contract at different times in healthy
volunteers with the inflow region reaching peak
contraction first, followed by the outflow region and lastly
the apex approximately 100 ms later.9 Although severe focal
regional wall motion abnormalities may exist in diseases
such as arrhythmogenic RV dysplasia/cardiomyopathy
(Movie clip 53.3B), these more subtle, normal regional
variations need to be recognized as normal or else they may
inadvertently lead to misinterpretation with important
downstream consequences.
ECHOCARDIOGRAPHY
Fig. 53.3: Cardiac magnetic resonance imaging midventricular
Echocardiography is and will likely remain a first-line
steady-state free precession (SSFP) images of a normal (control;
top) and pathological (tetralogy; bottom) heart demonstrating the diagnostic imaging modality for evaluating the RV
variation in time–volume (TV) curves (right side). Note the dilated structure and function because of its wide-spread availa
right ventricle (RV) and delay in contraction of the surgically re- bility and the fact that it is a noninvasive, rapid, and
paired tetralogy of Fallot patient (tetralogy). Right ventricular en- portable tool. It provides a comprehensive approach
docardial tracing and TV curves, green; left ventricular endocar-
dial tracing and TV curves, yellow.
to assess patients with suspected right heart disease.
Accurate evaluation of RV morphology and function
requires integration of multiple echocardiographic views,
necessarily due to a specific myopathic process of the including parasternal long- and short-axis, RV inflow,
RV myocardium.5 Given a normal pericardium, the LV apical (RV modified) four-chamber, and subcostal views.10
is estimated to contribute between 20% and 60% of the Although multiple quantitative methods for RV
function of the RV.6 Despite this knowledge, the ventricular assessment are provided, the routine assessment of RV
septum has been relatively ignored as a biventricular structure and function is mostly qualitative or semiqua
muscle region. It remains unknown how best to include ntitative in clinical practice (Fig. 53.4). Nor
mally, the
this region in calculation of LV and RV function. cardiac apex is formed by the LV, but when significantly
Some experts advocate measuring RV and LV volumes dilated, the RV is “apex-forming.” Methods commonly
at individual time-points of the cardiac cycle to identify the used to calculate the LV volume may be used to calculate
maximal diastolic volumes, while others ignore the extreme RV volumes, but are less accurate due to the complex
interventricular dependence and measure ventricular geometry. Due to these inherent limitations, a number of
volumes when the septum is at the midline. Results will geometry-independent parameters have been proposed.
often be strikingly different and these differences in the Recently published guidelines on the echo evaluation
timing of minimum and maximum ventricular volumes of the right heart recognize that there are limitations in
are particularly evident in disease states with disturbed available published normal references and therefore, most
electrical conduction as commonly seen in left bundle categories are reported as normal or abnormal, rather
branch block or repaired TOF (Fig. 53.3). Therefore, for than mild, moderate, or severe disease, which is common
serial investigations, research studies, or just clinical in reporting the left heart.11
consistency, these authors recommend selecting a lab
preference and being consistent. To reiterate, these authors
believe that interstudy and test–retest reproducibility is
M-MODE ECHOCARDIOGRAPHY
most important and, given the lack of a true gold standard, The myofibrillar arrangement of the RV consists of mainly
outweighs methods aimed entirely at accuracy. subepicardial circumferential fibers and subendocardial
The evaluation of regional RV wall motion must longitudinal fibers in the inflow region and both
take into consideration the normal variable contraction subepicardial and subendocardial longitudinal fibers in
patterns near the moderator, parietal, and septomarginal the outflow region. The majority of the RV myocardium
Fig. 53.4: Echo windows for right ventricle (RV) assessment. Graphical illustration of the 11 recommended two-dimensional (2D) echo-
cardiographic images from a transthoracic approach highlighting the parasternal long, parasternal short, apical, and subcostal views to
obtain a comprehensive assessment of RV size and function. For additional details, see Rudski LG, et al. Guidelines for the Echocar-
diographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography. J Am Soc Echocardiogr.
2010;23:685–713.
lacks the middle circumferential myofiber array that is Doppler tissue imaging (DTI) color display. Interestingly,
dominant in the LV and consequently is much more for such a simple marker of RV function, the correlation
dependent on longitudinal shortening for global ejection between TAPSE and right ventricular ejection fraction
than the LV.12 The longitudinal RV contraction at the base (RVEF) by CMR was superior to first pass radionuclide
is important in understanding and estimating RV function. techniques and three-dimensional (3D) echo estimates
Because the predominant RV fractional shortening is of RVEF in a single-center investigation of a population of
significantly greater longitudinally than circumferentially, patients with ischemia or pulmonary hypertension.13
an evaluation of longitudinal shortening provides a rela However, this population had a relatively narrow RVEF
tively simple and reliable estimate of global RV function. range (58% ± 3%), there was significant variation in results,
The total tricuspid annular descent or tricuspid annular and despite having the best correlation coefficient, it was
plane systolic excursion (TAPSE) is an important marker not high (r = 0.48). It is possible that the simplicity and
of RV global systolic function (Fig. 53.5). Combining the relative reproducibility of this displacement parameter
findings from 46 studies investigating this value (N = 2320), partially compensates for the single-dimensional nature
the normal range can be reported as 22–24 mm (95% CI that lowers the accuracy when regional RV dysfunction
15–31 mm).11 The TAPSE can be derived from M-mode is present. Other clinical investigators studying more
analysis of the lateral tricuspid annular ring or from variable patient populations have found this real-world
Fig. 53.5: Two different patients’ two-dimensional (2D) echocar- Fig. 53.6: Graphical comparison of tricuspid annular plane systolic
diograms, apical four-chamber orientation (top row), diastolic (left) excursion (TAPSE) and RVEF as measured by cardiac magnetic
and systolic (right) frames, and associated M-mode from the right resonance imaging in normal controls, repaired tetralogy of Fal-
ventricle (RV) tricuspid annulus (bottom row). The patient dis- lot, and patients with atrial septal defect and pulmonary hyperten-
played on the left has a normal RV and the patient displayed on sion. These authors found a greater ability of TAPSE over RVEF
the right has marked RV dilation and dysfunction. Arrows repre- measures to separate right ventricle (RV) volume overload and
sent the systolic excursion of the tricuspid annular plane systolic RV pressure overload clinical syndromes. The y-axis is both per-
excursion (TAPSE). centage (for RVEF; red columns) and distance in millimeters (for
TAPSE; blue columns). By multiplying the TAPSE value by 2.19,
the TAPSE result is converted to a value that equals the normal
clinical value of TAPSE to remain when compared with controls RVEF (green columns) and further demonstrates the
3D techniques (r = 0.64).14 Another potential consid greater ability of TAPSE compared to RVEF to demonstrate statis-
tical differences (P < 0.05). (ASD: Atrial septal defect; ASD + PHT:
eration might be that the TAPSE parameter, being Atrial septal defect and pulmonary hypertension; TOF: repaired
single-dimensional and limited to the RV myocardial tetralogy of Fallot).
performance, at times is superior to 3D parameters. Since
3D methods will invariably include the curved ventricular dilation is subject to significant potential error. Despite
septum, it is possible that the 3D data may contaminate attempts to obtain orthogonal RV images necessary for
the actual RV performance by including a component volume calculations, most commonly using the apical
of LV contractile function. This is suggested by recent four-chamber and subcostal views, it remains difficult
investigations of CMR parameters of RV displacement to validate that they are orthogonal.17 Image acquisition
using a tagging sequence that approximates TAPSE.15 In should obtain the maximal diameter of the tricuspid valve
this study, the investigators used CMR to demonstrate annulus to ensure appropriate relative alignment and
that the simple (and semiautomated) single-dimensional avoid cutting through the LV in a “noncenter” trajectory
marker of RV function out-performed the 3D traditional (Fig. 53.7).
parameter of RVEF (Fig. 53.6). This is an area of intense The RVOT is composed of a preponderance of circum
investigation in the hope of creating a potential automated ferential myofibers and carefully evaluating the motion in
and reliable CMR-based tool for quantifying RV function.16 this region provides an estimate of global RV function. The
RVOT fractional shortening (RVFS%) can be calculated as
TWO-DIMENSIONAL the percentage of the RVOT diastolic diameter minus the
systolic diameter divided by the diastolic diameter. Either
ECHOCARDIOGRAPHY 2DE or M-mode echocardiography of the basal parasternal
The RV is typically smaller than the LV when normal, and short-axis view at the level of the aortic root can be used and
normally viewed in the apical four-chamber view using has been shown to correlate with TAPSE.18 Importantly, it
2D echo (2DE). However, it may be difficult to confirm closely correlates with other physiological events, such as
the optimal alignment of these ventricles. Therefore, using the shortened pulmonary acceleration time recorded at
relative dimensions as the sole criterion to diagnose RV the cusp level in patients with pulmonary hypertension.
Fig. 53.7: Two-dimensional (2D) echocardiogram, short-axis orien- Fig. 53.8: See Movie clip 53.6A. Contrast-enhanced two-dimen-
tation, midventricular position (left), and accompanying schematic sional echocardiogram, zoom apical four-chamber orientation,
(inset). Images on the right represent the apical four-chamber focused on the right ventricle (RV) apex. The center dark line rep-
cut-planes and accompanying schematic (inset). When aligned resents the ventricular septum (myocardium). The unenhanced,
correctly through the midcavity of the left ventricle (LV: Solid white well-circumscribed, 2.0 cm × 1.5 cm filling defect in the RV apex
line), the LV/right ventricle (RV) ratio is > 1.5:1. When aligned represents a large RV thrombus. The dark region toward the base
incorrectly superior (dashed yellow line) or inferior (dashed pink of the RV cavity represents attenuation artifact from the dense
line), this normal ratio may change and the normal RV may inad- manufactured contrast agent.
vertently appear relatively dilated. Although these lines are graphi-
cally displayed as parallel, in actual clinical practice these arise
from the same transducer location point and are more divergent.
Global, systolic RV function can also be simply assessed DOPPLER ECHOCARDIOGRAPHY

quantitatively using 2DE as a percentage of change in the
RV cavity area from end-diastole to end-systole in the Conventional Doppler
apical four-chamber view. End-diastole is identified by the Importantly, all conventional Doppler techniques are
onset of the R-wave, whereas end-systole is regarded as the subject to increased error as the quality of the spectral
smallest RV cavity just before the tricuspid valve opening. Doppler signal worsens and therefore, special care
Endocardial borders of the RV free wall and septum should be taken during image acquisition. The rate of RV
are traced from base to apex and the RV fractional area pressure increase is derived from the continuous wave
change (RV FAC) is defined using the following formula: Doppler (CWD) spectral display of the TR signal. The time
(end-diastolic area – end-systolic area)/(end-diastolic area) interval (dt) necessary to increase the TR velocity from
× 100). Heavy RV trabeculation may render border tracing baseline to 2.0 m/s represents a change in pressure (dP) of
difficult and requires good image quality for accuracy. 16 mm Hg. When the TR velocity is elevated, time intervals
This technique incorporates the RV inflow tract and the from 1.0 m/s to 2.5 or 3.0 m/s (dP = 21 mm Hg and 32 mm
apex but excludes the RVOT and may overestimate RV Hg, respectively) can alternatively be obtained (similar to
function if focal regional dysfunction (e.g. after surgical LV dP/dt estimates) and reduce error from using very low
repair of TOF) exists in this region. Intravenous contrast velocities. The dP/dt value is considered normal when >400
agents designed for the LV may assist in image quality of mm Hg/s (Fig. 53.10).20 Since the dP/dt is dependent on
the RV and may unmask RV thrombus (Fig. 53.8; Movie preload, the maximal TR velocity (TRmax) can be included
clip 53.6A). The percentage of RV FAC is a relatively in the equation (dP/dt/TRmax) and partially compensate
simple parameter that is a surrogate marker of the RVEF for this.21 In patients with predominant RV failure, the TR-
and correlates well with CMR-derived RVEF (r = 0.80; derived dP/dt/TRmax, but not dP/dt alone, was shown to
Fig. 53.9).19 be a clinically useful index of global RV contractility.
Fig. 53.9: Two-dimensional (2D) echocardiogram, apical four- Fig. 53.10: Top Left: Two-dimensional (2D) echocardiogram,
chamber orientation, diastolic (left) and systolic frame (right). short-axis orientation, midventricular level demonstrating a mark-
The right ventricle (RV) endocardial border has been traced for edly dilated right ventricle (RV), concave septum toward the
estimating the volumes (and calculating the fractional area change left ventricle (LV), and RVH in a patient with severe pulmonary
[FAC]) using the Simpson’s method of discs. In this patient with hypertension and a reduced RV dP/dt of 320 mm Hg/s. Bottom
severe global RV systolic dysfunction, the FAC was 13%. (FAC: left: Continuous wave Doppler spectral display of the tricuspid
Fractional area change; RVd: RV diastolic volume; RVs: RV regurgitation (TR) signal confirming the elevated TR maximal
systolic volume). velocity (> 5 m/s; >100 mm Hg). Right panel: Zoom image of the
TR spectral continuous wave Doppler (CWD) display used to esti-
mate the dP/dt. White arrow = TR flow at 1 m/s (4 mm Hg); Black
The pulsed wave Doppler (PWD) spectral display of arrow = TR flow at 3 m/s (36 mm Hg); ΔP = estimated change in
the RV inflow can be used as an estimate of RV diastolic pressure from 1 to 3 m/s; Δt = measured time from 1 m/s to 3 m/s;
TRmax = maximal TR velocity.
function. Similar to LV inflow patterns, peak early velocity
(E-wave) and its deceleration time, late velocity during
atrial contraction (A-wave) and its duration, are parameters Tissue Doppler can be used to record the peak
that reflect right-heart pathology. The hepatic and vena systolic velocity of the tricuspid annulus (S'). In healthy
cava PWD patterns also reflect RV hemodynamics and individuals, the lower normal limit at the basal RV lateral
increased right-sided heart filling pressures lead to wall is ≥ 14 ± 2 cm/s for DTI spectral displays and ≥ 10 ± 2
increased flow reversal in the hepatic vein (HV > 20%) or cm/s for DTI color displays. This velocity has been shown
superior vena cava (SVC > 10%) during apnea. Increased to correlate more closely with CMR-derived RVEF than
flow reversals in response to inspiration or expiration the 2D fractional area change (FAC), DTI-derived tissue
can be seen in patients with restrictive or constrictive displacement, systolic strain, and strain rate.24 An S' < 9.5
cardiomyopathies, respectively.22,23 cm/s identifies patients with an RVEF < 40%.25 Thresholds
of > 12, 12–9, and < 9 cm/s allow differentiation between
normal (> 55%), moderately reduced (30–55%), and
Tissue Doppler severely reduced (< 30%) RVEF, respectively.26
The pulmonary circulation normally has a low vascular Myocardial velocity of the RV free wall as measured
resistance, and consequently, a very short (or unde by DTI during the IVCT phase (IVCv) has also been used
tectable) isovolumic contraction time (IVCT) and isovo to estimate RV contractility (Fig. 53.11). Although this
lumic relaxation time (IVRT). The superficial circum parameter appears more sensitive to loading conditions
ferential fibers contract during the IVCT and the deeper than myocardial acceleration and other listed parameters,
longitudinal fibers contract during ejection. The onset of this parameter demonstrated the ability to predict
RV ejection at the outflow tract is delayed after the onset of outcomes in patients with pulmonary artery hypertension
contraction of the inflow tract. This regional RV contractility (PAH).27 In 142 patients with PAH, the 6-minute walk test
requires high temporal resolution to be recognized and (≤ 400 M) and IVCv (≤ 9 cm/s) were the only clinical or
provides a basis for color mapping of the myocardium with echo parameters that predicted mortality. Myocardial
advanced tissue Doppler or speckle tracking techniques. acceleration during the earliest phase of IVCT is a novel
Fig. 53.11: Two-dimensional (2D) echocardiogram, apical four- Fig. 53.12: Illustration of tissue Doppler spectral display of the
chamber orientation, tissue Doppler color map display of mean right ventricle (RV) tricuspid annulus highlighting the RV myo-
myocardial displacement (derivative of velocity/time). The arrow cardial movements and representative measurements during the
points to the basal right ventricle (RV) myocardial region, which entire cardiac cycle. Inset: Actual tissue Doppler spectral display
we have found should remain purple (≥ 12 mm) for approximately of the RV tricuspid annulus in a patient. (Ea: Early diastolic veloc-
50% of the length of the RV free wall in normal individuals. This ity of the RV annulus that occurs during early RV filling; Aa: Late
is a quick semiquantitative tool that appears to correlate with diastolic velocity of the RV annulus that occurs during the atrial
tricuspid annular plane systolic excursion (TAPSE). contraction; Sa: Systolic velocity of the RV annulus that occurs
during RV systolic contraction; IVCT: Isovolumic contraction time;
IVRT: Isovolumic relaxation time; IVV: Isovolumic velocity;
At: Time to reach maximal IVV.
index for the assessment of RV contractile function that is (near the apex) myofibers.4 Unlike tissue Doppler analysis
less affected by preload and afterload changes.28 This index which is subject to error from cursor angle misalignment,
is calculated by dividing myocardial velocity during IVCT 2D strain (speckle tracking) is angle-independent,
by the time interval from the onset of this wave to the time allowing for the evaluation of regional function in all
at peak velocity. myocardial segments (including the apex). Ultrasound of
The RV index of myocardial performance (RIMP; or Tei the myocardium has natural small variations in decibels
index) is defined as the sum of the IVCT and IVRT divided (speckles) that are inherent to the characterization of the
by the ejection time and is increased in either systolic or RV wall and can be tracked throughout the cardiac cycle.
diastolic RV dysfunction.29 This parameter is relatively These provide a detailed regional determination of frame-
simple to obtain with high quality tissue Doppler or to-frame myocardial deformation. Peak systolic strain
conventional PWD, is a marker of early disease in cardiac and strain rate, particularly of the basal RV free wall, are
amyloidosis, and predicts symptoms in hypertrophic significantly impaired in patients with pulmonary arterial
cardiomyopathy (Figs 53.12 and 53.13).30 A value < 0.25 hypertension and have been used as an index of global RV
predicts an RVEF ≥ 0.50 (sensitivity 70%, specificity 89%) function.32 The longitudinal RV strain and strain rate values
and ≥ 0.40 predicts an RVEF < 35% (81%, 85%).31 are higher and more inhomogeneous than values reported
for the LV. Longitudinal strain and strain rate values are
TWO-DIMENSIONAL STRAIN lowest in the RV base and increase toward the RV apex.
Strain rate imaging is independent of overall motion.
(SPECKLE TRACKING) This technique has significant potential as the initial and
The RV myocardium is normally only 3–4 mm thin. serial diagnostic tool to assess patients with known or
Within this thin layer of myocardium resides a complex suspected RV pathology, and correlates with invasive
arrangement of circumferential (parallel to the aortic and noninvasive reference standards of RV performance
valve (AV) groove and encircling the RVOT) and spiral (Figs 53.14A and B).33,34 Moreover, strain rates are much
Fig. 53.13: Pulsed wave Doppler (PWD) spectral display of the

right ventricle (RV) inflow (upper left insert) and RV outflow (lower
right insert) and an illustrative drawing of these Doppler displays
demonstrating an alternative method from Figure 53.12 to
calculate the Tei index. (E: RV inflow early diastolic wave; A: RV
inflow late (atrial) diastolic wave; TRd: Tricuspid regurgitation
duration; ET: RV ejection time; RVOT: Right ventricular outflow tract
PWD flow; IVCT: Isovolumic contraction time; IVRT: Isovolumic
relaxation time; IMP: Index of myocardial performance.
A B
Figs 53.14A and B: Two-dimensional (2D) echocardiogram, apical four-chamber orientation, angulated slightly rightward to visualize
the entire right ventricle (RV) apex with 2D strain color map (speckle tracking) display of the RV myocardium. A. Diastolic frame (arrow =
apical variant hypertrophic cardiomyopathy); B. Systolic frame (arrow = abnormal distal ventricular septum due to adjacent pathological
LV myocardium). For additional details, see Abdy NA, et al. Apical Hypertrophic Cardiomyopathy in an Adolescent. Congen Heart Dis.
2010;5(2):182–87.
less load-dependent than strains, volumes, or ejection stroke volume, but the upper limit of normal RV volume
fraction, which is particularly important in the RV, where is greater than the LV. This explains why the lower limit
preload varies significantly with respiration.35 of normal RVEF is lower than the left ventricular ejection
Recently, velocity vector imaging of the RV (longitu fraction (LVEF; e.g. RV in diastole, 100 mL; RV in systole, 55
dinal strain), was used to predict RV failure after left mL; RV stroke volume, 45 mL; RVEF = 45/100 = 45%; LV in
ventricular assist device placement. In this report, a diastole, 90 mL; LV in systole, 45 mL; LV stroke volume, 45
peak strain cutoff of –9.6% predicted RV failure with 76% mL; LVEF = 45/90 = 50%).
specificity and 68% sensitivity.36 Three-dimensional echocardiography analysis of the
RV has recently been reported as a means to eliminate
THREE-DIMENSIONAL the geometric intricacies of the RV. Real time 3D echo
(RT3DE) has recently become a reliable, reproducible
ECHOCARDIOGRAPHY tool to measure the LV. Although less well reported, this
In the absence of cardiac shunting (or significant atrioven technique provides an evaluation of the RV independent
tricular valve regurgitation), the LV and RV have the same of geometric assumptions.37 The RV volumes and RVEF are
Table 53.1: Normal Right Ventricular Volume/Body Surface Area

Volume CMR (mL/m2) RT3DE (mL/m2)
n Mean (SD) Mean (SD)
EDV:
● All 71 71.3 (12.9) 70.0 (12.9)
● Male 36 67.1 (12.1) 56.4 (13.4)
● Female 35 75.6 (12.4) 74.7 (13.0)
ESV:
● All 71 33.5 (9.9) 33.4 (10.3)
● Male 36 28.6 (8.1) 29.2 (10.7)
● Female 35 38.4 (9.1) 37.8 (7.4)
RVEF:
● All 71 53.3 (8.7) 52.6 (9.9)
● Male 36 57.5 (7.0) 56.2 (9.1)
● Female 35 49.0 (8.8) 48.9 (9.5)
Normal right ventricular volumes (indexed to body surface area) as measured with CMR and RT3DE categorized by gender to end-
diastolic and end-systolic volumes and the resulting RVEF. Table modified from Reference 38. For additional details, see Gopal AS,
et al. Normal values of right ventricular size and function by real time three-dimensional echocardiography: comparison to cardiac
magnetic resonance imaging. J Am Soc Echocardiogr. 2007;20:445–55.
(CMR: Cardiac magnetic resonance imaging; EDV: End-diastolic volume; ESV: End-systolic volume; RT3DE: Real time three-di-
mensinal echocardiography; RVEF: Right ventricular ejection fraction).
determined by manual tracing of the endocardial borders technique matched the direct surgical measures (r2 = 0.99)
and require adequate image quality for this purpose. obtained by injecting saline solution through the tricuspid
However, due to the fact that the entire RV is acquired in valve using a graduated syringe. In the clinical setting of
a single pyramidal data set, any acoustic window may be tricuspid or pulmonic valve pathology, TEE is a valuable
used and this increases the likelihood that an adequate complementary diagnostic tool.
image is obtained. Head-to-head comparison of 3D
techniques to 2D techniques consistently demonstrate HEMODYNAMICS
larger volumes and closer agreement as well as higher
Although not a direct estimate of RV volume or function,
reproducibility relative to CMR. Normal RT3DE values of
the evaluation of right heart hemodynamics is exceedingly
the RV size and function have been reported (Table 53.1).38
valuable when right heart pathology is suspected. The
The technique of RT3DE has been validated in phantoms,
right atrial pressure (RAP) is readily estimated from
animals studies, adults with acquired RV pathology, and
the inferior vena cava (IVC) dynamics and the caval
children with congenital heart diseases.39
and HV flows. More recently, DTI has also been used to
evaluate this parameter. Classically, a dilated IVC, lack of
TRANSESOPHAGEAL inspiratory collapse, E/e' ratio > 6, atrial septal leftward
ECHOCARDIOGRAPHY bulge, predominant diastolic flow patterns in the SVC,
When the transthoracic ultrasound window is suboptimal or HVs suggest an elevated RAP. However, these features
and CMR is not available, transesophageal echo (TEE) may be normal in athletes, obese individuals, congenital
may be performed. Most reports on TEE evaluation of narrowing of the IVC–RA junction (Budd–Chiari), cor
the RV are from intraoperative studies and may not be triatriatum dexter, or mechanical ventilation. Importantly,
as clinically relevant due to the frequent administration IVC imaging should be performed in the supine (not left
of inotropic medications and rapid fluid shifts in this lateral decubitus) position. For specific RAP values, it is
population. In a study of 25 children operated on for ASDs, somewhat reliable to use 0–20 mm Hg range at 5 mm Hg
90% had adequate 3D TEE studies.40 RV volumes with this intervals. If the IVC size is < 21 mm and the inspiratory
RAP or LV systolic pressure (cuff pressure in absence of

outflow gradients), and provide an avenue to noninvasively
estimate intracardiac pressures.44
OTHER IMAGING MODALITIES

Extensive review of multimodal imaging of the RV is
available elsewhere, but a brief update on the capabilities of
CMR and computed tomography is worthwhile to provide
the reader with an understanding of these contemporary
diagnostic imaging capabilities.45,46 The development of
CMR has advanced significantly over the past two decades.
Since CMR is not limited by the ultrasound acquisition
window, the entire RV can be easily visualized (Fig. 53.15
and Movie clip 53.9). This allows qualitative assessment
Fig. 53.15: See Movie clip 53.9. Cardiac magnetic resonance
of right ventricular wall motion and reliable quantitative
imaging, diastolic frame from a cine image, four-chamber long-axis
orientation, demonstrating the clarity of the right ventricle (RV) that assessment of chamber size, mass, and ejection fraction,
is expected despite body habitus or lung disease that often limits and is considered the reference standard when assessing
the visualization of echocardiography. Chamber size and ejection new echo techniques.47 These properties have made
fraction can be measured by obtaining multiple slices that covers
the entire RV and manually tracing the endocardial borders.
CMR a valuable tool in the assessment of primary RV
cardiomyopathies and cor pulmonale.48,49 CMR can also
be used to evaluate vascular anatomy and quantify blood
narrowing is > 50%, the RAP is 0–5 mm Hg. Use 5 mm Hg if flow and is an important tool in the evaluation of patients
any other signs of elevated RAP exist. If either one of these with complex congenital heart disease.50,51 Despite being
two parameters is abnormal, use 5–10 mm Hg (10 mm Hg considered a reference standard due to the excellent
if other signs exist). If both size and respiratory variation interstudy reproducibility and lack of a gold standard,
are abnormal, use 15 mm Hg (or 20 mm Hg if IVC plethora highly skilled technologists and interpreting physicians are
exists). The dynamic inspiratory change > 50% is more as necessary for CMR studies as they are for echo studies.
important than the IVC size.41 A vital aspect of CMR is the ability to evaluate tissue
In mechanically ventilated individuals, more complex characteristics. RV infarction can be easily detected with
assessment is necessary to estimate RAP, such as the HV late gadolinium contrast-enhancement imaging.52,53
systolic filling fraction (HVFF = VsVTI/VdVTI) < 55%, This CMR sequence has also been demonstrated to be a
which has a sensitivity and specificity of 86% and 90%, reliable way to assess thrombus, not uncommonly found
respectively, for diagnosing RAP > 8 mm Hg.42 [VsVTI: in the RV.54–56 Yet another important strength of CMR for
Systolic (hepatic) venous velocity time integral; VdVTI: quantification of RV function is the ability to quantify
Diastolic (hepatic) venous velocity time integral]. Another regional and global RV mechanics (such as strains
formula successfully used in mechanically ventilated and strain rates) using techniques such as myocardial
patients incorporates the tricuspid inflow and DTI of the tagging.57 However, due to the thin walls of the RV, tagging
tricuspid annulus: 1.62 E/e' + 2.13 = RAP (r = 0.7).43 This methods are mostly limited to assessment of longitudinal
was assessed in patients with recent cardiac surgery. RV deformation. However, newer MRI techniques such as
When apnea cannot be performed for these right-sided cine displacement encoding with stimulated echoes
measurements, averaging multiple Doppler velocities (DENSE) show promise in their ability to acquire full
appears to be an adequate alternative. 3D data sets for quantification of circumferential and
Careful scrutiny of the transvalvular (when regur longitudinal strain in the RV from a single data set.9
gitation exists) or trans-septal (when shunts exist) Despite these strengths, CMR is still mostly limited
gradients provide the means to determine pressure to patients without pacemakers or defibrillators and
gradients across cardiac chambers. These gradients are not severely claustrophobic, although it is becoming
then added or subtracted to known pressures, such as the increasingly recognized that CMR is safe in patients with
differentiate the myocardium from blood. Depending

on the area of interest, the site of contrast injection
(intravenous line placement), contrast injection rate
and volume, and timing of the scan need to be carefully
considered to optimize opacification of the vascular
structures or cardiac chamber of interest. This is especially
important in the evaluation of the RV since intravenous
contrast is injected via a peripheral vein, and is mixed with
noncontrast blood from other peripheral veins (e.g. IVC
flow) prior to entering the RV. This can lead to incomplete
contrast/blood mixing and limit the optimal evaluation.
Despite the high spatial resolution that can be acquired
with CCT, this technique is limited by low temporal
resolution (> 80 ms), need for ionizing radiation, and
nephrotoxic contrast agents.
Fig. 53.16: See Movie clip 53.10. Cardiac CTA displayed in the Lastly, invasive RV angiography could be considered
four-chamber orientation demonstrating the clearly defined border
between the brighter blood pool and the darker right ventricular
the reference standard, but like all imaging modalities
myocardium. require specialized skills at acquiring adequate orientation
and contrast filling of the RV. Some investigators have had
success at creating quantitative off-line spline models of
these devices.58 Even when CMR is proven to be entirely regional contraction patterns to help separate normal
safe in patients with pacemakers, the RV lead will create
from abnormal motion patterns.61
havoc for optimal imaging of the RV by producing a
ferromagnetic artifact, limiting optimal visualization
of the RV wall near the lead. Gadolinium contrast use is CONCLUSION
limited to patients without severe renal insufficiency Continued investigation is warranted to improve our
[Glomerular filtration rate (GFR) > 30 mL/min/1.73 m2] understanding of the RV and to obtain robust noninvasive
due to the increased risk of nephrogenic systemic fibrosis. diagnostic methods to assess this chamber, which
Optimal imaging is achieved when patients are in normal continues to be proven to predict clinical outcomes.
sinus rhythm and able to breath-hold for 7–15 seconds, Evolving clinical settings demonstrate the importance of
which may not be possible in patients with significant evaluating the RV in an effort to predict RV failure such as
right heart pathology. Real time or single-shot imaging prior to left ventricular assist devices. Given the normally
can be performed in these patients in much shorter times complex shape, the further distortion with pathology, and
or during free breathing but at a cost of reduced spatial the intricate myofibrillar arrangement, it is likely that a
resolution. Similar to echocardiography, it is important combination of parameters (possibly imaging and clinical)
to have experienced specialists acquire high quality, will need to be used for optimal RV assessment rather than
comprehensive images to maximize the clinical value of a single diagnostic parameter. It may also be necessary to
the study. combine diagnostic imaging tools, but for the foreseeable
Cardiovascular computed tomography (CCT) can future, echo will be the initial technique for this purpose.
also be valuable in the assessment of the RV. With high
spatial resolution (approximately 0.5 mm), the right
ventricular anatomy, chamber size, and systolic function
DISCLOSURE
can be accurately determined (Fig. 53.16 and Movie clip Gadolinium contrast is not Food and Drug Administration
53.10).59 Since CCT obtains a volumetric data set with (FDA) approved for CMR.
isotropic voxels, images can be reoriented in any plane
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CHAPTER 54
Three-Dimensional Echocardiographic
Assessment of LV and RV Function
Aasha S Gopal
Snapshot

3D QuanƟtaƟon of the LeŌ Ventricle 
3D QuanƟtaƟon of the Right Ventricle
INTRODUCTION to cardiomyopathies. Despite its central importance,

evaluation of left ventricular (LV) structure and function
Significant progress has taken place in the last 25 years in routine clinical practice is largely subjective and
in moving echocardiography from a two-dimensional substantially relies on an expert knowledge of cardiac
(2D) imaging modality to a three-dimensional (3D) anatomy and physiology. The clinician integrates this
imaging modality that has found several routine clinical knowledge, views moving 2D cross-sectional images,
applications, an important one being quantification of and renders an eyeball estimation of LVEF as a first
cardiac structure and function. This progress has closely approximation. Indeed, in the hands of experts, this
paralleled the transition in transducer technology from method compares quite favorably when compared to many
conventional phased-array transducers to matrix array traditional M-mode and 2D techniques.1 However, there is
transducers. This chapter explores the limitations of substantial interoperator variability and standardization
quantifying left and right ventricular (RV) structure and is difficult. More objective measures of chamber
function by conventional M-mode [one-dimensional quantification rely on detection of endocardial borders at
(1D)] and 2D echocardiography (2DE), thereby providing end-diastole and end-systole. Linear dimensions may be
the rationale for developing and adopting new methods obtained by M-mode echocardiography that has excellent
such as 3D echocardiography (3DE) and speckle tracking temporal resolution, or by 2DE. Linear dimensions are
echocardiography (STE). still reported today because of simplicity of use. However,
these dimensions have high test–retest variability largely
3D QUANTITATION OF THE because of image positioning error, that is, the difficulty
faced by the sonographer to reproduce the same 2D
LEFT VENTRICLE
echocardiographic image with a high degree of fidelity.2
Limitations of 2D Echocardiography— This is because, standard 2D images are prescribed by
the sonographer solely on their basis of their knowledge
Lack of 3D Spatial Coordinates of cardiac anatomy and are not based on any external 3D
Left ventricular ejection fraction (LVEF) is a cardinal spatial coordinate system.
parameter that has been shown to have tremendous An external 3D spatial coordinate system can be
prognostic value in a variety of clinical situations varying specified by a variety of methods and the very early
from valvular heart disease, ischemic heart disease prototypes of 3DE systems utilized either an acoustic
or magnetic system of emitters and receivers that were views also exhibit a high degree of variability. A study
mounted on the transthoracic imaging transducer showed that the standard unguided 2D examination
(Figs 54.1 and 54.2).3–5 Using such a system, it was noted was associated with an interobserver variability of 9.1%
that only 24% of unguided standard images are optimally for ventricular measurements. Guided 3DE significantly
positioned within ±5 mm and ±15° of the ideal position reduced interobserver variability to 3.1% for the same
and 3DE improves positioning of standard images to measurements (p < 0.005 by McNemar’s test).6
80%, a threefold improvement (p < 0.001).2 If the standard The lack of 3D spatial coordinates by conventional
parasternal long-axis view is positioned with a high echocardiography also means that it is not possible to
degree of variability, measurements obtained from these relate one cross-sectional image with another cross-
sectional image without making assumptions related
to image position that is, biplane images are assumed
to be orthogonal to each other but rarely ever satisfy
that assumption because each cross-sectional image
is obtained independently by the sonographer in an
unguided manner.2 An external 3D spatial coordinate
system provides a means of precisely measuring the
relationship of one cross-sectional image with respect
to another, a necessary prerequisite for accurate
quantification of the left ventricle. An external 3D spatial
coordinate system, while providing the ability to scan
freely without constraints has limitations. Limitations of
the freehand acoustical spatial locating system are that it
requires a clear line of sight between the sound emitters
and the overhead receivers. An electromagnetic spatial
locating system may be limited by interference with the
Fig. 54.1: Freehand scanning. An external three-dimensional (3D) electromagnetic field by large ferromagnetic objects such
spatial coordinate system is provided by an acoustic spatial loca- as metal examination beds and other nearby metallic
ter which consists of a system of three sound emitters mounted equipment that may degrade system accuracy.7
on the transducer (shown on the examination bed) which provides
Another means of relating images to one another is
freehand scanning. These emit sound waves that are received by
an overhead microphone array (shown above the examination by providing a spatial coordinate system that is internal
bed). The 3D spatial information is fed into a computer (shown to the heart. This approach is based on the principle that
beside the ultrasound machine) which assigns a set of x, y, and z a 3D data set can be reconstructed from a series of 2D
Cartesian coordinates to each image. images in which the intervals and angles between the
A B C
Figs 54.2A to C: Freehand scanning. A modified ultrasound probe is tracked in three-dimensional (3D) space using an electromagnetic
field device; images then may be reconstructed offline to create 3D data sets. A schematic of the receiver and transmitting device and
the Cartesian coordinate system for tracking the location of the transducer is shown. (Courtesy of TomTec Imaging Systems, Munich,
Germany; with permission).
Chapter 54: Three-Dimensional Echocardiographic Assessment of LV and RV Function 1151
2D images are defined. In this method, serial 2D images the ventricle enlarges along its superior–inferior axis, that
are obtained using a mechanically driven transducer that increase is not reflected in the parasternal long-axis view
sequentially recorded images at predefined intervals from from which LV dimensions are traditionally reported. To
a fixed transducer position. The images may be acquired improve on simple linear dimensions, a plethora of models
in a parallel fashion or by pivoting around a fixed axis in a of ventricular size and shape have been devised. Popular
rotational, fan-like manner. A variety of transthoracic and among these is a prolate-ellipsoid model that utilizes two
transesophageal echocardiographic systems were devised apical views (two-chamber and four-chamber). Here the
in which fast rotating transducers provided a series of ventricle is assumed to conform to the shape of a prolate-
cross-sectional images that were all spaced relative to each ellipse and ventricular volume is calculated based on traced
other in precise fashion (Fig. 54.3).8–14 endocardial borders from these views.15 However, when
the ventricle is affected particularly by regional ischemic
Limitations of 2D Echocardiography— heart disease, the ventricle may enlarge and remodel in
ways that deviate from a prolate-ellipsoid shape, thereby
Geometric Assumptions posing a severe limitation to accurate quantification of
Since conventional echocardiography does not provide its size and function. 3DE overcomes these limitations
cross-sectional images that are spatially related to each by eliminating image positioning error and geometric
other in a precise fashion, it is necessary to make certain assumptions (Figs 54.4 and 54.5B).16 3D reconstruction
assumptions about ventricular geometry to arrive at a techniques have been used extensively in vivo to validate
more objective assessment of LV size and function. Linear determination of LV volumes and EF against several
measurements are popular and still reported today. reference clinical standards such as cardiac magnetic
However, they are difficult to reproduce when acquired resonance imaging (CMR), cineventriculography, as
in an unguided fashion and they assume that ventricular well as multigated radionuclide scintigraphy using the
enlargement occurs uniformly and is reflected faithfully in transthoracic approach17–20 and shown to be superior to
the increase in the linear dimension. For example, when conventional 2DE methods.
Fig. 54.3: Schematic of how three-dimensional (3D) transesophageal echo (TEE) was acquired. The TEE probe is shown with its range
of rotation from 180°. Typically, a two-dimensional (2D) image was acquired every 3°. The relationship between the heart and the TEE
rotation is shown. (Courtesy of TomTec Imaging Systems, Munïch, Germany; with permission.)
A B
Figs 54.5A and B: Field of view for two-dimensional (2D) echo
and real time three-dimensional (3D) echo. (A) Tomographic
Fig. 54.4: Three-dimensional (3D) reconstruction—left ventricular image field of conventional linear phased-array transducer; (B)
volume can be computed using a polyhedral surface reconstruc- Matrix array transducer used to obtain pyramidal volumetric data
tion algorithm from a series of images acquired from the base of set. Relation of image dissections is shown by orthogonal B-scans
the heart to the apex. and cross-sectional C-scan.
Limitations of 2D Echocardiography— to patient motion and respiration. Therefore, to obtain a

good 3D data set free from reconstruction artifacts, data
Apical Foreshortening and Boundary acquisition takes longer and can last up to 5 minutes
Recognition depending on the quality of the 2D images, the patient’s
2DE from the transthoracic approach has additional heart rate, and respiratory pattern. In addition, 2D images
limitations that are patient specific and inherent to the need to be exported to offline workstations and reprocessed
anatomy and position of the heart as it is situated in the manually using proprietary software. The time taken to
rib cage. Imaging is performed through the interspaces process the images depends on the size of the heart and
between the ribs and even though guided 3DE can achieve the number of images required obtaining an accurate
perfect image positioning, image quality may be degraded if estimate of LV volume. The greater the number of images,
there is no optimal transthoracic interspace. Inherent ways the less data interpolation is required between images
of compensating for the lack of an adequate rib interspace is and the greater the accuracy of the method. However, the
to utilize whatever echocardiographic window is available. greater the number of images obtained, the greater the
This can frequently lead to apical foreshortening as was processing time. Typically, four to six images are required
nicely demonstrated in a simultaneous echocardiographic for accurate determination of LV end-diastolic and
and cineventriculographic study.21 Similarly, it is possible end-systolic volumes (ESVs).
to obtain only tangential cross-sections of the heart that
may cause inaccuracies in estimating linear dimensions Real Time 3DE
and wall thickness.
Advances in transducer technology have experienced
a revolution due to the increasing computing power of
Limitations of 3D Reconstruction modern electronics as well as miniaturization. These
Although 3D reconstruction addresses the principal developments allowed the first matrix array transducer to
limitations to accurate quantitation of the left ventricle, be constructed by von Ramm et al.22–24 Here the transducer
namely image position error and geometric assumptions, elements are arranged in a grid that allows steering in
this methodology has several limitations. The images both the azimuth and elevation directions as compared
acquired for 3D reconstruction are nonsimultaneous. to a conventional transducer that allows steering only in
Therefore, it may be inaccurate in patients with significant the azimuth direction (Figs 54.5A and B). This seminal
intracardiac dyssynchrony. It also requires electrocardio- innovation with 16:1 parallel processing allowed imaging
gram (ECG) gating and is susceptible to inaccuracies due of a volume rather than a sector. Furthermore, real time
A B
Figs 54.6A and B: (A) First-generation matrix array transducer: Simultaneous display of parasternal long-axis (orthogonal B scan) and
short-axis views (C-scan); (B) First-generation matrix array transducer: Simultaneous display of apical (orthogonal B scan) and short-
axis views (C-scan). (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
scanning could be performed in a single heartbeat that did to a human hair within a phased-array transducer
not require ECG gating and allowed simultaneous display compared to a matrix array transducer.26 Most matrix
of the images derived from the 3D data set. This first- array transducers have technology that provides wider
generation matrix array transducer operated at a frequency bandwidth with higher sensitivity. This technique now
of 2.5 MHz and consisted of 256 nonsimultaneous firing permits tracking of the endocardial borders in real time
elements that acquired a pyramidal volume data set throughout the cardiac cycle. Whereas 3D reconstruction
measuring 60° × 60°. A limitation of this first-generation methods and first-generation matrix array transducers
matrix array technology was that the image quality was only permit calculation of static volumes at end-diastole
relatively poor and did not match conventional 2D image and end-systole, current matrix array transducers allow us
quality. Additionally, since this was not a fully sampled to tracking ventricular volume over the full cardiac cycle,
matrix but a sparse-array matrix, it did not allow for thereby allowing us to not only calculate overall EF, which
3D rendering, but did allow simultaneous orthogonal is a very important measure of cardiac performance, but
B-scans and cross-sectional C-scans to be displayed also to calculate rates of ejection and rates of filling, which
(Figs 54.6A and B). Furthermore, large hearts could not be may be important as well. With further reduction in the
imaged within the 60° × 60° volume. Nevertheless, it paved size of electronics, 2D arrays can now be integrated into
a volume that is small enough to fit on a transesophageal
the way for further advances to be made in matrix array
transducer probe, thus allowing for real time 3D scanning
transducer technology and spurred many commercial
of the heart, thus avoiding image degradation from ribs,
ultrasound manufacturers to develop it further.
lungs, and fat. As ingenious as real time three-dimensional
Currently, real time 3DE (RT 3DE) is offered on several
transesophageal echocardiography (RT 3DTEE) is, it has
commercial platforms. RT 3DE can be performed by either
not yet been feasible to perform accurate measurement of
switching among 2D and 3D transducers, or alternating volumes using this technique, primarily due to limitations
between 2D and 3DE modalities present within the same of the sector size, frame rate, and finding suitable boundary
all-in-one probe.25 3DE is the only imaging method that tracking algorithms that will perform wall on this complex
is able to view moving structures in the beating heart in data set.
real time. In contrast, cardiac computed tomography (CT) Current RT 3DE systems offer several data acquisition
and magnetic resonance imaging (MRI) provide only 3D modes that have varying sector sizes. Data acquisition
reconstructed images from multiple tomographic planes. is always a trade-off between sector (volume size),
In contrast to the first-generation matrix array transducers, line density (spatial resolution), and volumes/second
current systems are fully sampled because they typically (temporal resolution). At present, two different methods
have over 3,000 elements that make up the grid that allows for 3D data acquisition are available: “real time 3D”
360° focusing and steering and 3D rendering. Figures or “live 3D mode” and multibeat 3D mode. In the real
54.7A and B contrast the size of the elements compared time or live mode, a thin sector (typically 30° × 60°) of a
A B
Figs 54.7A and B: (A) Phased-array elements compared to the size of a human hair; (B) Matrix array elements compared to the size
of a human hair.
Fig. 54.8: Full-volume three-dimensional (3D) data set acquired Fig. 54.9: Full-volume three-dimensional (3D) data set acquired
by a second generation matrix array transducer consisting of four by a second generation matrix array transducer consisting of four
stitched volumes acquired over four to six heart cycles. The left stitched volumes acquired over four to six heart cycles. The left
hand panel depicts static reference images with the intersect- hand panel depicts static reference images with the intersecting
ing lines showing the intersection of sagittal and coronal planes. lines showing the intersection of sagittal, coronal and axial planes.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle). RV: Right ventricle).
pyramidal 3D data set is obtained and visualized live, beat To provide better spatial and temporal resolution,
after beat as during 2D scanning. In this mode, narrow multibeat acquisitions can be performed to yield a full-
volume, zoom, wide-angle, and color Doppler modalities volume data set. Typically a full-volume data set in second-
are available. Since data acquisition in this mode is done generation 3D echocardiographic systems comprises four
in a single heartbeat, no ECG or respiratory gating is of the small live sectors stitched together to provide a sector
required and heart dynamics is shown with instantaneous size that is much larger (e.g. 101° × 104° depending on the
online volume rendered reconstruction. This mode also particular commercial system used and the transducer
overcomes limitations posed by rhythm disturbances type). This technique is not real time and therefore requires
and respiratory motion. However, this mode suffers from ECG gating and is acquired over four to six consecutive
relatively poor spatial and temporal resolution. heartbeats (Figs 54.8 and 54.9). The full-volume mode is
A B
Figs 54.10A and B: (A) Full-volume three-dimensional (3D) data set acquired by a third-generation matrix array transducer. The data
set is displayed as a set of three image planes that are spaced 60° apart and one cross-sectional axial image; (B) Three equiangular
planes (triplane) that are spaced 60° apart can be generated selectively instead of acquiring a full-volume data set and used for calcula-
tion of 3D volumes and ejection fraction. The image quality of triplane imaging is superior to that of full-volume imaging and therefore,
may have some advantages for left ventricular quantitation.
prone to stitching artifacts if the patient moves or breathes and filling rates. However, if only 3D EF is desired, static
deeply during image acquisition. However, it has the volumes in end-diastole and end-systole are sufficient. In
advantage of imaging a large volume while preserving this case, simultaneous viewing of triplane images, which
high spatial and relatively good temporal resolution are derived from the same heartbeat and same 3D data
(volume rates). Stitching artifacts may be minimized by set but have higher resolution than the full-volume data
performing data acquisition during suspended respiration. set, may be more practical (Fig. 54.10B and Movie clip 3).
Arrhythmias such as atrial fibrillation may also produce Triplane LVEF is computed from static volumes obtained
stitching artifacts when patients with this arrhythmia are at end-diastole and end-systole from three equidistant
imaged in the full-volume mode. planes 60° apart. Even though the phantom appears as if it
Third-generation 3D echocardiographic systems have is contracting dynamically, the only true volumes that are
even higher processing power with fully sampled 2D obtained are from end-diastole and end-systole. Computer
matrix arrays such that a full-volume can be obtained in data interpolation is performed for all other points in the
a single heartbeat (Movie clip 1A) with little sacrifice in cardiac cycle (Movie clip 3). Triplane volumes tend to
image quality. A zoom mode is also typically provided. underestimate true volume since they are obtained only
This is an enlargement of a subsegment of the thin slice live from three images. However, the EFs derived from them
sector that is ~30° × 30° that provides even greater spatial are quite reliable. A further advance in LV quantitation
and temporal resolution (Movie clip 1B). An x-plane mode has been to provide automatic endocardial borders
allows simultaneous viewing of a cross-section and the without the need for any manual operator interaction.
plane orthogonal to it. Lateral tilting of this plane is also These sophisticated systems were designed by feeding
possible by manipulating the trackball (Movie clip 2). A data generated from thousands of manually endocardial
mode that is particularly helpful with LV quantitation is borders by experts and teaching the computer to recognize
the triplane mode. Figure 54.10A shows a full-volume the type of echocardiographic view being presented and
data set that may be obtained for LV quantitation. In this generating an automatic border based on recognizable
mode, the left ventricle is tracked by a border-tracking features. Examples of apical two-chamber (Movie clip 4)
algorithm using the full 3D data set for the full cardiac and four-chamber views (Movie clip 5) being automatically
cycle. Using this feature, dynamic LV volumes may be processed and borders generated for EF calculation
obtained that are helpful in evaluating not only EF, but are shown. Current 3D echocardiographic systems
also intracardiac dyssynchrony, as well as ejection rates also allow overlay of color information (Movie clip 6),
conventional tissue Doppler information (Movie clip 7) the long axis of the ventricle is maximized before manual
as well as color-coded strain information (Movie clip 8) boundary tracing or border tracking is applied. However,
that are helpful for evaluating regurgitant jets, intracardiac in patients with abnormal ventricles, the predominant
dyssynchrony, and wall motion, respectively. For these source of error is due to geometric assumptions that may
purposes, the triplane mode is particularly helpful because not be valid.35 This source of error can be minimized by
of its higher temporal resolution than the single-beat full- increasing the number of images utilized for sampling
volume mode. the ventricle and carefully editing computer-generated
endocardial boundary. However, despite controlling for
Validation Studies of Real Time 3DE for image positioning error and geometric assumption error,
underestimation is still present due to differences in
Left Ventricular Volumes and Ejection boundary tracing depending on the modality chosen as
Fraction the reference modality.35 This finding was confirmed by
a multicenter study36 as well as by a recent meta-analysis
Extensive validation studies of RT 3DE against independent
of 23 studies (1,638 echocardiograms) that compared LV
reference methods have been performed. Almost all
volumes and EF measured by RT 3DE and CMR examined
of them confirm the earlier validation studies with 3D
the overall accuracy of RT 3DE. A subset of those also
reconstruction against the same reference standards. Most
compared standard 2D methods with CMR. The pooled
show superiority over conventional 2D methods together
biases ± 2 SDs for 3DE were –19.1 ± 34.2 mL, –10.1 ±
with improved intraobserver, interobserver, and test–
29.7 mL, and –0.6 ± 11.8% for EDV, ESV, and EF, respectively.
retest variability.27–34 Correlation coefficients against CMR
Nine studies also included data from 2DE, where the
for end-diastolic volumes (EDVs) have varied from 0.92
pooled biases were –48.2 ± 55.9 mL, –27.7 ± 45.7 mL, and
to 0.98, and for end-systole from 0.81 to 0.98. Variable syste-
0.1 ± 13.9% for EDV, ESV, and EF, respectively. In this subset,
matic underestimation of 3D EDVs from 4 mL to 14 mL the difference in bias between 3DE and 2D volumes was
has been reported; underestimation of ESVs from 3 m L to statistically significant (p = 0.01 for both EDV and ESV).
18 mL has also been reported. However, since both EDVs The difference in variance was statistically significant
and ESVs are underestimated, EF calculations have been (p < 0.001) for all three measurements.37 Boundary-tracing
neither systematically overestimated nor underestimated. error depends largely on image quality38 and differences
Interobserver variability varies from 5% to 11%. Whereas in image segmentation, particularly with respect to the
3D reconstruction methods had longer acquisition times handling of papillary muscles and trabeculae. In CMR
and image-processing times, RT 3DE has significantly cut methods, these tend to be included in the cavity volume
down on data acquisition time in many cases, to a single to a greater extent, thereby resulting in larger volumes.
heartbeat or four to six heartbeats. In addition, border The larger the ventricle, the greater is the degree of
tracking has been increasingly automated, requiring only underestimation.35 The underestimation of 3DE volumes
specifying anatomic landmarks within the left ventricle is particularly noteworthy in subjects with heart failure.
(medial and lateral mitral annular points and the apex). Compared to CMR, RT 3DE is accurate for evaluation of
While border tracking has made the reporting of ventricular EF and feasible in heart failure patients, at the expense of
volumes more practical, the best results are obtained only a significant underestimation of LV volumes, particularly
when image quality is good and when computer generated when LVEDV is above 120 mL/m2.39
boundaries are manually corrected. Fully automated endocardial trabecular contouring
The underestimation of ventricular volumes and algorithms have also been used and validated to compute
sources of error have been studied extensively by several volumes and EF and compared to CMR in patients in sinus
investigators. Using the several modes of 3D data acqui- rhythm (67 subjects) as well as in atrial fibrillation (24
sition as well as processing of 3D data, it is possible to analyze subjects). To correct for the underestimation of volumes,
in detail the sources of error contributing to traditional an automated correction can be applied to track the
underestimation of volumes by echocardiography. Image compacted myocardium. Among all sinus rhythm patients,
positioning error includes nonorthogonal image positio- there was excellent correlation between RT 3DE and CMR
ning as well as apical foreshortening. This type of error for EDV, ESV and EF (r = 0.90, 0.96, and 0.98, respectively;
predominates in normally shaped ventricles. It can be p < 0.001). In patients with EF ≥ 50% (n =36), EDV and ESV
minimized by aligning apical images in such a way that were underestimated by 10.7 ± 17.5 mL (p = 0.001) and by
4.1 ± 6.1 mL (p < 0.001), respectively. In those with EF < 50% in comparison with the corresponding values obtained by
(n = 31), EDV and ESV were underestimated by 25.7 ± 32.7 conventional echocardiography.42
mL (p < 0.001) and by 16.2 ± 24.0 mL (p = 0.001). Automated In addition to gender- and age-specific reference
contour correction to track the compacted myocardium values, population-specific reference values may also be
eliminated mean volume differences between RT 3DE and important. A study of 978 subjects from the London Life
CMR. In patients with atrial fibrillation, LV volumes and EF Sciences Prospective Population (LOLIPOP) study, who
were accurate by RT 3DE (r = 0.94, 0.94, and 0.91 for EDV, were free of clinical cardiovascular disease, hypertension,
ESV, and EF, respectively; p < 0.001). Automated 3D LV and type 2 diabetes, showed that indexed 3DE LV volumes
volumes and EF were highly reproducible, as expected.40 were significantly smaller in female as compared with male
Newer ways of addressing the systematic undere- subjects and in Indian Asians compared with European
stimation of RT 3DE volumes compared to CMR have whites. Upper limit of normal (mean ± 2 SD) reference
utilized contrast agents. The use of contrast agents may values for the LVESV index and LVEDV index for the four
improve endocardial border recognition in RT 3DE. ethnicity–sex subgroups were, respectively, as follows:
However, automatic and semi-automated border-tracking European white men, 29 and 67 mL/m2; Indian Asian
algorithms for calculation of RT 3DE ventricular volumes men, 26 mL/m2 and 59 mL/m2; European white women,
have not been rigorously developed for use with contrast 24 mL/m2 and 58 mL/m2; Indian Asian women, 23 mL/m2
agents. In addition, contrast agents can sometimes cause and 55 mL/m2, respectively. Compared with 3DE studies,
attenuation and difficulty in identifying the valve planes, 2DE underestimated the LVESV index and LVEDV index by
which may result in variable inclusion or exclusion of an average of 2.0 and 4.7 mL/m2, respectively. LVEF was
the left atrium. An approach that shows some promise, similar between in all four groups and between 2D and
particularly in patients with poor acoustic windows, is 3D techniques, with a lower cutoff of 52% for the whole
the use of contrast agents in conjunction with power cohort.43
modulation (PM) imaging that uses low mechanical
indices and provides uniform LV opacification.41 New 3DE Parameters and RT 3DE
Left Ventricular Strain
Normal RT 3DE Values for Volumes and
The ability to generate time–volume curves from dynamic
Ejection Fraction RT 3DE volumes throughout the cardiac cycle have
Practical and routine clinical use of 3DE volumes and generated new 3DE parameters of LV diastolic and systolic
EF to detect LV remodeling and dysfunction require age- performance that have shown interesting results in small
specific and gender-specific reference ranges. In 226 pilot studies. These parameters may be particularly
consecutive healthy Caucasian subjects (125 women; age helpful in the detection of ischemia since diastolic LV
range, 18–76 years), comprehensive 3DE analyses of LV abnormalities are sensitive early signs of myocardial
parameters were performed, and values were compared ischemia and persist longer than systolic changes.44,45
with those obtained by conventional echocardiography. Abnormalities of peak filling rate (PFR), expressed in units
Upper reference values (mean + 2 SDs) for 3DE LV EDVs of EDV/s, have been reported in ischemic patients using
and ESVs were 85 and 34 mL/m2 in men and 72 and Doppler echocardiography.46 A pilot study by Gopal et al.
28 mL/m2 in women, respectively. Indexing LV volumes used RT 3DE to evaluate PFR in 19 subjects with an
to body surface area did not eliminate gender differences. intermediate-high risk of computer-aided design (CAD)
Lower reference values (mean –2 SDs) for EF were 54% together with 1-day rest/stress adenosine 99mTc-sestamibi
in men and 57% in women and for stroke volume were gated single-photon computed emission tomography
25 and 24 mL/m2, respectively. Upper reference values (GSPECT). Adenosine was infused at 140 mcg/kg/min
for LV mass were 97 g/m2 in men and 90 g/m2 in women over 6 minutes. Nuclear images were acquired at 16
and for end-diastolic sphericity index were 0.49 and 0.48, frames/R-R interval for 64 projections over a 180 arc at
respectively. Significant age dependency of LV parameters rest and poststress. 2DE and RT 3DE were performed
was identified and reported across age groups. 3D at rest and at peak stress (at 2–6 minutes of adenosine
echocardiographic LV volumes were larger, EF was similar infusion). GSPECT and RT 3DE data were analyzed by QGS
and LV stroke volume and mass were significantly smaller (Cedars-Sinai) and QLab (Philips) algorithms, respectively.
Fig. 54.11: Peak filling rate (PFR) derived from RT 3DE time volume Fig. 54.12: Peak filling rate (PFR) derived from RT 3DE time volume
curves fitted to third order harmonics normal response-rest and curves fitted to third order harmonics-ischemic response-rest and
stress. (RT 3DE: Real time, three-dimensional echocardiography). stress. (RT 3DE: Real time, three-dimensional echocardiography).
Nuclear and RT 3DE volume versus time curves were Figure 54.13 shows a representative patient with lateral
Fourier-fit to third-order harmonics to compute PFR perfusion defect whose 2DE WMSΔ was normal but whose
by custom software (RSI, Inc., Boulder, CO) for rest and PFRΔ was abnormal. Of the six patients with ischemia
stress. PFR difference (PFRΔ) was defined as PFR stress– (SDS > 2), four patients had abnormal PFRΔ compared
PFR rest. Nuclear perfusion defects were quantified by to those without ischemia (–0.12 ± 0.77 EDV/s vs +0.66
17-segment/5-point stress and rest nuclear perfusion ± 0.44 EDV/s, p = 0.02); two patients had both a normal
scores and a summed difference score (SDS) > 2 accepted WMSΔ and a normal PFRΔ. Two of the four patients with
as abnormal and indicative of stress-induced ischemia.47 abnormal PFRΔ also had an abnormal WMSΔ (Table 54.1).
A qualitative assessment of rest and stress wall motion PFRΔ correlated inversely with ischemia [Spearman’s
scores (WMS) were determined for 17 segments and 2DE coefficient = –0.55, p = 0.03 with 95% confidence
WMS difference (WMSΔ) were computed as follows based interval (CI) = −0.81 to −0.08, Fisher exact test, p = 0.006;
on systolic wall thickening48: normal wall thickening was Fig. 54.14]. An abnormal PFRΔ predicted myocardial
given a score of 1; hypokinetic wall = 2; akinetic was scored ischemia with 89% accuracy, 100% specificity, 66% sensi-
= 3; and dyskinetic = 4. The mean age of the study group was tivity, 100% positive predictive value, and 86% negative
68.4 ± 15 years, with 14 males. There were no significant predictive (ROC PFRΔ threshold > 0.0).
differences in the change in heart rate between rest and In addition to being able to assess ejection rates and
adenosine stress studies for patients with ischemia versus filling rates, LV performance may be assessed by 3D strain
patients without ischemia (heart rate change of 17 ± 12 analysis. Strain analysis can be performed by a technique
beats/min vs 13 ± 8 beats/min, p = 0.15). Of all the echo known as STE, which identifies a pattern of speckles in
parameters examined, only PFRΔ exhibited significant one frame and then tries to find the same pattern in the
associations with ischemia. The response to adenosine following frames using pattern-matching algorithms. This
stress in patients without ischemia was an increase or allows all anatomic features including the myocardium
no change in PFR (Fig. 54.11). An abnormal response present in the echocardiographic image to be tracked
to adenosine stress was a decrease in PFR (Fig. 54.12). through space and time. This technique offers advantages
Table 54.1: Comparison of mean PFR to SDS >2.

Stress-rest differences
Myocardial ischemia
PFR  > 0 PFR  < 0
SDS < 2 13 0
SDS > 2 2 4
Fig. 54.13: Lateral wall ischemia-adenosine 99mTc-Sestamibi

GSPECT. GSPECT, gated single-photon computed emission
tomography.
Fig. 54.14: Comparison of mean PFR to SDS >2. Mean values
and error bars representing one standard deviation is plotted.
in and out of the 2D imaging plane. To overcome this

limitation, 3D STE has been developed. Thus, displacement
measured by 3D STE is larger than the corresponding 2D
Fig. 54.15: One-dimensional Lagrangian strain. The length is the STE values, indicating that through-plane motion (motion
only strain component, and thus L is measured along the only perpendicular to the imaging plane) can be detected by 3D
coordinate axis, thus L = x.
Source: Reproduced with permission from: http://folk.ntnu.no/
STE but not 2D STE.49
stoylen/strainrate). Myocardial contractile motion is complex and 3D.
In addition, the complex arrangement of muscle fibers
over tissue Doppler imaging (TDI), which also measures contributes to varying extents to myocardial deformation.
myocardial motion, but which is angle dependent. Strain analysis is usually performed by using an external
Therefore, motion that occurs along the ultrasound beam Cartesian spatial coordinate system. 1D strain occurs only
can be detected by TDI. However, motion that occurs along the coordinate axis (Fig. 54.15). In 2D, the strain
perpendicular to the ultrasound beam is not detected. tensor has four components, two along the coordinate
This limitation is overcome by STE. Myocardial motion axis and two shear strains (Fig. 54.16). In 3D, the complex
can be expressed as myocardial displacement, velocity, 3D myocardial deformation can be decomposed into
or strain. However, the former two are affected by cardiac three normal and six shear strains (Fig. 54.17). Normal
translation that may occur with each heartbeat or with strains (longitudinal, circumferential, and radial) reflect
respiration. Strain, however, measures the deformation of changes in length along a spatial coordinate system.50
the myocardial muscle as the difference between its length Shear strains (longitudinal–radial, circumferential–radial
at a certain point in the cardiac cycle and its initial length and circumferential–longitudinal) are forces acting in
divided by its initial length. Thus, contraction is expressed opposite directions.50 Myocardial strain can be described
as a negative strain value whereas elongation is expressed without an external spatial coordinate system using three
as a positive strain value. Limitations of 2D STE include principal strains and three principal angles or axes of
difficulties tracking the speckles when the speckles move deformation that form an internal frame of reference.50 The
Fig. 54.16: Strain in two dimensions. Above are the two normal
strains along the x and y axes, where each strain component can
be seen as Lagrangian strain along one main axis. Below are the
two shear strain components, movement of the borders relative to
each other. Here there are two strain components, characterized
by the tangent to the shear angle alpha.
Source: Reproduced with permission from http://folk.ntnu.no/
stoylen/strainrate.
Fig. 54.17: Strain in three dimensions. Only the three strain components along the x axis (one normal, two shear) are shown, but the
y and z strains will be exactly the same and can be imagined by rotating the x images.
Source: Reproduced with permission from: http://folk.ntnu.no/stoylen/strainrate.
three principal strains are oriented along three mutually translation of the heart are no longer important with this
orthogonal directions and ranked from maximum internal frame of reference approach53 (Fig. 54.18).
contraction at the end of systole to maximum lengthening As described above, 3D STE offers an integrated
at the end of diastole.50,51 The principal strain is the approach toward analyzing the complex motion of
maximum contraction that occurs in an oblique direction the heart. In addition to providing more accurate and
within the circumferential–longitudinal plane and reproducible volumes compared to 2D STE,54 it provides
angled to spiral counterclockwise from the apex to base a new methodology for analyzing regional function.
(as viewed from the apex).51 It aligns in the general 3D STE shows that all strain components are reduced
direction of the subepicardial muscle fibers.52 The benefit in abnormal regional segments.49 3D STE also provides
of this approach of describing myocardial strain along the new 3D parameters to assess LV systolic function such
axes of deformation (internal frame of reference) rather as global area strain (percentage of deformation in the
than in terms of an external Cartesian spatial coordinate LV endocardial surface area defined by the longitudinal
system is that it can provide a more integrated perspective and circumferential strains vectors). Recent studies have
of all the major forces experienced at the tissue level. shown that this parameter is more accurate and
In addition, an internal frame of reference allows us to reproducible on a regional level than the other
eliminate shear strain since principal strain represents the components.55 Global area strain is strongly correlated
combined effect of shortening and shear.53 Rotation and with LVEF and to a lesser degree with cardiac output.56
Fig. 54.18: In the heart, the usual directions are longitudinal, Fig. 54.19: Three-dimensional (3D) line of intersection display
transmural (radial), and circumferential as shown to the left. In positioned short-axis images for left ventricular (LV) mass com-
systole, there is longitudinal shortening, transmural (radial) thick- putation. Traced epicardial and endocardial borders are shown.
ening and circumferential shortening. (This is an orthogonal coor-
dinate system, but the directions of the axes are tangential to the
myocardium, and thus changes from point to point).
Source: Reproduced with permission from: http://folk.ntnu.no/ et al.62 showed that the 95% CI width of a single replicate
stoylen/strainrate. measurement of LV mass was 59 g. This measurement
variability exceeds the usual decrease in mass during
A progressive decrease in global area strain is noted as treatment.62 Alternative imaging tools such as MRI
heart failure progresses from normal to stage D.57 A study and ultrafast CT not only use nonportable equipment
that compared a 3D strain vector (summing the radial, but also are expensive, cumbersome and not widely
circumferential and longitudinal vectors) and 3D area available for serially following LV mass in patients. LV
strain in patients with coronary artery disease showed that mass has been calculated by 3DE reconstruction by
while area strain correlated with the severity of transmural subtracting the endocardial volume from that of the
extent of necrosis, 3D strain decreased only when necrosis epicardium and multiplying the result by 1.05, the density
extent was > 75%.58 Though many of these alternative of myocardium (Fig. 54.19). It was calculated in vitro
approaches of quantifying LV performance appear in fixed animal hearts very accurately with a standard
promising, it is unclear how these indexes will correlate error of the estimate (SEE) of 2–3 g.63 Anatomic in vivo
diagnostically or prognostically given such constraints as animal validation provided the best results for 3D echo
vendor variability and proprietary algorithm differences, a reconstruction (r = 0.96, SEE = 5.9 g, accuracy 6.8%)
sentiment reflected by a recent consensus statement by the compared with the truncated ellipsoid (r = 0.88, SEE = 10.2 g,
American and European Societies of Echocardiography.59 accuracy 12.6%) and bullet (r = 0.83, SEE = 12 g, accuracy
= 12.7%) algorithms.63 In vivo validation of LV mass by 3DE
Left Ventricular Mass by 3DE reconstruction was carried out in normal subjects using
MRI as a standard of comparison and was shown to correlate
Several studies, including the Framingham Heart Study, highly (r = 0.93, SEE = 9.2 g) with good interobserver
have demonstrated that elevated LV mass is an indep- variability (6.3%) and statistically no different from
endent and strong predictor of morbid cardiac events and corresponding MRI values.64 In the same population, 3DE
death.60 Conventional 1D M-mode echo and 2DE methods reconstruction achieved a two- to threefold improvement
of measuring mass rely on geometric assumptions, lack in the correlation with MRI over conventional M-mode
spatial registration and are associated with comparatively and 2DE algorithms used to compute LV mass.64 In
high measurement variability, particularly in abnormally patients with abnormal ventricular geometry, the SEE
shaped hearts.61 As a consequence, the test–retest stability and limits of agreement between 3DE reconstruction
of 1D and 2DE for the serial measurement of LV mass in and MRI were roughly twice the values found in normal
patients with hypertension may be impaired. Gottdiener subjects.18 LV mass determination by 3DE reconstruction
has also been anatomically validated in vivo in humans 671 comparisons were analyzed showed that 3DE still
undergoing heart transplantation using the true weight of underestimated LV mass compared to CMR. However, the
the left ventricle of the explanted hearts (range 125–433 g) underestimation improved with time from −5.7 g, 95% CI
as the standard of reference and compared to M-mode −11.3 to −0.2, p = 0.04 in studies before 2004 to −0.1 g, 95%
Penn, 2DE area length and truncated ellipsoid algorithms. CI −2.2 to 1.9, p = 0.90 in studies published after 2008.68
The results showed that 3DE reconstruction is a highly
accurate and reproducible method (r = 0.993, accuracy Left Ventricular Remodeling, Sphericity,
= 4.6%, bias = −3.4 g, and interobserver variability 9.4%).
and Regional Function by 3DE
In addition, 3DE reconstruction accuracy was fourfold
superior to 2DE (r = 0.898, accuracy 19.2%, bias +21.7 g, 3DE reconstruction has been used to analyze LV endo-
interobserver variability 16.7%) and ninefold superior to cardial surface area, infarct subtended surface area,
M-mode echocardiography (r = 0.817, accuracy 43.4%, bias infarct subtended volume and volume/mass ratio, which
may be measures that supplement measures of LV mass
+85.1 g, interobserver variability 18.2%).65 Studies of LV
in studying LV remodeling.69–72 In addition, LV shape can
mass with 3DE reconstruction showed that conventional
be characterized in terms of a sphericity index. This is
M-mode calculations of LV mass significantly overestimate
done by calculating a 3D surface area/volume ratio and
true mass.
indexing it to a surface area/volume ratio of a sphere.
RT 3DE has also been used to calculate the feasibility
As the LV becomes more globular and spherical (i.e.
of calculating LV mass. While 3DE reconstruction utilized undergoes adverse LV remodeling), its sphericity index
parasternal short-axis images for tracing epicardial and approaches.1,73
endocardial borders, the RT 3DE utilizes apical views. The LV volume can be further broken down into 16 or 17
use of apical views for tracing epicardial and endocardial regional segments and volume in each of these segments
borders presents two problems: apical foreshortening and can be tracked over the full cardiac cycle to generate time–
off-axis longitudinal views. The first problem results in volume curves (Fig. 54.20). In a normal subject without
underestimation of LV mass that may be reduced by using significant intracardiac dyssynchrony, the minima of
a 3D-guided biplane technique.66 The second problem
occurs when the imaging plane does not pass through
the center of the left ventricle along its longitudinal axis.
This can result in tangential views of the myocardium that
show an artificially thick ventricle and may overestimate
mass. RT 3DE imaging with single-beat capture has been
used to validate LV mass measurements in 69 patients with
hypertrophic cardiomyopathy against a CMR reference
standard. RTDE and CMR values were also compared to
M-mode mass and the 2D-based truncated ellipsoid mass.
The mean time for RT 3DE analysis was 5.85 ± 1.81 minutes.
Intraclass correlation analysis showed a close relationship
between RT 3DE and CMR LV mass (r = 0.86, p < 0.0001).
However, LV mass by the M-mode or 2DE method showed
a smaller intraclass correlation coefficient compared with
CMR-determined mass (r = 0.48, p = 0.01, and r = 0.71,
p < 0.001, respectively). Bland–Altman analysis showed
reasonable limits of agreement between LV mass by RT
3DE and by CMR, with a smaller positive bias [19.5 g
(9.1%)] compared to that by the M-mode and 2D methods
Fig. 54.20: The left ventricle (LV) has been subdivided into 17
[−35.1 g (−20.2%) and 30.6 g (17.6%), respectively]. These
regional segments. The time–volume curve for each segment is
results confirm the finding by 3DE reconstruction that displayed. In this normal subject without significant intracardiac
conventional LV mass algorithms tend to overestimate LV dyssynchrony, the minima of the time–volume curves (shown on
mass.67 Although 3DE has shown to be more accurate than the top panel) and their first derivatives (bottom panel) are all
reached at roughly the same time.
M-mode and 2DE, a meta-analysis of 25 studies including
Fig. 54.21: The left ventricle has been subdivided into 17 regional Fig. 54.22: Time–volume curves are generated from the entire
segments. The time-volume curve for each segment is displayed. surface of the left ventricle (LV) and are color-coded in such a
In this patient with significant intracardiac dyssynchrony, the mini- manner that late contracting segments are color-coded in shades
ma of the time–volume curves (shown on the top panel) and their of red and normally contracting segments are color-coded blue.
first derivatives (bottom panel) have a wide temporal dispersion. This is a normal subject without dyssynchrony (no red areas are
noted). (LA: Left atrium).
are generated from the entire LV surface. These time–

volume curves can be color-coded in such a manner
as to shown late contracting segments as shades of red
and normally contracting segments in blue. A normal
subject with a perfectly synchronous heart is shown in
Figure 54.22. A patient with significant dyssynchrony is
shown in Figure 54.23 with large areas of late contracting
segments (shown in red). After receiving a biventricular
pacemaker, the areas of late contracting segments (shown
in red) have reduced in size (Fig. 54.24). Though these
tools may be very helpful in evaluating a patient for a
biventricular pacemaker, the reliability of the time–volume
curves depends critically on the 3DE image quality. In
addition, when regional volumes measured in a group
of patients were compared against CMR as a reference,
Fig. 54.23: Time–volume curves are generated from the entire
the levels of agreement were very high in basal and mid-
surface of the left ventricle (LV) and are color-coded in such a
manner that late contracting segments are color-coded in shades ventricular segments, but were considerably lower near
of red and normally contracting segments are color-coded blue. the apex. This difference could probably be explained by
This is a patient with significant intracardiac dyssynchrony with the limited endocardial definition near the apex on both
large areas of late contracting segments (shown in red). 3DE and short-axis CMR images that are affected by partial
volume artifacts at this level.74
RT 3DE also provides new ways of assessing regional
the time–volume curves and their first derivatives are all LV wall motion that may have implications for interpreting
reached at roughly the same time. However, in a patient stress echocardiograms. Instead of viewing the left
with significant intracardiac dyssynchrony there is usually ventricle in conventional short axis, and apical images that
widening of the QRS interval together with a temporal only display a limited portion of the myocardium, RT 3DE
dispersion of the time–volume curves (Fig. 54.21). Instead can display the entire myocardial volume in a multislice
of breaking the LV down only into 17 regional segments, panel, allowing a comprehensive assessment of regional
a bull’s-eye plot can be made of time–volume curves that wall motion (Movie clip 9).
Fig. 54.24: Time–volume curves are generated from the entire Fig. 54.25: Three-dimensional (3D) echo detected a statistically
surface of the left ventricle (LV) and are color-coded in such a significant decrease in LV mass which paralleled a decline in BP.
manner that late contracting segments are color-coded in shades (BP: Blood pressure; LV: Left ventricle).
of red and normally contracting segments are color-coded blue.
This is the same patient after receiving a biventricular pacemaker.
Note that the areas of late contracting segments (shown in red) 25.8 g or greater is considered significant, that is, not due
have reduced in size. to measurement variability alone. This is a greater than
twofold improvement over the value of 59 g reported by
Gottdiener et al. for the conventional M-mode method.
The importance of greater accuracy and reproducibility
of 3D echo in detecting biologically significant LV mass
regression is illustrated in a preliminary study of patients
with hypertension and LV hypertrophy that were treated
and imaged at baseline, after 6 weeks, and after 12 weeks
of antihypertensive therapy. Under the conditions of
this study, 3D echo detected a statistically significant
decrease in LV mass at both 6 and 12 weeks owing to its
lower measurement variability (narrower CIs) whereas
M-mode echocardiography showed a statistically signi-
ficant decrease in LV mass only after 12 weeks of treatment
(Figs 54.25 and 54.26). Using the standard deviation (SD)
of the decrease in LV mass at the end of 12 weeks by each
method, it was calculated that 3D echo was capable of
Fig. 54.26: Three-dimensional echocardiography (3DE) detected
detecting a 10 g reduction in LV mass at a power of 80% with
a statistically significant decrease in LV mass at both 6 weeks and
12 weeks whereas M-mode echocardiography showed a statisti- one-third the number of patients (n = 42) compared with
cally significant decrease in LV mass only after 12 weeks of treat- M-mode echocardiography (n = 148 patients; Fig. 54.27).
ment. (LV: Left ventricle). In addition, owing to the low intraobserver variability of
3DE, 85% of the measured change in LV mass could be
attributed to true biologic change. In contrast, since the
Serial Evaluation of Patients with 3DE intraobserver variability of M-mode echocardiography
The utility of a test to assess a parameter in a serial fashion exceeded the measured change in mass, the contribution
is measured by its test–retest variability. It is well known due to true biologic change could not be determined.75
that the test–retest variability of 3DE is lower than that of Sequential quantification of LVEF and volumes in
2DE. For example, test–retest variability studies of 3DE patients undergoing cancer chemotherapy are important
LV mass have shown that 95% of the time, a change of to clinical decision making. Marwick and colleagues
Table 54.2: Misclassification rate of EF postmyocardial infarc-

tion (comparison of clinical EF, 2DE EF, and 3DE EF to CMREF)
25% CMR EF = 40% CMR EF = 30%
Clinical EF 42.4% 15.2%
2DE EF 21.8% 10.9%
3DE EF 14.5% 5.4%
(EF: Ejection fraction)
studied the method for EF measurement with the lowest

temporal variability. Fifty-six patients were selected for
stable function in the face of chemotherapy for breast
cancer by defining stability of global longitudinal strain
(GLS) at up to five time points (baseline, 3, 6, 9, and 12
months). In this way, changes in EF were considered
to reflect temporal variability of measurements rather Fig. 54.27: Using the standard deviation of the decrease in LV
than cardiotoxicity. 2DE-biplane, 2D-triplane, and 3DE mass at the end of 12 weeks by each method, it was calculated
that three-dimensional (3D) echo was capable of detecting a 10 g
acquisitions with and without contrast administration reduction in LV mass at a power of 80% with one-third the num-
was performed at each time point. Stable LV function was ber of patients (n = 42) compared with M-mode echocardiography
defined as normal GLS (≤ −16.0%) at each examination. (n = 148 patients).
The best temporal variability of EF 0.06 was shown by
noncontrast 3DE while other 2DE methods showed a
temporal variability of > 0.10 with 2D methods over 1 year superiority of 3DE for risk stratification by EF post-MI. The
of follow-up.76 reduced test–retest variability of 3DE compared to 2DE
establishes its utility for serial monitoring.77
RT 3DE for Postmyocardial Infarct
Risk Stratification 3D QUANTITATION OF THE RIGHT
VENTRICLE
Perhaps the greatest utility of 3DE LV quantification
occurs in risk stratifying patients with heart failure or Anatomic Considerations and Prior
moderate LV dysfunction postmyocardial infarction (post-
MI). Decisions are made regarding offering lifesaving
Conventional Approaches
therapies such as implantable defibrillator placement Accurate estimation of RV size and function is essential for
and/or biventricular pacemaker based on the assessment the management of many cardiac disorders. Estimation
of the EF post-MI. Risk stratification by routine methods of RV size and function is of central importance for the
(2DE, planar multigated radionuclide angiography, and management of various congenital diseases.78 Echo-
cineventriculography) were compared to 3DE and CMR cardiographic variables that reflect the severity of right
in 55 patients with MI or congestive heart failure and EF heart failure in primary pulmonary hypertension (PH)
≤ 40%. Patients were stratified by CMREF into two groups: may help identify patients appropriate for more intensive
EF ≤ 30% and ≤ 40%. For CMREF ≤ 30%, the misclassifi- therapy or earlier transplantation.79 Assessment of RV
cation rates were: 42%, 22%, and 14.5% by routine methods, function is also important in determining treatment
2D, and 3DE; for CMREF ≤ 40%, misclassification occurred options for patients with pulmonary embolism, MI, and
in 15%, 11%, and 5% by routine methods, 2DE and 3DE. heart failure.80,81 Therefore, an accurate, easily repeated,
Regardless of the cutoff level chosen, 3DE had the lowest noninvasive method would be ideal for the serial
misclassification rate. 3DE also had a stronger correlation evaluation of patients.
and less bias than 2DE (Table 54.2). 3DE but not 2DE was However, evaluation of RV function has been
equivalent to CMR by analysis of variance (ANOVA). Test– hampered by its complex crescentic shape, large infundi-
retest variability of 3DE was threefold lower than 2DE. bulum, and trabecular nature. Its function by invasive
This study shows inadequacy of routine methods and the angiography can be characterized using area and length
Fig. 54.28: Two-dimensional echocardiography (2DE) long-axis of Fig. 54.29: Two-dimensional echocardiography (2DE) short-axis
RV (length) in diastole for purposes of RV volume calculation by of RV infundibular area in diastole for purposes of RV volume
the area-length method. (RV: Right ventricle). calculation by the area-length method. (RV: Right ventricle).
measurements or Simpson’s rule from single or biplane to have an orthogonal relationship to each other. However,
projections.82 Single plane methods provide limited the transducer is moved from one position to another
sampling of the RV, depend on the orientation of the based on the sonographer’s knowledge of cardiac anatomy
imaging planes with respect to intrinsic RV axes, and and orthogonality is assumed but not verified and
make shape assumptions. Biplane methods provide better rarely satisfied. Furthermore, a prolate-ellipsoid shape
sampling, but are invasive and often overestimate volume. assumption is made, which also may not accurately depict
While radionuclide ventriculography is not constrained RV anatomy. While area-length methods work in vitro
by geometric assumptions, results have been variable and and in animal models, they have wide confidence limits
scanning requires the injection of radioactive agents.83 in human subjects when compared to methods, which
The retrosternal location of the RV as well as the are not subject to geometric assumptions.94 Moreover, the
presence of ribs makes it difficult to access this chamber geometric models used to describe the shape of the RV can
fully by transthoracic echocardiography. Therefore, indi- be changed unpredictably by disease.
vidual aspects of its function can be assessed separately. Wide confidence limits also occur due to reliance on
Transverse shortening can be assessed by fractional anatomic visual information alone for determining image
area change (FAC) in each short-axis slice. Longitudinal plane orientation. Previous experience with freehand
contraction can be assessed by tricuspid annular plane 3DE reconstruction has shown wide operator variability
systolic excursion (TAPSE). TAPSE is measured as the in the optimal positioning of short-axis and apical image
distance in the four-chamber plane between the lateral planes.2 In addition, apical views are often foreshortened
aspect of the tricuspid annulus at end-diastole and end- during 2DE scanning, resulting in underestimation of the
systole. A TAPSE value of > 20 mm has been reported to be RV length that is used in area–length formulas.21 Sheehan
normal. Global function is assessed by calculation of right et al. found that standard 2DE monoplane and biplane
ventricular ejection fraction (RVEF) and several efforts RV algorithms performed better when the images were
have been made to find echocardiographic methods based positioned correctly using 3D electromagnetic guidance.95
on simple geometric models using single plane, biplane, Techniques such as the CT96 and CMR overcome limitations
or on multiplane methods based on Simpson’s rule.84–93 posed by other methods in that image planes are precisely
The most common method utilizes the area and length defined and geometric assumptions are unnecessary.97
from an apical four-chamber view and an RV outflow tract However, these imaging modalities are expensive and are
view93 (Figs 54.28 and 54.29). The two views are assumed not widespread.
Previous 3D Reconstruction Approaches

A variety of options for rapid 3DE image acquisition and
reconstruction of the right ventricle have been used.98–111
Early approaches to 3DE reconstruction occurred from
fixed transducer positions (apical or subcostal) and used
rotational or “fan-like” scanning. This approach works
if the patients are prescreened for good image quality, a
prerequisite for this approach. However, a failure rate of 18%
has been reported in postoperative subjects due to poor
transthoracic windows.112 Therefore, 3DE reconstruction
from a fixed transducer position provides mechanical
3D spatial registration of cross-sectional images, but is
feasible only in those subjects who are echogenic enough
to permit complete visualization of the right ventricle
from a single echocardiographic position. Acoustic
and electromagnetic tracking devices were developed
to provide 3D spatial registration while scanning in a
freehand fashion, permitting the sonographer to utilize
all available echocardiographic windows.95,101,113 Apfel et
al. studied 26 patients with PH with an acoustic spatial Fig. 54.30: Real time, three-dimensional echocardiography
(RT 3DE) data acquisition from an off-axis apical window. The top
locating system and found a good correlation to spin-echo
left, top right and bottom left panels show three orthogonal multi-
CMR but with 31–33% volume underestimation by 3DE.101 planar reconstructions (MPRs) of the RV. The bottom right panel
Since data acquisition occurs over several cardiac cycles shows a partial coronal view of the three-dimensional (3D) data
in the span of 8–10 minutes, respiratory, whole body, or set showing the intersecting sagittal and axial planes from which
the MPRs were derived. (RV: Right ventricle).
transducer motion will lead to data misregistration.
sector angle (60°× 60°) pyramidal data set. While the 3D

RT 3DE Approach to RV Quantification data set can be captured in one heartbeat, frame rates are
RT 3DE uses matrix array transducer technology, pioneered low and image quality is relatively poor. Due to the narrow
by von Ramm et al. and permits continuous acquisition sector angle, visualization of the right ventricle is difficult
of volumetric data, thereby allowing rapid scanning and since a large portion of it lies in the near field where the
minimizing the chance of motion artifacts. Cardiac motion sector is narrowest.
can be evaluated in a dynamic mode and the heart can Second-generation RT 3DE systems use fully sampled
be viewed from any desired plane. Ota et al. validated RV matrix array transducers utilizing 3,000 elements. This
volume measurements using a first-generation RT 3DE results in improved image quality, greater contrast reso-
system in excised canine hearts and in 14 normal subjects. lution, higher sensitivity, and penetration as well as capa-
Though their method performed accurately in vitro, their bilities for harmonic imaging. The full volume of the heart
in vivo standard of comparison was not a 3D method can be obtained by assembling four wedges of 15°× 60°
but a 2D monoplanar modified Simpson’s method. A each over eight consecutive cardiac cycles to obtain a
good correlation and interobserver variability (8.3−9.4%) pyramidal sector 90°× 90°. Some approaches have utilized
was noted between 3D right ventricular stroke volume an off-axis apical four-chamber view that highlights the
(RVSV) and monoplanar 2DSV.114 Shiota et al. validated right ventricle as the initial view taken for the acquisition
the same technology in sheep using electromagnetic flow of the RT 3DE data set (Fig. 54.30). Disc summation and
probes. The correlation obtained for RVSV was r = 0.8 apical rotation algorithms have been developed to quantify
and the Bland–Altman analysis showed a mean RVSV RV size and function in connection with RT 3DE. The RT
difference of –2.7 mL.106 First-generation RT 3DE systems 3DE-disc summation algorithm appears to be superior
use a sparse array matrix transducer, which utilizes 256 to an apical rotational algorithm because it is able to
nonsimultaneously firing elements to acquire a narrow handle data from the RV inflow and outflow tracts, which
Fig. 54.31: RT 3DE-apical rotation method—The top left, top right Fig. 54.32: RT 3DE-apical rotation method—the same 3D data set
and bottom left panels show three orthogonal MPRs of the RV shown in Figure 54.31 is now advanced to show three orthogonal
taken at the basal level showing discontinuity of the RV inflow and MPRs (top left, top right and bottom left panels) of the RV taken at
outflow tracts. (RT 3DE: Real time, three-dimensional echocardi- the mid-ventricular level. (RT 3DE: Real time, three-dimensional
ography; RV: Right ventricle). echocardiography; RV: Right ventricle).
may appear to be discontinuous when viewed in a basal CMR.117,118 Interstudy reproducibility of RVEDV, ESV, and
short-axis cross-section (Fig. 54.31), but not from a mid- EF by CMR has been reported to be 6.2%, 14.1%, and 8.3%,
short-axis section (Fig. 54.32). Whereas the apical rotation respectively, by Grothues et al.118
method appears to be appropriate for the simple shape of Despite encouraging preliminary results, there are still
the left ventricle, it is unable to handle data in which the many challenges associated with routine quantification
contours appear to overlap (Fig. 54.31). The short-axis disc of the right ventricle. Chief among them is image quality.
summation algorithm is identical to the algorithm used for RT 3DE is subject to error if the right ventricle is large and
analysis of CMR images and is able to handle discontinuous a large portion of the infundibulum falls outside the near
data and overlapping contours both at basal (Fig. 54.33) field afforded by the 90°× 90° pyramidal sector size. Thus,
and mid-ventricular levels (Fig. 54.34). Test–retest vari- while this method works well in normals, its application in
ability for RTDE by disc summation was 3.3%, 8.7%, 10%, markedly dilated right ventricles has not been established.
and 10.3%, respectively for EDV, ESV, and EF. Though Additionally, if the right ventricle is large, undersampling
test–retest variability for right ventricular end-diastolic can occur by the apical rotation method because the
volume (RVEDV), RVSV, and RVEF were acceptable (8.7%, ventricular surface is usually convex and the volume lying
10%, and 10.3%, respectively) and comparable to those between the true surface and the surface approximated by
reported for CMR,115 these values were somewhat higher the AR algorithm is omitted, resulting in underestimation.
than those noted for EDV, probably reflecting variability Underestimation may also occur because the RV
in end-systolic video-frame selection.116 Normal reference inflow and RV outflow tracts may be very large and may
ranges of indexed volumes (mean ± 2 SDs) for RVEDV, ESV, appear to be discontinuous when viewed on a single
SV, and EF were 38.6 to 92.2 mL/m2, 7.8 to 50.6 mL/m2, image plane and therefore not included by the volume
22.5 to 42.9 mL/m2, and 38.0 to 65.3%, respectively, for algorithm. This can be addressed by the short-axis disc
women and 47.0 to 100 mL/m2, 23.0 to 52.6 mL/m2, 14.2 summation algorithm in which portions of the right
to 48.4 mL/m2, and 29.9 to 58.4%, respectively, for men.116 ventricle that appear discontinuous on any given plane
These values are similar to the normal referenced indexed such as the inflow and outflow tracts can be included in
ranges of indexed volumes for RVEDV and RVESV by the volume by summating separate discontinuous discs.
Fig. 54.33: RT 3DE-disc summation method—the top left, top right Fig. 54.34: RT 3DE-disc summation method—the same three-
and bottom left panels show three orthogonal MPRs of the RV dimensional (3D) data set shown in Figure 54.4A is now advanced
taken at the basal level showing discontinuity of the RV inflow and to show three orthogonal MPRs (top left, top right and bottom left
outflow tracts. (RT 3DE: Real time, three-dimensional echocardi- panels) of the RV taken at the mid-ventricular level. (RT 3DE: Real
ography; RV: Right ventricle). time, three-dimensional echocardiography; RV: Right ventricle).
can be minimized by including 7–10 images.119 Variable

designation of end-diastolic and end-systolic frames by
RT 3DE and CMR is a source of error. Differences in image
acquisition approaches (RT 3DE long-axis rotational
approach vs CMR short-axis cross-sectional approach)
introduce different partial volume effects, which may
introduce error. Endocardial boundaries may be obscured
by tangential RT 3DE-apical slices, whereas variable
inclusion of the right atrium and RV outflow tract may
occur by CMR. Boundary tracing error remains the largest
source of error. Tracing the endocardium on the white side
of the black–white boundary minimizes underestimation
of RT 3DE volumes when compared to CMR. Variable
visualization of the apex can be minimized by carefully
manipulating the entire 3D data set so that the largest
long-axis is visualized and prescribing a series of short-axis
images such that they are perpendicular to the long axis.
Toggling between the traced endocardial boundaries as
displayed in the orthogonal multiplanar reconstructions
minimizes erroneous boundary tracing. Although best
Fig. 54.35: RT 3DE RV automatic boundary tracking algorithm at
the mid-ventricular level. (RT 3DE: Real time, three-dimensional results are obtained with manual boundary tracing an
echocardiography; LV: Left ventricle; RV, Right ventricle). automatic RV boundary recognition algorithm has been
utilized which tracks best at the mid-RV level (Fig. 54.35).
In addition, the thickness of the discs can be reduced to This algorithm requires manual inputs of the tricuspid
reduce interpolation of data between traced areas. Based annuli and the RV apex as starting points for the boundary
on the work of Weiss et al. significant underestimation tracking to take place. However, manual correction of the
automatically generated boundaries is necessary to avoid and the RVEF or tenting volume. Therefore, the most
significant underestimation.120 In particular, the base adverse remodeling was noted in the patients with PH.126
and the RV outflow tract is incompletely visualized 48% The same authors studied prognostic markers in these
of the time requires manual correction of the automatic patients. An increase of right atrial (RA) sphericity index
contours. A 3DTEE study has shown that the RV outflow > 0.24 predicted clinical deterioration with a sensitivity of
tract is not circular, but oval.121 Future developments in 96% and a specificity of 90% [area under the curve (AUC)
automatic image segmentation, possibly with the help of = 0.97]. RV sphericity index was less sensitive (70%) and
contrast agents may improve results. specific (62%) in predicting clinical deterioration (AUC =
0.649). The deterioration in RVEF had a sensitivity of 91.1%
RT 3DE Studies of Congenital and a specificity of 35.3% (AUC = 0.479) in predicting
clinical deterioration. The dilatation of RA > 14 mL over
Heart Disease and PH 1 year had high sensitivity at 82.6% but low specificity at
The underestimation of RV volumes is particularly marked 30.8% in predicting clinical deterioration.127
in patients who have massive ventricular dilation and In summary, the field of 3DE has made tremendous
is not ready for clinical use in patients with congenital progress over the last 25 years and is now being offered on
heart disease.122 In PH patients, RT 3DE has shown some every clinical platform. Though its efficacy and superiority
promise. In patients with PH, evaluation of the RV diastolic over conventional techniques is now well established, there
and systolic volume and EF by RT 3DE showed a higher is still considerable variability of products and algorithms
discriminating power in comparison, respectively, with 2D offered by the differing vendor platforms. This makes
RV diastolic area and the relative FACs.123 RV shape change standardization difficult among the various platforms
has been studied in PH by RT 3DE. In PH, the right ventricle and also makes it difficult to gather a large enough patient
is more spherical with increased cross-sectional area at database to offer long-term RT 3DE prognostic parameters.
the mid and basal ventricular segments, basal bulging With further improvements in transducer design, image
adjacent to the tricuspid valve and blunting or rounding of quality, temporal resolution, and standardization, it is
the apex.124 Additionally, a RT 3DE study in these patients anticipated that clinical guidelines regarding its routine
showed that RV inflow and global systolic function was use will emerge.
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CHAPTER 55
Newer Aspects of Structure/Function
to Assess Cardiac Motion
Gerald Buckberg, Navin C Nanda, Julien IE Hoffman, Cecil Coghlan
Snapshot

State-of-the-Art 
The Normal Heart

Composite of State-of-the-Art Reports 
The Septum

Novel Mechanical and Timing Interdependence 
The Right Ventricle
Between Torsion and UntwisƟng 
Other ConsideraƟons
INTRODUCTION Cardiac movement had been analyzed by two-dimensional

(2D) methods like the ventriculogram and echocardiogram
Cardiac motion, until recently, had been thought to follow that display its narrowing, shortening, lengthening, and
the observations of William Harvey, who dissected cadaver widening motions. Now, three-dimensional (3D) imaging
hearts and deduced that the heart underwent constriction is available due to development of magnetic resonance
for ejection and dilation for filling, “acting like a water imaging (MRI) and speckle tracking imaging (STI;
bellows”. Keith1 delivered the classic article on structure Figs 55.2A and B), so that the natural twisting movement to
and function during presentation of his 1918 Harveian develop torsion and uncoiling to permit suction for rapid
Lecture, and called Harvey the “functional anatomist” filling becomes evident; these motions become impaired
who emphasized that “we cannot claim to have mastered by a spectrum of cardiac diseases. The newer 3D obser-
the mechanism of the human heart until we have a funda- vations appropriately supplement the 2D measurements,
mental explanation of its architecture”. Keith described the as all six movements have become accepted descriptors
cardiac architecture to contain circumferential and helical of cardiac motion. Recognition of the form/function
fibers, as he perhaps relied upon the observations of Lower2 relationship is essential in order to determine how the
in the 1600s describing that the cardiac apex showed helical interweaving circular and helical fibers cause them.
fibers, or Senac in the 1700s,3 who defined an internal Just as the anatomist observes structure and deduces
helix and surrounding transverse circumferential fibers, or function, those that use the echocardiogram have
Krehl’s 1800s description4 of its powerful circumferential observed motion and deduced structure. Solution to this
fibers that cause cardiac constriction during ejection. problem occurs when structure is understood, so that
Physiological recordings of pressure and flow have cle- function can be explained, and that is the goal of this
arly defined the impact of ventricular performance on these review. The infrastructure for explaining this macroscopic
variables, but their cause is determined by the function structure/function relationship involves knowledge of
of the underlying ventricular structure (Figs. 55.1A to C). the functional anatomy of the heart. In 1942, Robb and
Chapter 55: Newer Aspects of Structure/Function to Assess Cardiac Motion 1177
Robb5 summarized the findings of anatomists over five functional HVMB provides an explanation for how the
centuries, and expressed generalized agreement that interacting circular and helical fibers cause each of these
the heart structure includes a helical configuration that actions, as this information was gleaned from motion
contains an apex, together with a circumferential muscle studies using sonomicrometer crystals, MRI, diffusion
mass that occupies upper two-thirds of the cardiac base. tensor magnetic resonance imaging (DTMRI), 2D echo-
Disagreement has existed as to the exact layering positions cardiogram, 3D STI, velocity vector imaging (VVI), and
that are occupied by the overlapping circumferential and radionuclide ventriculography.16–18
helical fibers. Grant,6 Lev,7 and Anderson8 have voiced
concern about how the microscopic connections between BASIC HEART FUNCTION
the fiber tracts are always dislodged during manual
dissection. The heart is a muscular pump that supplies blood
Francisco Torrent-Guasp did a hand dissection of the containing oxygen and nutrients to the body. This goal is
ventricles in his effort to define “functional pathways”, and achieved by electrical excitation that produces sequential
his work demonstrated that the unscrolled heart appeared ventricular emptying and filling. Figure 55.1 demonstrates
like a rope-like model when stretched from the pulmonary the physiological sequence of ventricular function—a
artery to the aorta.9,10 His dissection demonstrated that the contraction phase to develop pre-ejection tension, ejec-
intact heart contains two interconnected loops containing tion, and rapid and slow periods for filling. This report
a circumferential and helical muscle mass and his relates the function to the underlying precisely described
configuration is called the helical ventricular myocardial muscular anatomy, thereby providing novel structural
band or HVMB; this anatomy will be described in detail, as explanations for the contractile sequence that causes the
it forms the basis of the structure/function analysis in this ventricular directional motions of narrowing, shortening,
review11 (Fig. 55.3). lengthening, widening, and twisting and uncoiling
The role of this functional analysis is to adhere to (see Fig. 55.1A to C).
Harvey’s functional anatomist requirement so that we The observed functional patterns (see Figs 55.2A
can integrate helical and circular fiber tracts in order and B) are documented by MRI and include an initial
to explain reasons for the readily observed narrowing, global counterclockwise rotation and attendant narrowing
shortening, lengthening, widening, twisting, and uncoiling or “cocking” in the isovolumic contraction (IVC) phase
motions. Current echocardiography movement analysis before ejection, followed by twisting of the cardiac
demonstrates each of these motions, yet their conventional apex in a counterclockwise direction and of the base
“state-of-the-art” reports have consistently failed to in a clockwise direction as the ventricle longitudinally
consider the circumferential muscle.12–15 Conversely, the shortens during the ejection phase, followed by a vigorous
A B1
Figs 55.1A and B
B2 B3
B4 C
Figs 55.1A to C: (A) Currently accepted time frames of systole and diastole, with measurements of intravascular pressure in the aorta,
left ventricle (LV), left atrium (LA), and LV volume, together with their impact on the mitral and aortic valves. Aortic flow occurs between
the two intervals that define ejection. The physiological phases of cardiac cycle that include isovolumic contraction, ejection, isovolumic
“relaxation” (to be questioned in this report), rapid and slow filling, and atrial contraction are shown; (B) Two-dimensional images of the
LV in a longitudinal view that shows the normal sequence of narrowing, shortening, lengthening, and widening of the ventricular cavity
during a normal cardiac cycle. Images were obtained by epicardial imaging in an open-chest porcine preparation. The phases of the car-
diac cycle include end-diastolic state (B4), isometric phase (B1), ejection (B2), and isovolumic phase (B3). The broken-line markers are
within the ventricular cavity and define the transverse (between the midendocardial walls) and the longitudinal (from apical endocardium
to a line across the mitral annulus) dimensions. Muscle thickness is shown by the dark area adjacent to these intracavity dimensional
lines. The pale color is the cavity. The predominant changes exist with muscular thickening that narrows and widens the cavity rather
than the external wall dimensional changes. Note progressive muscular thickening (evaluated by wider distance between epicardial and
endocardial lines as myocardial mass narrows and shortens for ejection), together with maintained thickness as heart lengthens during
the rapid filling phase before substantial widening; (C) Twist of the heart: clockwise (below baseline) and counterclockwise (above base-
line) motions of the base and apex, respectively, during the cardiac ejection and filling periods are represented in rotational degrees with
the use of speckle tracking with marker placed at the LV endocardial surface (Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA).
The relationships between the initial uniform and then reciprocal twisting motions of the base and apex during the pre-ejection, ejection,
and rapid and slow filling periods are explained in the text.
Figs 55.2A and B: (A) Magnetic resonance imaging (MRI) phase con-
trast velocity mapping (tissue phase mapping) of systolic and diastolic
cardiac frames with a temporal resolution of 13.8 ms during free breath-
ing in a healthy volunteer. All motions are described in the text; the
arrows show the clockwise (marker to right) and counterclockwise
(marker to left) directions of transmural twisting motion during the
short-axis view and are obtained during isovolumic contraction, midsys-
tole, isovolumic “relaxation” phase, and slower filling in mid-diastole;
(B) Differences in mean values for tracing radial, tangential, and longitu-
dinal velocity motion, each 13.8 ms, for 12 volunteer subjects in whom
basal, mid, and apical segments are analyzed. Values above zero line
indicate contraction, clockwise motion, and shortening; below the zero
line, values define expansion, counterclockwise motion, and lengthen-
ing. The line expansion time is end systole (ES), with an average 320 ms
time frame. Note early radial expansion in basal segment (a), reversal of
twisting before end of systole (b), and supplemental late counterclock-
B wise base motion during systole (c).
A B
Figs 55.3A and B: (A) Myocardial fiber organization. (a) Mall and MacCallum’s suggestion of bundles, which include deep (circular)
and oblique bulbospiral tracts. (b) Rushmer’s functional model, which includes the central transverse constrictor muscle and oblique
clockwise and counterclockwise layers. (c) Torrent-Guasp’s fiber trajectory model showing an upper transverse circumferential muscle
(or basal loop) surrounding the oblique right- and left-handed helical apical loop; (B) (a) Diffusion tensor magnetic resonance imaging
(DTMRI) studies where water is diffused parallel to fiber orientation, showing a helical positive or right-handed helix or clockwise (red)
and negative or left-handed helix or counterclockwise (yellow) muscle of myofibers reflecting circumferential or horizontal with a zero
helix angle. Note absence of circumferential or circular fibers in the septum, and how these zero angle helix fibers encircle the left and
right ventricles. (b) Dissected heart showing the circumferential or basal loop fibers encircling the left and right ventricles that are not
present in septum, and overlapping left and right helical fibers of the apical loop in septum.
global untwisting in a clockwise direction as the ventricle fibers with an average angle of −50° (clockwise rotation
lengthens and slightly widens during a phase interval below the equator), and the remaining 55% of midwall
where no blood enters or leaves the ventricular chamber. muscle fibers had an approximately horizontal (equatorial
This untwisting motion continues into the rapid filling or circumferential) orientation. Streeter found that the
interval, and finally a phase of relaxation exists during apical one third of the LV had no circumferential fibers,
diastole as heart widening continues during the slower but there is less certainty about the composition of
filling period before the atrium contracts during initiation the septum. Many studies by DTMRI or polarized light
of the next organized beat. The helical and circumferential show three comparable layers,21–24 yet others show only
muscle mass of the intact heart causes these movements, two oblique layers without a circular component25,26
and explaining how they cause these integrated motions (Fig. 55.3B). The VVI method that will subsequently be
is our goal. shown will confirm the presence of two oblique layers, as
this functional measurement provides best evidence of its
structural arrangement.17 Moreover, ultrahigh frequency
STATE-OF-THE-ART
ultrasound functional studies show that these two septal
The underlying myocardial muscle mass is composed layers are separated by a thin midseptum bright echo line
of helical and circumferential fibers, even though their partition, and that they contract independently during
origins are uncertain.6,7,19 There is general agreement thickening to develop a similar longitudinal motion.18
from DTMRI studies that the basal two thirds of the left The thinner right ventricular (RV) side corresponds to the
ventricular (LV) free wall contains three layers of muscle. subepicardial fibers of the free wall, and the thicker LV side
These correspond to the layers defined by Streeter20 who conforms to the free wall subendocardial fibers.
found that the inner 20% of the wall had subendocardial Based on studies by Streeter and previous investigators,
fibers with an average angle of about +60°, where the the noncircumferential fibers form helices16,27,28 that
positive sign indicates counterclockwise rotation above are composed of oppositely wound oblique fibers
the equator, the outer 25% of the wall had subepicardial that comprise a right-handed arm within the deeper
(subendocardial) muscle and a left-handed arm that is unopposed during each pre- and postejection phase,
occupies superficial (subepicardial) muscle. The HVMB yet one of them becomes the dominant muscle when the
model of Torrent-Guasp shows that these right and left entire helix co-contracts during ejection, whereby the
helical arms form an apical loop; the right-handed arm is torque of the subepicardial muscle rotates the cardiac
called the descending segment and the left-handed arm apex counterclockwise, while the subendocardial muscle
is called the ascending segment.10,29 Circular fibers with contraction causes the basal clockwise motion that
transverse orientation19,29,30 surround the LVs and RVs, and produces shortening.16,17
these are called the right and left segments of the basal loop Similar distinctions exist during shortening for
within the HVMB (Figs 55.4A and B). The HVMB model is ejection, because the circular muscles cause compression,
displayed in the major anatomy texts written by Clemente31 yet the cardiac longitudinal dimension is reduced, thus
and by Moore and Dalley.32 The interaction between indicating that the helical fibers have dominant power to
the helical and circular fibers provide the mechanical oppose the elongation that would otherwise occur from
reasons for the rotational motions that are observed by constriction, as they did during IVC. Furthermore, the
imaging studies and will be subsequently defined for net counterclockwise and clockwise rotational directions
IVC, ejection, postejection isovolumic phase, and rapid that exist during IVC and uncoiling (untwisting)16,17 are
filling. Mathematic modeling by Sallin33 defined the vital governed by the most powerful component within these
importance of fiber direction in causing function, as the overlapping circular and helical muscular components.
oblique helical fibers produce a 60% ejection fraction,
while the transverse orientation of the circumferential COMPOSITE OF STATE-OF-THE-ART
fibers cause a 30% ejection fraction. The integrity of fiber
REPORTS
orientation is related to the extracellular collagen scaffold,
which governs muscle alignment, ventricular shape, and Prior imaging reports only address the helical compo-
size. The spiral fibrillar structure of endomysial collagen nent as a smooth change from a left-handed helix in
supports the spatial distribution of myocytes by a weave the subepicardium into a right-handed helix in the
that ensheathes the HVMB structure described in detail34 subendocardium, without considering actions of the
as profound heart failure follows collagen scaffold damage anatomical circular fiber structure that remains the
in hearts that do not have direct myocyte disease.35 centerpiece of anatomical descriptions (Fig. 55.5),4,37–39
The different muscular components contract asynchr- and whose presence is further confirmed by DTMRI
onously. For example, sonomicrometer crystal studies recordings25,26 (Fig. 55.3B). The mechanisms for twisting,
show that the subepicardial muscle does not contract whereby the apex rotates counterclockwise and the
during the isovolumic pre-ejection interval (IVC), both base rotates clockwise, has been based upon the Taber
subendocardial and subepicardial muscles contract model of a single helical layered architecture,40 where
and contribute to torsion during ejection, and only obliquely aligned muscle fibers are embedded in an
the subepicardial muscle continues to contract during isotropic matrix. This engineering design states that
postejection isovolumic phase when untwisting or recoil torsion develops within each layer so that epicardial fiber
occurs.16,36 This scheme of asynchronous contraction contraction rotates the apex in a counterclockwise and the
underscores the incorrectness of traditional thinking that base in clockwise direction, while subendocardial region
states that the ventricle contracts synchronously. contraction will rotate the LV apex and base in exactly the
“Dominance” defines the governing muscular force opposite directions. In contrast, this description of torsion
causing the directional and rotational motions during within each right- and left-handed helix differs from the
each heartbeat, as the interweaving helical and circular current mechanistic descriptions by showing that torsion
cardiac muscles co-contract and/or recoil during each develops between each arm of the helix, whereby there
of the IVC, twisting, and untwisting phases. For example, is clockwise motion of the entire right-handed arm and a
when considering the helical muscles, only the deep right- counterclockwise motion of the entire left-handed arm16,17
handed helical muscle contracts during IVC, whereas only (Fig. 55.6).
the superficial left-handed helical muscle contracts during When both helical layers contract simultaneously
the isovolumic pre-filling phase. These helical muscles during ejection, the larger radius of rotation for the
are antagonistic, so that the contracting helical muscle outer epicardial layer provides a mechanical advantage
B1 B2
B3 B4
Figs 55.4A and B: (A) Unscrolled myocardial band model of Torrent-Guasp that contains a circumferential basal loop and a helical apical loop.
Note (1) the transverse basal loop fiber orientation (b–e) representing circumferential fibers, and (2) the right- and left-handed apical loop helix
with predominantly oblique fibers, and (3) myocardial fold in (e) showing basal midwall twist to form the apical loop. The unfolded basal loop (d)
contains a right segment (RS) and left segment that surround the left and right ventricles. The septum does not have circumferential fibers. The
apical loop has helical fibers that form the right-handed helical arm or descending segment (DS) and left-handed arm or ascending segment
(AS). The unfolded myocardial band in (e) extends between the pulmonary artery (PA) and the aorta (Ao). Note (a) the intact heart contains
a circumferential basal loop wrap that surrounds the apical loop comprising helical fibers; (B) Architectural fiber orientation of (B1) intact heart
in upper left with circumferential fibers surrounding the inner helical fibers, (B2) detached circumferential fibers (basal loop) in upper right with
predominantly horizontal fibers compared with the conical apical loop containing right- and left-handed helical fibers in a helical design, (c) with
these segments super-imposed (top image); when the segments are separated (below), (B3) the right-handed helix or descending segment
(lower left) connected to the myocardial fold with oblique fibers aimed toward apex, and (B4) overlying left-handed helix or ascending segment
(lower right) with longer oblique fibers coursing toward aorta connection. This fiber orientation is used in all subsequent anatomical drawings.
Fig. 55.5: Conceptual cartoons of myofiber structural orientation from imaging and anatomy reports. The imaging drawing (left) separates the
left ventricle into a deep endocardium with right-handed helical clockwise fibers and a superficial epicardium with left-handed helical counter-
clockwise fibers. There is no circumferential or circular muscle. The anatomical drawing (right) displays similar right- and left-handed helical
arms in the deep endocardium and superficial epicardium regions but adds the prominent component of thick circular or circumferential fibers
that reflect the “Triebwerkzeug” described by Krehl.4 These circular fibers are considered to be constrictor fibers by anatomists.
(torque) to dominate the overall direction of rotation has been ascribed to lower temporal resolution by the
toward apical counterclockwise rotation14,41,42 (Fig. 55.5). same MRI modality that is simultaneously called “the gold
Untwisting was ascribed to endocardial or right-handed standard” of measurements. An alternate explanation is
helix recoil in an engineering model that also predicts that both methods are correct, but that this discrepancy
(a) no difference between a cylinder and an ellipse model may be related to imaging analysis depth as MRI quantifies
(as also described by Ingels41 in intact hearts),20,30 and transmural observations and STI focuses only upon tissue
(b) states that both endocardial and epicardial segments moving in the same observation plane.16,17
cause systolic shortening to bring the base toward
the apex.12,15,40,43 Conversely, this analysis employs an
DEFINITIONS
anatomically defined structure to define mechanical and
timing interdependence of twisting and untwisting, and “Rotation” is the circular or angular movement of the
differs from current state-of-the-art reports. The right- and LV about its long axis, and by convention is defined as
left-handed helical arms will be called the descending and clockwise or counterclockwise when looking up at the
ascending segments, respectively. heart from the foot of a supine patient. If the whole LV
Imaging reports using 2D STI recordings describe rotates en masse, there is no torsion. Conversely, if apex
subendocardial clockwise motion and state the transmural and base rotate in opposite directions, then torsion can
apical region moves clockwise, while the reciprocal be assessed by the angular difference between them
stretch of the subepicardium causes the base to move (Fig. 55.8).
counterclockwise during that interval. These 2D STI “Twisting” describes these differences without ref-
observations differ from the MRI evidence of transmural erence to a long-axis measurement. “Untwisting” expresses
IVC apical counterclockwise motions16,44 (Figs 55.7A the return of cardiac shape to its initial resting position.
to C). This disparity between different recording methods “Torsion” defines the difference between the rotation of
Fig. 55.6: Structural reasons for torsion from bioengineering drawings (above) and anatomical structure (below). Comparable find-
ings during torsion development are reported in bioengineering studies under conditions where myocardium structure is displayed
as either cylindrical (upper left) or conical (upper right), as the right-handed helical arm or deeper clockwise endocardium and is
covered by a left-handed helical or counterclockwise arm. Torsion is described as developing within each arm as shown by the
arrows in the cylinder on the right, and each arm develops clockwise and counterclockwise motion as shown on the left. The clockwise
layer is R1, counterclockwise layer is R2, and its larger torque causes apical counterclockwise rotation during torsion. Below, the ana-
tomical structure shows a right-handed helical arm with clockwise motion (lower right) and a left-handed helical arm with counterclock-
wise motion (lower right) and these arms are called the descending and ascending segments of the apical loop. Torsion is described as
developing between helices, as the entire right- and left-handed helix move in different directions.
the base and apex of the LV relative to the long axis, exists into why these interweaving circular and helical fiber
beyond the interval for systolic ejection, is measured components exert their dominant “global” action
in degrees, and defining its duration is a vital part of its becomes clearer by use of other imaging modalities such
measurement. as STI, VVI, and sonomicrometer crystals that define
“regional” analysis, and simultaneously characterize
NOVEL MECHANICAL AND TIMING their timing sequence.17,42,46 Harmonic interaction exists
INTERDEPENDENCE BETWEEN during rotational movement that relates to the coiling and
uncoiling actions of responsible muscles; the uniformity
TORSION AND UNTWISTING of global counterclockwise motion during IVC precedes
Torsion and untwisting are “transmural” rotational the apex and base differential motion during torsion,
movements well seen by MRI.44,45 Mechanistic insight and then recurs during untwisting as a uniform or global
A B
C
Figs 55.7A to C: Motion of apex and base during isovolumic contraction by speckle tracking imaging (STI; upper) and magnetic reso-
nance imaging (MRI) studies. (A) The STI study shows counterclockwise (above baseline) and clockwise motions of the base and apex,
respectively, during the isovolumic contraction. The speckle tracking with marker is placed at the left ventricular (LV) endocardial sur-
face (Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA). Tracings from Aman Mahajan laboratory; (B) MRI studies showing global
counterclockwise motion of the apex and base during isovolumic contraction in both studies. Tagged MR images were acquired on a
1.5 T whole body MR scanner (Magnetom Sonata, Siemens, Erlangen, Germany) with a temporal resolution of 14 ms. The hatched line
following twisting (apex counterclockwise and base clockwise) marks peak apical rotation that exists just prior to the postejection iso-
volumic interval. Note: apex begins clockwise motion at this stage and prolongation of clockwise base rotation; global clockwise motion
occurs during this postejection isovolumic interval; (C) Velocity vector imaging (VVI) short-axis views of endocardial rotational motion of
six segments at the apex and base employing the (Sequoia 512, Siemens, Mountain View, CA, USA 4.0 MHz transducer) derived from
three-dimensional (3D) images displays counterclockwise motion of the apex and base during the isovolumic contraction (IVC) phase.
The hatched purple lines show both the end of the IVC phase where AVo is aortic valve opening, and the separated purple hatched lines
between AVc or aortic valve closure and MVo or mitral valve opening show the postejection isovolumic interval.
clockwise movement.16 Consequently, despite emphasis THE NORMAL HEART

upon their interplay during torsion and untwisting,14,15,47,48
understanding their interweaving muscular interactions The Left Ventricle
during the IVC interval is fundamental requirement in
order to understand mechanistic reasons for torsion and
Isovolumic Contraction
untwisting motions. Moreover, untwisting cannot begin if Ventricular narrowing, elongation, and counterclockwise
torsion is prolonged. net rotation characterize the pre-ejection phase.16,17
descriptions of fiber orientation of the underlying muscle

during IVC may be misleading, because only oblique
helical fibers were measured at the selected sampling;51
this analysis did not evaluate the circumferential basal
segment, whereby their horizontal orientation49,50 exerts
the compressive force that was just described.
MRI evidence of global counterclockwise motion
contradicts STI documentation of clockwise motion by
the right-handed helix or descending segment, and VVI
recordings derived from real time 3D echocardiography
in Figure 55.7C confirm this counterclockwise movement.
Global counterclockwise movement must arise from
the governing circumferential muscle fiber, because the
clockwise motion arising from descending segment is not
dominant. Anatomical analysis of circumferential muscle
mass dimensions16 shows why the thicker, left-sided basal
Fig. 55.8: Ventricular torsion displayed by speckle tracking imag- circular fibers exert the mechanical advantage causing
ing (STI) on left side (From Aman Mahajan laboratory) and mag- the dominant counterclockwise motion that accompanies
netic resonance imaging (MRI) on right side (From Jurgen Hennig the ventricular narrowing that exists during LV cavity
laboratory), where twisting motions between the left ventricular
apex and base are displayed. STI study shows counterclockwise
compression (Fig. 55.9A).
(above baseline) and clockwise motions of the apex and base, Moreover, recent VVI recordings show that as the
respectively, during the cardiac ejection. The speckle tracking with compressed or narrowed LV chamber develops pressure,
marker is placed at the left ventricular (LV) endocardial surface the blood flow velocity from apex to base closes the
(Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA). MRI
phase contrast velocity mapping (tissue phase mapping) of sys- mitral valve,16 and there is simultaneous expansion or
tolic ejection cardiac frames with a temporal resolution of 13.8 ms rightward motion of the upper portion of noncontracting
during free breathing in a healthy volunteer shows arrows that ascending segment fibers within the septum (Fig. 55.9B).
demonstrate clockwise (marker to right) and counterclockwise
This rightward movement, which resembles the bulging
(marker to left) directions of transmural twisting motion during the
short-axis view. of an aneurysm, occurs because of the presence of
noncontracting or relaxed ascending segment fibers within
this subaortic valve region that is anatomically uncovered
Sonomicrometer crystal recordings during this 50-ms by the contracting right-sided deep descending segment
interval display the contributions of individual muscle fibers, as shown in Figure 55.9B. Rotational motions
masses by documenting that (a) the right and left sides during IVC similarly relate to dominant interactions
of the circumferential circular fibers shorten almost because (a) the coiling of basal loops circular fibers that
simultaneously (10 ms delay between right and left causes cardiac compression, simultaneously produces a
segments of basal wrap)16,49,50 with the subendocardial net counterclockwise global movement that overcomes
descending segment helical fibers during IVC, and (b) clockwise rotation caused by the descending segment
there is no ascending segment helical subepicardial fiber fibers as intraventricular pressure rises before ejection,
shortening during that interval.49,50 STI measurements of especially because (b) the ascending segment muscle is
subendocardial shortening and lengthening during pre- not shortening, so that it cannot contribute to pre-ejection
ejection systole provide further evidence of stiff outer counterclockwise basal rotation. Most importantly,
shell dominance.13 They show that the initial longitudinal the clockwise descending segment motion during IVC
shortening is followed immediately by apical lengthening,
produces a persistent clockwise rotational motion that
as the circular base compresses the inner helix; shortening
continues as torsion develops during the next phase of
would otherwise occur if the co-contracting descending
ejection.
segment was dominant. Moreover, the overlying nonco-
ntracting ascending segment fibers stretch during
ventricular elongation,42,51 so that they cannot be
Torsion
responsible for global counterclockwise rotation of the The presystolic isovolumic interval is followed by
cardiac base during the pre-ejection interval. Previous ventricular ejection, whereby torsion develops as the
B
Figs 55.9A and B: (A) On the left side is cranial view of the model of the helical ventricular myocardial band showing how the circular
and circumferential fibers or basal loop surrounds and embraces the conical right- and left-handed helix or apical loop. Note that (a)
circumferential fiber muscle thickness is greatest in the left component or segment, and thinner in the right component or segment and
(b) there are no circumferential fibers in the septum. On the right side are VVI images of isovolumic contraction (Sequoia, 512, Siemens,
Mountain View, CA, USA; 4.0 MHz transducer), where there is shortening of the entire circumference of basal loop, and of the right-
handed helical armor descending segment. No left-handed arm or ascending segment shortening occurs, yet right-sided upper septum
motion exists in area of uncovered noncontracting left-handed helix or ascending segment as shown in B images; (B) Topographical view
of septum architecture in the wrapped heart where the right ventricle is intact (left side) and unwrapped form where the circumferential
fibers are separated. Note that the apical loop has a septum segment above the overlap of right- and left-handed helical fibers, where
the left-handed arm or ascending segment is the only muscle mass. This segment does not shorten during the isovolumic contraction,
and is the only segment that shortens during the postejection isovolumic phase (Fig. 55.8A, left panel and Fig. 55.11B, in “a” figure).
LV cavity shortens and its wall thickness increases due pathway achieved by the outer ascending segment helical
to deformation of all co-contracting circular and helical arm as the entire helix coils.16,17 The figure-of-eight spiraling
fibers, as the ascending segment begins to contract.49,50 arms of these shortening vectors dominate (Fig. 55.11A), as
MRI and STI images13,15,16,44,46,52 document that the apex the descending segment fibers exert a downward velocity
and base twist in different directions, and VVI recordings16 vector direction toward the apex during their contraction,
show consistent inward systolic subendocardial motion while the ascending segment helical fibers are pulled
at the apex, midwall, and base to provide evidence that downward to follow a downward velocity vector toward
contradicts theoretical concepts that counterclockwise the apex caused by dominance of the descending segment;
twisting exists within the subendocardial muscle.14,15 the upward motion of the ascending segment only
Compression is a central feature of the torsion becomes uncovered during the postejection isovolumic
sequence as the LV shortens and twists to eject, because phase, where the antagonistic descending segment stops
such narrowing reflects the functional contribution of contracting. Consequently, shortening forces overcome
circumferential or circular fibers, as well as being due to the predominant compressive action of the circular fibers
helical fiber deformation. Conversely, shortening reflects that existed when torsion was initiated.
the principal coiling motion of oblique helical fibers There is complementary action between the recipro-
cally helical coiling forces that become maximal at the
within the descending segment. The initial phase of torsion
apical vortex,53 because the circumferential basal fibers act
furthers ventricular compression without imparting
as a buttress to prevent explosion or unlimited expansion
substantial shortening, as the ascending segment fibers
of the vortex forces of helical fibers basal components as
only begin to contract at this beginning stage. VVI studies
they develop reciprocal outward forces to balance the
during this phase at the onset of torsion are shown in Figure
downward motion toward the apical vortex (Fig. 55.11B).
55.1 (occupying approximately 50 ms or approximately
From a rotational aspect, the torque from the larger
20% of the torsion interval at 72 beats/min), as they provide
curvature of the ascending segment helix rotates the
insight into why the circular fibers remain dominant as
apex counterclockwise, while the more forceful strain in
torsion starts. Figure 55.10 displays motion immediately
the descending segment helical fibers16,36 continues the
following the QRS wave on the electrocardiogram by
clockwise rotation of the base that began during the IVC.
showing that (a) the septum twists (different motion of its The extent of deformation and strain increases toward
basal and apical components) as its inward velocity vector the LV apical vortex, which is formed by the helical fibers
reflects descending segment helical shortening, while the that exist without any surrounding circumferential muscle
simultaneously outward velocity vector motion is caused at the apical region.17,54 The endocardium of the entire
by the ascending segment helical arm that is not covered by septum, including its apex, midwall, and base, displays
circumferential muscle, (b) minimum shortening occurs a consistent leftward motion16 to provide evidence that
because the dominant circular muscle mass that occupies contradicts suggestions of separate torsion development
the upper lateral ventricular wall becomes the governing within the “subendocardial” layer and the “subepicardial”
force of narrowing or compression, and thereby overrides layer.14,15,40 Instead, torsion and shearing relate to the
the counterclockwise motion of the underlying oblique entire descending segment or right-handed helix rotating
or ascending segment lateral LV free wall component, clockwise and to the entire ascending segment or left-
whose shortening has just begun. Figure 55.1C also shows handed helix rotating counterclockwise (see Fig. 55.6).
that initiation of clockwise basal motion is delayed due to
ongoing counterclockwise motion of the circular fibers Postejection Isovolumic Phase
that started during IVC, and (c), inward movement persists
within the lower lateral wall, because no circular fibers The isovolumic interval follows ejection and is chara-
exist in that region (Fig. 55.10). cterized by untwisting, lengthening, and widening.16,17 This
The next phase of torsion during ejection involves interval was previously called the isovolumic relaxation
longitudinal shortening and reflects its most important phase (IVR), but ongoing shortening of the ascending
component. Helical fiber dominance governs this motion, segment continues (albeit at a reduced force so that no
even though there is simultaneous compression arising ejection occurs), so that the term postejection isovolumic
from deformation of co-contracting circular and helical interval is a more precise description. The lengthening
fibers, as well as from narrowing by the more horizontal and widening components are 2D echocardiography
observations that do not define the 3D geometry required during torsion.16 Third, the circumferential or basal loop
to maintain a constant volume. Insight into this geometric contributes a dominant clockwise motion, which of course
change is provided by echocardiography studies that contrasts to its counterclockwise movement during the
document how the mitral annulus becomes less oval,55 IVC phase; ventricular widening is also caused by these
thereby explaining how an unchanged volume is recoiling circular fibers.
maintained as the heart lengthens and widens during this Lengthening is evident from the elegant studies of
interval. Karwatowski,57 who used MRI and echocardiography
The reciprocal relationship of contributions from to demonstrate that isovolumic long-axis lengthening
circular and helical muscles becomes revealed by comp- preceded flow across the mitral valve by 46 ms. Lengthening
arison of their pre and postejection isovolumic volume during untwisting is determined by both the left- and right-
interactions. Before ejection, the entire circumferential handed arms of the helix or the ascending and descending
muscle and only the descending segment helical arm segments of the apical loop. One component is the ongoing
shorten, without shortening of the ascending segment contraction of the ascending segment that had become
helical arm. In contrast, during the postejection isovolumic more spiral during torsion. The helical coil becomes more
phase, shortening stops in the left and right segments of taut, so that this segment becomes thicker and straighter
the circular muscles and in the descending segment, but (thus longer) when the counterforce of the right-handed
continues in the ascending segment. There is normally arm becomes removed after its shortening stops.16 In
a 80- to 90-ms “timing hiatus” between the cessation of nature, this resembles the mechanisms within the snake,
the descending segment shortening and the time when which elongates before striking due to differences in the
the ascending segment stops shortening (Fig. 55.13A).
contractile sequences in paraspinal muscles (Figs 55.11A
Disturbance of this normal relationship during torsion
to C). The second lengthening component involves retur-
affects untwisting because apical untwisting cannot begin
ning the recoiling right-handed helix or descending
if torsion is extended by prolonged descending segment
segment to its neutral and thus uncoiled longer helical
coiling.
position (Fig. 55.11C).
Recoil is determined by when shortening stops.
The untwisting sequence during the postejection
Regional and global motion depends upon the dominance
isovolumic phase mirrors torsion by having two
of recoiling contributions of the interweaving circular
components. The first phase begins just before the aortic
and descending segment arms of the helix. This recoil
valve closes57,58 and is characterized by reduced torsion and
process is attributed to expansion of the Titin and collagen
continued shortening as the ascending segment continues
pathways that were compressed during ejection.56 During
the isovolumic phase, rotation during recoil reflects the to shorten (Fig. 55.12A). Moreover, VVI demonstration
dominance within the overlapping circular and helical that the ascending segment arm’s directional vector points
muscle groups, because the ascending segment is still toward the apex does not contradict our prior suggestion
shortening and thereby cannot contribute to this process. that the ascending segment exerted an upward force.
Cardiac spatial configuration is a vital factor in the Instead, this force is overcome by the dominant right-
untwisting process because the LV wall would otherwise handed arm during torsion.16
implode if only helical fibers caused this untwisting An intriguing imaging parallel exists between the initial
motion that precedes suction during rapid filling. This phases of IVC and untwisting (Figs 55.8A and 55.12B in its
dynamic collapse is prevented by the support of the left panel), whereby the same VVI image results are related
stiff outer shell formed by circular fibers that maintain a to entirely different causes. During untwisting, the early
circumferential buttress (Fig. 55.11B). The interplaying appearance of lack of significant motion of the lateral wall
forces of three sets of muscle motions determine the net and lower septum relates to their absence of contraction,
or global clockwise rotation of the apex and base that whereas this same image reflects shortening of the circular
exists during untwisting. First, the ascending segment muscle and descending segment during IVC. The upper
cannot be responsible because it contributes an ongoing septum beneath the aortic valve displays right-sided
counterclockwise motion as it continues to shorten, but and downward motion during untwisting that reflects
with diminished force during that interval.36,49,50 Second, the counterclockwise motion from ongoing ascending
the descending segment arm recoils counterclockwise, segment shortening. In contrast, this right-sided vector
a motion that contrasts with its clockwise movement during IVC reflects the bulging of this noncontracting
Fig. 55.10: Beginning of torsion (upper left) with co-contraction of base and entire helix. There is essentially no longitudinal ventricular
shortening at this time. Note (a) twisting septum with upper septum showing left-sided motion of the right-handed arm of the descending
segment and lower septum, and lower lateral wall has right-sided motion of left-handed arm or ascending segment, and (b) upper lateral
wall has essentially no motion as it is compressed by shortening circular basal muscle, which does not exist in the septum, and (c) the
lower lateral wall has no circumferential compression and shows leftward motion of left-handed arm or ascending segment. Slightly later
in torsion (upper right), with more twisting of septum, and increased left-sided motion of lateral wall that is occupied by the more fully
contracting left-sided arm or ascending segment. The lower images show the responsible architecture, whereby the lower left shows
the wrapped heart, where the uncovered left-handed helix displays more prominent counterclockwise motion. The lower right displays
unwrapped heart to show how the circumferential muscle covers the same upper lateral wall to exert a compressive force and limit early
rightward counterclockwise motion.
A B
C
Figs 55.11A to C: (A) Velocity vector imaging forces during maximal torsion during ejection as longitudinal shortening occurs. Vector
angulation is directed toward the apex, as the helical left- and right-sided arms dominate to change force direction toward the apical vor-
tex, despite ongoing circumferential or circular muscle shortening; (B) Drawing of simulated cardiac anatomy with circumferential wrap
and internal helix (lower left) with spiral motion for ejection (in center) and suction (lower right). The outward spiral forces with an apical
vortex during ejection would expand the basal wall laterally to potentially cause explosion. Lower right shows suction where inward
forces at the base would cause implosion. This circumferential wrap at the base becomes a buttress to prevent these events, and this
configuration resembles a gothic cathedral (upper center) where the buttress protects the downward forces of the dome from loss of the
base due to downward forces from the tip at the peak of the dome; (C) This drawing shows normal anatomy (upper left) with right and
left segments of basal loop [right segment (RS) and left segment (LS)], and right and left helical arms of apical loop or descending and
ascending segments [descending segment (DS) and ascending segment (AS)]. The bottom shows the coils in the right-handed helix
arms in diastole, ejection, and isovolumic phase. Note that (a) both shorten during ejection, but the descending segment is stronger,
(b) ascending segment becomes more horizontal as spiral shortens, and (c) ascending segment continues shortening during isovolumic
phase and causes elevation or lengthening. This action mirrors cobra shown in upper right that lengthens as its spiral becomes elon-
gated in its pose before striking (Fig. 55.11A).
region due to increased intraventricular pressure even though the valve cusps may be open. Untwisting
(Fig. 55.8A compared to 55.12B in its left figure). reflects the opposite of twisting, yet global motion
The second untwisting component involves the shows no differential differences between the apex and
prominent left-sided vectors (Fig. 55.12B, panels b base, as both segments rotate clockwise (MRI image).
and c) that match similar rotational motions recorded Consequently, the term “untwisting” is not matched by
by MRI and STI imaging methods.15,44,46,59 No flow crosses global differential rotational action. A better term may be
the mitral valve during this counterclockwise movement, either unwinding, uncoiling, or recoiling as these terms do
B
Figs 55.12A and B: (A) On the left, tracings of endocardial and epicardial sonomicrometer crystals placed into the fiber orientation
pathways of the right- and left-handed helix, or descending and ascending segments in anterior myocardium of open-chest pig. The
solid line shows the beginning and ending of the right-handed helix shortening, and the hatched lines show the left-handed helix or
ascending segment. The postejection isovolumic phase (within yellow color overlay) shows (a) approximately 80 ms time hiatus, and
(b) lengthening of the right-handed helix as the left-handed helix or ascending (Asc.) anterior fibers are still shortening; the left ventricle
(LV) pressure and dP/dt tracings indicate the timing. On the right is unfolded myocardial architecture showing left- and right-handed
or ascending and descending segments of the helical ventricle, surrounded by the circumferential muscle of the basal loop. Note that
(1) central ventricular cavity is composed of overlapping left- and right-handed helices or ascending and descending segment fibers in
the septum region, (2) left-handed helix wraps around and overlaps the right-handed helix in septum, and (3) absence of overlap in the
lateral wall, which is composed of the left-handed helix or ascending segment. The lack of overlap in the left-handed helix or ascending
segment also occurs in the septum, below the aortic valve, as displayed in Fig. 55.9B; (B) In (a), beginning of “postejection isovolumic
phase” with right-sided motion of the upper septum, where there is ongoing shortening of the left-handed arm or ascending segment
without overlap of right-sided arm or descending segment. Simultaneously, there is essentially no motion of the lower septum or lateral
wall, where the circular and right-handed arm and descending segment fibers just stopped shortening. In (b), slightly later in postejection
isovolumic phase, there is left-sided motion of the septum and lateral wall due to recoil of the circumferential or circular basal muscle,
and the upper septum now shows similar left-sided movement despite the ongoing shortening of the left-sided arm or ascending seg-
ment that just showed right-sided motion before these recoiling forces became dominant. In (c), end of postejection isovolumic phase
with both septum and lateral wall showing left-sided movement as dominant recoil exists in the circumferential or circular base, which
thereby counteracts the simultaneous counterclockwise recoil of both the right-handed helical arm or descending segment and ongoing
shortening with counterclockwise motion of the left-handed helical arm or ascending segment.
not require the differential action that would be needed if

untwisting is used. The dominance of recoiling by circular
or circumferential fibers that cause this counterclockwise
motion parallels how these circular fibers also cause
dominant global counterclockwise rotation as they coil
during the IVC interval. Consequently, a balance becomes
apparent between fiber orientation and rotational motion
before and after torsion. The circumferentially controlled
global motions before and after torsion effectively
surround the twisting motions of the differential clockwise
and counterclockwise rotations of the base and apex
during torsion, which are principally determined by the
helical fibers.
A B
Untwisting during elongation sets the stage for rapid
Figs 55.13A and B: (A) First phase of rapid filling with elongation
filling by creating a deceleration of ventricular pressure
(note apical lengthening) and further leftward motion of the sep-
and circumferential force that creates a potential vacuum tum and lateral wall as there is recoil of the left-handed helical arm
that causes suction after ventricular pressure falls below or ascending segment, together with termination of recoil or the
atrial pressure.16,57 The causative mechanism of untwisting circumferential or circular base and right-handed arm or descend-
ing segment whose forces interacted during the preceding poste-
during rapid filling differs from recoil of the noncontracting jection isovolumic phase; (B) Completion of the rapid filling phase,
circumferential fibers during the postejection isovolumic whereby increasing ventricular volume is the dominant force as
phase, and relates to recoil of the left-handed arm fibers the left ventricle (LV) chamber further lengthens and widens. Ve-
locity vector imaging (VVI) displays expansion and outward veloci-
that starts immediately after they stop shortening. ties because filling forces overcome recoil action in septum and
Consequently, the left-handed helix or ascending segment lateral wall.
cannot be the cause of the initiation of untwisting during
elongation59 because it maintains strain, continues to
shorten,16 and its counterclockwise motion is maintained
Rapid Filling
until its contraction stops. Under normal circumstances, Untwisting has two components, as the initial unwinding
such ascending segment shortening is normally called occurs during the postejection isovolumic interval and
“post-systolic contraction”, a term that shows why the is dissociated from the untwisting, causing rapid filling
term “IVR” is inaccurate.16 This early postejection time but the causes are tightly interrelated.66 Early untwisting
interval for post-systolic contraction is extremely impo- during the postejection isovolumic interval is the reason
rtant in determining the interdependence of torsion and for subsequent suction, despite this temporal separation;
untwisting, because prolonged descending segment recoil continues from a different cause (the ascending
shortening is caused by the right-handed helical arm due segment) during the subsequent phase of rapid filling
to a spectrum of causes, and will interrupt the onset of that develops after ventricular pressure falls below atrial
untwisting and interfere with rapid filling patterns that are pressure (Figs 55.13A and B). The first phase of untwisting
subsequently considered under clinical implications.60–63 is characterized by transmural clockwise motion that is
The aforementioned temporal and mechanical factors initially caused by dominant recoil of circular muscle
underlying untwisting cause a dynamic geometric change during this postejection isovolumic interval. The second
in ventricular size and shape that result in a series phase develops from elastic recoil of compressed titin coils
of imaging and hemodynamic changes that include within the left-handed helix or ascending segment fibers.
measuring the rate of untwisting48,59 as well as tau (change The interaction of these dual recoiling forces is critical for
in time related to change in deceleration in LV pressure)64 suction, because 50–60% of untwisting normally occurs
and development of an intraventricular pressure gradient before the rapid filling phase.64,67 Moreover, a fundamental
that becomes maximal immediately after the rapid filling component relates to the normal 80 ms timing hiatus
that follows the postejection isovolumic interval.65 Each between the completion of shortening of the descending
factor is a result of the functional geometric change that segment and the later completion of shortening in the
produces such measurements. ascending segment. Suction increases if inotropic drugs
enhance isovolumic phase untwisting,48,59 or diminishes The interaction between noninvasive methods and
if untwisting is delayed by prolonged torsion when structure is enhanced by conventional low-resolution
shortening of the descending segment extends torsion ultrasound imaging of the working ventricular septum,
during this “temporal hiatus interval.”16,63 which has previously identified a hyperechogenic
Unwinding of the apex to return to its original position “septal line” that matches the septal separation line,
in order to create suction for rapid ventricular filling which runs in a basal–apical direction (Figs 55.15A
requires relaxation of all muscle segments, so that the and B)18 as demonstrated by postmortem contrast
isovolumic interval untwisting component becomes an tomography studies by Lunkenheimer et al.27 High-
essential prelude to this process. Conversely, prolonged resolution ultrasound imaging allows identification of
descending segment shortening allows ongoing torsion the structural and functional separation of the ascending
during the isovolumic interval, thereby diminishing and descending septum components along the previously
this response, retards LV pressure deceleration, reduces observed “septal line”, along with temporal and sequential
ventricular compliance, and impairs the 50–60% of filling movement of these muscle layers toward the respective
that normally occurs during this period.67 Augmented ventricular cavities (Figs 55.16A and B). Visualization of
filling pressures are then needed to achieve proper end- different fiber orientation in the working heart using high-
diastolic volume after cessation of the apical clockwise resolution echocardiography strongly supports the helical
unwinding, in order to stretch the LV satisfactorily during anatomical models displaying the muscle bands that
the later slower or passive filling phase. form the ventricular septum and free LV wall. Figure 55.17
displays similar functional contractions of the descending
THE SEPTUM and ascending segments that are similar between these
structures.
The ventricular septum is a thick structure composed Recent animal studies in the working heart, using
of discrete muscular bands that separate the LV and RV. higher magnification of this septum midline in porcine
The septum comprises approximately 40% of ventricular and rabbit models, document that a space exists between
muscle mass and contributes to biventricular cardiac the edges of the septal line (Fig. 55.16B).18 The line is
function.68 Analysis of this structure/function relationship approximately 100 microm (0.10 mm) wide, and its thin-
requires a full understanding of how existing normal edge components attach to the overlying ascending
anatomical form translates into hemodynamic perfor- and descending segments of the septum muscle. The
mance. Satisfactory accomplishment of this task shall septum muscle on either side of this line in the working
answer the 1790 supposition of Weber,19 who indicated hearts shows a relative uniformity that depends on the
that actions of muscular heart would not be understood echocardiogram probe placement position in relation to
until the muscle bundles of the septum are clarified. fiber orientation planes that pass along or across working
Our initial experimental evaluation of septal structure/ muscle (Fig. 55.16B). Further analysis of the midseptum
relationships was acquired by use of sonomicrometer line demonstrates that the space between its edges (a) is
crystal measurements that demonstrated how fiber retained during systole (when intramyocardial vessels
orientation determines the maximum rate of systolic are collapsed by the surrounding contracting muscle);
shortening. Findings validated the hypothesis that the (b) is unchanged during early diastole (when flow
configuration of septum anatomy conformed to the through vessels is greatest and would expand the space
descending and ascending segments of the HVMB, as if it was vascular); and (c) becomes nearly obliterated
described by Torrent-Guasp11 (Figs 55.3 and 55.4). This when function of the overlying ascending and descending
spatial composition has been recently supported by segments is removed during cardiopulmonary bypass
DTMRI recordings (Figs 55.14A to C).25,26 Oblique fibers by inducing ventricular fibrillation, and completely
of the endocardial regions of the left and right sides of the obliterated or cardiac arrest by cardioplegia (Fig. 55.16B).
septum displayed the same functional characteristics that The space between this line conforms to the pathway
exist within in the free LV wall, thereby confirming the followed by Torrent-Guasp during his cardiac dissection as
spatial structural configuration required for development he separated the ascending and descending loops during
of twisting. postmortem analysis. Most importantly, the collapse of
B
Figs 55.14A to C: (A) Fiber orientation relationship of the septum,
composed of oblique fibers that arise from the descending and
A ascending segments of the apical loop, surrounded by the trans-
verse muscle orientation of the basal loop that comprises the free
right ventricular (RV) wall. Note the conical arrangement of the
septum muscle and the basal loop wrap, forming the RV cavity; (B)
(a) Diffusion tensor magnetic resonance imaging (DTMRI) studies,
where water is diffused parallel to fiber orientation, showing a heli-
cal positive or right-handed helix or clockwise (red) and negative or
left-handed helix or counterclockwise (yellow) muscle of myofibers
reflecting circumferential or horizontal with a zero helix angle. Note
absence of circumferential or circular fibers in the septum, and how
these zero angle helix fibers encircle the left and right ventricles.
(c) Dissected heart showing the circumferential or basal loop fibers
encircling the left and right ventricles that are not present in septum,
and overlapping left and right helical fibers of the apical loop in sep-
tum; (C) Diffusion tensor magnetic resonance imaging (MRI) from
the work of Zhukov and Barr26 showing the helical inner or endo-
cardial (clockwise) and outer or epicardial (counterclockwise) fiber
orientation (in purple and blue colors) and a central left ventricular
(LV) free wall that is white to reflect a more horizontal or very small
C angle pitch that does not involve the septum.
A B
Figs 55.15A and B: (A) Cross-section images demonstrating the oblique crisscross endocardial and epicardial fibers contained within
a circumferential midseptal wall; (B) Computed tomography scans demonstrating the interweaving collagen support of the connective
tissue skeleton that is likely the scaffold for reciprocally oblique septal muscular fibers. Note the space between the two septum regions.
A B1
B2 B3
Figs 55.16A and B: (A) Low- and high-resolution echocardiogram

showing the mid-hyperechogenic and midseptal line; (B) High-res-
olution ultrasound image of the septum at the base of the heart
acquired using high ultrasound transducer frequency (12 MHz).
Septal images showing a bilayer structure with an inner dimension
of 100 to 150 mm. B-mode or echocardiographic pattern of the
septum on either side of the septal bilayer is different, demon-
strating the different directionality of the myocardial fibers on the
respective sides of the septum. The septal bilayer is recorded dur-
ing a normal cardiac cycle, during ventricular fibrillation, and during
B4 cardiac arrest.
Fig. 55.17: Comparison of ultrasonic crystal tracings of descending and ascending segments of left ventricular (LV) free wall, and
M-mode and Doppler M-mode imaging of the septum. The beginning and end of descending segment shortening and motion (sol-
id lines), and the ascending segment (hatched lines). Strain in the right (red) and left (blue) sides of the septum is noted in sys-
tole. M-mode shows displacement of the left and right sides of the septum toward their respective ventricular chambers. Note the
delay of initiation of ascending segment and right septal motion and lengthening of descending segment during phase after ejection
and continuing displacement of the right side of the septum toward the right ventricular (RV) cavity, despite the beginning of LV cavity
expansion. (LV: Left ventricle).
this space between the border edges of this midseptal Torrent-Guasp’s unfolding diagrams.50 Torrent-Guasp
line precisely reflects the conditions encountered by the et al. realized this error when they were made aware of
anatomist or pathologist in the cadaver or biopsy specimen. the 1971 functional studies by Armour and Randall.69
Structural differences between dead vs live conditions can Their subsequent dissections display the proper location,
lead to artifacts, but matching form to performance must and they encouraged valid correlation of structure and
remain the goal of the functional anatomist. function during efforts to understand architectural reasons
We concur with problems related to cadaver dissection for living heart motions.
limitations that were suggested by Grant,6 and Lev and The physiological implications of these observations
Simkins,7 and that are now supported by Anderson et al.8 is that the oblique nature of the septum structure is a
An example is the incorrect posterior papillary muscle vital component needed for generating the twisting
position that accompanies the myocardial band during motion required for efficient ventricular ejection against
increased peripheral vascular resistance. In contrast, are needed to define the septum’s twisting capacity.
constriction or bellows action is the predominant An example of this interface is shown by the way that
function of the basal loop as the result of its predominant circumferential basal loop constricts the RV during IVC,
transverse fiber orientation, because its circular fibers an action that precedes the septal shortening that occurs
surround the LV and RV septum. Consequently, right- during ejection when afterload is encountered. Wiggers
sided heart function may become impaired after loss of in 191474 showed that IVC compressive movements were
sequential septum contraction with attendant pulmonary unaltered by RV afterloading because the pulmonary valve
hypertension, an effect that sometimes follows septum did not open during this interval. In contrast, inotropic
hypokinesia or akinesia or dyskinesia in cardiac surgical drug stimulation directly affected circumferential free wall
procedures with impaired myocardial protection,70 or after muscle contraction to accentuate function.74
temporary ischemia, or when the septum is stretched after The interaction between septum and free wall
LV or RV volume overload. Conversely, recognizing and performance has been experimentally tested by investi-
using knowledge from this form–function relationship gators who demonstrated that RV performance was
has resulted in developing innovative RV reconstructive not significantly impaired by either cauterization of the
procedures that restore the septum into the midline entire RV free wall,75 its replacement by a semirigid patch
position, recover its twisting action, and result in favorable material,76 or regional ventricular fibrillation after its
clinical outcomes.71 isolation,77 so long as the septum was intact. Conversely,
RV failure developed if the septum was either cauterized,
THE RIGHT VENTRICLE made ischemic by further dye embolization after right
coronary artery occlusion, or damaged by pulmonary
RV architecture involves two components. First, the free hypertension;78 each intervention deteriorated perfor-
wall is predominantly composed of a basal loop containing mance by interrupting the natural septal wringing motion.
transverse fibers, which constrict or compress the
chamber. Second, the septum contains helical fibers with
OTHER CONSIDERATIONS
an oblique orientation that cause a twisting movement;
there is no septum transverse component (Figs 55.18A Subendocardial Muscle; Correct
and B). Comparison of RV and LV architecture reveals
Anatomical Location But Architectural
marked differences because the RV has no global helical
configuration, even though its outflow tract free wall and Functional Confusion
contains oblique ascending segment fibers that Torrent- Subendocardial muscle mass surrounds the LV inner
Guasp termed aberrant fibers.11 The septum is a central surface, and drawings14,15,40,51 (Fig. 55.19) left side of the
biventricular helical structure, rather than a LV structure.72 transmural ventricle imply a circumferential line that
The interaction of its free wall’s predominant transverse bisects the LV wall to separate the deeper oblique clockwise
fibers and septum’s oblique fiber orientations determine fibers of the descending segment or right-handed helix
RV function, which was inaccurately called a bellows from the overlying counterclockwise ascending segment
action due to (a) free wall’s horizontal fibers that constrict or left-handed helix that occupies the outer LV shell. In
or compress the RV chamber against the septum73 and contrast, anatomical studies show that different parts of
(b) the incorrect notion that the septum was a LV right- and left-handed helices form the circumferential
structure.72 A twisting action is needed, especially against subendocardial muscle.10,11,31,32 This difference conveys
increased pulmonary vascular resistance (PVR), and that a different functional effect, as previously described
movement is provided by the septum’s helical fibers that during cardiac motions of the normal heart. The dissected
becomes quantified by its shortening and lengthening or unraveled cardiac architecture shown in Figs 55.4
movement. and 55.9 shows that the upper septal oblique orientation
Prior concepts that RV has a bellows-like action differs from reciprocally oblique fibers of the lower
are related to measurement from septum to free wall septum, and that the oblique lateral ventricular wall
dimension by ventriculogram,73 but these 2D recordings subendocardial fiber arrangement mirrors that of the
can only demonstrate narrowing, shortening, lengthening, subepicardium, because there is no overlap of descending
or widening movements. In contrast, 3D measurements and ascending segment helical fibers (Figs 55.9 and 55.10).
A1 A2
A3 A4
Figs 55.18A and B: (A) Model and anatomical preparations

showing the orientation of the ventricular myocardial band of the
(A1 and A2) intact heart and (A3 and A4) after exposing the septum
by unfolding of the right ventricular (RV) free wall. Note the similar
configuration of the septum and left ventricular (LV) free wall com-
posed of the ascending segment of the apical loop; (B) Anatomical
unwrapping of the right segment of the basal loop, which surrounds
the septum composed of the helical fibers of the descending and
B ascending segments of the apical loop.
VVI recordings in Figures 55.9A and 55.12B demonstrate confusing conclusions. Dynamics of the anatomically
dissimilar subendocardial motion during the time frames visible subendocardial muscle are linked to both an
of IVC, twisting for ejection, and untwisting before rapid architectural configuration that evolves from its left- and
filling. Presumptions of subendocardial function that are right-handed cardiac helical form, as well as from how the
only based upon bioengineering models containing a wrapped circular muscles within the upper LV influence its
uniform or homogeneous inner shall wrap40 may provide motions. The global counterclockwise rotation during IVC,
Fig. 55.19: The left drawing shows a bioengineering concept of circumferentially overlapping endocardium and epicardium, whereby the
endocardium reflects the right-handed helical arm and surrounds the left ventricular (LV) inner surface. The right architectural reflection
of the anatomical endocardium shows that it is formed by both the right- and left-handed helical arms, and has fiber pathways that have
both clockwise and counterclockwise directions. This anatomy is described in Figure 55.10.
despite clockwise motion of the endocardium becomes changing the natural 60° angulations of the right- and left-
explained by such insight into powerful circular muscle handed helical components toward a more horizontal82,83
during normal cardiac architecture. configuration will geometrically alter functional twisting
and untwisting. For example, Borg recently examined
Torsion and Untwisting/Preload and changes after increased preload and reduced afterload
in patients with mitral insufficiency (whose regurgitation
Afterload Relationships causes these intrinsic spherical ventricular shape changes)
Changes in torsion and untwisting within normal conical and demonstrated decreased torsion and reduced
hearts has been defined in regard to alterations in preload, untwisting.84 Moreover, initiation of the untwisting
which increases them due to volume dependency, and movement began 23 ms before aortic valve closure. This
raised afterload that increases torsion while reducing reliable echocardiographic finding, thereby poses a series
untwisting.15 Conversely, dilated and failing hearts have of questions about responsible muscular mechanisms that
a more spherical form and exhibit completely different produce such reproducible changes. Untwisting normally
torsion and untwisting responses following similar begins during the postejection isovolumic phase, yet mitral
hemodynamic loading alterations.79 Consequently, LV valve insufficiency continues after aortic valve closure58 to
geometric alterations influence motion observations thereby support the functional role of ongoing ascending
in a manner that is independent of loading conditions, segment contraction during an interval that previously
because cardiac fiber orientation is primarily responsible was called IVR.
for such rotational actions.80,81 van Dalen reinforced this
observation by showing that LV sphericity index is the Mitral Valve Opening Relationships
strongest independent predictor of apical rotation and twist
when comparing normal subjects with an elliptical cardiac
with Untwisting
shape against a cohort with dilated cardiomyopathy.79 Echocardiographic calculations of torsion and untwisting
Figs 55.20A and B shows such architectural changes when are traditionally related to mitral valve opening (MVO)
the conical form becomes spherical, and implies that alterations.15,59,85 This terminology is based upon Doppler
A B
Figs 55.20A and B: Comparison of fiber orientation in the normal heart (above) and the dilated or spherical form (below) where there
is detachment of the circumferential or basal loop with horizontal fibers, and exposure of the normal and spherical configuration of the
right- and left-sided arms of the helical structure. Note that the normal 60° fiber orientation becomes more horizontal in the spherical
configuration and this angulation begins to resemble the more transverse fiber pathways of the circumferential or basal loop.
inflow and outflow recordings, rather than conforming timing provides insight into how measuring torsion’s
to knowledge of the separation of the mitral leaflets, peak, velocity, or rate and duration uncovers their
which provide the only valid confirmation of MVO. Lee interconnection. Torsion duration is an essential feature
in 199058 called this MVO observation “the mitral valve that improves a fuller understanding of untwisting,
artifact that correlates with the E point in the mitral because its knowledge integrates with the vital “timing
echogram, but is unrelated to actual mitral valve opening”. hiatus” that exists between the end of descending
Moreover, concepts of untwisting existing during the “IVR and then subsequent ascending segment shortening.
interval” that are based upon this MVO observation need Untwisting is unchanged if this interval mirrors the
reevaluation, because subepicardial muscle continues to approximately 80-ms interval existing at normal heart
contract and myocardial strain is maintained during this rates. Conversely, untwisting accentuates if this interval
phase.16,59 Untwisting during the postejection isovolumic is extended by positive inotropic drug intervention,59 or
phase is due to uncoiling of the transversely oriented becomes impaired if this interval is shortened by either
circumferential muscle surrounding the cardiac base negative inotropic drug intervention59 or by several other
that both rotates and houses the helix from which the factors described below. Extending torsion by prolonged
papillary muscles arise. Ventricular untwisting caused shortening of the right-handed helical arm or descending
by these horizontal fibers may simultaneously open the segment will impair untwisting because this delay
mitral valve leaflets by changing papillary muscle position, compromises the postejection isovolumic time frame
and thereby may explain Lee’s 1990 findings.58 Moreover, when unwinding should start. Consequently, a unifying
untwisting simply cannot start if there is ongoing torsion, influence of mechanical events during this “hiatus time
as exists in a spectrum of diseases to be subsequently frame” evolves into a torsion/untwisting interdependence
discussed. Mitral valve inflow (MVI) is a more accurate that plays a major role that results in diastolic dysfunction.
term than MVO, because this term dissociates the earlier
existing anatomical observation of leaflet separation from Diastolic Dysfunction
a later flow effect that is only initiated during the rapid
filling phase. The term diastolic dysfunction has been used because
heart failure occurs in patients with normal ejection
fraction, implying that the problem is diastolic in origin.
Clinical Implications However, Tan86 has done an extensive echocardiographic
Recognizing how the interdependence of torsion analysis and emphasized that it is not an isolated diastolic
and untwisting relates to underlying mechanics and disorder; each patient displays combined systolic and
Myocyte Factors
Myocyte causes relate to thickened LV mass due to
increased afterload from aortic stenosis, hypertension,
or hypertrophic cardiomyopathy.14,15 Stuber employed
MRI to demonstrate that aortic stenosis causes increased
peak torsion, longer interval to peak torsion,60 (Fig. 55.21)
and impaired untwisting. Similar alterations happen after
concentric hypertrophy in hypertension90 and hypertro-
phic cardiomyopathy. Treatment options should address
the specific cause of hypertrophy, because alleviating the
cause of LV hypertrophy allows regression of LV mass to
Fig. 55.21: The average relation between apical velocity rotation return twisting and untwisting capacity toward normal
and timing for human left ventricle (LV) in controls, and physi- by aortic valve replacement.91 Similar results likely follow
ological (rowers) and pathological hypertrophy (aortic stenosis). pharmacological management to reduce the increased
Data from magnetic resonance imaging (MRI) tagging show rapid
systemic vascular resistance, or alcohol or surgical removal
changes in early and late systole and early diastole, demonstrat-
ing prolongation of late systolic velocity into the early diastolic of the hypertrophied ventricular segment.92
phase in aortic stenosis. (ES: end-systole).
Calcium-Related Factors
diastolic abnormalities, particularly involving ventricular Sarcolemmal calcium flux efficiency is a central underlying
twist and deformation (strain) patterns leading to reduced event in both ischemia and aging.63,93 Kroeker studied94
ventricular suction, delayed untwisting, and impaired twist dynamics during early ischemia and observed
early diastolic filling.87,88 These observations emphasize that counterclockwise apical rotation was prolonged
the interdependence of twisting and untwisting. Diastolic into the isovolumic phase. A similar event occurs with
dysfunction has clear echocardiography characteristics aging,95 and is also associated with prolonged shortening
relating to changes in the velocity waves during rapid of the descending segment after reperfusion following
filling and atrial contraction. The role of the reported longer ischemic intervals.94 Although prior suggestions
increased untwisting during early diastolic dysfunction for defining the aging mechanism include left atrium
is uncertain because the E-wave is reduced and impaired considerations and LV pressure deceleration changes,95,96
filling occurs.16,89 Diastolic dysfunction’s characteristic neither has addressed the impaired subendocardial
impaired untwisting is associated with either (a) increased muscle function described by Lumens, that will prolong
torsion and preserved ejection fraction or (b) reduced inner shell shortening.96 Management options for
torsion from reduced systolic function. improving torsion and untwisting imbalance from these
The underlying problem is prolonged shortening of causes may relate to enhancing calcium flux by reperfusion
the right-handed helical arm or descending segment after ischemia, or via pharmacological management
that causes extended torsion duration with resultant with aging; the compromised “time hiatus” is improved
compromise of the vital postejection isovolumic by sodium hydrogen exchange inhibitors63 (Figs 55.22A
phase “timing hiatus”. The keynote echocardiographic to D). Moreover, favorably modifying calcium efficiency by
observation is loss of longitudinal strain, a process caused levosimendan similarly reverses diastolic dysfunction.97
by the prolonged descending segment contraction causing
prolonged torsion, so that there is a delay in allowing the
noncontracting descending segment to become a fulcrum
Dilated Cardiomyopathy
for lengthening. Several reasons exist for this descending Geometric reasons for diminished torsion and impaired
segment prolongation, and the resultant treatment untwisting become apparent as the normal conical
options are determined by whether the causative factor ventricular shape becomes spherical in dilated cardio-
relates to (a) regional muscle anatomy, (b) physiological myopathy, as increased sphericity index is a primary
calcium flux, or (c) geometric interruption of normal fiber determinant of abnormal twisting and associated diastolic
orientation by cardiac dilation. dysfunction.79 Geometric changes thereby become the
A B
C D
Figs 55.22A to D: Sonomicrometer crystal tracings of “hiatus between termination of right- and left-handed helix or descending and
ascending segment contraction” during the isovolumic phase in the normal heart. A yellow shade defines this interval, and there is
recording of left ventricular pressure and dP/dt. (A) Normal or control intervals; (B) Bulging of both segments during ischemia or tempo-
rary coronary occlusion, without shortening; (C) 15 minutes after reperfusion shows reduced shortening, prolongation of right-handed
helix, or descending segment contraction that markedly reduces the hiatus between termination of the right- and left-handed helices
or descending and ascending shortening; (D) Diastolic dysfunction is percentage prolongation of “hiatus” between end of shortening
of right-handed helix (descending segment) and left-handed helix or ascending segment. Prolonged right-handed helix or descending
shortening defines this interval. The treated animals received cariporide, a sodium hydrogen ion inhibitor called HOE pretreatment.
Control values are shown below. Values expressed as mean ± SEM. Note return of normal hiatus following this intervention (*P < 0.05
HOE pretreatment vs no treatment).
unifying theme of torsion and untwisting dysfunction relationship. Consequently, returning the helical form
in dilated cardiomyopathy from ischemic, valvular, with a spectrum of restoration procedures may reverse
and nonischemic origin.98 Sallin33 showed that ejection the adverse torsion and untwisting interdependence in
fraction diminishes as the oblique helical configuration dilated hearts.82
develops a more horizontal fiber orientation, and dilation
simultaneously stretches the fibers to also impair their
Pacing Factors
electrophysiological function.99 Moreover, diminished
untwisting is a hallmark sign of dilated cardiomyopathy, Cardiac motion studies show that normal cardiac
so that this event stems from prolonged torsion to thereby movement is sequential due to spread of the electrical
solidify the interdependence of the torsion and untwisting impulse from its earliest action within the conduction
system toward its transition across the matrix and A close relationship exists between the septum and
into the muscle fibers.100 Heart motion after a single tricuspid valve function, because its base anchors the
transmural electrical excitation is synchronous, so that it part of the A to V valve annulus and RV septum papillary
is not surprising why Wiggers in 1925 demonstrated that muscles arise from its body. Tricuspid valve regurgitation
ventricular pacing disturbed cardiac muscle movement (TR) develops when ventricular dilation stretches the
adversely.101 Recent studies of torsion and untwisting septum, a change that is caused by tethering of valve
during isolated ventricular pacing102,103 confirm how leaflets; this mirrors the reason for MR development
a single pacing stimulus interferes with these natural occurring in patients with a wide QRS interval (Figs 55.23A
patterns. Biventricular pacing or cardiac resynchronization and B). Moreover, studies from our laboratory show that
therapy (CRT) also causes an inconsistent torsion and acute pulmonary hypertension causes septum bowing
untwisting improvement, because this fixed dual stimulus and resultant TR, both of which become reversed by
differs from the natural spread of impulses via the His supplementing phenylephrine with intra-aortic balloon
Purkinje system that produces sequential motion. A recent pumping. This treatment restores the midline RV septum
study in CRT responders demonstrated the apex and base position, while simultaneously avoiding LV vasoconstrictor
moving in the same direction or synchrony,52 instead drugs induced after loading.106
of twisting, thereby demonstrating their production of RV failure treatment protocols are linked to under-
transmural stimulation rather than sequential activation standing the functional HVMB causes of performance
along the His Purkinje system. Sonomicrometer crystal impairment. Postoperative paradoxical septum motion is
studies document loss of the sequential motion following totally avoided by use of the integrated blood cardioplegia
atrium and then biventricular stimulation.104 Conversely, during open heart surgery.68 Abolition of postoperative
there is restoration of the natural twisting and untwisting, arrhythmias and right heart failure was achieved by
as sequential motion returns following atrial and then a “valve ventricular approach” that reconstructed the
high septal pacing;104 this pattern reflects the natural His stretched septum and replaced the pulmonary valve in
the dilated failing hearts.71 RV dysplasia was successfully
bundle pacing. Torsion and untwisting thereby require a
treated by normalizing the size of the aneurysmal free
coordinated spread of impulses to the circular and helical
wall,107 as the septum is not diseased in 80% of these
pathways to ensure the natural excitation contraction
patients.108 RV failure is reversed in left ventricular assist
coordination that does not interfere with the postejection
device (LVAD) patients by reducing LV suction to return the
isovolumetric interval “time hiatus”.
bowed septum to the midline position.109 Realization that
septal twisting is further impaired by the high pulmonary
Right Heart Failure pressures in RV failure patients has resulted in avoidance
of vasoconstrictor drugs (epinephrine, dopamine), and
The septum is the “lion of RV function,”105 because the RV
selection of the amrinone or milrinone agents, which
must rely upon the requisite twisting of its helical fibers to
combine vasodilator and inotropic actions.
maintain RV cardiac output against increased PVR.105 In
The theme of these clinical implications is that
contrast, paradoxical septum motion follows its stunning
unbalanced torsion and untwisting have a common
during cardiac surgery70 or from its stretch following
premise related to the impaired “timing hiatus” or longer
volume overloading. The consequent bulging septal
torsion duration during the postejection isovolumic
geometric change causes its oblique fibers to become
phase. Decisions about management become linked to
more transverse to decrease its twisting capacity; this focusing upon how this common abnormality is altered
infrastructure progresses to RV failure during pulmonary within different muscular, physiological, structural, and
hypertension. In contrast, the power of the RV free wall’s electrical disease processes. Treatment options then
compressive capacity is apparent when PVR is low; RV become geared toward efforts to reverse the initiating
failure did not occur in approximately 50% of the 3,292 event. Readily available torsion and untwisting monitors
consecutive surgical patients who developed paradoxical may be employed to gauge their effectiveness.
septal motion following conventional methods of myo-
cardial protection.70 Most importantly, the septum and
LV free wall are made of the same helical muscle, so that
CONCLUSION
LV diastolic dysfunction should occur whenever the Ventricular torsion is due to the twisting of the ventricle
septum is globally stunned. during systole, and its subsequent untwisting is the prelude
Figs 55.23A and B: (A) Left intraventricular view of the septum.

Note that the posterior medial papillary muscle arises from the left
ventricular (LV) wall immediately adjacent to the septum. Para-
doxical or bowing septal motion causes it to bulge into the right
ventricle, so that the adjacent posterior papillary motion moves in
that direction, and results in tethering the mitral valve leaflets to
cause mitral regurgitation from this geometric reason; (B) Right
intraventricular appearance of the septum. Note attachments of the
posterior papillary muscles to the septum wall as well as tricuspid
valve leaflets. Observe that leftward bowing of septum will produce
traction upon the septum cusp leaflets and alter coaptation and
B cause valve incompetence.
to subsequent diastolic filling. These interdependent untwisting is related to preserving the 80-ms “timing
rotational events arise from the mechanical actions and hiatus” between the end of shortening of the descending
timing relationships of the heart’s underlying circular and and then the ascending arms of the helical muscle.
helical muscle pathways. Explanation of the presystolic Central to understanding torsion and untwisting
IVC period is essential for analysis of these interactions. interdependence is knowledge of the mechanics of normal
Circular fibers dominate to cause pre- and post-twisting cardiac motion during the timing of the coiling and
net or global counterclockwise and clockwise movement, recoiling actions of circular and helical fiber pathways.
whereas the helical fibers govern torsion. Normal Longer torsion duration results from prolonged right-
handed helical arm or descending segment contraction 16. Buckberg G, Hoffman JI, Mahajan A, et al. Cardiac
that compromises this “timing hiatus” and thereby mechanics revisited: the relationship of cardiac architecture
to ventricular function. Circulation. 2008; 118(24):2571–87.
interferes with untwisting. Clinical implications result from
17. Buckberg G, Hoffman JI, Nanda NC, et al. Ventricular
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duration becomes their common theme. Management timing interdependence: a viewpoint. Echocardiography.
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CHAPTER 56
Echocardiography in Assessment of
Complications Related to Permanent
Pacemakers and Intracardiac
Defibrillators
Ahmed Almomani, Khadija Siddiqui, Masood Ahmad
Snapshot
Normal Echocardiographic Findings in Permanent
¾¾ Masses: Lead Infection and Thrombus
¾¾
Pacemakers/Implantable Cardioverter-Defibrillators Myocardial Perforation
¾¾
Pacemaker and Implantable Cardioverter-Defibrillator-
¾¾ Deleterious Effects of Right Ventricular Apical Pacing on
¾¾
Related Complications Left Ventricular Function
¾¾Tricuspid Regurgitation
INTRODUCTION Endocardial lead-related complications have not been

very well recognized. In recent years, there has been an
Over the past decades, technical advances in permanent increasing awareness of device-related complications such
pacemakers (PPMs) and implantable cardioverter-defibri as tricuspid regurgitation (TR), lead infection, thrombosis,
llators (ICDs) have led to a tremendous increase in the perforation, and left ventricular dyssynchrony. We will
use of these medically important devices. This trend is review the roles of transthoracic echocardiography (TTE)
likely to continue due to the increased life expectancy of and transesophageal echocardiography (TEE) in the
the population and the increasing number of indications assessment of pacemaker/ICD endocardial lead-related
for their use including placement of PPMs for cardiac complications.
resynchronization therapy (CRT) and ICDs for primary and
secondary prevention of complications from arrhythmias
in patients with left ventricular dysfunction.1–3 The 11th
NORMAL ECHOCARDIOGRAPHIC
World Survey of Cardiac Pacing and ICDs reported that FINDINGS IN PERMANENT
737,840 new devices were implanted in 2009 worldwide, PACEMAKERS/IMPLANTABLE
with the largest number of new implants, 225,567, in the
United States. These numbers showed a huge increase
CARDIOVERTER-DEFIBRILLATORS
compared to a similar survey done in 2005, and the Transthoracic echocardiography can be used to visualize
numbers are expected to be much higher in 2013.4 the intracardiac portion of the PPM or ICD lead. Device
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators 1211
A B
C D
Figs 56.1A to E: Two-dimensional transthoracic echocardiog-

raphy (2D TTE) with pacer lead shown in modified parasternal
long-axis (A), short-axis (B), apical four-chamber (C), focused
right-sided chambers (D), and subcostal (E) views. Arrowheads
point to the lead, arrow in Figure A points to the tricuspid valve.
(AO: Aortic valve; LA: Left atrium; LV: Left ventricle; RA: Right
E atrium; RV: Right ventricle).
leads may be imaged in the right atrium or right ventricle demonstrated due to poor acoustic windows resulting
(RV) in a number of views, including RV inflow, parasternal in limited visualization, and due to artifacts related
short-axis at the level of the aortic valve, apical four- to lead reverberations. Real time transthoracic three-
chamber, or subcostal view (Figs 56.1A to E). However, in dimensional echocardiography (RT3DE) can overcome
some patients, the presence of lead cannot be satisfactorily some of these limitations by visualizing the entire route
A B
C D
Figs 56.2A to D: Real time transthoracic three-dimensional echocardiography (RT3DE) in apical four-chamber view demonstrating the
pacer route and its relation to intracardiac structures in simultaneously obtained multiple views derived from the same data set. Arrow-
heads point to the lead. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: right ventricle).
of the lead in multiple views from the same data set TRICUSPID REGURGITATION
(Figs 56.2A to D, and Movie clips 56.1 and 56.2).
In addition, in some patients with RV pacing, there may The development of significant TR and its progression
be paradoxical septal motion due to early activation of the over time is an important device-related complication.
RV. This, however, is not a finding isolated to the presence Prospective data on TR secondary to PPM and ICD
of cardiac devices but can also be seen in the presence of leads are lacking. Multiple small retrospective studies
other conduction abnormalities, cardiac surgery, or RV and small case series have reported this complication
volume/pressure overload. (Table 56.1). TR in patients with PPMs or ICDs may not
be exclusively caused by the endocardial lead, as pre-
PACEMAKER AND IMPLANTABLE existing abnormalities such as tricuspid valve (TV) annular
dilatation or pulmonary hypertension may be present.5
CARDIOVERTER-DEFIBRILLATOR-
A number of different mechanisms of RV intracardiac
RELATED COMPLICATIONS lead-related TR have been described. In one retrospective
Transthoracic echocardiogrphy and Transesophageal study, it was noted that in 41 patients with PPM or ICD
echocardiography are useful in evaluating pacemaker- lead-induced severe TR requiring surgery, 7 patients had
related complications including TR, valvular or lead perforation of the TV leaflet by the PPM or ICD lead, 4 had
vegetations, pericardial effusion, abnormal lead position, lead entanglement in the TV, 16 had lead impingement
perforation, and lead thrombus. of the TV leaflets, and 14 had lead adherence to the TV.6
Table 56.1: Summary of the Studies on Pacemaker-Related Tricuspid Regurgitation

Study Sample Size Study Design Follow-up TR Before Lead TR After TR Severity
Implantation Implantation
Sakai et al. (1987)58 18 Prospective NA NA 5 TR severity not assessed
Paniagua et al. (1998) 59
374 cases Case control NA NA 27 Moderate to severe TR
683 controls NA NA 12 (P < 0.0001)
Leibowitz et al. (2000) 60
35 Prospective 1.2 ± 0.7 days 10 7 Moderate to severe TR
(P = Not significant)
Lin et al. (2005)6 41 Retrospective NA NA Out of 1,465 patients
with severe TR that
required surgery,
41 were secondary to
endocardial leads
Kucukarslan et al. (2006)61 61 Prospective 6 ± 3 months 9 12 Moderate to severe TR
(P = Not significant)
Seo et al. (2008)7 87 Retrospective 36 months NA 32 Moderate to severe
TR3D echocardiography
was used
Kim et al. (2008)62 248 Retrospective 93 days 69 NA TTE before and after
(range, pacer showed signifi-
23–199) cant increases in TR,
moderate to severe in
24.2% and severe in 4%
Klutstein et al. (2009)63 410 Retrospective 75 days NA NA TTE before and after
(range, pacer showed that 75
1–4,367 d) patients (18.3%) had
significant worsening
of TR
Vaturi et al. (2010)64 23 Prospective 48.6 ± 32.7 0 9 Moderate to severe TR.
months Number increased to
18 when mode changed
to active RV pacing
(P < 0.001)
Alizadeh et al. (2011)65 115 Prospective 4.1 ± 0.8 10 36 Moderate to severe TR
years (P < 0.001)
(RV: Right ventricle; TR: Tricuspid regurgitation; TTE: Transthoracic echocardiography).

Source: Modified with permission from Al-Mohaissen MA, Chan KL. Prevalence and mechanism of tricuspid regurgitation following
implantation of endocardial leads for pacemaker or cardioverter-defibrillator. J Am Soc Echocardiogr. 2012;25(3):245–52.
In the same study, severe lead-induced TR causing right- over weeks to months leads to fibrous tissue formation,
heart failure that required TV surgery accounted for 2.8% which encapsulates the pacemaker lead and may result
of all TV operations (41 of 1,465 consecutive patients) in fusion and adherence of the endocardial lead to the TV
between 1993 and 2003 at the Mayo Clinic. leaflets, chordae, and papillary muscles, resulting in TR.8
The time course for TR development and progression Transthoracic echocardiography is often used to
after endocardial lead placement in the RV is not well assess TR after implantation of PPMs (Fig. 56.3 and
defined. A large retrospective study reported an increase Movie clip 56.3). However, the ability to define the
in TR acutely after RV lead implantation, and progressive precise anatomical relationship between the TV and
worsening with time, while other studies have mainly the pacemaker lead is quite limited using this modality.
focused on the chronic effects of the leads on the In a study done at Mayo Clinic, the valve malfunction
valve.7 Pathological studies have demonstrated major due to PPM or ICD lead was diagnosed preoperatively
inflammatory changes occurring within the heart only days in only 5 of 41 patients by TTE.6 On the other hand,
after lead implantation. The progression of inflammation RT3DE enables visualization of the entire TV, in
also been a notable increase in the detection of masses

on these leads. Of the available imaging modalities,
magnetic resonance imaging still continues to be relati
vely contraindicated in a majority of pacemakers and
defibrillators, therefore limiting evaluation of these devices
by either computed tomography (CT) or echocardiography.
However, since the metal in the device and the movement
of the leads create artifacts on CT, the most desirable
imaging modality remains an echocardiogram. A mass
detected on an implanted lead on an echocardiogram,
almost invariably represents either a thrombus or vege
tation, and distinguishing between the two can often be
quite difficult. Since ultrasound imaging alone cannot
determine the etiology of the mass, clinical presentation
Fig. 56.3: Two-dimensional transthoracic echocardiography (2D
and lab data play a crucial role in the interpretation and
TTE) with focused view of right-sided chambers, demonstrating
the tricuspid regurgitation (TR) jet (arrow) between the right ven- management of these abnormal findings.
tricle (RV) lead (arrow head) and the septal leaflet of the tricuspid
valve (TV).
Infection
Initial cases of pacemaker endocarditis were described
particular, en-face short-axis views of the valve, which
in the early 1970s.10 Reported device-related infective
may facilitate the enhanced ability of this technique in
endocarditis (IE) has ranged from 10% to 23% in the
assessing the route and position of the lead at the TV
literature.11,12 The incidence of infection following implan
and in visualizing the actual movement of the leaflets
tation of PPMs has been variably assessed, ranging from
(Figs 56.4A and B, and Movie clips 56.4 and 56.5). A
0.13% to 19.9%,13,14 with the majority of infections repo
high efficacy (96%) of 3DE in complete assessment of
rted to be in the pacemaker generator pocket. Incidence
the TV structure has also been reported.9 Another study
of infection in ICDs has been reported in the literature
reported that among the 87 patients involved in the study,
ranging from 0.7% to 1.2%.15,16 Technical advances have
leads passing through the TV were identified in only 15
allowed transvenous implantation of ICDs, thereby
(17.2%) patients by 2D. In contrast, on 3DE examinations,
eliminating the need for thoracotomy and epicardial lead
lead routes were identified in 82 (94.2%) patients. In the
placement. These advances have contributed to the overall
remaining five patients, appropriate 3DE images for lead
route analysis could not be obtained because of artifacts decline in infection associated with ICD.
caused by the lead.7 In patients with device-related endocarditis, the
Tricuspid regurgitation is a preventable complication of presence of vegetation is limited not only to the TV but
PPM and ICD leads, and is related to the lead position, lead can be found anywhere along the course of the lead,
route, and interaction with the valve leaflets. Prospective including the endocardium of the right atrium or RV.17
studies are needed to establish the temporal relationship Echocardiography plays an important role by allowing
between placement of the device and development/severity direct visualization and measurement of the mass along
of TR. Real time 3D echocardiography appears superior with the ability to assess for cardiac involvement. On
to two-dimensional transthoracic echocardiography (2D echocardiography, vegetations have been defined as
TTE) in assessing the TV in patients with PPMs and ICDs oscillating intracardiac masses on the device leads, valve
(Figs 56.4A and B). leaflets, or endocardial surface, confirmed by imaging in
more than one echocardiographic plane. In these cases,
MASSES: LEAD INFECTION AND the valve or lead infection was confirmed by positive
blood or lead tip culture.12,18 Valvular vegetations have
THROMBUS been further characterized on the basis of four physical
As there has been an increase in the number of implanted properties, as assessed by echocardiography: vegetation
cardiac devices over the past couple of decades, there has size, mobility, extent, and consistency.18
A B
Figs 56.4A and B: Real time transthoracic three-dimensional echocardiography (RT3DE) with pacer and en-face view of the tricuspid
valve from the right atrium (A). Pacer and the tricuspid valve from the right ventricle (B), the lead position (arrowhead) in relationship
to the tricuspid valve (arrow).
Although TTE has an improved sensitivity later in the cases of large right atrial thrombi that were found in a
course of the disease, even then the overall sensitivity is series of 53 necropsies performed in patients with PPM.
quite poor.19 This is in part due to the difficulty in precisely Both patients had evidence of hemodynamic impairment
distinguishing between abnormal masses, the TV, and the with signs of congestive heart failure. This series suggested
lead itself—mainly as a result of limited visualization, poor that a right atrial thrombus should be considered in the
echogenicity in some patients, and artifacts due to lead setting of refractory heart failure, despite a normally
reverberations in others. In contrast, use of a multiplane functioning pacemaker and adequate medical treatment.
echocardiographic probe with TEE improves the quality Other serious complications such as superior vena cava
of exploration and has a much more established role as syndrome have also been reported but are beyond the
a diagnostic technique. The ability to visualize the entire scope of this review.26
intracardiac route of the leads, from the upper vena cava to As stated, the diagnosis on TTE may be technically
the RV apex, provides TEE with its much higher sensitivity challenging in patients with limited acoustic windows and
and specificity. In three of the larger studies that used lead reverberations. On the other hand, TEE allows direct
strict criteria for entry and compared results with surgical visualization of the lead, the entire right atrium, interatrial
and microbiological endpoints, the sensitivity of TTE was septum, superior vena cava, and inferior vena cava
22–30%, while that of TEE was 92–96%.19–21 In addition, (Fig. 56.5 and Movie clip 56.6). TEE has also been sugg
TEE also has a role in defining the most appropriate ested to provide information that can possibly determine
extraction technique by identifying patients with whether the thrombus is recent or long-standing.27 Long-
myocardial abscess or extremely large (> 5 cm) lead standing thrombi tend to be sessile and sometimes contain
vegetations that may necessitate surgery rather than a calcium. In contrast, fresh thrombi are highly mobile and
percutaneous method of extraction.12 appear less echo-dense. The use of 3D with TTE or TEE has
an incremental value in evaluating masses attached to the
Thrombus leads (Fig. 56.6 and Movie clip 56.7). As discussed above,
differences in clinical presentation and laboratory findings
Venous thrombosis and stenosis have been described play a significant role in distinguishing lead infection from
as the most common complications associated with thrombus.
transvenous pacemaker implantation with incidence
ranging between 30% and 45%.22 Right atrial thrombus
MYOCARDIAL PERFORATION
is a rarely reported event, and can present either as an
incidental finding on echocardiogram,23 or as symptoms Myocardial perforation is a relatively rare complication
of right-sided heart failure,24 obstruction, or embolization of endocardial leads of cardiac implantable devices. The
of the pulmonary artery.25 One study described only two incidence of this complication is estimated to be < 1%.
Fig. 56.5: Two-dimensional transesophageal echocardiogra- Fig. 56.6: Real time transthoracic three-dimensional echocardi-
phy (2D TEE) showing the pacer lead (arrowhead) and attached ography (RT3DE) of the pacer lead shown in Figure 56.5; right
mobile thrombus (arrow). ventricle (RV) lead (arrowhead) with a thrombus (arrow).
perforation defined as the perforation of the lead through

the myocardium more than 1 month after implantation.28–35
In a review of 51 reported cases of delayed lead
perforation, the demographics and characteristics of this
group showed that elderly women and patients with lower
body mass are more vulnerable to this complication.36
Table 56.2 summarizes 35 cases reported in the literature.
Unlike acute lead perforation, one of the distinguishing
features of delayed lead perforation is the low risk of
cardiac tamponade and death.37–39 Lead perforation can
sometimes be identified by chest X-ray showing the lead’s
migration outside the heart.36,40 Chest CT plays a crucial
role in the diagnosis of lead perforation when other
modalities are nondiagnostic.40,41 In one report, 15 out of
Fig. 56.7: Echocardiography (four-chamber apical view): implant-
100 asymptomatic patients with pacemakers or ICDs were
able cardioverter-defibrillator (ICD) lead perforation across the
right ventricular apex. (LV: Left ventricle; PE: Pericardial effusion; found to have late lead perforation on chest CTs performed
RV: Right ventricle). for other reasons.40 However, CT images may be limited by
Source: Reproduced with permission from Sassone B, Gabrieli L, artifacts created by the leads.
Boggian G, Pilato E. Management of traumatic implantable car-
dioverter defibrillator lead perforation of the right ventricle after car
Two-dimensional echocardiography is a valuable tool
accident: a case report. Europace. 2009;11(7):961–2. for the diagnosis of lead perforation and dislodgement
(Fig. 56.7).42 This modality can also demonstrate pericardial
In some studies the lead perforation rates range from effusion and tamponade if present. Difficulty or failure of
0.1% to 0.8% for PPMs and 0.6% to 5.2% for ICD leads. 2D TTE to visualize leads is not uncommon. Transthoracic
Lead perforation rates may depend on the lead model.28 RT3DE is superior to 2D TTE in detecting lead perforation
Furthermore, lead perforation can be categorized into two and its route (Fig. 56.8). RT3DE is complementary to
groups: acute perforation after lead placement, which is 2D TTE in clinical practice and should be used if a lead
mostly related to the procedure, and subacute or delayed complication is suspected.43,44
Table 56.2: Summary of the Reported Cases of Lead Perforation

Characteristics Total Number = 35
Mean Age 64.0 ± 20.2 years
Gender:
• Male • 16 (45.7%)
• Female • 19 (54.3%)
Mean time from implant 35.9 ± 48.8 weeks
Type of device:
• Pacemaker • 19 (54.3%)
• ICD • 15 (42.8%)
• NA • 1 (2.9%)
Type of lead fixation:
• Active • 24 (68.6%)
• Passive • 6 (17.1%)
• NA • 5 (14.3%)
Evidence of Perforation on echocardiography:
• Yes • 24 (68.6%)
• No • 4 (11.4%)
• NA • 7 (20.0%)
(ICD: Implantable cardioverter-defibrillators; NA: Not available).
Table includes the analysis of 35 cases reported in the listed references.29,31,32,34-37,41,44,66-84
DELETERIOUS EFFECTS OF RIGHT

VENTRICULAR APICAL PACING ON
LEFT VENTRICULAR FUNCTION
Left ventricular mechanical and electrical dyssynchrony
are poor prognostic factors in patients with systolic
heart failure.45,46 Abnormalities in the timing of regional
mechanical left ventricular activation, known as
intraventricular dyssynchrony, appears to be the principal
factor associated with contractile impairment that is
improved by CRT. The classic type of dyssynchrony
resulting from abnormal electrical activation is seen with
left bundle branch block (LBBB), where there is early
activation of the interventricular septum and late activation
Fig. 56.8: Three-dimensional echocardiogram of an apical four- of the posterior and lateral left ventricular walls.47 The early
chamber view, demonstrating the pacemaker lead tip (arrow) septal contraction occurs before normal ejection when
going through the interventricular septum (arrowhead); (LV: Left pressure in the left ventricle is low, thereby generating
ventricle; RV: Right ventricle).
asynchronous stress and strain in the left ventricle, with
Source: Reproduced with permission from Daher IN, Saeed M,
Schwarz ER, Agoston I, Rahman MA, Ahmad M. Live three- one wall exerting forces on the contralateral wall.48
dimensional echocardiography in diagnosis of interventricu- Right ventricular apical pacing is frequently used
lar septal perforation by pacemaker lead. Echocardiography. with implantable pacemakers, and it has been shown
2006;23(5):428–9. to be a well-established risk factor for left ventricular
A B
Figs 56.9A and B: Three dimensional (3D) segmental time–velocity curves of left ventricle (LV) before pacing, left ventricular ejec-
tion fraction (LVEF) 48.5%, SDI 2.7% (A) and after right ventricle (RV) pacing, ejection fraction (EF) 45.7%, SDI 10.9% (B), showing
increased LV dyssynchrony and decrease in LVEF after pacing.
dyssynchrony leading to systolic dysfunction.49 Dyssyn Figures 56.9A and B.53–57 Patients with RV pacing can be
chrony with RV pacing has a similar mechanism to LBBB. evaluated for left ventricular dyssynchrony and followed
In the long run, RV pacing and dyssynchrony may trigger over time to detect pacing-related left ventricular systolic
ventricular remodeling by causing both systolic and dysfunction.
diastolic left ventricular dysfunction, increases in end-
systolic volume and wall stress, leading to asymmetrical CONCLUSION
hypertrophy and abnormal histopathology. Clinically,
these changes manifest as worsening of heart failure.48,50,51 The increasing indications and uses for implantable
Furthermore, one study demonstrated that RV apical pacing cardiac devices have led to a continuous increase in the
number of implanted devices each year. Implantation
can cause regional myocardial perfusion and wall motion
of endocardial leads for these devices can cause many
abnormalities near the sites of electrical stimulation,
delayed complications. Some of the complications are
which increase with the duration of pacing. These changes
mechanical and related to the presence of foreign body
are associated with impairment of left ventricular diastolic
and its interaction with the valves and endomyocardium,
function and progressive deterioration of regional left
for example, perforation, infection, and thrombosis, while
ventricular ejection fraction over time in regions remote
others are related to the electrical pacing of the myocardium
from the site of electrical stimulation, resulting in a and conduction abnormalities, for example, dyssynchrony
significant reduction in global left ventricular function.52 and TR. It is important to have a high index of suspicion
Left ventricular dyssynchrony can be assessed by to diagnose these complications, using the appropriate
tissue Doppler imaging and more recently by speckle imaging modality. Based on the preceding review, it is
tracking echocardiography. Measurements of mechanical clear that echocardiography plays an important role in the
dyssynchrony index guide CRT in patients with heart diagnosis of the device-related complications. Both 2D TTE
failure and left ventricular dysfunction.53 Real time 3D and TEE provide extremely useful diagnostic information
echocardiography provides a unique and powerful tool for that is helpful in detecting the endocardial lead-related
the evaluation of left ventricular dyssynchrony by allowing complications. Real time 3D echocardiography is a novel
comparison of the time–velocity curves of the various left technique that can precisely track the intracardiac route of
ventricular segments in the same cardiac cycle. The impact the lead and accurately detect most of the pacemaker/ICD
of RV pacing on left ventricular dyssynchrony is shown in lead-related complications.
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CHAPTER 57
Echocardiographic Evaluation of
Ventricular Assist Devices
Peter S Rahko
Snapshot

Clinical Uses of Ventricular Assist Devices 
ComplicaƟons of LeŌ Ventricular Assist Devices

Reverse Remodeling 
Evidence of Underfilling of the LeŌ Ventricle

Types of Devices 
OpƟmizing LeŌ Ventricular Assist Device Seƫngs

PreoperaƟve Echocardiographic EvaluaƟon 
ExplantaƟon

Immediate Postsurgical EvaluaƟon 
Percutaneous ConƟnuous Flow Devices

Serial Changes in Cardiac Structure and FuncƟon
INTRODUCTION required heart valves at the inflow and outflow cannulas,

and thus had many possibilities for failure. Furthermore,
Mechanical circulatory support began in the 1960s when the device was never conceived of as anything but a bridge
Michael DeBakey and his colleagues first began concei- to transplantation; thus, very long-term utilization of the
ving of a left ventricular assist device (LVAD). Early devices device was not feasible. Finally, the device was relatively
were pneumatic with an external drive system, and large and could only be implanted in full-sized adults.
the first device was implanted in 1963 in a patient who To address the shortcomings of pulsatile devices, the
suffered a cardiac arrest. By 1966, a second pneumatic, concept of a continuous flow assist device was developed.
pulsatile LVAD was developed and utilized in a patient.1,2 These rotary pumps use the principle of the Archimedes
Considerable development in pulsatile LVADs, namely, screw. Pump rotation creates force on blood and propels it
devices that had a chamber that filled and then ejected forward longitudinally through the device. The impeller of
using a pusher plate design, expanded throughout this device must rotate at a relatively faster speed (8,000–
the 1970s and into the 1980s. The first Food and Drug 12,000 rpm) to create a moving force on the blood. The
Administration (FDA)-approved intracorporeal LVAD was advantage of this design is a marked reduction in overall
the Thoratec HeartMate XVE. Initially, it was pneumatically size. Indeed, it was possible to miniaturize these devices
powered but later became electrically powered. This was to the point that there was a marked improvement in
the first realistic device that gave patients independence tolerability and also an expanded range of body size that
since everything was internal except a driveline that came could benefit from this device. The first clinical use of a
out from a skin tunnel, and attached to a controller and device of this type occurred in 1998 with the introduction
battery pack. For the first time, patients could be relatively of the MicroMed DeBakey VAD. The first approved
mobile and realistically go home and even go back to work device by the FDA was the HeartMate II built by Thoratec
in some situations. The superiority of device therapy over Corporation (Figs 57.1A to D).4
medical therapy was established in the REMATCH trial for The third generation of assist devices is centrifugal
end-stage heart failure patients.3 However, the pulsatile (Figs 57.2A to D). These devices propel blood like a
chamber–pusher plate design had many moving parts, spinning top inside of a chamber. Blood enters the device
Chapter 57: Echocardiographic Evaluation of Ventricular Assist Devices 1223
A B
C D
Figs 57.1A to D: (A) The Thoratec HeartMate II draws blood from the left ventricle (LV) apex through the inflow cannula into the pump
and then ejects blood through the outflow cannula to the central aorta; (B) Closer view showing the impellar design. (Used with permis-
sion of Thoratec); (C) Chest X-ray of patient with a HeartMate II showing inflow cannula position and relative position of the left ventricu-
lar assist device (LVAD) in the chest; (D) Three-dimensional reconstruction of a chest CT scan of same patient as (C). Note position of
cannula and in this case the tortuous course of the outflow cannula.
A B
Figs 57.2A and B
C D
Figs 57.2A to D: Example of a centrifugal pump, the HeartWare device. (A) Relative size is small; (B) The device open shows the
rotating disk that spins blood outward from the center; (C) Diagram showing relative location of device at the left ventricle (LV) apex and
the outflow cannula to the central aorta; (D) View showing relative location in the chest of the components of the device.
Source: Used with permission from HeartWare International.
conceived as bridge to recovery devices to allow tempo-

rary support of patients expected to recover from a
temporary but severe clinical situation. Devices designed
for this purpose are in clinical use today and typically are
percutaneously deployed rapidly in urgent situations.
These devices provide a greater level of assist and output
response than an intra-aortic balloon pump. Devices may
be small, impeller-type systems such as the Impella series
of recovery devices (Abiomed) designed for very short-
term use, or the TandemHeart (Cardiac Assist), which is
an extracorporeal centrifugal flow pump. This device may
also be used for relatively brief periods of time (Figs 57.3
and 57.4).
Fig. 57.3: The Abiomed Impella is moved retrograde from the Long-term assist devices are now predominantly axial
femoral artery to the central aorta and across to the left ventricle. flow devices or centrifugal pumps. These devices may be
Blood is pulled from the left ventricle (LV) cavity by the Impella and directly implanted in individuals who are severely ill or in
ejected into the central aorta.
Source: Used with permission from Abiomed.
individuals who are chronically severely debilitated and
require emergent, urgent, or elective placement of the
at the center, where the spinning generates a vortex of device. The most detailed data on the use of these devices
low pressure, with progressively higher pressures as one comes from the Interagency Registry for Mechanically
reaches the outside of the spinning disk. These devices Assisted Circulatory Support (INTERMACS).5 INTERMACS
typically rotate between 2,000 and 3,000 rpm. The first has defined seven different profiles of patients based upon
clinical application of a third generation device occurred clinical severity and is tracking these patients over time
in 2005 with implantation of the VentrAssist.4 to determine outcomes. Ongoing evaluation of patients
having these devices placed will help determine best
CLINICAL USES OF VENTRICULAR practices. The basic INTERMACS profiles are shown in
Table 57.1.
ASSIST DEVICES As assist devices have evolved, so have indications.
Ventricular assist devices have multiple uses for mul- Bridge to recovery is predominantly confined to acute
tiple clinical circumstances. The original devices were devices, with the larger devices showing only very
A B
Figs 57.4A and B: (A) The TandemHeart device. An inflow catheter is inserted in a femoral vein and moved up to the
right atrium and then across to the left atrium. Blood is removed from the left atrium outside to the pump and then via
the outflow cannula ejected into the central aorta; (B) Closer look at ideal position of the inflow cannula. Note there are
14 side holes and 1 end-hole.
Source: Used with permission from Cardiac Assist, Inc.
Table 57.1: Classification of Levels of Severity of Patients who are Potential Candidates for a Ventricular Assist Device: The
Interagency Registry for Mechanical Assisted Circulatory Support (INTERMACS)
Profile Percentage of Implants* Profile Name Profile Description
1 16 Critical Cardiogenic Shock Life threatening despite inotropic support, hypotensive, and
hypoperfused
2 38 Progressive Decline Declining systemic function, nutrition, and renal function despite
inotropic support
3 28 Staple Inotropic Dependent Achieved stable blood pressure, organ perfusion, and nutri-
tion but unable to be weaned from inotropic or other temporary
mechanical support
4 12 Resting Symptoms Stable with normal volume status on oral medications. Daily
symptoms of limiting congestion at rest or with minimal activity
5 3 Exercise Intolerant No symptoms with rest or minimal activity. All other activity
causes congestion. Frequent episodes of volume overload
6 2 Exertion Limited No resting symptoms. No fluid overload. Minimal activity can be
performed but fatigues in a few minutes
*Percentage of implants for the year 2011.
Source: Adapted from INTERMACS, accessed at www.intermacs.org January 27, 2013.
Table 57.2: Frequency of Ventricular Assist Device Implantation by Device Strategy: The INTERMACS Registry
Device Use Strategy Implant Year
2007 2009 2011
Bridge to Transplant 147 (42%) 505 (49%) 424 (22%)
Bridge to Candidacy 134 (38%) 432 (42%) 695 (37%)
Destination Therapy 48 (14%) 59 (6%) 742 (39%)
Bridge to Recovery 14 (4%) 13 (1%) 17 (1%)
Other 8 (2%) 11 (1%) 20 (1%)
Total Implants 351 1,020 1,898
Source: Adapted from INTERMACS, accessed at www.intermacs.org January 27, 2013.
modest use for this indication. With greater longevity REVERSE REMODELING
of use, indications have expanded into several other
large categories. Bridge to transplant is for patients who Placement of a LVAD generally causes significant reverse
are expected not to survive until a transplant becomes remodeling. While it was hoped initially that substantial
available. As the demand for transplants has increased reverse remodeling would include both volumetric
but the availability has remained static or actually slightly reduction and functional improvement, to the point that the
decreased, the waiting time for a heart transplant continues LVAD might be removed, later studies in wide varieties of
to prolong. Thus, bridge to transplant now requires long- individuals have shown that the ability to explant an LVAD
term support of these newer devices. The success of bridge is relatively uncommon.6 In an analysis of neurohormonal
to transplantation has led to considerations for use of blood levels in patients with LVADs, some studies have
these devices as an ultimate end in itself. This is called shown a decline in levels of endothelin-1 and B-type
destination therapy. Patients in this category are not natriuretic peptide associated with some improvement
candidates for cardiac transplantation but are candidates in function. Analysis of myocardium taken from the core
for prolonged assist to help improve longevity and quality sample at the time of LVAD placement and then at the
of life. A final major category is called bridge to candidacy. time of either explantation or transplantation has shown
These patients typically, at the time of evaluation, are improvements in cardiac myocytes. In particular, there has
found not to be candidates for transplantation. However, been regression of cardiac cell hypertrophy and reduction
the impediment to transplantation may not be permanent in overall size. In addition, there has been evidence of a
and could be reversed by ongoing therapy. Thus, there is a reduction in total collagen back toward control levels.
possibility that these patients might be listed in the future Replacement fibrosis for dead myocytes generally does
not revert, but the interstitium may change favorably
for transplantation. If this occurs, the patient reverts to
with unloading from the LVAD. Unfortunately, these
bridge to transplantation. If this does not ever occur, the
positive changes in matrix and myocytes have generally
patient reverts to destination therapy.
not been translated into enough improvement to allow
Current data from INTERMACS, as of September 30,
explantation.7,8
2012, lists 7,290 patients in the registry. In 2007, during the
first full year of the registry, bridge to transplant was the
most frequent reason for placement of an LVAD. Over the TYPES OF DEVICES
next 2 years, bridge to transplant and bridge to candidacy
were the predominant reasons for LVAD implantation.
Short-Term Circulatory Support
With changes in approval of devices, 2010 saw a dramatic Short-term devices are indicated for patients with acute
increase in destination therapy, and in 2011 destination cardiogenic shock or postcardiotomy shock, which could
therapy became the most frequent indication for an LVAD. involve the left ventricle, right ventricle, or both. In some
The relative frequency of these categories of implantation circumstances, these devices may be placed prior to
is shown in Table 57.2 for selected years.5 performing a high-risk percutaneous intervention.
Impella Catheter-Based Assist Device Abiomed AB5000

This device is a continuous flow system that is quite small This is a first generation pulsatile assist device. It is designed
and can be placed percutaneously across the aortic valve for short-term support. The cannulas are surgically
or pulmonic valve. It is a continuous flow device designed implanted and the device may be used in LVAD, right
to provide partial circulatory support for up to 6 hours. ventricular assist device (RVAD), or biventricular assist
The output of the smaller version is about 2.5 L/min. The device (BiVAD) configurations. It is a bridge to recovery or
inlet area of the device sits in the ventricle, pulling blood bridge to decision device.
out of the ventricle and ejecting it on the other side of the
valve into the central aorta or pulmonary artery. The pump
is 12 Fr in diameter. A positive response to placement of
Thoratec Paracorporeal Ventricular
this device consists of (1) a reduction in filling pressure Assist Device
within the ventricle, (2) a reduction in mechanical work This device can be used as an external or internally
and wall tension that reduces oxygen demand, and (3)
implanted device. The device has been available since the
an improvement in cardiac output increasing oxygen
1990s, approved for patients with end-stage heart failure as
supply to the periphery. A larger version using a 21 Fr
a bridge to cardiac transplantation or for postcardiotomy
pump motor is the Impella 5, capable of 5 L/min of output
support. The device is a pneumatic-driven, pulsatile
(see Fig. 57.3).9
device.
TandemHeart System
Long-Term Axial Flow Devices
The TandemHeart is an extracorporeal centrifugal pump.
The inflow catheter is inserted via a femoral vein, up the For all of these devices, output across the LVAD is
vena cava and into the right atrium, and then via trans- determined by three major factors: (1) pump rotation
septal puncture placed in the left atrium. The device speed, (2) LVAD “preload”, and (3) LVAD afterload. Factors
removes oxygenated blood from the left atrium, out of the (1) and (2) are inter-related and determine the pressure
body to the external pump, which then returns this blood via differential across the pump. For a given rotational speed,
a femoral artery into the central aorta. The inflow catheter flow across the pump increases as the pressure differential
has 14 side holes and an end hole. Transesophageal across the pump declines. For example, consider a patient
echocardiography is used by some operators to help under two circumstances: a mean arterial pressure of
assist in trans-septal puncture and placement of the 55 mm Hg versus a mean pressure of 80 mm Hg. In the
inflow catheter. The TandemHeart has been compared former situation, particularly during diastole, the LV–aorta
to intra-aortic balloon pulsation in patients presenting pressure differential is relatively small and pump flow will
with cardiogenic shock. The hemodynamic effects of the be higher, giving the patient a higher systemic output.
TandemHeart appear to be superior (see Figs. 57.4A and B). Doppler flow velocity, both inflow and outflow, will be
However, overall mortality was not reduced.10 higher. As aortic pressure rises, if all other things stay the
same, pump flow declines.
Thoratec CentriMag System The most significant determinant of LVAD “preload”
is residual contractility of the LV. During systole, a larger
This device is a magnetically levitated centrifugal pump
that is extracorporeal. It comes in an adult and pediatric pressure rise in the LV will decrease the LV–aorta pressure
version, and can be used for up to 30 days as a bridge to difference and propel more blood forward. The patient
decision for cardiogenic shock of either the right or left will demonstrate greater pulsatility and a greater degree of
ventricle. It has been approved for use in various formats flow velocity during systole in both the inflow and outflow
in several countries. This device has the capability of cannula. The degree to which the aortic valve opens shows
producing up to 10 L/min of flow and can be configured to the relative amount of time in which the cardiac cycle LV
provide left ventricular, right ventricular, or biventricular systolic pressure exceeds aortic pressure. This will be an
support, and also can be configured to work with an independent contributor to pulsatility.
extracorporeal membrane oxygenator (ECMO) circuit.11 It All long-term axial devices share a relatively similar
does require a sternotomy and surgical placement of the configuration. The inflow cannula is at the left ventricular
cannulas. apex and the pump is in series with the cannula. Blood flow
12,500 rpm, and can generate flow as high as 10 L/min. A

pediatric version has also been developed.
Jarvik-2000 FlowMaker
The pump is different from other axial flow devices in that
there is no inflow cannula. The pump is directly implanted
in the apex of the left ventricle but can also be used in the
right ventricle. This pump is also relatively small at 90 g
(Fig. 57.5).
Long-Term Third Generation

Centrifugal Flow Systems
These devices represent the newest design for LVADs.
They operate at lower speeds than axial pumps and may
have a longer life expectancy than the axial flow models.
Configuration with the heart is similar to that of the
axial flow system. Current examples of these devices are
discussed below.
Fig. 57.5: Diagram of the placement of the Jarvik-2000 Flow-
maker. The device has no inflow cannula; it is inserted directly into HeartWare Ventricular System
the left ventricular apex.
Source: Used with permission from Jarvik Heart. This device is a centrifugal pump with blood flow coming
into the center of the device and exiting from the periphery.
is boosted by the pump and returned to the aorta, usually The pump is designed to be implanted entirely in the
the ascending segment. The lifespan of these devices is pericardial space, without the need for any secondary
projected for several years (see Fig. 57.1). Some examples pocket. The device is inserted directly onto the apex, with
of current devices are discussed below. a very short inflow cannula. The outflow cannula is placed
at the central aorta (see Fig. 57.2).
Berlin Heart INCOR Assist Device
This pump is an axial flow pump with a magnetically
DuraHeart Magnetically Levitated
levitated impeller. The pump provides flow of up to 6 L/ Centrifugal Assist System
min at a speed of 7,500 rpm. This device is manufactured This is a third generation system with no mechanical
by Berlin Heart. contacts; instead, it uses magnetic levitation between
the impeller and pump housing. The device is attached
HeartMate II Continuous Flow via a cannula to the left ventricle, and outflow goes to the
Left Ventricular Assist Device central aorta.
This system is manufactured by Thoratec and is an axial

flow pump that is electrically driven. The rotor is suspended
Levacor Ventricular Assist Device
between the inlet and outlet sites with ball bearings. This This is a centrifugal device operating between 800 and
device has been used for bridge to transplant, bridge to 3,000 rpm, providing flows up to 9 L/min.
decision, and destination therapy (see Fig. 57.1).
EvaHeart Left Ventricular System
HeartAssist System This is a larger centrifugal pump implanted into the
This is an axial flow pump that is relatively small, being left abdominal wall, weighing 420 g. The device output
only 95 g in weight. The device rotates between 7,500 and depends on head pressures within the pump similar to
Table 57.3: Partial List of Ventricular Assist Devices

Name Manufacturer Pump Type Inflow/Outflow Speed Range Current Usage
Anatomic Site
Percutaneous Placement for Short-Term Support
Impella Abiomed (Danvers, MA) axial/ LV/aorta RV/PA 25–50 K (2.5) Short-term up
catheter based 10–30 K (5) to 6 hours
TandemHeart Cardiac Assist, Inc. (Pitts- centrifugal/extracorporeal LA/femoral artery 3–7.5 K Short-term up
burgh, PA) to 30 days
Short-Term Support (surgical placement)
Abiomed AB5000 Abiomed (Danvers, MA) Pulsatile/extracorporeal LV apex/aorta RA/ Pulsatile Short-term
PA support up to
30 days
CentriMag Thoratec Corp. Centrifugal/extracorporeal LV apex/aorta RA/ 0–5.5 K Up to 30 days
(Pleasanton, CA) PA BiVAD, ECMO support
Long-Term Support
Incor Berlin Heart (Berlin, Axial/intracorporeal LV apex/aorta 7.5 K Long-term
Germany) support
HeartMate II Thoratec Corp (Pleasan- Axial/intracorporeal LV apex/aorta 6–15 K Long-term
ton, CA) support
HeartAssist 5 MicroMed Technology Axial/intracorporeal LV apex/aorta 7.5–12.5 K Long-term
(Houston, TX) support
Jarvik 2000 Jarvik Heart (New York, Axial/intracorporeal LV apex/aorta RV/ 8–12 K Long-term
NY) PA BiVAD support
HeartWare HVAD HeartWare International Centrifugal/intrapericardial LV apex/aorta 1.8–4 K Long-term
(Framingham, MA) support
DuraHeart Terumo Med Corp. Centrifugal/intrapericardial LV apex/aorta 1.2–2.4 K Long-term
(Somerset, NJ) support
Levacor World Heart Inc. (Salt Centrifugal/intracorporeal LV apex/aorta 1–3 K Long-term
Lake City, UT) support
EvaHeart Sun Medical Technology Centrifugal/intracorporeal LV apex/aorta 1–2.8 K Long-term
(Naguno, Japan) support
(LV: Left ventricle, PA: Pulmonary artery, BiVAD: Biventricular assist device, ECMO: Extracorporeal membrane oxygenator; RA: Right
Atrium).
axial devices. The greater the pressure differential between comprehensive echocardiogram should be performed to
LV and central aorta, the lesser the flow that goes through further refine information about the patient’s candidacy.
the pump. On the other hand, when the LV contracts in It is important that a full, comprehensive echocardiogram
systole, the pressure between LV and aorta falls, facilitating be performed, with full two-dimensional (2D) views from
more pump flow. In this way, even though a continuous all standard imaging planes, along with color Doppler,
flow device is used, the patient maintains a degree of pulsed wave, and continuous wave Doppler assessment,
pulsatility.12 and appropriate dimension measurements. In some
A summary of more commonly available devices is circumstances, three-dimensional (3D) imaging may
listed in Table 57.3. Not all devices are universally available. help further define potential abnormalities, and the use
of contrast may be necessary to fully define structures and
function.13,14
PREOPERATIVE ECHOCARDIOGRAPHIC
EVALUATION Left Ventricle
Once clinical criteria have been established that The left ventricle should be carefully examined with full
suggest a ventricular assist device may be necessary, a long-axis and short-axis imaging. Particular attention
A B
Figs 57.6A and B: Two examples of apical thrombi. (A) Large thrombus is easily seen in the apical region of the ventricle; (B) This
thrombus was not apparent with routine imaging but was much better characterized with contrast.
Fig. 57.7: Severe dilated cardiomyopathy baseline study. Dimen- Fig. 57.8: Apical four-chamber view of the heart. The septal
sions serve as a frame of reference for serial follow-up after place- contour preleft ventricular assist device (LVAD) is rightward and
ment of the device. (Ao: Aorta; LA: Left atrium; LV: Left ventricle; left atrial volume is markedly increased. (LA: Left atrium; LV: Left
RV: Right ventricle). atrium; RA: Right atrium; RV: Right ventricle).
should be paid to the left ventricular apex. Care should (Fig. 57.7). The ejection fraction should be quantified;
be taken to determine if there is any evidence of a typically, patients qualifying for ventricular assist devices
possible thrombus in the apex since this part of the heart should have a severely reduced ejection fraction of < 30%.
will be cored out for attachment of the inflow cannula Views should be obtained in both short-axis and apical
(Figs 57.6A and B). In addition, characterization of the views to adequately define the baseline contour of the
apex for any unusual shape changes or trabeculations is of septum so that changes in septal shape can be judged
value to the surgeon. If there is a question about visibility appropriately after placement of the LVAD (Fig. 57.8). The
of endocardial ventricular function or the apex, contrast apex should also be assessed in ischemic heart disease
agents should be used to enhance visualization. Careful patients for the possibility of an apical aneurysm.
2D dimensions should be measured from the parasternal An evaluation of diastolic performance is also
long-axis view to document overall ventricular size at end- important as a baseline. Full diastolic evaluation including
diastole and end-systole, and also wall thickness. These mitral inflow patterns and velocities, tissue Doppler
values will serve as a baseline for serial evaluation of the evaluation of the medial and lateral mitral annulus, and
patient after placement of the ventricular assist device pulmonary vein inflow should be recorded.
Fig. 57.9: Example of a patient with severe mitral regurgita- Fig. 57.10: Aortic valve (AV) with significant calcification and on
tion due to papillary muscle dysfunction. In most cases, severe evaluation was severely stenotic.
mitral insufficiency is not a contraindication for left ventricular assist
device (LVAD) placement. In many patients, decompression of the
left ventricle (LV) improves coaptation. (RV: Right ventricle).
Left Atrium of calcification, stenosis, or significant insufficiency could

create significant problems for this device and result in
It is important to assess the size of the left atrium. This is ineffective support (Fig. 57.10).
best done by evaluating left atrial volume from a biplane For placement of standard apical continuous flow
volume calculation (see Fig. 57.8). In some cases, it may devices, baseline evaluation of the aortic valve is also
also be necessary to evaluate the left atrium and the left critical. Careful evaluation of the leaflets should be
atrial appendage with transesophageal echocardiography performed in short-axis views, and in some circumstances
if there is a strong suspicion of a thrombus in the atrial might be enhanced by 3D views to determine how much
chambers. sclerosis, calcification, and leaflet motion restriction is
present. Forward flow velocities should be quantified,
Mitral Valve and if there is any evidence of aortic stenosis, this should
The mitral valve morphology should be evaluated. be calculated and quantified. Most importantly, the
LVADs are contraindicated in patients with significant severity of aortic insufficiency should be fully quantified.
mitral stenosis. This is readily evaluated by Doppler Patients with more than mild aortic insufficiency could
flows across the mitral valve. The motion of the leaflets be at substantial risk if the valve is left alone. If significant
stenosis is present, the valve may need to be replaced,
should be determined and the severity of mitral regur-
since the patient could be put at substantial risk if the
gitation evaluated (Fig. 57.9). In most circumstances,
LVAD suddenly failed.
mitral regurgitation will be expected to improve with
decompression of the left ventricle after placement of the
LVAD. Appropriate quantification of the severity of mitral Pericardium
insufficiency preoperatively helps establish a baseline to The pericardium should be assessed to determine if any
evaluate the effect of the LVAD after it is implanted. significant effusion is present or if there is any evidence
that suggests the presence of constriction.
Aortic Valve
Aortic valve anatomy and function is particularly important
Atrial Septum
if the temporary impeller device is to be utilized. This The atrial septum is an important structure, particularly
device works well if the aortic valve is normal, but presence since relative filling pressures may change dramatically
A B
Figs 57.11A and B: (A) Dilated cardiomyopathy with small secundum atrial septal defect (ASD) with shunt shown in orange going
left to right. This was not detected in the past and may be due to stretching open of the foramen caused by chamber enlargement;
(B) Different patient with heart failure and a larger atrial septal defect. Note large orange left to right shunt jet. Both require closure during
left ventricular assist device (LVAD) surgery.
after placement of the LVAD. The patient should be can be demonstrated. Size of the annulus, sinus of Valsalva,
evaluated for the possibility of a patent foramen ovale sinotubular junction, and proximal ascending aorta
in the preoperative assessment. Careful evaluation should be determined and as much of the aorta examined
of the atrial septum from multiple views, particularly as possible for the possible presence of atherosclerosis that
the subcostal view, may detect shunting. In some might affect placement of the cannula. Suprasternal views
circumstances with very large hearts and persistently high of the arch should also be performed and subcostal views
filling pressures, a previously patent foramen ovale may can be obtained to evaluate the upper abdominal aorta.
stretch out, allowing a left-to-right shunt to be present The presence of an untreated ascending aortic aneurysm
(Figs 57.11A and B, Movie clips 57.1 to 57.3). This is usually > 5 cm in diameter is considered a contraindication.
readily quantified in the subcostal view. If no color flow
abnormalities are present, a bubble study should be Tricuspid Valve
performed with injection of agitated saline intravenously.
The tricuspid valve can pose particular challenges in the
This should be done with and without Valsalva. Careful
perioperative period. Careful evaluation of the severity
attention should be paid to an assessment of left atrial
of tricuspid valve insufficiency is essential from all
filling pressures. In some circumstances, there may be
standard views. Characterization of leaflet motion and
such a differential between left and right atrial pressures
morphology, along with measurement of the diameter of
that even a Valsalva maneuver may be ineffective in
the annulus, can be of considerable help to the surgeon in
detecting a patent foramen ovale. If there is a question
determining whether or not tricuspid valve repair is indi-
about the presence of a foramen ovale, a transesophageal
cated during the procedure (Figs 57.12A to D and Movie
echo can be considered for further evaluation. Unusual
clips 57.4 and 57.5).15–17
anatomy of the atrial septum, such as a large atrial septal
aneurysm or substantial lipomatous hypertrophy of the
atrial septum, could affect the trans-septal puncture Pulmonic Valve
necessary for placement of the TandemHeart. If need be, The pulmonic valve should be evaluated to exclude
transesophageal echocardiographic guidance may be any unknown stenosis and also carefully evaluated to
necessary to help placement of a device. determine if there is any significant pulmonic valve insuffi-
ciency. This is particularly important in patients who may
Aorta have underlying congenital disease. Substantial amounts
Examine as much of the aorta as possible. Off-axis views of pulmonic valve insufficiency could contribute to right
may be of value to show as much of the ascending aorta as ventricular overload following placement of the LVAD.
A B
C D
Figs 57.12A to D: Tricuspid valve. (A) Valve morphology shows evidence of mild leaflet thickening and reduced excursion due to right
ventricle (RV) enlargement and dysfunction; (B) Severe valve insufficiency is present; (C) Coaptation is severely compromised, leaving
a visible regurgitant orifice (arrow); (D) The annulus is markedly dilated. This patient had tricuspid valve repair when the left ventricular
assist device (LVAD) was placed. (LV: Left ventricle; RA: Right atrium).
Inferior Vena Cava always that of how the ventricle will respond during
surgery and how it will respond to placement of the LVAD
The inferior vena cava should be imaged and its diameter after the fact. Will the ventricle maintain good function
measured, and an evaluation of estimated central venous
and not be a limiting factor on the patient’s overall cardiac
pressure made. This, combined with the tricuspid
output and functional performance, or will it deteriorate
regurgitant jet, can be used to assess the severity of
and become the rate limiting structure that controls right-
pulmonary hypertension. This is particularly important if
sided congestion and cardiac output? Significant right
there is disproportionate elevation of pulmonary pressures
ventricular heart failure occurs in 20–30% of post-LVAD
that might indicate independent pulmonary artery disease
patients. Survival is reduced in right heart failure patients,
or reactive pulmonary hypertension.
morbidity is increased, and length of stay prolonged.18,19
Frequently, clinical characteristics of patients do not differ
Right Ventricle between those who do well post-LVAD and those who
Perhaps the most difficult and perplexing pre-LVAD develop right ventricular failure. Detailed assessment of
evaluation is that of the right ventricle. The concern is the right ventricle is important and frequently overlooked
in standard echocardiographic evaluations. A special in this situation is whether or not right ventricular function
effort should be made to characterize right ventricular is adequate to sustain an adequate cardiac output in the
size and function from multiple views, particularly apical, immediate postoperative setting (Fig. 57.15).
subcostal, and short-axis views. As 3D imaging improves, Another potential issue, particularly if there is leftward
better transthoracic assessment of the right ventricle may deviation of the atrial septum, is the evaluation for a
be possible in the future. patent foramen ovale. A large right-to-left shunt could
Because the right ventricle is so difficult to evaluate, cause significant hypoxemia. Sometimes this is missed in
a large number of proposed tests, measurements, ratios, the preoperative assessment due to excessively high left-
fractional changes, excursion measurements, indices, sided pressures. If the preoperative contrast bubble study
and point score systems have been proposed (Figs 57.12 is abnormal and suggests a significant right-to-left shunt,
to 57.14 and Movie clip 57.6). No one measurement consideration needs to be given for closure of the patent
is foolproof and no particular measurement is consi- foramen ovale during the procedure.30
dered a gold standard. Table 57.4 summarizes only The inflow and outflow cannulas are inspected.
echocardiographic-based measurements, including a The inflow cannula should be inspected to determine
point score system based on left-sided measurements. the orientation. It should be angled toward the mitral
The point score system is a single center study that uses valve and aligned with the left ventricular outflow tract.
simple LV measurements. This study suggests that smaller Doppler evaluation should be performed. A normally
LV size with better preserved LV systolic function but functioning inflow cannula generally has relatively low
higher levels of filling pressures (represented by the larger velocity laminar color Doppler flow with low velocities
LA) are more likely associated with a worse RV prognosis.26 by pulsed wave Doppler. Flow signals deviating from
Table 57.5 is an overall summary of the preoperative this should raise suspicion about possible obstruction
echocardiographic assessment. or pump malfunction. Particularly concerning would
be a regurgitant flow signal at the site of the cannula,
IMMEDIATE POSTSURGICAL indicating backflow through the pump. One exception is
EVALUATION the Jarvik-2000. The pump is actually in the LV apex.
The outflow cannula is generally visible in high
Immediate postoperative evaluation is generally esophageal views and should show relatively low velocities
performed by the cardiac anesthesiologist after the patient outward from the cannula (Figs 57.16A and B).
comes off the pump.29 The immediate surgical results of
the LVAD placement should be evaluated, along with the
SERIAL CHANGES IN CARDIAC
response of the ventricles and valves.
Before the assist device is activated, an immediate STRUCTURE AND FUNCTION
evaluation for residual air bubbles is undertaken in the
ventricular chambers, ascending aorta, and cannulas.
Ventricular Size and Function
Activation of the LVAD should begin appropriate The echocardiography laboratory and the heart failure
unloading and should result in a slight change in program should set up a regular schedule for evaluation of
interventricular and intra-atrial septum contours to patients with an LVAD. This allows for regular surveillance
the left. Lack of effective decompression will result in of effectiveness of the device and for serial changes in
substantial rightward deviation of the septum, indicating performance of the device. A suggested schedule could be
suboptimal support from the assist device or inadequate month 1, month 3, month 6, month 12, and every 6 months
settings to unload the left ventricle. This may require rapid thereafter. A comprehensive study should be performed
assessment of LVAD function and the device cannulas. similar to a regular echocardiographic exam. As many
The opposite can also occur. Extreme leftward shift of the normal views as is possible should be obtained, realizing
septum suggests the possibility of an excessively high pump that all views may not be available. Certain specialized
speed that may be unloading the left side too vigorously. It views are also necessary; some of these are outlined in
could also be caused by right ventricular dysfunction; thus, Figure 57.17.30,31
right ventricular function should be assessed immediately The left ventricle should be assessed particularly
along with tricuspid valve performance, particularly for for changes in size and function. The expected response
severity of tricuspid regurgitation. An immediate concern to placement of an outflow tract device would be a
Table 57.4: Evaluation of the Right Ventricle: Factors that May Impact Response to Placement of Left Ventricular Assist Device
Factors Comment
Tricuspid Valve
Severity of regurgitation (Fig. 57.12) May identify patients who would benefit from tricuspid valve
repair. Repair may have a survival benefit, recovery benefit, and
RV function benefit.15,16,18–21
Size of annulus diameter (Fig. 57.12) Annular diameter > 43 mm is associated with reduced sur-
vival.17
Duration of tricuspid regurgitation in systole (Fig. 57.13) A rate corrected value of less than 461 ms indicates a worse
2-year prognosis.22
Right Ventricular Function
Semiquantitative evaluation of function Severe systolic dysfunction associated with worse outcome.23
Fractional area change Values < 20% are associated with increased risk of post LVAD RV
dysfunction.24
TAPSE (Fig. 57.14) Annular motion < 7.5 mm predicts post LVAD RV failure.25
Right Ventricular Geometry
Short-axis/long-axis ratio of the RV Ratio > 0.6 associated with worse outcome
RVEDD/LVEDD ratio (by transesophageal echocardiography) Ratio > 0.72 associated with adverse outcome.17,26
Right-sided Hemodynamics
Central vanous pressure (CVP) Elevated pressure and ratio of CVP to PCWP > 0.64 associated
with worse outcome.19
Estimated RV systolic pressure Conflicting findings, higher25 or lower27 RV systolic pressure
associated with worse outcome.
Left ventricle parameters point score system for predicting RV The point score system for detecting RV failure showed the
failure after LVAD following performance.28
Parameter Points
LVEDD Sensitivity Specificity
> 78 mm 0
70–78 mm 1 3 points 88.6% 47.4%
< 70 mm 2
LVEF 4 points 71.4% 67.1%
< 19% 0
19–33% 1 5 points 42.9% 80.3%
> 33% 2
LAD/LVEDD
< 0.63 0
0.63–0.68 1
> 0.68 2
(CVP: Central venous pressure; LAD: Left atrial dimension; LVEDD: Left ventricular end-diastolic dimension; LVAD: Left ventricu-
lar assist device; RV: Right ventricle; RVEDD: Right ventricular end-diastolic dimension; TAPSE: Tricuspid annular plane systolic
excursion).
Table 57.5: Points of Emphasis for the Pre-Left Ventricular Assist Device Echocardiogram
Left Ventricle: Size, geometric shape, systolic function, diastolic function, and filling pressure
Left Ventricular Apex: Shape, trabeculae, and thrombus
Aortic Valve: Leaflet motion, morphology, severity of valve insufficiency, and presence of stenosis
Mitral Valve: Presence of stenosis, motion of leaflets, and severity of valve insufficiency
Tricuspid Valve: Annular dimension and severity of valve insufficiency
Atrial Septum: Patent foramen ovale and atrial septal defect
Pulmonic Valve: Severity of valve insufficiency
Inferior Vena Cava: Estimate of central venous pressure
Aorta: Ascending aortic size and atherosclerotic plaque
Pericardium: Effusion or constrictive changes
Right Ventricle: Size, geometric shape, and systolic function
A B
Figs 57.13A to C: Two examples of calculation of tricuspid regurgi-

tation duration. In (A) the duration is quite prolonged, giving a rate
corrected value of 631 ms. This puts the patient in a prognostically
favorable group; In (B) the rate corrected value is 458 ms, putting
the patient in the prognostically less favorable group even though
the severity of the valve regurgitation was moderate; (C) and
right ventricular systolic pressures were only mildly increased.
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
C ventricle).
20– 30% reduction in dimensions. These are most effectively should be made to reproduce the same views each time the
performed using direct 2D diameter measurements at the study is obtained. Measurements of ventricular ejection
tips of the mitral valve in the parasternal long-axis view fraction and function are sometimes difficult to obtain due
(Figs 57.18A and B and Movie clips 57.7 and 57.8). Attempts to the inability to get true long-axis views from the apex.
A B
Figs 57.14A to C: Evaluation of right ventricular function.

(A) Tricuspid annular plane systolic excursion (TAPSE) in this
case is markedly reduced consistent with a reduced prognosis;
(B) Transthoracic measurement of the mid-right ventricle (RV) and
mid-left ventricle (LV) diameter, this example the ratio is 0.41, a
favorable prognostic finding; (C) Evaluation of fractional area
change of the RV in the apical four-chamber view. The end dias-
tolic and end systolic areas are shown. The fractional area change
C is only 11%, an unfavorable prognostic finding.
Since loading conditions are no longer natural, evaluation

of an ejection fraction may not have the same meaning in
an LVAD patient.
While imaging the ventricle, it is also important to
obtain views of the inlet cannula at the apex. This should
be visible over 90% of the time and may be imaged from
low parasternal off-axis views, from distal short-axis views
in the parasternal view, and from various apical or off-axis
apical views. Cannula position and direction should be
shown as best as possible. Color flow Doppler should be
utilized in these views and under normal circumstances
should show laminar relatively low velocity flow. Pulsed
wave inflow velocities should be obtained at the origin
of the cannula. These velocities should generally be fairly
Fig. 57.15: Immediate postoperative transesophageal echo. The low, gently pulsatile, and average about 1.0–1.5 m/s, and
left ventricular assist device (LVAD) has been ramped up to 8,800
rpm but the left ventricle has become suctioned down to a small always less than 2.0 m/s (Figs 57.19A and B).32
volume. In systole, the inflow cannula is now obstructed causing In most patients, the outflow portion of the cannula
flow acceleration at the inflow site that aliases the display. As soon attaches to the pump in the abdomen, thorax, or
as systole concludes, inflow abruptly slows to normal.
pericardium. The course of a typical cannula can be
A B
Figs 57.16A and B: (A) Transesophageal echo high esophageal view showing location of the outflow cannula in the ascending aorta
with color flow signal. The arrows show aorta landmarks for reference of location; (B) Continuous wave Doppler of outflow showing
pulsatile flow preservation. (AV: Aortic valve annular plane; ST: Sinotubular junction).
followed using off-axis modified parasternal and right improve some, lessening the severity of papillary muscle
parasternal views. The relative position in the chest of dysfunction. The tricuspid valve is less predictable. The
outflow cannulas is shown in Figures 57.1 and 57.2. severity of insufficiency may or may not change depending
The transthoracic view on the cannula anastomosis is on changes in size and function of the right ventricle.
shown in Figure 57.20A with a typical color flow signal in In some situations, the tricuspid valve will have been
Figure 57.20B and pulsed Doppler in Figure 57.20C. Off repaired, and so the repair should be evaluated for both
axis images from the suprasternal notch or right paras- stenosis and insufficiency.
ternal imaging positions are usually needed to see the Of considerable importance is the aortic valve.
outflow cannula and anastomotic site. Motion of the aortic valve should be carefully evaluated
The right ventricle should be assessed for size and from multiple views to determine if the valve is opening
function. This should be done from standard views and or not, and if it is opening, if it is opening every beat,
apical views that emphasize the right ventricle. The septal intermittently, or partially on each beat. Changes in
contour should be assessed. Generally most favorable is aortic valve morphology should also be assessed by
a relatively neutral position between the two ventricles. careful evaluation of the short-axis view. Color Doppler
This indicates adequate but not excessive unloading examination is also important for determining if aortic
(Figs 57.21A to C and Movie clips 57.9 and 57.10). Similarly, insufficiency has developed.33,34 Motion of the aortic valve
the septum can be assessed in the parasternal short-axis will help determine whether speed settings of the device
views as one sweeps from base to apex. Under normal are optimal. At higher rotational speeds, the LVAD takes
circumstances, the contour of the curvature should over relatively greater amounts of output, and in many
remain reasonably normal. The atrial chambers should be circumstances the valve no longer opens. As speeds
evaluated as with normal studies and the volume of the left are reduced or as the left ventricle starts improving,
atrium measured. It is expected that left atrial volume will some native contribution to outflow is again observed
diminish over time. The junction of the inferior vena cava (Figs 57.22A to D and Movie clips 57.11 and 57.12).
and the right atrium should also be evaluated and central Diastolic performance of the ventricle can also be
venous pressure estimated. Hepatic vein Doppler signals assessed by standard indices of tissue Doppler, and
can also be assessed throughout a continuous respiratory mitral valve flow and pulmonary venous inflow. In
cycle. Valvular performance should be evaluated. The general, successful unloading of the left ventricle results
mitral valve is expected, with appropriate unloading, in a reduction in left-sided filling pressures, but there is
to show a reduction in regurgitation. With reduction in no evidence that any intrinsic improvement in diastolic
left ventricular size, motion of the leaflets might also function occurs (Figs 57.23A and B).35 Doppler assessment
Fig. 57.17: Specialized views obtained to help visualize structures in the patient with a left ventricular assist device (LVAD).
Source: Reproduced with permission from Elsevier.
A B
Figs 57.18A and B: Example of pre- and post-left ventricular assist device (LVAD) dimensions taken at 3 months following LVAD place-
ment. There has been significant decompression of the left ventricle (LV), with a 25% decline in LV end diastolic dimension.
A B
Figs 57.19A and B: (A) Example of off-axis apical view showing the inflow cannula having relatively low inflow velocity (B).
of LV filling shows these changes. On the right side of the over time. In some cases, there is evidence of recurrent
heart, the typical response is a reduction in pulmonary enlargement of the ventricular chamber. This can be
artery pressures and also central venous pressures. detected qualitatively or more precisely quantitatively
Right ventricular function does not show significant by noting dimension changes in the left ventricle. If the
improvement over time in most patients. ventricle dilates again, the septum may deviate more
Table 57.6 shows some selective data from a single rightward as filling pressures increase on the left side.
center study demonstrating some of the typical serial Reduced cardiac output may be associated with evidence
changes expected after placement of an axial flow device. of stasis, causing visible spontaneous contrast in both the
left ventricle and left atrium. Mitral regurgitation may
increase in severity if the mitral valve apparatus is stretched
COMPLICATIONS OF LEFT
and the annulus increases in size. In most settings, there is
VENTRICULAR ASSIST DEVICES evidence of over-filling associated with a relative reduction
in cardiac output moving through the assist pump and a
Evidence of Left Ventricular Over-Filling relative increase in stroke volume across the aortic valve.
Normally, the LVAD is expected to decompress the left This typically is manifested by an increase in amplitude
ventricle, reduce its size, and maintain the changes and frequency of aortic valve opening from none, minimal,
A B
Figs 57.20A to C: Transthoracic echo example of the right

parasternal view of the outflow cannula as it joins the central aorta.
(A) Note relative size of cannula; In (B), typical color flow signal of
flow in same location; In (C) pulsed Doppler at the outflow is shown
C with normal velocities. (Ao: Aorta).
A B
Figs 57.21A and B
Figs 57.21A to C: (A) Apical four-chamber view showing a normal

septal contour with mild residual right ventricular dysfunction. In
(B) and (C) the patient has right ventricular dysfunction causing
an abnormal septal contour in the parasternal long- and short-
axis views. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
C RV: Right ventricle).
A B
C D
Figs 57.22A to D: (A) Example by M-mode of intermittent opening of the aortic valve (AV); (B) Typical central jet that can develop after
left ventricular assist device (LVAD) placement; (C) M-mode with color of same patient as (B) shows continuous nature of insufficiency
flow; (D) Continuous wave (CW) Doppler from the apical view in the same patient. Note that even though the left ventricle (LV) contracts,
it does not generate enough force to open the valve.
A B
Figs 57.23A and B: (A) Mitral flow prior to placement of left ventricular assist device (LVAD) when the patient was in class IIIb heart failure; In
(B) the LVAD has been in place for 2 months. Note the improvement in deceleration even though the E/A ratio remains elevated.
Table 57.6: Early Serial Changes in Ventricular Size and Function after Placement of a Left Ventricular Assist Device
Pre-Op Three Months Six Months
LVEDD (mm) 70 61 60
LVESD (mm) 62 54 54
Biplane LVEF (%) 17 18 16
RVEDD (mm) 35 34 34
*
RV Function 1.5 1.6 1.4
CVP (mm Hg) 12 9 9
RVSP (mm Hg) 46 26 27
Mitral E/A Ratio 2.5 1.6 1.5
Mitral DT (ms) 146 200 195
E/e’ Ratio 13.3 11.8 11.4
Diastolic Grade* 2.5 2.0 2.1
LA Volume (mL) 95 73 65
*For both RV function and severity of diastolic dysfunction, the score was: 0 = normal, 1 = mildly abnormal, 2 = moderately abnormal,
3 = severely abnormal.
(CVP: Central venous pressure; DT: Deceleration time; EDD: End-diastolic dimension; EF: Ejection fraction; ESD: End-systolic
dimension; LA: Left atrium; LV: Left ventricle; RV: Right ventricle; SP: Systolic pressure).
Source: Adapted from reference 28.
or intermittent to present on every beat. There also may pulsed wave Doppler. It is also possible that the over-filling
be evidence of direct pump failure, which sometimes is and reduced flow is due to an abnormality of the outflow
nonobstructive. Forward flow velocity is reduced and from the pump; thus, the outflow cannula should also
LVAD regurgitation may occur (Figs 57.24A and B, Movie be very carefully analyzed for evidence of turbulence or
clip 57.13). In some patients, the increase in flow velocity change in velocity from one part of the cannula to another
may only occur at certain times in the cardiac cycle (Figs 57.26A to C and Movie clip 57.14).
(Figs 57.25A and B). If there is obstruction of the inflow Specific potential causes of over-filling are categorized
cannula, there may be a change in flow velocity, from the in Table 57.7.
normal laminar, relatively low velocity flow to an increase One specific difference must be noted for the case of
in velocity that is turbulent. The cannula inflow area high afterload due to hypertension. In this case, the aortic
should be interrogated with color, continuous wave, and valve stays closed due to increased central aortic pressure.
A B
Figs 57.24A and B: Patient who suffered sudden power failure of his left ventricular assist device (LVAD). (A) There was a significant
change in the aortic valve M-mode that began fully opening in each beat; (B) The flow signal at the inflow cannula showed evidence of
weak forward flow, upper arrow, and regurgitant flow (lower arrow). The device did not clot in the 6 hours before surgery. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
A B
Figs 57.25A and B: This patient had normal inflow cannula velocity (A). Two months later, there is a change in the inflow pattern con-
sistent with obstruction. In this case, velocity peaks near end-systole (B). Peak velocity has increased from about 40 cm/s to almost
200 cm/s.
A B
Figs 57.26A and B
Figs 57.26A to C: This patient showed evidence of reduced output.

The inflow cannula appeared normal by three-dimensional imaging
with low inflow velocity (A). The aortic valve began to open fully on
each beat (B) as shown by the arrows compared to previous inter-
mittent opening (Fig. 57.22D). An increase of flow velocity was seen
in the outflow cannula (C) where a kink in the cannula developed.
The location of the kink was similar to that seen in Figure 57.1D
C close to the anastomosis with the aorta.
Table 57.7: Causes of Left Ventricular Overfilling

Clinical Signs of Heart Failure
Possible echocardiographic Increased LVEDD
changes Septal contour shift rightward
Spontaneous contrast in LV, LA
Increase in mitral regurgitation
Change in mitral inflow signal, E/e’ suggesting increased filling pressure
Increased opening of aortic valve to every beat
Atrial septal contour shifted rightward
Cause Inflow obstruction Pump failure pump thrombus Outflow obstruction or high
afterload
Pump readout changes Reduced flow Reduced flow Low pump flow
Increased power Increased power consumption Normal power consumption or
consumption
Spikes in power Sudden flow reduction High afterload: Increased
pulsatile index
Specific echocardiographic Apical thrombus Reduced inflow velocity Thrombus in outflow cannula
findings
Apical vegetation Regurgitation signal from flow Kink in outflow cannula
reversal
Adverse change in septum Doppler with different velocities
contour at different part of cannula
Increased inflow velocity Anatomic obstruction of cannula
at aorta
Increased inflow turbulence High afterload: High velocity
pulsatile LVAD flow
(LA: Left atrium; LV: Left ventricle; LVAD: Left ventricular assist device; LVEDD: Left ventricular end-diastolic dimension).
Outflow through the cannula becomes more pulsatile with is right ventricular failure. If the right ventricle deteriorates
higher outflow velocity. after the LVAD is placed, septal contour may push from
right to left, indicating overload of the right ventricle,
EVIDENCE OF UNDERFILLING OF THE reduced systolic performance of the right ventricle, and
LEFT VENTRICLE thus a reduced cardiac output (Fig. 57.28). This situation
can be further complicated by worsening of tricuspid valve
A second major abnormality is that of under-filling.
insufficiency, which will further reduce forward flow out
Under-filling may be associated with low flow but normal
of the right side of the heart and reduce volume delivered
overall power usage of the LVAD device. This could result
from an excessive reduction in preload. This may be a to the left ventricle. In some situations, a third possibility
consequence of an excessive speed setting of the device exists: right ventricular function may be preserved, but
and can be associated with “set-down,” in which the device the patient is excessively volume depleted. This could
is intermittently obstructed at its inflow cannula site by be due to bleeding, poor intake, or excessive diuretic
changes in contour of the interventricular septum or use. There will be a decline in overall cardiac output due
apex (Figs 57.27A to C and Movie clip 57.15). The device is to an excessive reduction in right ventricular preload
typically programmed to reduce rpms to a default rate until (Table 57.8). Restoration of normal fluid status brings
the obstruction is relieved. A second cause of this situation outputs back to baseline.
A B
Figs 57.27A to C: The left ventricle (LV) cavity is small and the gap
between the cannula and septum satisfactory in systole (A)
but very small toward end-diastole (B). The inflow signal shows
increased flow velocity at end-diastole corresponding to the
findings (C). Diuretics were stopped and the left ventricular as-
sist device (LVAD) speed reduced to 8,800 rpm. Inflow velocity
C normalized.
Table 57.8: Causes of Left Ventricular Underfilling

Possible echocardiographic changes Further reduction in LV end diastolic dimension
Leftward septal shift
Change in mitral inflow suggesting reduced LV filling and filling pressures
Aortic valve continuously closed
Atrial septum position variable
Potential causes Excessive LVAD speed setting
Right ventricular failure
Increased tricuspid valve insufficiency
Dehydration (bleeding, excessive diuresis, poor intake, etc.)
Pump readouts Reduced output
Normal power usage
Occasional “set down” episodes due to inflow cannula obstruction
Specific possible echocardiographic findings Low cannula inflow velocity
Change in septal contour to left or evidence of septal obstruction of inflow
Worsening RV function and high central venous pressure
Dehydration with reduced RV size, inferior vena caval signs of low or normal
central venous pressure
(LV: Left ventricle; LVAD: Left ventricular assist device; RV: Right ventricle).
development of aortic insufficiency. With the position of

the pump circuit routing blood from LV to central aorta,
the presence of aortic insufficiency causes a direct loss of
forward cardiac output. Aortic insufficiency and changes
in the aortic valve have been evaluated in several serial
studies. The phenomenon of deterioration of aortic valve
leaflets, changes in coaptation, reduction in excursion of
the leaflets, and the development of continuous aortic
insufficiency have all been documented in multiple
series.32,34,36,37 Morphological changes on the valve have
been demonstrated. Changes in the valve may be related
to lack of opening motion. Some groups now suggest
that the aortic valve should be carefully observed at the
time of setup of pump speed, with pump speed adjusted
Fig. 57.28: Example of right ventricle (RV) failure which causes to allow at least intermittent or modest opening motion
the septum to deviate to the left. (LV: Left ventricle). of the valve. This may prevent deterioration of the valve
leaflets over time. More serial information is necessary
High Left Ventricular Assist Device Flow to determine more definitively the etiology and the
most appropriate settings. Aortic insufficiency is best
with Low Net Forward Cardiac Output evaluated in the parasternal long-axis view. It may be
In most circumstances, this is associated with an continuous depending on the status of LV ejection of blood
abnormality of the aortic valve, most frequently the (see Figs 57.22 A to D and Movie clip 57.12).
Pericardial abnormalities may occasionally occur. of a respirometer may enhance the information by
Development of a significant pericardial effusion, and allowing motion of the septum to be tracked with
particularly cardiac tamponade physiology, may substa- the respiratory cycle. Under ideal circumstances, the
ntially change the loading conditions. The exact effect septum should stay at the midline. Turning device
on the heart is determined by the actual location of rpms up too high may result in the septum drifting
the effusion (loculated or not). Hemopericardium can leftward and actually collapsing the left ventricle. This
complicate the early post-LVAD recovery period. Normal may also be observed in ramp protocols when one
diagnostic criteria for cardiac tamponade are not reliable, is trying to quickly adjust the device to achieve an
particularly Doppler criteria. Chamber collapse is the best optimal speed. On the other hand, device settings that
indicator of tamponade.24,31 are too slow, that reduce flow out of the left ventricle,
may result in the septum drifting rightward to the right
ventricle. This can result in a reduced efficiency of right
OPTIMIZING LEFT VENTRICULAR ventricular performance and reduced output.
ASSIST DEVICE SETTINGS • Atrial septum: Position of this structure indicates
a relative difference between LA and RA pressure.
The goal of device optimization is to (1) preserve cardiac
One group40 has suggested that estimation of RA
output as much as possible, (2) eliminate congestion,
pressure using the inferior vena cava dimension and
and (3) help the patient feel as well as possible. The LVAD
its response to respiration can be used to estimate LV
devices display certain pieces of information on the
unloading. They assign LA pressure = RA pressure if
controller system. For example, the HeartMate II displays
the atrial septum is midline, LA pressure = RA pressure
revolutions per minute (rpm; range 6,000–15,000 rpm,
– 5 mm Hg if the septum is deviated to the left, and LA
most commonly 8,000–10,000 rpm), power consumption
in watts, flow, and the pulsatile index. The device is capable pressure = RA pressure + 5 mm Hg if the atrial septum
of directly measuring rpms and power consumption. It is deviated to the right. If RA pressure is 15 mm Hg
calculates flow and the pulsatility index based on the other or greater and LA pressure is equal to or greater than
two pieces of data. The device calculations give reasonable RA pressure, these authors would suggest increasing
estimates of flow; however, at extremes of the rpm range, pump speed to further unload the LV.
for instance below 8,000, calculated flow rates become • Evaluation of LV filling characteristics may be done
less reliable. Flow is also determined by the pressure directly by evaluation of the grade of diastolic filling
gradient across the device. As speeds go up, left ventricular and estimation of filling pressures. Successful unloa-
systolic pressure will fall within the left ventricular ding should cause a reduction in diastolic grade, E/e'
chamber relative to the central aortic pressure. ratio, and left atrial volume.35 The early mitral decele-
A comprehensive echocardiogram is necessary to ration time can be measured and should prolong
obtain full information in order to optimize speed settings. as unloading occurs. The deceleration time can
In some circumstances, an interactive echocardiogram be indexed by dividing deceleration time by peak
may be performed using a ramp protocol to follow the E velocity (e.g., 150 ms/50 cm/s = 3 ms/cm/s). A value
effect of serial pump adjustments on the echocardiogram. < 2 is associated with adverse outcomes and is another
From the echocardiogram, particular attention should indicator that suggests an increase in pump speed may
be made to obtain full information on the following:38–40 be beneficial (see Figs 57.23A and B).
• Cannula flow velocities: Flow velocities should be • The aortic valve should be carefully observed. There
examined carefully at both the inflow site and the has been much controversy about the optimal settings
outflow site. Flow velocities that are not elevated or with regard to aortic valve motion. It has been well
unusually shaped or turbulent indicate normal flow. documented by multiple studies that the aortic
One should maneuver the transducer to optimize valve can deteriorate over time. Valve thickening
obtaining flow in the most parallel position possible. occurs, some fusion of leaflets may occur in certain
This will usually be from some type of an off-axis low circumstances, and valvular insufficiency can develop
parasternal or apical view. (see Figs 57.22A to D). It now appears that the best
• Ventricular septal contour position: The interven- policy is to set the speed so that there is intermittent
tricular septum should be carefully evaluated. Use opening of the aortic valve. This has the salutary effect
of allowing a slight increase in pulsatility that could be tolerates this, in increments of 400 rpm. From an
beneficial for the patient and also, more importantly echocardiographic standpoint, ventricular dimensions
from the valve standpoint, it may reduce the likelihood are serially measured, the septal contour is observed, and
of fusion abnormalities, thickening, or development aortic valve opening and severity of aortic insufficiency are
of aortic insufficiency over time. It is no longer observed, as are mitral insufficiency and right ventricular
recommended to set the speed so that the aortic valve systolic pressure estimates. From these measurements the
stays closed. Proof of long-term benefit of this policy is authors have been able to show characteristic changes in
not yet available. their protocol, particularly when related to simultaneous
• Mitral valve performance: Optimal unloading of the plotting of the pulsatility index, left ventricular end-
left ventricle should result in a reduction of mitral diastolic dimension, and power output of the device.
valve insufficiency if it was present before placement Protocols of this nature can be used to optimize settings
of the LVAD. In most settings, mitral valve insufficiency by observing all the variables discussed above, and in
is not due to primary valve disease; rather, it is due certain circumstances may also be used to evaluate for
to secondary valve disease from papillary muscle the possibility of thrombosis in and around the device
dysfunction, annular enlargement, and reduced because the characteristics of the ramp protocol change
excursion of the leaflet apparatus. In many patients, when thrombus is present. A summary of optimization
as ventricular size is reduced, the severity of mitral goals and measurements is shown in Table 57.9.
valve insufficiency may diminish. Ideally, the severity
of mitral insufficiency should be reduced to mild or EXPLANTATION
nothing with unloading.
• Arterial pressure: An ideal setting would be a mean A small group of patients show recovery of function
pressure > 65 mm Hg. sufficient to allow consideration for explantation.6 A few
• Right ventricle: Optimal settings of the LVAD should disease processes are inherently self limited and have
result in a reduction of filling pressures on the left side an optimistic outlook for recovery. However, in cases of
of the heart that should be translated backward to the true dilated cardiomyopathy, the chance of recovery is
right side. Optimization of the LVAD should result in very low. For instance, in one series of 1,108 patients,
a reduction of right ventricular pressures over time. 20 (1.8%) had the HeartMate II explanted due to recovery
Similarly, central venous pressure should come down of left ventricular function. This trial and others have
over time, particularly if there is fluid optimization that also suggested that a nonischemic etiology is more likely
occurs. A goal would be to lower RA pressure to 5–10 to recover. Of the 1,108 patients cited above, 531 were
mm Hg through a combination of pump settings and nonischemic; they had a recovery rate of 3.4%. A group of
medical management.38 578 of the patients were ischemic; they had a recovery rate
• Cardiac output: Total cardiac output is best estimated of only 0.3%. Age also appears to be a significant factor.
using pulsed Doppler flow at the right ventricular Patients < 40 years of age have a higher rate of recovery,
outflow tract just above the pulmonic valve. Views and patients who have a shorter duration of heart failure
should be obtained to allow accurate measurement of symptoms tend to have a higher rate of recovery.41,42 These
the diameter of the region. The flow velocity integral is general findings have also been consistently found in
used in the formula: trials evaluating medical management of dilated cardio-
RV outflow diameter/2 × π × RV outflow TVI = stroke myopathy.
volume Echocardiography plays a central role in identifying
Stroke volume can be multiplied by heart rate to obtain patients who may be considered for explantation. Weaning
a cardiac output. An indexed value of 2.2 L/min/m2 or protocols were more easily performed with pulsatile
greater is a reasonable goal for total outputs. pumps since they could be temporarily slowed down to
A formalized ramp test protocol for the HeartMate II a very low rate while patients were carefully monitored.
has been developed by Uriel et al.38 This protocol serially The transition to continuous flow devices has made
evaluates device indices such as pulsatile index, power weaning more difficult. Some devices can be shut down
output, and flow calculations from the device, along with temporarily; others can only be partially slowed down.
blood pressure and heart rate. The device is changed Specific weaning protocols are beyond the scope of this
through a range of 8,000–12,000 rpm, if the patient chapter and tend to be institution-specific.43 It should be
Table 57.9: Optimization of Left Ventricular Assist Device Performance: Echocardiographic Measurements Indicating a Favorable
Response
Cardiac Index: Cardiac Index > 2.2 L/m2/min
Ventricular Septum: Neutral at midline
Atrial Septum: Neutral at midline with an estimated right atrial pressure at 5–10 mm Hg
Mitral Flow Deceleration Slope Index: greater than 2 ms/cm/s
Left Atrial Volume: Reduced compared to baseline
Left Ventricular End-Diastolic Dimension: Reduced by 20–30% compared to baseline
Aortic Valve Motion: Intermittent partial opening
Mitral Valve Regurgitation: Reduced compared to baseline
Tricuspid Valve Regurgitation: Reduced compared to baseline
Right Ventricle Size and Function: Stable or improved compared to baseline
noted that dobutamine stress testing has been tried in a device should be considered when reviewing either a
limited number of patients. Positive findings of enhanced transthoracic or transesophageal echocardiogram. Prior to
inotropic responsiveness combined with evidence of implantation, besides the usual information obtained in a
improved cardiac output were shown to be valuable comprehensive echocardiogram, one should pay parti-
predictors of explantation success in one study. cular attention to the presence of large atheromas in the
A more recent report from Dandel et al.44 suggested the ascending aorta, size of the ascending aorta, abnormalities
following echocardiographic parameters be considered, of the aortic valve, particularly in regard to significant
and if met, the patient might be considered for weaning. stenosis that might restrict leaflet motion, increasing
• The left ventricle should have an end-diastolic the difficulty of placing the device across the valve, or
diameter < 55 mm and an ejection fraction ≥ 45%. substantial aortic regurgitation, which might make the
• The right ventricle should not be dilated. device of little or no value. The left ventricular outflow
• Valvular insufficiency of all four valves should be either tract should be evaluated for possible unusual shape
not present or only mild. or narrowing, and the mitral valve should be evaluated
These factors are combined with right heart cathe- for baseline insufficiency and any unusual chordal
terization parameters. Recommended is a cardiac index abnormalities. During or immediately following positio-
> 2.6 L/min/m2, a pulmonary capillary wedge pressure ning of the device, appropriate location of the device
< 13 mm Hg, and a right atrial pressure < 10 mm Hg. across the aortic valve can be assessed echocardiographi-
If patients achieve this level of improvement of cally by noting the location of the device relative to the
performance and, importantly, show evidence of stable valve and also using color Doppler to verify inflow comes
maintenance of these changes over time, then patients from the left ventricle and outflow goes to the aorta
might be considered for a weaning protocol. (Figs 57.29A to C).45 Transesophageal echocardiography
can be particularly valuable in assessing the location and
PERCUTANEOUS CONTINUOUS function of the device.46 For example, failure of the device
to improve patient status could be related to malposition
FLOW DEVICES
of the device. One case report recently described distal
Impella Device positioning of the device with entrapment in the papillary
muscles, inhibiting outflow into the aorta. A 3D transes-
This device is devised for short-term implantation. The ophageal echo (TEE) was demonstrated to be of particular
device is inserted generally through a large femoral value in assessing the position of the device.47
artery and retrogradely brought into position across
the aortic valve so that the distal portion of the device
suctions blood out of the left ventricle, across the
TandemHeart
valve, and deposits the blood in the ascending aorta The TandemHeart (see Fig. 57.4) typically utilizes
(Fig. 57.3). Since this is a retrograde percutaneous device, a femoral vein and femoral artery with an external
several different considerations before placement of the pump.10 These devices are frequently placed using
A B
Figs 57.29A to C: (A) Parasternal long-axis view showing the

position of the Impella device (arrow); (B) Typical artifact generated
C by the device; (C) M-mode of the device and aortic valve leaflets.
A B
Figs 57.30A and B
Figs 57.30A to C: Subcostal view confirming cannula position in

the inferior vena cava (IVC), arrow, (A). Apical four-chamber view
confirming correct location of the cannula tip (arrow); (B). In some
cases the cannula may migrate; in this case (C) the tip moved into
C LV (arrow). (LA: Left atrium; RA: Right atrium; L: Liver).
echocardiographic imaging support, commonly trans- Congestive Heart Failure (REMATCH) Study Group. Long-
esophageal echocardiography. Preprocedure TEE term use of a left ventricular assist device for end-stage
heart failure. N Engl J Med. 2001;345(20):1435–43.
screening is of considerable value to evaluate not only
4. Nose Y, Motomra T, Miyamoto H. History of Mechanical
issues of ventricular and valvular function but also to Circulatory Support. In: Joyce DL, Joyce LD, Loebe M,
carefully evaluate the left and right atrial chambers for editors. Mechanical Circulatory Support. New York, NY:
any unusual anatomical abnormalities or thrombi that McGraw Hill Medical; 2012:3–12.
would complicate placement of the large-bore catheter 5. Interagency Registry for Mechanically Assisted Circulatory
Support. Quarterly Statistical Report 2012, 3rd Quarter.
moved retrograde up through the vena cava and then
Available from www.intermacs.org. 2012; Accessed January
trans-septally across the atrial septum. TEE is frequently 2013.
used for guidance of the trans-septal puncture, and 6. Mancini DM, Beniaminovitz A, Levin H, et al. Low incidence
once the device is placed for correct positioning of the of myocardial recovery after left ventricular assist device
retrograde catheter in the left atrium. Color Doppler can implantation in patients with chronic heart failure.
Circulation. 1998;98(22):2383–9.
assist in detecting flow since the device has multiple
7. Bruckner BA, Stetson SJ, Farmer JA, et al. The implications
holes at the end of its inflow catheter (see Figs 57.4A for cardiac recovery of left ventricular assist device
and B). As with other assist devices, echocardiography can support on myocardial collagen content. Am J Surg. 2000;
serially evaluate the effect of the device. Also, if the device 180(6):498–501; discussion 501.
appears to be working improperly, echocardiographic 8. Bruckner BA, Stetson SJ, Perez-Verdia A, et al. Regression of
fibrosis and hypertrophy in failing myocardium following
imaging, particularly to evaluate the position of the inflow
mechanical circulatory support. J Heart Lung Transplant.
cannula, may be of great value (Figs 57.30A to C and Movie 2001;20(4):457–64.
clips 57.16 to 57.17, 57.18). In some circumstances, this 9. Abiomed, product description. Available at www.abiomed.
cannula could migrate either further inward or backward com. 2013; Accessed January 2013.
across the atrial septum and become malpositioned. These 10. Burkhoff D, Cohen H, Brunckhorst C, et al. A randomized
multicenter clinical study to evaluate the safety and
catheters also can form clots and sometimes become efficacy of the TandemHeart percutaneous ventricular
obstructed, which echocardiography can identify. assist device versus conventional therapy with intraaortic
balloon pumping for treatment of cardiogenic shock. Am
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33. Soleimani B, Haouzi A, Manoskey A, et al. Development of
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26. Kukucka M, Stepanenko A, Potapov E, et al. Right-to-left II Clinical Investigators. Young patients with nonischemic
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right ventricular failure with continuous-flow left ventricular recovery during left ventricular assist device support.
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42. Mano A, Nakatani T, Oda N, et al. Which factors predict 45. Catena E, Milazzo F, Merli M, et al. Echocardiographic
the recovery of natural heart function after insertion of evaluation of patients receiving a new left ventricular
a left ventricular assist system? J Heart Lung Transplant. assist device: the Impella recover 100. Eur J Echocardiogr.
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43. Osaki S, Sweitzer NK, Rahko PS, et al. To explant or not to
46. Patel KM, Sherwani SS, Baudo AM, et al. Echo rounds: the
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to determine the need of continued ventricular assist use of transesophageal echocardiography for confirmation
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44. Dandel M, Weng Y, Siniawski H, et al. Pre-explant stability 2012;114(1):82–5.
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outcome after weaning from ventricular assist devices. dimensional echocardiography in Impella placement.
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CHAPTER 58
Echocardiographic Assessment of
Left Atrial Function
Utpal N Sagar, Hirohiko Motoki, Allan L Klein
Snapshot
Anatomy
¾¾ Functional Assessment
¾¾
Physiology
¾¾ ¾¾Left Atrial Pathophysiology
INTRODUCTION and is varied in the number of lobes which comprise its

structure. The wall of the appendage also has variable
As the role of echocardiography has evolved to assess thickness, with alternating muscle bundles. The vestibule
hemodynamic status and diastolic function in addition to of the LA includes the region around the mitral valve
characterizing two-dimensional structure and function, orifice and is noted for its generally smooth endocardial
there has been an emphasis on improving echocardi
surface. Encompassing the posteroinferior wall of the
ographic assessment of the left atrium (LA). This has
LA is the mitral isthmus, which extends between the
become of increasing importance as left atrial volume
entrance of the left inferior pulmonary vein and the mitral
and function have been described as strong predictors
valve. Proximally, the vestibule merges with the septal
of major adverse cardiovascular events. In this chapter,
component, which joins with the posterior wall and the
we will review the structure and multifaceted function of
roof of the LA. The fossa ovalis, which is a remnant of the
the LA, the physiology of the LA, modalities of functional
embryonic septum primum, may be seen from the left
assessment, and review left atrial function in the context of
atrial aspect of the septum as a crescent-shaped edge.
various cardiovascular disease states.
The posterior portion of the LA into which the pulmonary
veins drain is referred to as the venous component. There
ANATOMY are small ridge-like structures between the entrance of the
The LA is the most posteriorly situated chamber of the superior and inferior pulmonary veins, and a larger ridge
heart and is oriented superiorly and to the left of the right between the left atrial appendage and the entrance to the
atrium. The pulmonary veins normally drain into the left atrial appendage.1
posterior aspect of the LA. These vessels are covered by the The walls of the LA that border the regions of the LA
visceral or inner layer of pericardium. The serous layer is described previously are not of uniform thicknesses. Most
fused with the outer fibrous pericardium.1 notably, the wall surrounding the venous portion of the
The LA may be divided into different regions, LA is composed of varying amounts of musculature with
including the left atrial appendage which is small, tubular, differing orientations.1
Fig. 58.2: Graphical representation of the pressure–volume

relationship of left atrial (LA) function. Note the dynamic changes
in the pressure and volume that occur during the cardiac cycle.
(MVC: Mitral valve closure; MVO: Mitral valve opening; Pre-A:
Pre-atrial contraction).
decrease in pressure. The LA then receives blood that

returns via the pulmonary veins. The difference between
the passive LA emptying volume and the pulmonary blood
flow is the reservoir volume. The next phase of left atrial
function is the conduit phase in which the LA conducts
blood into the left ventricle with the opening of the mitral
valve and occurs until left atrial contraction. Essentially,
the mitral valve annulus descends toward the cardiac base,
Fig. 58.1: Left atrial (LA) phasic functions and their relationship decreasing LA volume. This volume may be determined
with the cardiac cycle. (TMF: Transmitral flow; PVF: Pulmonary
vein flow, ECG: Electrocardiogram). Note the changes in left atrial by the difference in LV stroke volume and the sum of the
volumes through the various phases of left atrial function. active and passive LA volumes. In the final phase, the left
atrial pectinate muscles contract in late diastole with an
increase in LA pressure, and development of a pressure
gradient between the LA and LV, resulting in blood flow
PHYSIOLOGY across the mitral valve. This so-called “atrial kick” has a
Although the role of the LA could be simply described as a significant contribution to the stroke volume of the left
chamber that receives oxygenated blood, its function is far ventricle and may be defined as the difference in the left
more complex. Below, we will discuss the phasic function atrial volume at the onset of the P-wave and minimal
of the LA and the factors that affect its function. LA volume.2 Figure 58.2 graphically demonstrates this
relationship of left atrial pressure and volume throughout
the cardiac cycle, and its relation to left ventricular filling.
Phasic Left Atrial Function The LA also functions as a volume and pressure sensor
Broadly, there are three phases of left atrial function, of diastolic function. And, through the release of natriuretic
including the reservoir, conduit, and contraction phases peptides and interactions with the sympathetic nervous
(Fig. 58.1). The LA first acts as a reservoir during left system, as well as the renin–angiotensin–aldosterone
ventricular systole as the mitral valve annulus is displaced system, it communicates with various neurohormonal
apically, augmenting LA volume with a concomitant systems.3
Chapter 58: Echocardiographic Assessment of Left Atrial Function 1257
Physiological Effects on pressures over time,5,6 making it a useful marker of both

the chronicity and severity of LV diastolic dysfunction.7,8
Left Atrial Function Left ventricle size measurement is routinely perfor
It follows that the previously described left atrial function med by transthoracic echocardiography (Figs 58.3A to E).
do not occur in isolation and are related to left ventricular LA antero posterior dimension can be measured by
compliance. Abnormalities in left ventricular, valvular, or M-mode or B-mode in the parasternal long-axis view. This
atrial disease are often reflected as an increase in left atrial method is convenient and has been widely adopted in
filling pressures, which may be observed as enlargement routine clinical practice. However, LA volume measured
of left atrial size.4 Hence, LA afterload increases as by either the ellipsoid model or the Simpson’s method
left ventricular (LV) filling pressures increase and as is a more reliable measure of true LA size than M-mode
LV diastolic dysfunction worsens. This increase in LA LA dimension9 and is the recommended method for the
afterload and LA volume results initially in an increase in accurate assessment of LA size.10 Measuring the maxi
LA size and an improvement in LA function. However, LA mum LA volume at the time of mitral valve opening
contractility declines once a threshold has been reached, is now routinely performed with echocardiography,
similar to the Frank–Starling curve of the LV.2 Various although it only represents a snapshot of LA function at
examples of the relationship between LA and LV size, and a specific point of the cardiac cycle. Maximal LA volume
functional assessment will be discussed in the left atrial index is a predictor of adverse cardiovascular outcomes
pathophysiology section of this chapter. such as AF, stroke, and congestive heart failure and/or
death in various conditions, including community-based
FUNCTIONAL ASSESSMENT populations,11–14 postmyocardial infarction,15,16 heart
We have already described the significant interplay failure,17–19 hypertrophic cardiomyopathy,20–22 AF,23 and
between LA and LV function, such that events during postcardiac surgery patients.24 However, measurement
each phase of “LA phasic function” are affected by factors of LA phasic volumes using 2DE is time consuming, and
from both the LA and LV. However, despite considerable errors can arise from geometric assumptions of biplane
investigation, the magnitude and relative importance volume calculations, as well as from difficulties with
of the atrial contribution to LV filling and cardiac output echocardiographic window and the timing of various atrial
remain controversial, and provide a motivation for a more events.
complete evaluation of the atrial cycle. To improve the accuracy of LA size measurement,
Recent advances in catheter ablation for the treat 3DE has been studied (Fig. 58.4). The 3DE measurements
ment of atrial fibrillation (AF), in dual- and three- demonstrate favorable test–retest variability25 and good
chamber pacemakers that maintain atrioventricular and agreement with cardiac magnetic resonance imaging.25–27
biventricular synchrony, and in the pathological and Among the newer techniques including 3DE, cardiac
clinical relevance of chamber-specific structural, elec computed tomography, and cardiac magnetic resonance
trical, and ionic remodeling have increased the interest imaging, 3DE shows the most promise of adoption in
in accurately imaging the LA structure and function. routine clinical practice as it is noninvasive, readily
With respect to the assessment of the LA function, two-
available, and can be added onto the routinely performed
dimensional echocardiography (2DE), three-dimensional
2DE examination. 3DE also offers the possibility of meas
echocardiography (3DE), Doppler echocardiography,
uring LA volumes at different phases of the cardiac cycle,
and speckle tracking echocardiography have distinctly
yielding information on LA phasic function.
different strength and weaknesses, and are complementary
in specific clinical scenarios. In this section, we discuss
the role of each modality to assess LA function with Spectral Doppler
an emphasis on the relative merits of newer imaging Pulmonary vein flow, transmitral flow, and mitral annular
techniques and how these may be applied in the various velocity are routinely measured. These parameters are
clinical settings. determined by LA function, as well as LV systolic and
diastolic performance. Peak velocity of mitral A-wave
Volume indicates LA mechanical function, although it is also
Left atrial size has been compared to the hemoglobin A1c affected by heart rate and loading conditions.28 Peak
in diabetes as a measure of the average effect of LV filling mitral A velocity has been shown to be associated with
A B
C D E
Figs 58.3A to E: Various methods of echocardiographic determination of left atrial size. Figure A demonstrates M-mode through the
left atrium (LA), Figures B and C demonstrate volume measurements of the LA using the biplane method of discs (modified Simpson’s
rule) from apical four-chamber and two-chamber views, respectively. Figures D and E demonstrate the measurement of LA volume from
area–length method using the images from apical four-chamber and two-chamber views.
atrial mechanical function30 and has been shown to be

prognostic for cardiovascular events in a population with a
high prevalence of hypertension and diabetes,31 although
the method is limited by the robustness and variability of
the measurements.
Mitral annular velocity during atrial contraction (a') is
another indicator of atrial contractile function, measured
by tissue Doppler imaging (TDI) of the mitral annulus to
quantify the low-velocity, high-amplitude myocardial
velocities. Its amplitude is related to both atrial contractility
and LV end-diastolic pressure.32 Increased a' is also seen
Fig. 58.4: Multiplanar imaging of the left atrium, with three-dimen- in those with LV hypertrophy, indicating increased LA
sional rendering and determination of the left atrial volume.
active ejection force.33 Decreased a' has been shown to
be a predictor for elevated pulmonary pressures in mitral
AF recurrence postcardioversion.29 Atrial ejection force, regurgitation, clinical deterioration in aortic stenosis, AF
calculated from the mitral annulus area and mitral postcoronary artery bypass grafting, and progression from
A-wave velocity, has been suggested to be a useful paroxysmal to persistent nonvalvular AF. In heart failure,
of AF, left ventricular systolic dysfunction, and clinical

heart failure, the role of LA functional assessment and its
pathophysiological applications has continued to evolve.
In this section, we will discuss the role of left atrial
functional assessment in various clinical settings.
Hypertension
The left ventricular hypertrophy and LA dilation that
is observed in patients with moderate and severe
hypertension often is not observed in patients with
mild hypertension. Earlier in this chapter, we noted
that strain and strain rate (SR) imaging may be useful in
characterizing the components of LA phasic function. In
the setting of mild hypertension, SR imaging may show a
reduction in the early diastolic LA SR, which is associated
Fig. 58.5: An example of speckle tracking, used here to determine
with a decrease in LA conduit volume. These changes may
left atrial strain and strain rate. reflect early LV diastolic dysfunction, which may herald
the development of LV hypertrophy and LA dilation, and
low a' is associated with poor exercise tolerance and is a possibly AF and clinical symptoms.34
better predictor of cardiac events than E/e' and decele
ration time of transmitral E-wave. Atrial Fibrillation
Speckle Tracking Impairments in LA structure and function may commonly
lead to AF. Various echocardiographic modalities have
Measures of myocardial deformation have been been studied to evaluate the degree of LA fibrosis that
increasingly adapted to study LA mechanics. Both echo has been associated with AF. Atrial conduction time
cardiographic methods of measuring strain and SR, 2D as measured by TDI has been shown to predict the
speckle tracking imaging, and color TDI have been adapted development of AF in the general population when
to measuring LA deformation. Speckle tracking calculates prolonged greater than 190 ms.35 Recently, percutaneous
strain by tracking tissue deformation via characteristic catheter ablation has been used more frequently to
myocardial speckles frame-by-frame, with SR given by the successfully manage symptomatic and medically refractory
rate of such deformation (Fig. 58.5). Color TDI generates AF. In these patients, the best predictors for maintenance
a spatial map of myocardial velocities, from which SR of of sinus rhythm following ablation were parameters of LA
the region of interest is derived, with strain calculated by reservoir function, determined by SR imaging.36,37 Studies
integrating the SR data. have shown that LA reservoir function has been related to
The advantage of analyzing LA mechanics with strain LA structural remodeling and fibrosis of the atrial wall.38,39
and SR imaging is the information that can be obtained Following conversion to sinus rhythm, LA volume and
about each component of LA phasic function. One could function may be monitored, with those patients who have
use this method to resolve the exact change in LA phasic greater degrees of dysfunction being selected for further
function with different disease states and investigate the therapy, such as antiarrhythmics.40
effect after treatment. Direct current cardioversion is a widely used treatment
Thus, deformation-based parameters of LA reservoir modality for both emergent and elective manage
function could provide the prognostic information in the ment of AF. However, there is significant risk of stroke
population at risk for adverse cardiovascular events. and thromboembolism associated with direct current
cardioversion.41–44 Classically, this was thought to be
due to pre-existing left atrial thrombus or continuous
LEFT ATRIAL PATHOPHYSIOLOGY or prominent spontaneous echo contrast. Earlier, we
While left atrial size has been shown to have a significant described the location and anatomy of the left atrial
prognostic role in the prediction of the development appendage. Transesophageal echocardiography (TEE) is
the best method of detection of thrombi in the left atrial cardioversion.47,48 TEE-guided cardioversion may be impo
appendage and LA that could be embolized in the setting rtant with the use of the new oral anticoagulants such as
of cardioversion (Figs 58.6A to C). However, significant dabigatran, rivaroxaban, and apixaban since there is no
work in this area has described myocardial stunning that anticoagulation monitoring in patients with AF.
follows cardioversion, giving rise to conditions in the LA
that may lead to thrombus, even if no LA thrombus was Cardiomyopathies
present prior to cardioversion.45 Nevertheless, TEE prior Assessment of LA function may help to diagnose, to
to cardioversion has become standard of care, and has differentiate, and to guide therapy of various cardio
been studied extensively.45,46 Techniques have evolved myopathies. For example, patients with hypertrophic
now to involve the functional assessment of the left atrial cardiomyopathy have been shown to have decreased
appendage, evaluating emptying velocities with pulsed LA longitudinal function in addition to decreased LA
wave Doppler at the entrance of the appendage. Peak reservoir function. This may be used to differentiate
LA appendage emptying velocity has been shown to be hypertrophic cardiomyopathy from other forms of left
a predictor of maintenance of sinus rhythm following ventricular hypertrophy.49,50 Figures 58.7A to E show an
A B C
Figs 58.6A to C: The spectrum of left atrial appendage pathology. Figure A shows the presence of left atrial spontaneous echocontrast,
or “smoke”; Figure B demonstrates prominent, persistent spontaneous echocontrast in the left atrium and left atrial appendage (LAA),
consistent with “sludge”; Figure C shows the presence of a minimally mobile echodensity within the left atrial appendage, consistent
with thrombus (arrow).
A B C
D E
Figs 58.7A to E: Patient with hypertrophic obstructive cardiomyopathy (HOCM), as seen in Figure A. Figures B and C demonstrate
severe left atrial enlargement. Mitral inflow shows decreased A-wave (Figure D) and tissue Doppler image (Figure E) shows moderate
to severe decreased atrial contraction with decreased a' velocity. (LA: Left atrium; LV: Left ventricle).
A B
C D
Figs 58.8A to D: Echocardiogram of a patient with cardiac amyloidosis. The apical four-chamber view in Figure A demonstrates the
degree of left atrial enlargement; Figure B shows the degree of increased left ventricular wall thickness and the characteristic echotex-
ture of a patient with cardiac amyloidosis. The transmitral flow demonstrating restrictive diastolic pattern with small A-wave is seen in
Figure C. Tissue Doppler in Figure D shows severely depressed a' velocity, consistent with restrictive cardiomyopathy. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
echo-Doppler evaluation of LA function in a patient 2. Blume GG, McLeod CJ, Barnes ME, et al. Left atrial function:
with HOCM. Also, in patients with cardiac amyloidosis, physiology, assessment, and clinical implications. Eur
we see that the LA is severely dilated and a' is reduced, J Echocardiogr. 2011;12:421–30.
conferring a high risk for thromboembolism. Figures 58.8A 3. To AC, Flamm SD, Marwick TH, et al. Clinical utility of
multimodality LA imaging: assessment of size, function,
to D show an echo-Doppler evaluation of LA function in
and structure. JACC Cardiovasc Imaging. 2011;4:788–98.
cardiac amyloidosis. In a study of a population of patients
4. Leung DY, Boyd A, Ng AA, et al. Echocardi ographic
with ischemic and idiopathic cardiomyopathy, LA evaluation of left atrial size and function: current under
reservoir function predicted a positive response to cardiac standing, pathophysiologic correlates, and prognostic
resynchronization therapy, although the study did not implications. Am Heart J. 2008;156:1056–64.
report strain and SR for the other phases of LA function.51 5. Abbas AE, Fortuin FD, Schiller NB, et al. A simple method
for noninvasive estimation of pulmonary vascular resis
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CHAPTER 59
The Use of Echocardiography to
Assess Cardiac Hemodynamics and
Guide Therapy
Roy Beigel, Robert J Siegel
Snapshot

Right Atrial Pressure/Central Venous Pressure 
AddiƟonal Parameters for EsƟmaƟon of

Pulmonary Artery Hemodynamics LeŌ Atrial Pressure

LeŌ-Sided Filling Pressures 
Stroke Volume, Stroke Distance, Cardiac Output and
Systemic Pulmonary Shunts (QP/QS)
INTRODUCTION RIGHT ATRIAL PRESSURE/CENTRAL

Accurate noninvasive hemodynamic assessment has the VENOUS PRESSURE
potential to greatly improve patient management with
Central venous pressure (CVP; Table 59.1) and RAP are
regard to volume status, pharmacological treatment,
the same, provided that there is no obstruction of the
and clinical outcomes. Several studies have shown that
vena cava. Traditionally, RAP is measured with a central
the “time-honored physical examination” has very
limited sensitivity and specificity for right atrial pressure venous catheter (normal range is between 1 and 7 mm Hg
(RAP), pulmonary artery pressure (PAP), as well as for mean RAP). Elevated values have adverse prognostic
left atrial (LA) filling pressures. Since the 1970s, the implications for morbidity and mortality,6–9 making
standard for hemodynamic assessment has been invasive the accurate assessment of RAP an important factor in
measurements made by pulmonary artery (PA) cathete- patients’ assessment, management and outcome.10,11
rization. However, use of the PA catheter has been subject Accurate evaluation of RAP is also a necessary for the
to criticism1–3 as it can increase patient morbidity.4,5 noninvasive estimation of the systolic and diastolic PAP
Doppler-echocardiographic measurements of right- and (DPAP). Table 59.1 lists various methods used for the
left-sided filling pressures, pulmonary vascular resistance echocardiographic evaluation of RAP.
(PVR), and cardiac output (CO) are possible to obtain in
most patients. Echocardiography can potentially provide Inferior Vena Cava Parameters (Fig. 59.1)
adequate alternative hemodynamic data, which are more
accurate than the physical examination without the risks The most commonly utilized method uses the inferior
of invasive monitoring. vena cava (IVC) size and its respiratory variation for
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy 1265
Table 59.1: The Various Methods Utilized for the Echocardiographic Evaluation of Right Atrial Pressure (RAP)
Method Criteria Used Strength and Limitations
IVC parameters—2D or M-mode IVC diameter, collapse: Most validated method
subcostal imaging of the IVC • < 2.1 cm, > 50%—normal Above a certain elevation of RAP, the IVC may be fully
(Fig. 59.1) RAP ~ 3 dilated and not collapsing, making estimation above
• > 2.1 cm, > 50%—intermedi- this point difficult
ate* RAP ~ 8 Increased IVC diameter and/or decreased collapse in
• > 2.1 cm, < 50%—High the presence of RAP can be seen with:
RAP ~> 15 • Low respiratory compliance
*In cases which the IVC diameter • Mechanically ventilated patients
and collapse do not fit the normal • Trained athletes
or high criteria. • Prominent Eustachian valve
• Narrowing of the IVC–RA junction
• Web or tissue present in the IVC
Systemic and hepatic venous flow— Vs > Vd—normal RAP Obtaining flow velocity curves from the SVC is simple,
Doppler flow in the vena cava, Vs < Vd—elevated RAP less obtainable with hepatic veins
jugular, or hepatic veins (> 8 mm Hg) Severe tricuspid regurgitation can alter the venous
(Figs 59.2A and B) flow pattern without correlation to RAP
Atrial compliance and relaxation and tricuspid
annular descent can affect flow patterns and make
them less reliable
Atrial fibrillation or past cardiac surgery can cause the
hepatic vein systolic flow to be diminished regardless
of RAP
Hepatic vein filling fraction VsVTI/(VsVTI + VdVTI) Validated in mechanically ventilated patients
(HVFF)—pulsed Doppler of < 55—High RAP > 8 mm Hg Single study24
hepatic veins Atrial fibrillation or past cardiac surgery can cause
the hepatic vein systolic flow to be diminished
regardless of RAP
Doppler and TDI—pulsed Doppler E/e′ > 6—RAP > 10 mm Hg Validated in mechanically ventilated patients
of the tricuspid inflow and TDI of the May not be an accurate method in patients who
tricuspid valve (Figs 59. 3A and B) have undergone cardiac surgery
Right ventricular regional > 59 ms correlates to Studied on a limited number of patients (n = 21)
isovolumic relaxation time RAP > 8 mm Hg in a single study25
(RV rIVRT)—tricuspid TDI
3D RA dimensions—3D 3DE maximal RA volume Single study in a selective group of heart failure
transthoracic imaging of the RA ≥ 35 mL/m2 combined with IVC patients with EF < 35%27
diameter ≥ 2 cm—correlates with
RAP > 10 mm Hg
(3DE: Three-dimensional echocardiography; IVC: Inferior vena cava; RA: Right atrial; SVC: Superior vena cava; TDI: Tissue Doppler
imaging; VTI: Velocity time integral).
the echocardiographic evaluation of RAP. As the IVC is systolic flow, reduced IVC collapse with inspiration and
a highly compliant vessel, its size and flow dynamics eventually IVC dilatation (Fig. 59.1). Current guidelines14,15
vary with changes in CVP and volume. As shown in recommend using the IVC maximal diameter (IVC max)
Figure 59.1, during inspiration (which produces negative 1–2 cm from the RA–IVC junction at end-expiration
intrathoracic pressure), vena cava pressure decreases and and the IVC collapsibility index (IVCCI, which equals
flow increases.12,13 At low or normal RAP, there is systolic [IVCmax – IVCmin]/IVCmax). For RAP assessment, as noted in
predominance in IVC flow, such that the systolic flow Table 59.2, an IVC with a diameter < 2.1 cm and collapse
is greater than the diastolic flow. As RAP increases, it is > 50% correlates with a normal RAP of 0 to 5 mm Hg. An
transmitted to the IVC, resulting in blunting of the forward IVC < 2.1 cm with < 50% collapse and an IVC > 2.1 cm with
> 50% collapse correspond to an intermediate RAP of 5 to

10 mm Hg. An IVC > 2.1 cm with <50% collapse suggests
a high RAP of 15 mm Hg. Using midrange values of
3 mm Hg for normal and 8 mm Hg for intermediate RAP
is recommended. However, if there is minimal collapse of
the IVC (<35%) and/or secondary indices of elevated RAP
are present, upgrading to the higher pressure limit (i.e. 5
and 10 mm Hg in case of normal and intermediate RAP,
respectively) should be done. Patients should be supine
during assessment of the IVC as other positions may lead
to either under- or overestimation of IVC diameter and/
or collapsibility.16 Patients with low compliance with deep
inspiration may have a diminished IVC collapse. A “sniff ”
maneuver that causes a sudden decrease in intrathoracic
pressure and by that accentuating the normal inspiratory
response can be used to differentiate those with normal
IVC collapsibility from those with a diminished IVC
collapsibility.
IVC size and collapsibility are helpful to identify RAP
as being high or low, but this method does not provide
precise numeric values for RAP. It should be noted that
the IVC can be dilated in individuals with a normal RAP.
Fig. 59.1: Imaging of the inferior vena cava (IVC, marked with Common causes of a dilated IVC in the setting of normal
asterisk) using 2D echocardiography (top, and middle images), RAP17,18 are listed in Table 59.1.
and M-mode echocardiography (bottom images) from the
subcostal view. The left three images show respiratory variations In order to overcome some of the limitations of RAP
of the IVC in a patient with normal right atrial pressure (RAP). The estimation through IVC indices, additional Doppler-
right three images show no respiratory variation of the IVC, which echocardiographic parameters have been evaluated and
is also dilated. This patient was found to have an elevated RAP.
proposed to better quantify RAP (Table 59.1).
Table 59.2: The Various Methods Utilized for the Echocardiographic Evaluation of Pulmonary Artery Pressure (PAP)
Systolic Pulmonary Artery Pressure (SPAP):
Using the TR jet—Simplified ΔP = 4 × V2 + RAP = SPAP Widely validated
Bernoulli equation (Fig. 59.5) Simple equation
Underestimation can occur if:
• RAP underestimated
• Misalignment of Doppler signal
• Poor TR signal
• Severe TR
Overestimation can occur if:
• RAP overestimated
• Mistakenly using the tricuspid valve closing
spike
Pulmonary flow acceleration Lesser range of 100 ms indicates • Not widely studied
time (Fig. 59.6) elevated SPAP • Validated only in patients with chronic heart
failure
• Measurements can be affected by extremes of
heart rate (below 60 or above 100 beats per minute)
Contd....
Contd....
Diastolic Pulmonary Artery Pressure (DPAP):
Using the pulmonary ΔP = 4 × V2 + RAP = DPAP Simple equation
regurgitation (PR) Not widely validated
jet—Simplified Bernoulli PR jet not always acquirable
equation (Fig. 59.9) Underestimation can occur if:
• Poor PR signal
TR velocity at time of ΔP = 4 × V2 + RAP = DPAP TR jet more detectable then PR
pulmonic valve Not widely validated
opening—Simplified Underestimation can occur if:
Bernoulli equation • RAP underestimated
• Poor PR signal
• Severe PR
Mean Pulmonary Artery Pressure (MPAP):
Using the peak pulmonary ΔP = 4 × V2 + RAP = MPAP Simple equation
regurgitation (PR) Not widely validated
jet—Simplified Bernoulli PR jet not always acquirable
equation (Fig. 59.9) Underestimation can occur if:
• Poor PR signal
Tracing the TR jet (Fig. 59.10) Validated in a single study49
Easy to obtain
Empirical formulas MPAP = 0.61 SPAP + 2 mm Hg Validated only invasively51
MPAP = DPAP + 1/3 (SPAP-DPAP) Validated echocardiographically in a single study43
Not widely validated
Same limitations as SPAP and DPAP
PVR:
Formula using surrogates for PVR (WU) = 10 × TR Not widely validated
the transpulmonary pressure velocity/RVOT VTI + 0.16 Underestimation can occur if:
gradient and transpulmonary • Misalignment of Doppler signal
flow • Poor TR signal
• Severe TR
• RVOT not well visualized in patients
with poor acoustic window
Formulas using the pre-ejec- PVR = –0.156 + 1.154 × [(PEP/AcT)/TT] Single study of heart failure patients with EF
tion period (PEP), AcT, and < 35%, in sinus rhythm59
total systolic time (TT)
Estimation of the PVR PVRI = 1.97 + 190 × Single study of patients with PH60
index (PVRI) [SPAP/(HR × RVOT VTI)]
SPAP/(HR × RVOT VTI) >0.076 correlated
with severe pulmonary vascular disease
with PVRI > 15 WU/m2
(PH: Pulmonary hypertension; PVR: Pulmonary vascular resistance; RAP: Right atrial pressure; RVOT: Right ventricular outflow tract;
TR: Tricuspid regurgitation; VTI: Velocity time integral).
Systemic Venous Flow (Figs 59.2A and B) integrals (VTIs): VsVTI/(VsVTI + VdVTI), can be obtained.
A value < 55% was found to be the most sensitive (86%)
The central venous Doppler flow pattern seen in the vena and specific (90%) sign of an RAP > 8 mm Hg. With higher
cava, jugular, and hepatic veins is characterized as seen by RAP, there was a decrease in systolic filling fraction.24 In
three distinct waveforms.19 The first is the systolic wave (Vs) this single study, the best model for prediction of mean
caused by RA relaxation and descent of the tricuspid ring RAP was = 21.6 – 24 × HVFF. Although IVC collapsibility
associated with right ventricular (RV) systole. The second cannot be evaluated in mechanically ventilated patients,
is the diastolic wave (Vd), which occurs during rapid hepatic vein flow velocities have been validated in this
ventricular filling when the tricuspid valve is open. The third situation,24 provided that the velocities are averaged over
is a positive A-wave, which occurs with RA contraction and ≥5 consecutive beats and comprising ≥1 respiratory cycle.15
represents reverse flow. The A-wave is small and might not The maximal early filling velocity through the tricuspid
be present in normal individuals. In the majority of normal valve during diastole (E-wave) increases as the RAP rises.
adults, inspiration increases the magnitude of Vs and Vd, The use of tissue Doppler imaging (TDI) allows recording
whereas the A-wave, if present, decreases in amplitude. At of myocardial and annular velocities, and can measure the
low or normal RA pressures, there is systolic predominant velocity of tissue relaxation of the lateral tricuspid annulus
venous flow, such that the velocity of Vs is greater than the in diastole (e'-wave) (Figs 59.3A and B). It has been shown
velocity of Vd (Figs 59.2A and B). With elevation of the RA that there is a relationship between RAP and the E/e' ratio:
pressure, the systolic flow predominance is lost, such that A high E velocity combined with a low e' giving an E/e' ratio
Vs is substantially decreased and Vs/Vd is < 1. The higher of > 6 was found to be predictive of a RAP > 10 mm Hg. This
the RAP the lower the pressure gradient between these correlation was found to also be accurate in patients on
veins and the RA causing diminished forward systolic flow. mechanical ventilation. However, this method may not be
This blunted gradient is present in patients with restrictive an accurate in patients who have undergone prior cardiac
heart disease and elevated right-sided filling pressures.20–23 surgery.
The RV regional isovolumic relaxation time (RV rIVRT)
Doppler and Tissue Doppler is the time period between the end of systolic annular
motion and the onset of the e'-wave upon TDI of the lateral
Imaging (Figs 59.3A and B) tricuspid annulus as evaluated in the apical four-chamber
Using pulsed wave Doppler, the hepatic vein systolic filling view. Using this index, it was found that an RV rIVRT of
fraction (HVFF), which is the ratio of the velocity time < 59 ms corresponds to a RAP > 8 mm Hg.25
A B
Figs 59.2A and B: Evaluation of the central venous flow pattern using pulsed wave Doppler imaging. (A) Systolic (S) and diastolic (D)
flow in the superior vena cava of an adult with a normal biphasic pattern. The S/D > 1 is supportive of normal right atrial pressure (RAP);
(B) S and D flow velocity in the hepatic vein in a patient with elevated RAP. There is diminished S, increased D, and an S/D < 1.
A B
Figs 59.3A and B: Doppler and tissue Doppler imaging (TDI) of the tricuspid valve. (A) Tricuspid inflow velocity Doppler recording
(E = 36.1 cm/s); (B) Tricuspid annular velocity, e , is 5.2 cm/s. In this patient, the E/e ratio is >6, which supports that the RAP is
>10 mm Hg.
Doppler and TDI provide an alternative for RAP

evaluation when subcostal views cannot be obtained and
when there is inability to assess the IVC and hepatic indices.
They can also be used to corroborate the prediction of RAP
using HVFF in patients on mechanical ventilation where
IVCCI is inaccurate.26
RA Dimensions (Fig. 59.4)

Chronically elevated RAP generally leads to RA enlarge-
ment. RA size and volume can be assessed from many two-
dimensional echocardiographic (2DE) views but it is most
commonly measured in the apical four-chamber view15
(Fig. 59.4). Three-dimensional echocardiography (3DE)
provides tomographic imaging of cardiac chambers and
Fig. 59.4: Measuring right atrial (RA) dimensions. 2D echocardio-
has the potential to be a more accurate modality for atrial graphic images from the apical view focusing on the right heart.
volume quantification than 2DE. In one study,27 3DE RA On the left, the measurements of the RA major and minor axis
volume correlated with RAP (r = 0.51, p < 0.001) in heart diameters are estimated at 4.87 and 3.07 cm, respectively. On the
failure (HF) patients. Conversely, 2DE measurements of the right, the RA area tracing, which is 13.7 cm2.
RA (both size and 2DE RA volume) have not been shown
to correlate with RAP.24 Compared to the American Society available is to yield even more accurate estimation of
of Echocardiography (ASE) recommendations of using an RAP. It is likely that the combination of measurements
IVC diameter of ≥ 2 cm and decreased respiratory collapse using echocardiographic findings that measure dynamic
of <40%, 3DE measured maximal RA volume of ≥35 mL/m2 changes, flow, and dimensions will provide the best
combined with an IVC diameter ≥ 2 cm improves the noninvasive assessment of RAP.
sensitivity in identifying RAP > 10 mm Hg.27
Currently, there is no single ideal parameter for PULMONARY ARTERY
noninvasive RAP estimation. Using the 2010 ASE
criteria,15 which is based on IVC parameters, RAP can
HEMODYNAMICS (TABLE 59.2)
be categorized as low (0–5), normal (6–10), or elevated Pulmonary arterial hemodynamics are important for
(11–20). A multiparameter approach from other methods patient diagnosis management, and prognosis. Current
echo-Doppler modalities allow evaluation and estimation can be due to variations in Doppler angle of interrogation,
of numerous parameters from the pulmonary vasculature: underestimation of RAP (especially when it is very
systolic PAP (SPAP), DPAP, and mean PAP (MPAP) as well elevated; Figs 59.7A and B), the presence of severe TR, or a
as indirect estimation of other parameters such as PVR. poor TR signal.15,37 Overestimation of SPAP is less common
and often is due to overestimation of RAP; it can also
Systolic Pulmonary Artery Pressure result from overestimating the TR signal peak velocity, as
The SPAP is equal to the RV systolic pressure in the well as mistakenly using the tricuspid valve closing spike
absence of pulmonary valve stenosis or other RV outflow due to tricuspid valve closure for the tricuspid maximal
tract (RVOT) obstruction. The normal value for the SPAP velocity. Nonetheless, Doppler estimation of SPAP is used
with invasive measurements is between 15 and 30 mm Hg. as a standard reliable method for screening patients for
The SPAP equals the pressure gradient between the PA suspected PH. A TR velocity of >2.8 m/s (corresponding to
and the RV plus the RAP. The pressure gradient (ΔP) can a pressure gradient of 31 mm Hg) is regarded as the cutoff
be calculated using the Bernoulli equation: ΔP = 4 × V2, velocity to define elevated SPAP. However, in obese38 and
where V is the velocity of the tricuspid regurgitation (TR) elderly39 patients, the “normal” cutoff may be higher.
jet in cm/s (Fig. 59.5). In apparently healthy individuals, To reduce false-negative results, multiple imaging
the prevalence of TR upon Doppler echocardiography planes and color Doppler should be used for optimal align-
varies within a range of 20 to 94% (depending on the age ment with the regurgitant jet. In cases with a poor Doppler
of the cohort being studied).28–30 Figure 59.6 shows the signal, it can be enhanced with either agitated saline,
pulmonary flow acceleration time (PAcT), an alternate, contrast, or an air–blood–saline mixture40–42 (Fig. 59.8).
less widely used method for screening patients for the However, this can also lead to overestimation of the TR
presence of pulmonary hypertension (PH). PAcT is the velocity due to signal artifacts.
interval between the onset of the forward flow in the PA
to its peak velocity. Values of <100 ms are associated with
Diastolic Pulmonary Artery Pressure
PH.31,32 While the use of the Bernoulli equation has been
widely validated,33,34 it can be imprecise, especially in The DPAP is equivalent to the LA and LV end-diastolic
patients with lung disease.35,36 pressure (LVEDP) when evaluated in individuals without
Table 59.3 lists common causes of inaccuracy using moderate or severe PH. Normal range is between 6 and
echo-Doppler for determination of PAP. Underestimation 12 mm Hg. In patients with a PVR of >200 dynes/s/cm–5
Fig. 59.5: Evaluation of systolic pulmonary artery pressure (SPAP) Fig. 59.6: Evaluation of systolic pulmonary artery pressure (SPAP)
using Doppler imaging of the tricuspid regurgitation (TR) jet. The using PA acceleration time (PAcT), which is the interval between
maximal velocity (Vmax) is 368 cm/s. Using the Bernoulli equation: the onset of the forward flow in the PA to its peak velocity (yellow
P = 4 × V2, a maximal pressure gradient (Max PG) of 54 mm Hg line). A value < 100 ms is associated with an elevated PAP. In this
between the right ventricle and atrium is calculated. When added patient (the same patient in Figure 59.5), the PAcT is 63 ms, con-
to the right atrial pressure, the SPAP can be estimated. sistent with an elevated SPAP.
Table 59.3: Causes for Inaccurate Estimation of Pulmonary Artery Pressure by Echocardiography
Cause Resolution
Underestimation:
Variations in Doppler angle of interrogation Multiple imaging planes to receive best Doppler signal, use of color Doppler
for optimal alignment with the regurgitant jet
Underestimation of right atrial pressure (RAP) Adequate assessment of RAP using a multiparameter approach
Severe tricuspid regurgitation
Poor Doppler signal Enhance signal with either, agitated saline, contrast, or an
air–blood–saline mixture.
Overestimation:
Overestimation of RAP Adequate assessment of RAP using a multiparameter approach
Mistakenly using the tricuspid valve closing Adequate analysis of the tricuspid Doppler signal
spike for the tricuspid maximal velocity
Contrast artifacts Adequate use of contrast, and adequate analysis of the tricuspid Doppler signal
A B
Figs 59.7A and B: Underestimation of systolic pulmonary artery pressure (SPAP). In this patient the estimated tricuspid regurgitation (TR)
gradient was 42 mm Hg. The estimated right atrial pressure (RAP) using inferior vena cava diameter of 2.78 cm and absence of respira-
tory collapse (bottom) was 15 mm Hg, giving an estimated SPAP of 57 mm Hg. However, the actual pulmonary artery catheter reading
for SPAP was 84 mm Hg. This underestimation was due to underestimation of the RAP, which was measured invasively as 40 mm Hg.
A B C
Figs 59.8A to C: Enhancing a poor tricuspid regurgitation (TR) Doppler signal (A) with saline (B) or a saline + blood mixture (C), which
gives even more enhanced results.
Fig. 59.9: Evaluation of diastolic and mean pulmonary artery pres- Fig. 59.10: Estimation of the mean pulmonary artery pressure
sure (DPAP and MPAP) using Doppler imaging of the pulmonic (MPAP). The RA–RV mean systolic gradient is derived from trac-
regurgitation (PR) jet. The maximal velocity (Vmax) is 281 cm/s ing the tricuspid regurgitation profile and equals 42 mm Hg; adding
(asterisk), and the velocity at end diastole is 195 cm/s (arrow). the RAP gives the MPAP.
Using the Bernoulli equation: P = 4 × V2, the maximal pressure
gradient of 32 mm Hg between the pulmonary artery (PA) and the
right ventricle (RV) during diastole is calculated and corresponds
with the MPAP. When added to the right atrial pressure (RAP), this
Mean Pulmonary Artery Pressure
improves accuracy. The gradient at end diastole between the PA
In individuals with a “normal” lung, the pulmonary
and the RV is also calculated and is 15 mm Hg. When added to
the RAP, the DPAP is given. capillary hydrostatic pressure is equivalent to the PCWP.
However, in the presence of pulmonary venoconstriction
or a MPAP > 40 mm Hg, the DPAP is higher (>5 mm Hg and PH, there can be a great difference between the lower
difference) than the mean pulmonary capillary wedge PCWP and the higher pulmonary capillary hydrostatic
pressure (PCWP).43 As demonstrated in Figure 59.9, pressure. In these situations, the DPAP does not necessarily
Doppler echocardiography can be used to estimate reflect adequate LA and LVEDP. It is then more important
DPAP15 by using the simplified Bernoulli equation with to predict the MPAP, which reflects and classifies more
the velocity of the pulmonic regurgitation (PR) jet at end adequately the PAP. The peak PR jet identifies the diastolic
diastole providing the end-diastolic PA–RV gradient. pressure gradient between the RV and the PA. Masuyama
The pulmonary artery diastolic pressure (PADP) can be et al.45 found that application of the Bernoulli equation to
estimated by adding the end-diastolic PA–RV to the RAP.44 the peak PR jet velocity provides an estimation of MPAP
This measurement correlates well with invasive measure- (Fig. 59.9). Addition of the RAP improves the accuracy of
ments. The most common errors in DPAP estimation this estimate.48 Figure 59.10 demonstrates another simple
have been attributed to inaccurate estimation of RAP.45 method to evaluate MPAP by adding the RAP to the RA–RV
However, the PR jet is not always detected (even with the mean systolic gradient, which can be derived from the TR
use of saline). profile.49 As the relationship between SPAP and DPAP has
The pulmonic valve opens when the RV and PA been shown to be constant,50 several empirical formulas
pressures transiently equalize.46 The gradient between listed in Table 59.2 have been suggested for the estimation
the RA and RV can be measured using the TR velocity of the MPAP.42,51 However, these data are derived from
and the velocity at time of pulmonary vein (PV) opening invasive studies and have not been validated by Doppler
combined with the RAP, allowing an estimate of the DPAP47 echocardiography.51
(with the use of superimposed QRS complexes from
the pulmonic flow and TR Doppler signals). However, as
Pulmonary Vascular Resistance
these measurements are made on a steep portion of the
TR slope, any small timing error can lead to inaccurate The PVR is directly proportional to the pressure gradient
calculations of the DPAP. across the entire lungs from the PAP to the left atrial
pressure (LAP). PVR equals: [(MPAP – mean PCWP) pulmonary vascular disease.57 This ratio has been validated
× 80]/CO and is a hemodynamic variable, which in several studies, but in patients with a very high PVR
contributes to the management of patients with advanced (>8 WU), its reliability as a quantitative measurement is
cardiovascular and pulmonary disease. Normal values poor.58 Other additional, more complicated methods for
range between 20 and 130 dynes/s/m/cm–5, which equals estimation of PVR are available59–61 (Table 59.2). To date,
0.25 – 1.6 woods units (WU). While increased SPAP may there have not been any comparative studies of the various
be secondary to increased backflow from the heart, it echo-Doppler methods used to assess PVR.
can also be the cause of pulmonary vascular disease.
An elevated PVR is used to define PH, and it is also LEFT-SIDED FILLING PRESSURES
an essential component in the evaluation of patients
awaiting heart and lung transplantation52 as well as in the
(TABLE 59.4)
determination of which patients should have closure of Invasive measurements for left-sided filling pressures
their intracardiac shunt.53 Elevated values of PVR correlate include the PCWP that reflects the LAP. PCWP also reflects
with worse clinical outcomes and prognosis in many LVEDP, which is the pressure within the left ventricle at the
different patient populations.54,55 Initial studies evaluating onset of the QRS complex on electrocardiography (ECG)
PVR noninvasively found only weak correlations with aside from several conditions, which can cause overesti-
invasive monitoring.56 However, using the maximal TR mation (mitral stenosis) or underestimation (aortic insuffi-
velocity and the RVOT VTI has recently been shown to ciency and a noncompliant left ventricle) of it. Noninvasive
correlate well with the transpulmonary pressure gradient assessment of left-sided filling pressures (LA and LV) is
and transpulmonary flow, respectively (which are the done using the diastolic function parameters listed in
parameters used for invasive estimation of PVR). Using Table 59.4. Left-sided filling pressures are considered
the simple equation: PVR (WU) = 10 × TR velocity/RVOT elevated when the PCWP is > 12 mm Hg or the LVEDP is
VTI + 0.16.57 In patients with a ratio of <0.175, there is a > 16 mm Hg62 and elevated filling pressures are the main
low likelihood to have a PVR > 2 WU, practically excluding physiological consequence of diastolic dysfunction.63
Table 59.4: The Various Methods Utilized for the Echocardiographic Evaluation of Left-Sided Filling Pressures (PCWP, LVEDP)
Mitral inflow parameters: E-wave, • See Table 59.4 for normal values. • Obtainable in nearly all patients
A-wave, DT, E/A ratio, IVRT. • Impaired LV relaxation: E/A < 1 or E/A > 2 • U shape relation with LV diastolic
Measured using pulse wave Dop- DT prolonged > 240 ms, IVRT prolonged function—similar values for healthy and
pler in the apical four-chamber • Pseudonormal: 1< E/A < 2, 160 < DT those with disease observed
view (Fig. 59.11) < 240 ms Difficult to interpret in the setting of:
• Restrictive filling: E/A > 2, DT short – Sinus tachycardia
< 160 ms, IVRT short < 70 ms – Conduction system abnormalities
See also Table 59.7 – Arrhythmias
• Poor correlation in patients with coronary
artery disease and those with hypertrophic
cardiomyopathy with EF ≥ 50%
Pulmonary venous flow S > D—Normal • Depth limitation
(Figs 59.12A and B) S < D—elevated LA pressure or normal in • Marked cardiac enlargement
young (<40 years) individuals. • Left atrial motion artifact
‘Ar’ velocity < 35 cm/s—normal • Influenced by age (D dominant in young
‘Ar’—A-wave duration < 30 ms—normal LA individuals < 40 years)
pressure Ar duration and velocity:
• Not influenced by age
• Applicable also in normal LVEF, MV
disease, and HCM
• Hard to obtain good quality images for
interpretation
Contd...
Contd...
Tissue Doppler imaging (TDI), é, é
see Table 59.5 for normal values. á, Reduced with:
(Fig. 59.13) E/é ratio < 8—normal LV filling pressures • Aging
≥15 (for septal é) or > 12 (for lateral é)— • The presence of annular calcifications,
elevated LV filling pressures annular rings, prosthetic MV, MS
Increased with:
• Moderate to severe MR
• Constrictive pericarditis (lateral may be
less than septal in this situation)
May be affected by:
• Preload in those with normal LVEF
• LV relaxation
• Systolic function
Lateral values higher than septal values
á increased by:
• Increased LA contractility
• Decreased LVEDP
E/é
• Lateral ratio lower than septal
• In patients with normal LVEF has low sen-
sitivity and high specificity
• In patients with mitral annular calcifica-
tion, severe MR, or constrictive pericarditis
might not give an adequate estimate of
filling pressures.
• Might not be valid for patients with acute
decompensated heart failure100
Propagation velocity—Vp Vp > 50 cm/s—normal Validated in patients with reduced LVEF
E/Vp > 2.5—elevated PCWP > 15 mm Hg Poor reproducibility
Aortic (Fig. 59.14) and mitral LVEDP = diastolic BP – (4 × AR jet velocity2) Validated only if no mitral stenosis is present
(Fig. 59.15) regurgitation jet LAP = systolic BP – (4 × MR jet velocity2)
RA and LA shunt (Fig. 59.16) Intra atrial pressure difference = 4 × V2
(DT: Deceleration time; HCM: Hypertrophic cardiomyopathy; IVRT: Isovolumic relaxation time; LA: Left atrium; LAP: Left atrial pres-
sure; LV: Left ventricle; LVEF: Left ventricular ejection fraction; PCWP: Pulmonary capillary wedge pressure, LVEDP: Left ventricular
end diastolic pressure; MR: Mitral regurgitation; MS: Mitral stenosis; MV: Mitral valve; RA: Right atrium).
Mitral Inflow Parameters (Fig. 59.11) Normal values of the mitral inflow parameters vary with
aging. With increasing age, the E-wave decreases and the DT
Using the pulsed wave (PW) Doppler in the apical four- and A-wave increases in amplitude, causing the E/A ratio to
chamber view, assessment of the mitral inflow velocities decrease as well. The normal values as per age are shown in
can be obtained, with images obtainable in nearly all Table 59.5.62 As listed in Table 59.6, heart rate and rhythm, PR
patients. The primary measurements include the peak interval, CO, mitral annular size, and LA function62 as well as
diastolic early filling (E-wave) and the diastolic late atrial other specific factors affect the mitral inflow.
filling (A-wave) velocities, the ratio between these (E/A), It is well established that the mitral E-wave velocity
the peak velocity deceleration time (DT), A-wave duration, primarily reflects the LA–LV pressure gradient during the
and the IVRT (derived by placing the curser in the left early stage of diastole and is thus amenable to changes
ventricular outflow tract [LVOT] to display simultaneously in the preload and alterations in LV relaxation.62,64 The
the end of aortic ejection and onset of mitral inflow). mitral A-wave velocity reflects the LA–LV pressure
A B
Figs 59.11A and B: Mitral valve inflow Doppler. Primary measurements include the peak early filling (E) and the late diastolic atrial
filling (A) velocities, deceleration time (yellow line) of the E-wave. (A) patient with a normal filling pattern, the E-wave is greater than the
A wave with a deceleration time of 206 ms (>160 ms); (B) Restrictive filling pattern, the E-wave is greater than the A-wave with a very
short deceleration time of 137 ms.
Table 59.5: Normal Values by Age Groups for Doppler and Tissue Doppler Variables for Estimation of Left Heart Hemodynamics
Measurement Age Group
16–20 21–40 41–60 > 60
E/A ratio 1.88 ± 0.45 1.53 ± 0.4 1.28 ± 0.25 0.96 ± 0.18
DT (ms) 142 ± 19 166 ± 14 181 ± 19 200 ± 29
IVRT (ms) 50 ± 9 67 ± 8 74 ± 7 87 ± 7
Septal é (cm/s) 14.9 ± 2.4 15.5 ± 2.7 12.2 ± 2.3 10.4 ± 2.1
Lateral é (cm/s) 20.6 ± 3.8 19.8 ± 2.9 16.1 ± 2.3 12.9 ± 3.5
Ar duration (ms) 66 ± 39 96 ± 33 112 ± 15 113 ± 30
Source: Adopted from Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic
function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):107–33.62
Table 59.6: Variables Affecting Mitral Inflow Parameters gradient during the late stage of diastole, which is affected
Parameter Variables
by LV compliance and the LA contractile function.
The DT of the mitral E-wave is influenced by the LV
General parameters • Heart rate and rhythm
• PR interval relaxation, LV diastolic pressure after mitral valve (MV)
• Cardiac output opening, and the LV compliance. Alterations in the LV
• Mitral annular size end-systolic and/or end-diastolic volumes, LV elastic
E-wave • Aging recoil, and/or LV diastolic pressures directly affect the
• Preload mitral inflow velocities (E-wave) and the time intervals
• Alterations in left ventricular (DT and IVRT).62
(LV) relaxation
In patients with dilated cardiomyopathy, the mitral
A-wave • Aging
inflow velocity parameters correlate better with functional
• LV compliance
• Left atrial contractile function class, filling pressures, and prognosis than the calculated
left ventricular ejection fraction (LVEF).65–77 However, in
E-wave deceleration • Aging
time (DT) • LV relaxation patients with coronary artery disease,78 mitral regurgitation
• LV diastolic pressure after mitral (MR), or hypertrophic cardiomyopathy (HCM),79,80 where
valve opening the LVEF is >50%, mitral inflow variables do not correlate
• LV compliance as well with hemodynamic measurements.
With the use of these parameters, especially the E/A an increase in LA pressure, there is a decrease in ‘S’ and
ratio and the DT, the echocardiographic filling pattern increase in ‘D’ velocities resulting in an S/D ratio of <1.
can be classified to either normal (E > A, DT > 160 ms), When there is an increased LVEDP, the Ar velocity and
impaired LV relaxation (E < A, DT > 240 ms), pseudonormal duration increase as well as the time difference between
LV filling (E > A, DT > 160 ms), or restrictive LV filling ‘Ar’ duration and the mitral A-wave duration.86,87
(E >> A, DT < 140 ms; Fig. 59.11). Normal values of pulmonary venous inflow are strongly
related to age. In young normal subjects, there is usually
Pulmonary Venous Flow prominent D velocity, reflecting their mitral E-wave. This
gradually declines (age > 40) with an increase in the S/D
(Figs 59.12A and B) ratio. The ‘Ar’ velocity also usually increases with age but
Pulmonary venous flow also provides important infor- normal values do not usually exceed 35 cm/s. A duration
mation for the assessment of LV diastolic function and difference of >30 ms between the ‘Ar’ and the mitral inflow
LA filling pressure. In most patients, the best Doppler A-wave (‘Ar’–A duration) is the only age-independent
recordings are obtained from the apical four-chamber indication of LV A-wave pressure increase,88 which can
view with the pulmonary venous flow obtainable in classify patients with abnormal LV relaxation into those
~approximately 90% of adult patients.81 As seen in Figures with elevated LVEDP but normal mean LA pressure,
59.12A and B, variables include the peak systolic velocity which is the first hemodynamic abnormality seen with
(S), which is composed of two systolic components (S1, diastolic dysfunction. Other variables such as maximal
S2), peak anterograde diastolic velocity (D), the S/D LA size, E-wave DT, and a pseudonormal filling pattern,
ratio, and the duration and peak of the atrial reversal (Ar) are all indicative of an increase in the mean LA pressure
velocity waveform. and a more advanced stage of diastolic dysfunction.62
The S-wave is primarily influenced by changes in the Importantly, unlike mitral inflow parameters, the ‘Ar’–A
LA pressure, contraction, and relaxation (S1 component) duration difference is still accurate in various patient
and by the stroke volume (SV) and pulse wave propagation populations such as those with: normal ejection fraction
in the PA vasculature tree.82,83 The ‘D’-wave is influenced (EF),78 MV disease,89 and HCM.80 Yet, one of the impor-
by the same factors that influence the mitral E velocity.84 tant limitations using the ‘Ar’ parameters is obtaining
‘Ar’ duration and velocity are influenced by the LV late high-quality images suitable for accurate reproducible
diastolic pressure, atrial preload, and contractility.85 With measurements.
A B
Figs 59.12A and B: Doppler of the pulmonary vein flow pattern from the apical four-chamber view. (A) normal Doppler study demonstrates
the peak systolic velocity (S), which is composed of two systolic components (S1, S2), peak anterograde diastolic velocity (D), and peak
of the atrial reversal (Ar) velocity waveform; (B) An examination from a patient with an elevated left atrial filling pressure demonstrating
a decrease in S and increase in D, resulting in an S/D ratio of <1.
Tissue Doppler Annular Early and Late additional time intervals and ratios using a combination
of TDI and mitral inflow parameters such as the E velocity
Diastolic Velocities (Fig. 59.13) to é ratio (E/é), which plays an important role in the
TDI measurements include both systolic (S) and diastolic estimation of LV filling pressures. The time differential
velocities. The early diastolic velocities are expressed as between the QRS and both E and é gives the TE-é and also
Ea, Em, É, or é, and the late diastolic velocity as Aa, Am, Á, provides additional information on diastolic function. TE-é
or á, with mostly used terms being é and á. TDI is acquired is prolonged in patients with diastolic dysfunction.
using PW Doppler from the apical views to acquire the The combination of TDI and mitral inflow velocities
mitral annular velocities.90 ASE guidelines62 recommend allows for prediction of LV filling pressures. However, it is
acquiring and measuring TDI signals from both the septal important to take into context the clinical parameters in
and lateral sides of the MV annulus. The normal values of order to make a reliable assessment (i.e. age, presence of
these parameters are influenced by age like other indices of cardiovascular disease, other echocardiographic abnor-
LV diastolic function, with a decrease in é and an increase in malities). Unfortunately, these criteria are limited in their
á and the E/é ratio;91 normal values are shown in Table 59.5. accuracy for LV filling pressures.
The hemodynamic determinants of the é velocity include It is recommended to use the average é velocity
LV relaxation, preload, systolic function, and LV minimal obtained from both the lateral and septal sides of the
pressure.62 A significant association between é and LV mitral annulus for the prediction of LV filling pressure. The
relaxation has been shown in several studies.92,93 While septal é is usually lower than the lateral é velocity, so the
preload has minimal effect on é in the presence of LV E/é ratio from the septal signal is usually higher than the
impaired relaxation,94,95 it increases é in patients with lateral é. In the context of regional myocardial dysfunction,
normal or enhanced LV relaxation.94–97 Due to this, in it is recommended by the ASE to use the average é
patients with cardiac disease, é velocity can be used to velocity, and in patient with atrial fibrillation an average
correct for the effect of LV relaxation on mitral E velocity, of measurements from 10 cardiac cycles is most accurate.
and the E/é ratio can be applied for the prediction of An E/é ratio of <8 (for either septal or lateral) is usually
LV filling pressures (Figs 59.13A and B).62 The main associated with normal LV filling pressures. Conversely, a
determinants of á include LA systolic function and LVEDP ratio ≥ 15 for septal or ≥ 12 for lateral é is associated with
such that increased LA contractility leads to increased á increased LV filling pressures.62 When the value is midrange
velocity, whereas increased LVEDP leads to decreased á (between 8 and 15), other indices should be used to assess
velocity.94 Once acquired, it is possible to also calculate LV filling pressures. In these patients and also in those with
A B
Figs 59.13A and B: Evaluation of left-sided filling pressures. (A) Mitral inflow velocities are measured (E, A), showing an E > A with a
pseudonormal filling pattern (deceleration time > 160 ms); (B) Lateral tissue Doppler imaging (TDI) that includes both systolic (S) and
diastolic velocities. The early diastolic velocity is expressed as é, and the late diastolic velocity as á. In this patient, the E/é is elevated
and equals—89/4.66 = 19, implying elevated left-sided filling pressures.
normal EF, or with MV disease, using the TE-é might provide However, Vp is often not routinely evaluated due to
additional input. It has been also shown in more recent poor reproducibility and limited sensitivity and specifi-
studies that in those with normal EF, the lateral é has the city. In addition, other signs of ventricular impairment
best correlation with LV filling pressures and invasive and filling pressure are usually apparent during the
parameters of LV stiffness.98,99 It is controversial whether echocardiographic evaluation, making Vp useful as
in patients with acute decompensated heart failure E/é is a complimentary index when there are inconsistent
valid.100–102 findings. In patients with a normal LVEF, Vp can be falsely
normal despiteelevated filling pressures.105,106
Color M-mode Flow Propagation
Left Atrial Dimensions
Velocity (Vp)
Chronic elevation of left-sided filling pressures leads to LA
The left ventricular filing is dominated by an early wave enlargement. There is a significant association between
and an atrial-induced filling wave. In a normal ventricle, LA dimensions and elevated left-sided filling pressures
the early filling forces are attributed to suction of blood and evaluation of LA dimensions is an important adjunct
from the atria and the filling wave propagates rapidly to the echocardiographic evaluation of the left-sided filling
toward the apex, driven by the pressure gradient between pressures.107 LA measurements are usually obtained most
the LV base and the apex. Using of color flow imaging and accurately from the apical views.14 However, LA enlarge-
M-mode echocardiography placed through the center of ment is not a specific sign for elevated filling pressures as it
the LV inflow from the MV to the apex, the propagation can accompany also situations where the left-sided filling
velocity can be estimated. A Vp > 50 cm/s is considered pressures are not elevated and diastolic dysfunction is not
normal,103,104 while in patients with myocardial ischemia present such as in trained athletes, patients with chronic
or heart failure there is slowing of the mitral to apical atrial fibrillation or flutter, bradycardia, high output states
flow propagation measured. In patients with reduced and mitral valvular disease.
LVEF (< 50%), the E/Vp ratio can be used to predict the LV Mitral inflow pattern, PV flow, TDI and Vp can serve
filling pressures,104 with E/Vp ≥ 2.5 predicting a PCWP of as useful tools for evaluation and estimation of left-sided
>15 mm Hg.98 filling pressures. Table 59.7 lists the main differences
Table 59.7: Variables Used for the Evaluation of Filling Pressures in Those with Normal and with Decreased Ejection Fraction (EF)
Variable Patients with Depressed EF Patients with Normal EF
E/A < 1 Normal FP (if E ≤ 50 cm/s) NA
≥ 1–<2, < 1 and E > 50 cm/s—indeterminate
≥ 2 – elevated FP (with DT < 150 ms)
E/é < 8—normal FP ≤ 8 (Sep, Lat, Avg)—normal FP
8–12—indeterminate Sep ≥ 15, Lat ≥ 12, Avg ≥ 13—elevated FP
≥ 13—elevated FP
Vp > 50 cm/s—normal Can be falsely normal despite elevated FP
E/Vp > 2.5—elevated FP >15 mm Hg
Ar-A < 0 ms—normal FP < 0 ms—normal FP
0–29—indeterminate 0–29—indeterminate
≥ 30 ms—elevated FP ≥ 30 ms—elevated FP
IVRT/TE-é > 2—normal FP > 2—normal FP
< 2—elevated FP < 2—elevated FP
Systolic pulmonary artery pressure < 30 mm Hg—normal FP < 30 mm Hg—normal FP
> 35 mm Hg—elevated FP > 35 mm Hg—elevated FP
Pulmonary vein S/D > 1—normal FP Limited accuracy
< 1—Elevated FP
Left atrial volume Some LA dilation may occur in this population < 34 mL/m2—normal FP
in the presence of normal FP. Thus, this param- ≥ 34 mL/m2—Elevated FP
eter should not be used in this population.
(Avg: Average; EF: Ejection fraction; FP: Filling pressure; Lat: Lateral; NA: Not applicable; Sep: Septal).
in the evaluation of filling pressures in patients with LVEDP can be calculated as the diastolic aortic pressure
either a decreased or a normal LVEF. While in patients minus the pressure gradient at end diastole which, by
with impaired myocardial function the primary tool for using the modified Bernoulli equation, is equal to 4V2
evaluation is the E/A ratio, in those with a normal LVEF the (Fig. 59.15). Diastolic blood pressure should be obtained
estimation can be more challenging, primarily assessed at the same time the AR jet is being interrogated. The
using the E/é ratio preferably with the use of other maximal pressure gradient between the LV and LA during
parameters.107 A simplified diagnostic algorithm for the systole can be determined by CW Doppler in patients with
evaluation of different patient populations based on the MR. LAP can be calculated as the difference between the
ASE regarding the evaluation of left ventricular diastolic systolic blood pressure and the MR gradient, by using
function62 is shown in Figure 59.14. the modified Bernoulli equation as well (Fig. 59.16). In
patients with mitral stenosis, the LA diastolic pressure is
ADDITIONAL PARAMETERS FOR ESTI- the sum of the LVEDP and the transmitral gradient. Again,
care must be taken to obtain blood pressure at the same
MATION OF LEFT ATRIAL PRESSURE
time as Doppler measurements are being obtained.108
Aortic and Mitral Regurgitation
Continuous Wave Doppler Signal Atrial Septal Defect/Patent
The LAP equals the LVEDP in the absence of mitral
Foramen Ovale Flow
stenosis. In patients with aortic regurgitation (AR), using As shown in Figure 59.17, by adding the estimated RAP
the continuous wave (CW) Doppler signal of the AR jet to the pressure gradient between the RA and the LA
gives the pressure gradient between the aorta and the LV as evaluated by CW Doppler, it is possible to estimate
at end diastole. Peak AR velocity should be >4 m/s, and the the LAP.
Fig. 59.14: Schematic approach for the evaluation of left-sided filling pressures using the various obtained echocardiographic param-
eters. (DT: Deceleration time; IVRT: Isovolumic relaxation time; LA: Left atrium; PV: Pulmonary vein; SPAP: Systolic pulmonary artery
pressure).
*Refers for averaged values from both septal and lateral tissue Doppler; for specific values see Table 7.
**See text for further details. Adopted from Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left
ventricular diastolic function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):107–33.62
Fig. 59.15: Measurement of left ventricular end diastolic pres- Fig. 59.16: Estimation of the pressure gradient between the left
sure (LVEDP) using the aortic regurgitation (AR) signal. Using ventricle and left atrium (LA). Using continuous wave Doppler
continuous wave Doppler through the aortic valve in diastole, the through the mitral valve in systole, the peak MR velocity gives
end-diastolic AR velocity gives out the pressure gradient (white ar- out the maximal pressure gradient (white arrow), which equals to
row) which equals to 4 × 3.42 = 46 mm Hg. Subtracting this value 4 × 4.872 = 95 mm Hg. Subtracting this value from the systolic
from the systolic blood pressure gives an estimate of the left atrial blood pressure gives an estimate of the LA pressure during
pressure during systole (V-wave). The LVEDP equals the diastolic systole (V-wave).
blood pressure—4V2.
STROKE VOLUME, STROKE DISTANCE, the absence of pulmonic shunting, the pulmonic outflow
tract can be used instead of the LVOT.111
CARDIAC OUTPUT, AND SYSTEMIC
By determining the transmitral and transpulmonary
PULMONARY SHUNTS (QP/QS) flow, it is possible to noninvasively obtain the pulmonic
to systemic flow ratio (QP/QS) in various shunt disease,
CO equals SV multiplied by the heart rate (CO = SV ×
if present. However, calculation of pulmonary flow can
heart rate). Using Doppler, it is possible to measure the
sometimes result in significant errors, mainly due to the
stroke distance, which refers to the distance traveled by a inability to adequately visualize and measure the PA
column of blood during a fixed time (the cardiac cycle). diameter or RVOT diameter. As the case in severe AR,
Multiplying the stroke distance with the cross-sectional significant PR can lead to overestimation of the right
area through which the column moves gives the SV. This ventricular SV. While in patients with severe AR, Doppler
method can be obtained at several sites, with the most can falsely underestimate the QP/QS, in those with
common and accurate being the LVOT109,110 (Fig. 59.18). It significant PR it can lead to falsely overestimating it.
is necessary that Doppler velocities be obtained parallel
to the direction of flow and that the cross-sectional area A WORD TO CONCLUDE
through which the flow is occurring is obtained. As the As noninvasive evaluation utilizes indirect indexes for
outflow tract is often elliptical and not circular, it may be estimation of hemodynamic parameters, it is far superior
more appropriate to use the stroke distance rather than the to bedside physical examination but it also has limitations.
CO, as it may be miscalculated due to the aortic annulus However, in addition to providing hemodynamic data and
being elliptical. Significant AR will lead to overestimation volume status, the Doppler data provides insight into the
of the SV and consequently the CO. When present, and in pathophysiology of ventricular filling and emptying.
Fig. 59.17: Estimation of the left atrial (LA) pressure. On the top Fig. 59.18: Estimation of stroke volume (SV) and cardiac output
image: color flow imaging demonstrating a shunt between the (CO). Using Doppler, the left ventricular outflow tract velocity time
right atrium (RA) and LA. On the bottom image: continuous wave integral (LVOT VTI) is measured, and is 11.5 cm. This is the stroke
Doppler for estimation of the pressure gradient between the RA distance (SD). Multiplying the stroke distance with the cross-
and the LA (white arrow), which equals 4 × 2.742 = 30 mm Hg. sectional area through which the SD moves gives the SV. As the
Adding this gradient to the estimated RAP gives the estimated LVOT diameter is 2 cm, the cross-sectional area is × (2/2)2 =
LA pressure. 3.14. The SV is thus 36 mL. The CO = SV × heart rate, which is
3.2 L/min. *As the outflow tract is elliptical and not circular, aver-
aging measurements from at least two different planes is recom-
mended for better estimation.
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alterations on mitral annulus velocities, strain, and strain 111. Kirkpatrick JN, Lang RM. Heart failure: hemodynamic
rates due to abrupt changes in preload elicited by parabolic assessment using echocardiography. Curr Cardiol Rep.
flight. J Appl Physiol. 2007;103(1):80–7. 2008;10(3):240–6.
SECTION 5
Ischemic Heart Disease,
Cardiomyopathies, Pericardial
Disorders, Tumors and Masses
Chapters
Chapter 60 Echocardiography in Ischemic Heart Disease Chapter 67 Echocardiographic Differentiation of Ischemic and
Chapter 61 Stress Echocardiography Nonischemic Cardiomyopathy: Comparison with
Chapter 62 Squatting Stress Echocardiography Other Noninvasive Modalities
Chapter 63 Three-Dimensional Stress Echocardiography Chapter 68 Pericardial Disease
Chapter 64 Echocardiographic Assessment of Coronary Chapter 69 Three-Dimensional Echocardiographic
Arteries —Morphology and Coronary Flow Reserve Assessment in Pericardial Disorders
Chapter 65 Echocardiography in Hypertrophic Cardiomyopathy Chapter 70 Echocardiographic Assessment of Cardiac
Chapter 66 Echocardiographic Assessment of Nonobstructive Tumors and Masses
Cardiomyopathies
CHAPTER 60
Echocardiography in Ischemic
Heart Disease
Chetan Shenoy, Hamid Reza Salehi, Francesco F Faletra, Natesa G Pandian
Snapshot

DetecƟon of Ischemia 
Novel Echocardiography Techniques in Ischemic

Role in Acute Coronary Syndromes Heart Disease

Mechanical ComplicaƟons of Myocardial InfarcƟon 
Future

Role of Echocardiography in Chronic Ischemic
Cardiomyopathy
INTRODUCTION imaging technology such as tissue harmonic imaging and

contrast echocardiography have significantly improved
Coronary artery disease (CAD) is the leading cause of death the accuracy and reliability of the modality in ischemic
for both men and women in the United States. Each year, heart disease.
an estimated 785,000 Americans will have a new coronary In this chapter, we will discuss established as well as
event, and nearly 470,000 will have a recurrent attack.1 It is novel and emerging applications of echocardiography in
estimated that an additional 195,000 silent first myocardial ischemic heart disease.
infarctions (MIs) occur each year.1 Approximately every
25 seconds, an American will have a coronary event, and
approximately every minute, someone will die of one.1
DETECTION OF ISCHEMIA
Noninvasive diagnosis and evaluation of the effects of Stress echocardiography is commonly used for the
CAD are important in risk stratification and guides disease detection of chronic ischemia in patients with known or
management. Since the 1980s, echocardiography has been suspected ischemic heart disease.3–6 Stress echocardio-
the mainstay of cardiac imaging in the field of noninvasive graphy can be performed either as an exercise test or as a
evaluation of CAD.2 pharmacological stress test. For patients who are capable
Echocardiography has a multifaceted role in ischemic of performing an exercise test, exercise stress rather than
heart disease.2 It can be used for the noninvasive detection pharmacological stress is recommended, as the exercise
of chronic ischemia, acute coronary syndrome (ACS), capacity is a reliable predictor of outcomes.
complications of ACS, and consequences of ischemic heart While either treadmill or bicycle exercise may be
disease. The role of echocardiography in ischemic heart used for exercise stress, the treadmill is widely used in the
disease is well established, time-tested, and supported United States.3–7 Symptom-limited exercise according to a
by extensive literature. Advances in echocardiographic standardized protocol in which the workload is gradually
1290 Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
increased in stages is performed.3–5 The Bruce protocol is sensitivity of dobutamine echocardiography in patients
most commonly used for treadmill exercise echocardio- receiving -blockers and in those with single-vessel
graphy in the United States. Imaging is performed at rest disease8 (Fig. 60.2).
and immediately after cessation of exercise.3–5 Exercise Both dobutamine and exercise echocardiography
stress echocardiography provides valuable information result in a marked increase of heart rate. The increase in
for detection of ischemic heart disease and assessment of blood pressure is much less with dobutamine compared
valvular heart disease (Fig. 60.1). with exercise. With both techniques, the induction of
Pharmacological stress testing is performed in ischemia is related to an increase in myocardial oxygen
patients who cannot exercise.5,6 Commonly used agents demand.
for pharmacological stress echocardiography include Vasodilator stress testing may be performed with
dobutamine and vasodilators. Although vasodilators adenosine or dipyridamole.6 Atropine is routinely
may have advantages for assessment of myocardial used with vasodilator stress to enhance test sensitivity.
perfusion, dobutamine is preferred when the test is based The addition of handgrip at peak infusion enhances
on assessment of regional wall motion.8 The standard sensitivity. Vasodilator stress echocardiography usually
for dobutamine stress testing is a graded dobutamine produces a mild-to-moderate increase in heart rate and a
infusion starting at 5 μg/kg/min and increasing at mild decrease in blood pressure. While adenosine stress
3-minutes intervals to 10, 20, 30 and 40 μg/kg/min.8 The is used to assess myocardial perfusion with contrast
low-dose stages allow detection of viability and ischemia echocardiography, it has not been widely used as a clinical
in segments with abnormal function at rest, even when tool.6
assessment of viability is not the primary objective of Interpretation of stress echocardiographic images
the test.8 involves visual assessment of endocardial excursion and
Endpoints of a dobutamine stress echocardiography wall thickening.6 Either a 16- or 17-segment model of the
study are achievement of target heart rate (defined as left ventricle (LV) may be used.6 Function in each segment
85% of the age-predicted maximum heart rate), new is graded at rest and with stress as normal or hyperdy-
or worsening wall-motion abnormalities, significant namic, hypokinetic, akinetic, dyskinetic, or aneurysmal.6
arrhythmias, hypotension, severe hypertension, and Images from low or intermediate stages of dobutamine
intolerable symptoms.6,8 Atropine, in divided doses of 0.25 infusion should be compared with peak stress images to
to 0.5 mg to a total of 2.0 mg, could be used as needed maximize the sensitivity for detection of coronary disease6
to achieve the target heart rate.8 Atropine increases the (Fig. 60.2).
Fig. 60.1: Example of an exercise stress echocardiogram using Fig. 60.2: Example of a dobutamine exercise stress echo-
contrast, demonstrating anterior wall ischemia (arrows). Upper cardiogram, demonstrating inferior wall ischemia (arrows). Upper
panels—rest, lower panels—stress. Left panels—diastolic frames, panels—rest, lower panels—stress. Left panels—diastolic frames,
right panels—systolic frame. right panels—systolic frame.
Chapter 60: Echocardiography in Ischemic Heart Disease 1291
A normal stress echocardiogram is defined as wall-motion abnormalities may also be seen in patients
normal LV wall motion at rest and with stress.6 Abnormal with hypertension or underlying cardiomyopathy, in the
study findings include those with fixed wall-motion absence of ischemia. Finally, tethering of LV myocardium
abnormalities (i.e. resting wall-motion abnormalities, due to patients with significant mitral annular calcification
unchanged with stress, which most often represent or prior mitral valve replacement could lead to a reduction
regions of prior infarction), or new or worsening wall- in motion of adjacent basal inferior and basal inferoseptal
motion abnormalities indicative of ischemia6 (Figs 60.1 segments and result in false-positive stress studies.6
and 60.2). In addition to the evaluation of segmental A normal stress echocardiogram is associated with an
function, the global response of the LV to stress should be annual mortality risk of 0.4 to 0.9%, equivalent to that of an
assessed. Stress-induced changes in LV shape, cavity size, age- and sex-matched population, based on a total of 9,000
and global contractility are also indicators of the presence patients; thus, in patients with suspected CAD, a normal
or absence of ischemia and may indicate multivessel stress echocardiogram confers an excellent prognosis
disease.6 The total amount of myocardium in jeopardy and coronary angiography can safely be avoided.6 With
predicts risk, and prolonged persistence of systolic wall- an abnormal stress echocardiogram, the risk of future
thickening abnormality may also identify severe CAD. mortality is directly related to the extent of the wall-motion
Stress echocardiography can also predict the presence of abnormalities. Patients with extensive stress-induced
myocardial hibernation when wall-motion abnormalities abnormalities in a multivessel distribution are at a high
at rest improve or resolve with stress.6 risk of mortality and cardiac events.
Based on pooled data, stress echocardiography has an Variables on a stress echocardiogram that are
average sensitivity of 88% and an average specificity of 83% associated with adverse outcomes include baseline LV
for the detection of coronary artery stenosis (generally dysfunction, wall-motion abnormalities in multivessel
>50% diameter stenosis by angiography).6 Studies com- distribution, extensive ischemia, location of wall-motion
paring the accuracy of nuclear perfusion imaging and abnormalities in left anterior descending coronary artery
stress echocardiography in the same patient population distribution, poor ejection fraction (EF) response or
have shown that the tests have similar sensitivities for the failure to reduce end-systolic volume with exercise, a low
detection of CAD, but stress echocardiography has higher ischemic threshold and LV hypertrophy.6
specificity. In a pooled analysis of 18 studies in 1,304
patients who underwent exercise or pharmacological Myocardial Contrast Stress
stress echocardiography in conjunction with thallium- or
technetium-labeled radioisotope imaging, sensitivity and
Echocardiography
specificity were 80 and 86% for echocardiography, and Development of ultrasound contrast agents containing
84 and 77% for myocardial perfusion imaging, respectively.6 microbubbles that mimic red blood cell rheology, now
The relatively high specificity of stress echocardiography allows simultaneous assessment of both function and
contributes to its use as a cost-effective diagnostic method. perfusion via myocardial perfusion imaging, making it a
False-negative stress echocardiography studies result unique technique for the assessment of CAD.9,10
primarily from suboptimal stress. They are more common Contrast stress echocardiography allows enhanced
in patients with single-vessel disease or disease of the assessment of wall motion both at rest and during stress
left circumflex artery because of the smaller amount of by improving visualization of the endocardial border, by
myocardium supplied by the left circumflex coronary improving the confidence in wall-motion assessment, and
artery, in patients with a small LV cavity and increased by reducing the number of uninterpretable images. Thus,
relative wall thickness, and in patients with significant by increasing the accuracy of wall-motion assessment,
aortic or mitral regurgitation (MR) leading to a hyper- contrast echocardiography enhances the diagnostic value
dynamic LV.6 of stress echocardiography for the detection of CAD9,10
False-positive stress echocardiography studies can be (Fig. 60.1).
seen in patients with coronary artery spasm or in patients The onset of ischemic wall-motion abnormalities
with decreased myocardial perfusion reserve in patients is preceded by development of regional disparities in
with LV hypertrophy, syndrome X, diabetes mellitus, myo- coronary perfusion that can be assessed by contrast agents.
carditis, and idiopathic cardiomyopathy.6 Stress-induced Contrast echocardiography can, therefore, be employed
for the detection of myocardial perfusion and several in the diagnosis of ACSs.13 Assessment of decreased
studies have demonstrated the clinical effectiveness of this myocardial contractility as noted by resting wall-motion
technique for the detection of CAD.9,10 abnormalities is the primary technique used in patients
Similar to stress echocardiography without myocardial with ACS, while other less established techniques include
contrast, completely normal perfusion during myocardial assessment of diastolic dysfunction, and assessment of
contrast stress echocardiography is very reassuring with myocardial perfusion using contrast echocardiography.14
a <1% annual risk of adverse outcomes, while abnormal Stress echocardiography is the other important technique
perfusion accompanied by wall-motion abnormalities used in these patients.11,12
identifies very high-risk patients with an approximately With unstable angina, the duration of ischemia
15% annual event rate.9,10 and consequently the wall-motion abnormalities may
be short-lived. Hence, assessment of wall-motion
ROLE IN ACUTE CORONARY abnormality in patients with suspected ACS should be
performed early after the onset of symptoms; normal wall
SYNDROMES motion in a patient with a normal ECG and without chest
Echocardiography today plays an essential role in the pain does not exclude an ACS. Conversely, if the patient
diagnosis of ACSs and their related complications.11,12 In has ischemic chest pain, a normal or equivocal ECG,
patients presenting to the emergency room with chest and no regional wall-motion abnormalities on resting
pain suggestive of an ACS but with negative biomarkers echocardiography during or immediately after the acute
and a nondiagnostic electrocardiogram (ECG), echo- episode, the presence of acute ischemia is unlikely. The
cardiography plays an important role for diagnosis and sensitivity of echocardiography in detection of ACS via
prognostication.11,12 resting wall-motion abnormalities is 90 to 95% with a
With a critical stenosis and interruption of blood flow negative predictive value of 90%15,16 (Figs 60.3 to 60.5).
in an epicardial coronary artery, the loss of myocardial After an ACS has been diagnosed, echocardiographic
function and development of clinical signs and symptoms assessment of wall motion aids in the assessment of site
proceed in a stepwise process referred to as the ischemic and severity of the acute CAD. The site of the myocardial
cascade. It starts with a defect in perfusion and progresses insult and consequently, the infarct-related coronary
through abnormalities in left ventricular diastolic artery, can be readily identified and the extent of the
function, decreased myocardial contractility, increased left subendocardial damage determines the degree of wall-
ventricular end-diastolic pressure, ST-segment changes, thickening that takes place in the affected segments. Mild
and occasionally, chest pain.13 This ischemic cascade hypokinesis suggests only a small amount of myocardial
forms the basis of the application of echocardiography damage affecting a small part of the endocardium.
A B
Figs 60.3A and B: Example of a wall motion abnormality in the mid-distal anteroseptum in a parasternal three-chamber view (arrows).
(A) Diastolic frame; (B) Systolic frame.
A B
Figs 60.4A and B: Example of a wall motion abnormality in the mid inferoseptum and inferior walls in a short-axis view (arrows).
(A) Diastolic frame, (B) Systolic frame.
A B
Figs 60.5A and B: Example of a wall motion abnormality in the mid-distal lateral wall in an apical four-chamber view (arrows).
(A) Diastolic frame, (B) Systolic frame.
Severe hypokinesis is unusual in patients with small, Global LV systolic function in patients with ACS
nontransmural infarctions. However, it could occur in may be assessed through either wall-motion scoring or
the presence of myocardial stunning overlying a small calculation of global LV ejection fraction (LVEF). Wall-
infarction. It is not uncommon for segments adjacent to motion scoring analysis assigns a numeric value to the
the ischemic ones to become hypokinetic due to factors degree of contractile dysfunction in each LV segment and
such as tethering. Therefore, wall-motion abnormalities a wall motion score index can be derived from the sum
following acute ischemia may be due to myocardial of individual segment scores divided by the number of
necrosis, stunned or hibernating myocardium, or more evaluated segments. The wall-motion score index been
commonly, combinations of these factors.15 shown to be an important prognostic indicator in patients
Echocardiography also provides useful prognostic with CAD.
information for identification of patients at risk of future There are extensive data showing that LVEF is one of the
cardiovascular events after an ACS. These data are based most powerful predictors of adverse outcomes including
on assessment of global LV systolic function.15 mortality in patients with LV systolic dysfunction of any
cause including ischemic heart disease. LVEF is the single approximately 10% of the patients who die after an acute
most powerful predictor of mortality and the risk for life- MI.19,20 It occurs within the first 24 hours in about half the
threatening ventricular arrhythmias after MI. patients and within a week in 85%. Any wall—anterior,
After resolution and management of an ACS, the lateral, or inferior—can be involved. In up to 40% of cases,
residual LVEF is important for treatment from both ruptures have a subacute course with ongoing myocardial
medical and device therapies standpoint. Even after acute tearing and bleeding into the pericardial space. A
treatment and clinical stabilization of heart failure, LVEF pseudoaneurysm is a manifestation of a subacute tear
has a strong and close relationship with survival in patients that is spontaneously sealed. Since; sudden death is often
with decreased LVEF. a presentation of complete rupture of the LV free wall,
The right coronary artery also commonly supplies the echocardiography has a bigger role in diagnosis of patients
right ventricle (RV), which may also be involved in the MI. with subacute free wall rupture.19,20
This has important implications in the acute management These patients often have pericardial effusions
of patients; so assessment of the RV in patients with ACS is secondary to hemopericardium, layered thrombus
important to assess the degree of RV dysfunction.15 in the pericardial space, or cardiac tamponade with
In patients with clinically suspected RV MI, echo- hemodynamic instability.19,20 A finding of a pericardial
cardiography showing RV cavity enlargement and effusion of 5 mm during diastole and layered thrombus
impaired RV free wall motion confirms the diagnosis of within the pericardial space are highly sensitive for the
RV MI and helps exclude other possibilities for the clinical detection of free wall rupture. The ruptured site is seldom
presentation, such as pericardial tamponade, which may detected with two-dimensional (2D) echocardiography;
present with the same clinical picture. however, this may be possible with color-flow Doppler.19,20
Echocardiography can be misleading for the diagnosis Contrast echocardiography may be helpful in
of RV MI in patients with chronic pulmonary disease, establishing the diagnosis of free wall rupture in patients
such as chronic obstructive lung disease or significant with a subacute presentation.21 Passage of contrast agent
pulmonary arterial hypertension, or acute pulmonary into the pericardial space after an intravenous injection
disease such as pulmonary embolism. confirms a connection between the heart and the
pericardial space, while lack of contrast in the pericardial
MECHANICAL COMPLICATIONS OF space helps exclude a LV free wall rupture.21
MYOCARDIAL INFARCTION
Left Ventricular Pseudoaneurysm
Echocardiography is the primary diagnostic tool for
detection of potentially life-threatening complications An LV pseudoaneurysm is formed when a subacute tear
of acute MI.17,18 Portability, immediate availability, in the LV wall is spontaneously sealed and contained by
quick imaging, and the detailed information it provides adherent pericardium or scar tissue22 (Figs 60.6A and B).
both on cardiac function and blood flow are critical in As opposed to a true LV aneurysm (Figs 60.6 and 60.7)
the management of patients with known or suspected where the outer layer consists of thinned, necrotic, or
mechanical complications of MI. By helping in diagnosis scarred myocardium, myocardium is not present in the
and decision-making in these often critically ill patients, external layer of the pseudoaneurysm.22 A true aneurysm
echocardiography can be life-saving.17,18 and a pseudoaneurysm carry greatly different implications
After the diagnosis of a mechanical complication has since a true LV aneurysm rarely ruptures, while there is a
been made, echocardiography could also be used to guide the high risk of rupture with a pseudoaneurysm.22 Surgical
intraoperative management (usually with transesophageal repair is indicated in most cases of pseudoaneurysm,
echocardiography) and in assessing the outcome of while it is not indicated in most cases of true LV aneurysm.
complicated surgeries and therapeutic procedures.17,18 Therefore, echocardiography plays a crucial role not only
in the identification of a LV pseudoaneurysm, but also for
the confirmation of the absence of a true LV aneurysm.22
Rupture of Left Ventricular Free Wall LV pseudoaneurysm is often detected when echo-
Rupture of the LV free wall is the most common form cardiography is performed routinely post MI or for
of myocardial rupture and the cause of death in indications of congestive heart failure or an embolic
A B
Figs 60.6A and B: Example of a left ventricular aneurysm (arrow Fig. 60.7: Example of a left ventricular apical aneurysm (arrows).
in Fig. A) and a left ventricular pseudoaneurysm (arrow in Fig. B).
The asterisk in the right image shows the narrow neck.
event.23 A sharp discontinuity of the endocardial border at the anterior two-third being supplied by branches of the
the communication site of the pseudoaneurysm with the LV left anterior descending coronary artery and the posterior
cavity, a saccular or globular contour of the pseudoaneu- third by the posterior descending artery of either the right
rysm, and a relatively narrow neck at the communication or circumflex coronary arteries.26 The timing of occurrence
site of the pseudoaneurysm with the LV cavity are of ventricular septal rupture varies depending on whether
often seen on echocardiography23 (Figs 60.6A and B). the patient was reperfused or not. It occurs within the first
Careful examination and use of off-plane imaging may week after MI in patients who did not receive reperfusion
sometimes be required for the identification and complete therapy, with peaks on the first day and on days 3, 4 and 5
characterization of the site of rupture. A thrombus may be after the MI. In patients treated with thrombolytic therapy,
visualized within the pseudoaneurysm cavity.23 most ruptures occur within the first 2 days.26
Color Doppler can also aid in the detection of LV Septal ruptures can be discrete with a direct through-
pseudoaneurysm and the characteristic finding is the and-through communication at the same level on both
bidirectional flow between the cavities of the LV and sides of the septum or be complex and irregular with
the pseudoaneurysm.23 A jet of bidirectional flow helps serpinginous tracts. In anterior MIs, septal ruptures are
distinguish a LV pseudoaneurysm from a pericardial usually apical and discrete, whereas in inferior MIs they
effusion or true aneurysm. The use of contrast agents are more commonly in the basal inferoposterior septum
may also help in the diagnosis of LV pseudoaneurysm.24 and more often complex.26
Intravenous contrast agents can clearly demonstrate the The ventricular septal defect can be visualized with 2D
narrow neck and the communication between the cavities echocardiography alone in approximately 50% of patients18
of the LV and the pseudoaneurysm.24 (Figs 60.8 to 60.10 and Movie clip 60.1). Multiple imaging
planes should be used and careful off-plane imaging may
be required to visualize the ventricular septal defect.
Ventricular Septal Rupture
Nevertheless, 2D echocardiography may not detect small
Ventricular septal rupture most commonly occurs after defects and therefore, color Doppler imaging is crucial
a transmural MI and is more frequent in acute anterior when looking for a ventricular septal rupture. Typically
infarction, extensive infarction, right ventricular infarction, seen are turbulent transseptal flow and systolic flow
and in total occlusion of the infarct-related artery.25,26 This disturbance within the RV. In cases of complex defects,
complication of MI is rare (incidence of 0.2–0.3%), due color Doppler imaging may demonstrate multiple holes
to the dual blood supply of the ventricular septum, with or serpinginous tracts between the LV and the RV. Using
Fig. 60.8: Example of a postmyocardial infarction ventricular

septal rupture with flow across the defect (arrows).
A B
Figs 60.9A and B: Example of a postmyocardial infarction ventricular septal rupture with flow across the defect (arrows).
A B
Figs 60.10A and B: Example of a postmyocardial infarction ventricular septal rupture (asterisk); 2D image (A) and 3D image (B).
color Doppler imaging, the ventricular septal defect can be muscle. The most frequent site of MI is, therefore, the
characterized in terms of the location and the size of the inferolateral wall.17,18 Often, the infarct size and expansion
defect. The left to right jet seen on color Doppler is typically are relatively small, and up to half of the patients have
pansystolic. In about half the patients, an aneurysm of single-vessel coronary disease, with many of them having
the ventricular septum can be seen protruding into the had their first MI. This complication generally occurs 2 to
RV. Similarly, in about half the patients, hyperkinetic LV 7 days after an MI and is responsible for approximately 5%
segments can be seen opposite the segment of the septal of acute MI-associated mortality. Mortality is very high if
defect. Decreased RV systolic function is not uncommon left untreated. About half die within the first day of rupture
and significant tricuspid regurgitation is frequently noted and 90% within a week without surgical therapy. Therefore,
secondary to high RV pressure.18,27 echocardiography is crucial for an early and accurate
Transesophageal echocardiography (TEE) is frequently diagnosis17,18 (Figs 60.11A and B and Movie clip 60.2).
performed to assess for mechanical complications in a Typical findings on 2D echocardiography include
critically ill or acutely decompensated patient following a flail mitral valve leaflet and often a mobile mass seen
an MI.27 TEE may provide better imaging compared attached to the chordae and prolapsing into the left atrium
to transthoracic echocardiography and may provide during systole, which is the head of the ruptured papillary
improved visualization of the morphology, number, muscle. Partial rupture of a papillary muscle, defined as a
location, and size of the ventricular septal defect(s), and partial disconnection of the base of the papillary muscle
consequently better guide management decisions.27 or rupture of one of several heads is seen more often than
complete rupture.17,18 In such cases, 2D echocardiography
Papillary Muscle Rupture shows a thin and excessively mobile papillary muscle. The
Papillary muscle rupture is the rarest mechanical LV systolic function is often hyperdynamic.
complications of MI.17,18 The posteromedial papillary Color Doppler imaging usually reveals severe MR, and
muscle is the most affected, having its blood supply the direction of the regurgitation jet points to the affected
only from the posterior descending coronary artery as mitral leaflet: posteriorly directed regurgitation jet in flail
opposed to the anterolateral papillary muscle that has a anterior leaflet and anteriorly directed jet in posterior
dual blood supply, both from the diagonal branch and the flail leaflet. If both leaflets are involved, the jet is centrally
left circumflex artery. Because of its single-vessel blood directed. The severity of MR may be underestimated
supply, the posteromedial papillary muscle is 6 to 12 times with eccentric regurgitant jets, and a TEE may provide
more vulnerable to rupture than the anterolateral papillary incremental data in such cases.17,18
A B
Figs 60.11A and B: Examples of papillary muscle rupture (arrows) in two different patients.
Left Ventricular Aneurysm into the lumen, and therefore have a greater risk of
embolization17,18 (Figs 60.12A and B).
A true LV aneurysm is a saccular protrusion or a bulge of Echocardiography is fairly sensitive and specific for the
the LV wall during both systole and diastole, demonstrating
detection of LV thrombus.29 Reasons for false identification
dyskinetic or akinetic motion28 (Fig. 60.6). As opposed to a
of LV thrombus include trabeculations, aberrant bands,
LV pseudoaneurysm where there is no myocardium in the
papillary muscle, tumors, or noise and artifacts that are
external layer of the pseudoaneurysm, the outer layer of a
mistaken for LV thrombus. Contrast agents improve
true LV aneurysm consists of thinned, necrotic, or scarred
visualization of LV thrombi and the sensitivity and
myocardium.17,18,28
specificity of echocardiography for the detection of LV
LV aneurysms are frequent complications of acute MI,
thrombus.24
and may be a cause of heart failure, LV thrombus formation
predisposing to the risks of systemic embolization, and
ventricular arrhythmias. LV aneurysms occur from ROLE OF ECHOCARDIOGRAPHY IN
infarct expansion and adverse remodeling of the involved CHRONIC ISCHEMIC CARDIOMYOPATHY
segment, usually the apex, which is the thinnest segment
of the normal LV myocardium.28 Echocardiography has Chronic long-standing CAD, with or without infarction, may
excellent sensitivity and specificity for the detection of LV result in progressive deterioration of LV function and other
aneurysms after MI.17,18,28 accompanying pathology such as ischemic MR, increased left
LV thrombus formation occurs almost exclusively in atrial pressure, and so on. Echocardiography is very valuable
the akinetic or dyskinetic apex with or without aneurysm in the evaluation of chronic ischemic cardiomyopathy and
formation.17,18 Most thrombi are formed within 48 hours associated conditions30 (Figs 60.13A and B).
to 1 week of the MI. Late formation of thrombus may be The assessment of LV systolic function and particularly
seen in patients with severe congestive heart failure and regional wall motion is primarily used for the diagnosis of
deteriorating LV systolic function. Chronic LV thrombus, ischemic cardiomyopathy, determination of the severity,
occurring 3 months or more after infarction, is seen with and prognostication of patients with CAD.30
a true LV aneurysm and nearly half of all patients with Ischemic cardiomyopathy is diagnosed on the basis
a true LV aneurysm have a LV thrombus. In spite of its of LV systolic dysfunction, typically with regional wall-
high prevalence, chronic mural thrombi are likely to be motion abnormalities. There are several possible reasons
organized and therefore, rarely embolize. Acute thrombi for LV systolic dysfunction in patients with chronic
within an aneurysm are likely to be mobile and projecting ischemic heart disease.
A B
Figs 60.12A and B: Example of central mitral regurgitation due to ischemic cardiomyopathy [2D image (A) and color Doppler
image (B)].
A B
Figs 60.13A and B: Examples of a left ventricular apical thrombus (arrows) in two different patients, one without (A) and one
with contrast (B).
During an acute MI, the acute occlusion of blood flow is termed “hibernating myocardium.”33 When blood
in an epicardial coronary artery leads to a rapid reduction flow to the hibernating myocardium is restored through
in resting myocardial blood flow and progressive death revascularization of the stenotic artery, contractile function
of myocardium within the perfusion territory, starting returns gradually. Systolic dysfunction in chronic CAD
from the subendocardium and extending out to the may also occur due to myocardial stunning resulting from
subepicardium in a wavefront phenomenon, as described repeated ischemic episodes occurring at rest (vasospasm)
in 1979 by Reimer and Jennings.31 When blood flow is or during increased myocardial oxygen demand such as
restored, the myocardial necrosis is halted and some exercise.33
of the acutely ischemic myocardium at risk is salvaged. Therefore, wall-motion abnormalities in patients with
The extent of myocardium salvaged depends on the ischemic cardiomyopathy may be due to multiple rea-
promptness and efficacy of the revascularization therapy. sons—myocardial necrosis, myocardial fibrosis, stunned
LV systolic function is affected soon after decrease in or hibernating myocardium, and is likely due to combina-
resting myocardial blood flow and persists after the acute tions of these factors.
MI.31 Determining the exact cause of wall-motion abnor-
In the acute and subacute stages of a MI, the dead malities in patients with ischemic cardiomyopathy carries
myocardium is necrosed and causes segmental wall- significant implications for management decisions. For
motion abnormalities. The necrosed myocardium gradu- example, hibernating myocardium may warrant revascu-
ally thins and reorganizes into scar tissue or fibrosis over larization, as opposed to fibrotic and scarred myocardium,
weeks to months. Thus, patients with a prior MI and wall- which may not respond as well to revascularization.33
motion abnormalities have myocardial fibrosis and scar Determination of LV fibrosis on rest echocardiography
tissue leading to contractile dysfunction. is based on wall thickness and echobrightness of the
After an acute MI, wall motion may not return to segments. Segments thinner than 5 mm in diastole have
normal for several hours to weeks after blood flow has been traditionally felt to be nonviable; there has been
been restored and the MI has completed. The LV systolic a long-standing thought that the likelihood of recovery
dysfunction in an area of uninfarcted myocardium is of contractility in such thinned segments is very low.34
called “myocardial stunning” and is reversible.32 However, recent data using cardiac magnetic resonance
In patients with chronic CAD, increasing reduction imaging (CMR) have challenged this paradigm and
in myocardial blood flow due to the progression of demonstrated that regional wall thinning could be
CAD may result in down-regulation of myocardial associated with limited scar burden in approximately 18%
contractile function with preserved metabolic activity. of patients and associated with improved contractility and
This is also reversible and this area of the myocardium resolution of wall thinning after revascularization.35
Myocardial contrast echocardiography permits the function following revascularization, with lower chances
assessment and quantification of myocardial blood of functional recovery with revascularization when there
flow, both at rest and during hyperemia.24,36 The basic is either no contractile improvement at either low or high
pathophysiological principle behind this technique is dose, or sustained improvement with both low and high
that viable myocardium has an intact microcirculation, doses of dobutamine.8
whereas infarcted, nonviable tissue loses this micro-
vasculature. Myocardial contrast echocardiography allows Ventricular Function in Chronic
measurement of myocardial blood flow and blood volume,
and thus provides a direct evaluation of microvascular Ischemic Heart Disease
integrity of the dysfunctional segments, and therefore,
viability and the likelihood of functional recovery after Assessment of Systolic Function
revascularization.24 Global LV systolic function in patients with ischemic car-
Contractile reserve and therefore viability can be diomyopathy may be assessed through either calculation
assessed using provocative stimuli such as exercise, of global LVEF or wall-motion scoring.38
nitroglycerin, dipyridamole, postextrasystolic poten- Left ventricular EF is most commonly computed
tiation, and catecholamine (e.g. isoprenaline, adrenaline, from echocardiography by using Simpson’s method
dopamine, or dobutamine) infusion in conjunction of using systolic and diastolic volumes obtained from
with echocardiography. Of these options, dobutamine biplane planimetry of paired orthogonal long-axis apical
stress echocardiography is the most accepted and views (Figs 60.14A and B). While quantitative techniques
widely performed of these techniques. Its diagnostic and generally are more accurate, grading based on visual
prognostic value in the detection of myocardial viability assessment is not inferior to other reference methods.
has been well established.8 In most echocardiography laboratories, the LVEF is
Dobutamine at low doses of <10 μg/kg/min demon- determined by subjective visual assessment of a single
strates a relatively more potent inotropic effect than experienced reader.
chronotropic effect, allowing stimulation of myocardial There are extensive data showing that LVEF is one of the
contractility before significant increases in heart rate, most powerful predictors of adverse outcomes including
and ischemia, occur. This is useful for the assessment mortality in patients with LV systolic dysfunction of any
of contractile reserve. Dobutamine at higher doses cause including ischemic heart disease. LVEF is the single
leads to both inotropic and chronotropic stimulation, most powerful predictor of mortality and the risk for life-
resulting in increased cardiac output, myocardial oxygen threatening ventricular arrhythmias after MI.
consumption, and ischemia in the presence of significant Wall-motion scoring analysis assigns a numeric value
CAD.8,37 to the degree of contractile dysfunction in each LV segment
The protocol for assessment of viability by dobutamine and a wall motion score index can be derived from the sum
starts with two low-dose stages (5 and 10 μg/kg/min), with of individual segment scores divided by the number of
each stage lasting 3 minutes, and the dose is increased in evaluated segments.38
10 μg/kg/min increments to a maximum dose of 50 μg/kg/ Doppler echocardiography can also provide useful
min. Atropine may be given if the target heart rate is not noninvasive estimation of left ventricular stroke volume
achieved. The test is terminated when the patient achieves and cardiac output.
85% of the age-predicted maximum heart rate, or clinical
or echocardiographic evidence of ischemia occurs. Viable
Assessment of Diastolic Function
segments will demonstrate improvement of contractility
at doses of 5 or 10 μg/kg/min. Even when viability or By definition, all patients with systolic dysfunction have
contractile reserve is noted at a low dose, higher doses varying degrees of diastolic dysfunction. Assessment of
should be administered to observe a biphasic response, diastolic function is more useful in patients with normal
wherein there is hypokinesis and decreased contractility at systolic function but clinical features of heart failure or
high doses due to ischemia. The biphasic response has the those at risk for heart failure, in whom diastolic dysfunction
best predictive value for prediction of improvement in LV is clinically silent.39
A B
Figs 60.14A and B: Example of Simpson’s method of quantification of LV ejection fraction. Diastolic frame (A) and systolic frame (B).
(LV: Left ventricle).
Details of assessment of diastolic function are beyond In patients with decreased LVEFs, the mitral inflow
the scope of this chapter and are discussed elsewhere in pattern can be used to estimate filling pressures with
this textbook. In patients with normal LVEFs, LV filling reasonable accuracy. Changes in the inflow pattern can
pressures can be calculated using measures of diastolic be used to track filling pressures in response to medical
function. An E/e' ratio (E = peak early passive diastolic therapy. In patients with impaired relaxation patterns
transmitral flow recorded by pulsed wave Doppler imaging and peak E velocities < 50 cm/s, LV filling pressures are
and e' = peak mitral annular velocity recorded by tissue usually normal. With restrictive filling, mean LA pressure
Doppler imaging) of ≤8 identifies patients with normal LV is increased.39 The use of additional Doppler parameters
filling pressures, whereas a ratio ≥13 indicates increased is recommended in patients with E/A ratios of ≥ 1 to
LV filling pressures.39 When the ratio is between 9 and 13, < 2. A change in E/A ratio with the Valsalva maneuver of
other measurements are useful. An Ar – A duration (Ar = ≥ 0.5, a systolic peak velocity/diastolic peak velocity ratio
duration of the atrial reversal waveform on pulmonary in pulmonary venous flow < 1, Ar – A duration ≥ 30 ms, E/e
venous flow recorded by pulsed wave Doppler imaging = (using average e) ≥ 15, IVRT/TE – e' > 2, and pulmonary
and A duration = duration of late diastolic wave across the artery systolic pressure ≥ 35 mm Hg (in the absence of
mitral valve recorded by pulsed wave Doppler imaging) pulmonary disease) are suggestive of the presence of
≥ 30 ms, a change in E/A ratio (E = peak early passive diastolic increased filling pressures.39
transmitral flow recorded by pulsed wave Doppler imaging
and A = peak late diastolic transmitral flow recorded by NOVEL ECHOCARDIOGRAPHY
pulsed wave Doppler imaging) with the Valsalva maneuver
TECHNIQUES IN ISCHEMIC HEART
of ≥ 0.5, IVRT/TE – e' [IVRT = isovolumetric relaxation
time, or the time interval between aortic valve closure DISEASE
and mitral valve opening as recorded by pulsed wave
Doppler imaging and TE – e' = the time interval between
Three-Dimensional Echocardiography
the QRS complex and the onset of mitral E wave (TE) on While 3D echocardiography (3DE) has potential roles
pulsed wave Doppler imaging subtracted from the time in assessment of ischemia by stress testing, wall-
interval between the QRS complex and e' onset on tissue motion abnormalities, left ventricular aneurysm, and
Doppler imaging] < 2, pulmonary artery systolic pressure pseudoaneurysm, other complications of MI, the primary
≥ 35 mm Hg (in the absence of pulmonary disease), and application in patients with ischemic heart disease, is in
maximal left atrial volume ≥ 34 mL/m2 are all indicative of the quantification of cardiac chamber volumes, function,
increased LV filling pressures.39 and LV mass.40–42
LV chamber and mass quantification have been Myocardial Ischemia

studied extensively using 3DE and have been shown
The subendocardium is the most vulnerable area to the
to be more accurate and reproducible than 2D echo-
effects of hypoperfusion and ischemia; so, LV longitudinal
cardiography techniques. A distinct advantage of 3DE
mechanics (mainly generated by subendocardium) as the
over 2D echocardiography is the ability to manipulate
most sensitive parameter may be attenuated in patients
the plane to align the true long axis and minor axis of the
with CAD even at rest. Circumferential strain and twist may
LV, thereby avoiding foreshortening and oblique imaging
remain normal or show exaggerated compensation for
planes.40–42 LV volume assessment by real time 3DE has preserving LV systolic performance. A lower longitudinal
been demonstrated to be rapid, accurate, reproducible, strain value in asymptomatic patients without wall-
and superior to conventional 2D methods.40–42 The LV motion abnormalities can be a strong predictor of stable
volumes can be segmented, which allows for regional LV ischemic cardiomyopathy. Recently, postsystolic index
function assessment. LV volume and mass obtained by has been proposed as an important quantitative marker
real time 3DE correlate well with those obtained with CMR for analysis of the ischemic myocardium. LV endocardial
or radionuclide imaging. Similarly, real time 3DE allows area change ratio by 3D speckle tracking coupled with both
improved accuracy of RV size and function assessment. longitudinal and circumferential strain have proposed
Use of real time 3DE in stress echocardiography to assess induced acute myocardial ischemia in a sheep
decreases imaging time (standard views can be obtained model.46
with only 1 or 2 image acquisitions) with accuracy
comparable to 2D stress imaging.40–42 Myocardial Infarction
Use of ultrasound contrast agents with 3DE potentially
Longitudinal strains are significantly reduced in patients
improves quantification of LV volumes and EF. Another with MI, proportionately within the area of infarction,
evolving application of contrast 3DE is in the evaluation and correlate closely with peak infarct mass and EF. While
of myocardial perfusion.43 Contrast 3DE offers the ability longitudinal strain has been used to detect MI, radial and
to image the entire LV and to quantify the full extent of circumferential strain values have been used to distinguish
hypoperfused myocardium.43 nontransmural infarction from transmural infarction47
Thus, 3DE is complementary to 2D imaging and can be (Figs 60.15 and 60.16). The pattern of postsystolic
used to improve assessment of cardiac function and mor- shortening peak strain after aortic valve closure has been
phology in patients with ischemic heart disease. Further also reported helpful. In some studies, longitudinal strain
technological developments will lead to improvements was related to peak levels of cardiac troponin T and the
and will contribute to more practical applications of 3DE LV infarct size. It has also been shown that longitudinal
in this group of patients.40–43 strain correlates with the global and regional extent
(transmurality) of scar tissue as evaluated by contrast-
Speckle-Tracking Echocardiography enhanced MRI.47
Strain imaging potentially has incremental value in the

Reperfusion
ability of echocardiography to detect and objectively
qualify ischemia and infarction.44,45 Speckle-tracking Longitudinal strain (measured immediately after reper-
echocardiography as a new noninvasive imaging tech- fusion therapy) has been suggested as an excellent
nique can be helpful for an objective and quantitative predictor of LV remodeling and adverse events, such as
evaluation of global and regional myocardial function. congestive heart failure and death.45 When combined
In contrast to tissue Doppler imaging, it works almost with wall-motion scoring, strain rate imaging offered
independently from the angle of insonation and from incremental value over wall-motion scoring alone for
cardiac translational movements. This technique is prediction of functional recovery after revascularization.48
potentially a better tool to detect myocardial ischemic
segments and also differentiate active contraction from Myocardial Viability
passive motion such as passive expansion, and recoil and Strain imaging as an adjunct to low-dose dobutamine
tethering from adjacent segments.44–46 stress echocardiography has been suggested to assess
A B
Figs 60.15A and B: Polar map derived from speckle-tracking echocardiography showing longitudinal strain in different segments of
two cases, (A) Normal subject (homogeneous yellow) and (B) A patient with a LAD lesion with lower (orange) and positive strain (blue)
in the anteroseptal and septal segments.
A B
Figs 60.16A and B: Speckle-tracking echocardiography demonstrating segmental longitudinal strain in a normal subject (A) and in a
patient with hypokinesis and dyskinesis due to a LAD lesion (B).
myocardial viability.49 In addition, a favorable correlation FUTURE

between resting longitudinal LV strain and the extent
of scarring by CMR imaging has been reported. Both Despite the emergence of competing imaging modalities,
myocardial velocity and deformation parameters have echocardiography remains and will continue to be the
been analyzed at rest and during dobutamine stimulation primary imaging modality for patients with ischemic
to define the functional reserve.49 heart disease due to its portability, ease of use,
Although speckle tracking is regarded as a revolution safety, and technological advancements in terms of
in echocardiography, prospective clinical trials for the techniques and instrumentation such as tissue Doppler
validation of this technique in large populations are still imaging, 3DE, speckle-tracking imaging, and contrast
lacking. To date, strain rate has not replaced conventional echocardiography. Future advances in technology
gray-scale imaging in the assessment of regional LV and increased clinical data will lead to overcoming of
function and the implementation of these new indices into current limitations and further increase the value of
routine clinical practice will need additional clinical and echocardiography in the everyday clinical use for patients
large-scale studies.44 with ischemic heart disease.
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CHAPTER 61
Stress Echocardiography
Azhar Supariwala, Siu-Sun Yao, Farooq A Chaudhry
Snapshot
Fundamentals of Stress Echocardiography
¾¾ Interpretation of Stress Echocardiography
¾¾
Types of Stress Echocardiography
¾¾ Stress Echocardiography: Future Directions
¾¾
INTRODUCTION cascade6 (Fig. 61.1). Normal coronary arteries adapt

to stress by coronary vasodilatation (coronary flow
Stress echocardiography was first introduced by Wann
reserve) to maintain flow to meet the increased oxygen
and coworkers with supine bicycle exercise,1 but the
demand. In the presence of a flow-limiting coronary
application was limited until the development of digital
stenosis, coronary flow reserve is impaired resulting in
acquisition.2,3 Stress echocardiography is now routinely
ischemic cascade including wall motion abnormality.
used in the diagnosis, risk stratification, and prognosis of
Abnormalities of regional relaxation of the left ventricle
patients with known or suspected coronary artery disease
precede systolic wall motion abnormalities. The
(CAD).4,5 In recent years, there have been further advances
segmental wall motion abnormality is characterized
in the application of stress echocardiography, including
echocardiographically as a reduction in systolic
the evaluation of valvular and anatomic abnormalities
thickening and endocardial excu rsion. The severity
of the heart, hemodynamics, myocardial perfusion,
quantitation of wall motion abnormality with speckle and extent of wall motion abnormality depends on
tracking, and three-dimensional (3D) imaging. multiple factors including severity of stenosis, level of
stress, coronary flow reserve, and collateral circulation.
Electrocardiographic changes and symptoms may or
FUNDAMENTALS OF STRESS
may not occur until late in the ischemic cascade and
ECHOCARDIOGRAPHY may not even be present in patients with mild ischemia.
The most common application of stress echocardiography Endothelial dysfunction secondary to hypertensive
is the detection of flow limiting stenosis. The use of heart disease and diabetes mellitus can also cause wall
stress echocardiography to diagnose flow-limiting CAD motion abnormalities during stress in the absence of
is based on a sequence of events known as the ischemic flow-limiting epicardial coronary stenosis.7,8
Chapter 61: Stress Echocardiography 1307
echocardiography is that it is more physiologic and higher

peak heart rates are achieved. The disadvantage is that
images are acquired postexercise as compared to bicycle
stress where images are obtained at peak stress. However,
bicycle stress achieves lower peak heart rate, and image
acquisition is more difficult during peak exercise.
Dobutamine Stress Echocardiography

In patients who are unable to exercise or attain adequate
levels of workload, pharmacological stress is preferred.
With stress echocardiography, dobutamine is preferred
as it is more likely to provoke ischemia as compared to
vasodilator agents such as adenosine or dipyridamole.
Dobutamine is a sympathomimetic drug that has
Fig. 61.1: Ischemic cascade: the sequence of events that occur
after a flow-limiting stenosis. The decrease in flow produces
both b-1 and b-2 adrenergic and a-1 activity. The affinity
biochemical metabolic abnormalities first, followed by detectable of dobutamine for b-1 cardiac muscle receptors results
perfusion defects by positron emission tomography (PET) or in positive inotropic, and, to a lesser extent, positive
single photon emission computed tomography (SPECT), followed chronotropic response. These actions are dose-dependent.
by perfusion, diastolic, and systolic abnormalities that could be
At lower doses, the inotropic response prevails, and
detected by stress echocardiography with microbubble and
speckle strain, eventually leading to ischemic electrocardiographic at higher doses, chronotropic activity is predominant.
changes and anginal symptoms. Dobutamine induces myocardial ischemia in patients
with flow-limiting coronary artery stenosis by increasing
left ventricular contractility, heart rate, wall stress, and
TYPES OF STRESS therefore, myocardial oxygen demand.12
Dobutamine is administered as an infusion starting
ECHOCARDIOGRAPHY at 5 or 10 μg/kg/min and is increased by 5–10 μg/kg every
There are various ways to induce stress such as dynamic 3 minutes until a maximal dose of 40–50 μg/kg/min or until
exercise (treadmill or bicycle), pharmacologic stress 85% of the age-predicted maximum heart rate is achieved.
(dobutamine, dipyridamole, and adenosine), and pacing Atropine is used in ~30% of patients in conjunction with
stress.9 Hand grip and leg raise are commonly employed dobutamine in 0.25 mg increments up to a maximum
as an adjunct to the above-mentioned stress modes to dose of 2 mg. Low-dose images are obtained at 5 or 10
further augment heart rate.10 μg/kg/min or when increased contractility is observed.
Peak stress images are obtained after ≥ 85% of maximal
Exercise Echocardiography predicted heart rate is achieved. The images are displayed
as four digitized cine loops that show rest, low dose, peak
Treadmill exercise using the Bruce protocol is the most dose, and recovery.
widely used stress modality in the United States. Supine
or semi-supine bicycle stress is widely used in Europe
Vasodilator Stress Echocardiography
and in some centers in the United States.11 The images in
treadmill exercise are acquired immediately postexercise Major coronary vasodilators used for stress echocardi
(Figs 61.2A and B). All images are acquired within 30–60 ography are adenosine and dipyridamole. Adenosine
seconds, and the entire sequence is repeated to acquire a is an endogenously produced substance with potent
second set of images. vasodilator properties. Adenosine induces blood flow
Reasons for termination of stress test are symptoms of heterogeneity by stimulating A2 receptor inducing vasodi
typical angina, marked dyspnea, significant ST segment latation. Dipyridamole exerts its effects indirectly by
depression or elevation, sustained arrhythmias, or hemo reducing the reuptake of adenosine. In the setting of a
dynamic compromise (hypotension or systolic blood critical coronary artery stenosis, the flow may increase
pressure > 220 mm Hg). The advantage of exercise stress in the epicardium but falls in the subendocardium distal
A B
Figs 61.2A and B: The equipment and set-up for performing a treadmill exercise stress echocardiography.
6 minutes after initiation of adenosine. Recovery images

for both dipyridamole and adenosine are obtained at
10- and 12-minute intervals from the start of infusion.
Adjunctive atropine and handgrip and leg lift exercise may
be used to further increase myocardial demand and induce
ischemia.14 The advantage of the vasodilator stress test is
that if an adverse event occurs, it resolves after termination
of infusion or after the administration of aminophylline.
The disadvantages are that it is contraindicated in patients
with severe chronic obstructive lung disease and those
taking theophylline preparations or who have recently
ingested caffeine. Vasodilators are also less likely to
precipitate ischemia compared to dobutamine.
Fig. 61.3: This is an example of a technically difficult dobutamine Contrast Echocardiography

stress echocardiogram. The noncontrast study had very poor
endocardial definition. Administration of contrast improved endo- As many as 20–30% of patients referred for stress
cardial delineation. The rest images show akinesis in the apex imaging have suboptimal visualization of the left
and apical anterior wall. The low-dose images show increased ventricular endocardial border echocardiographically.15
contractility throughout the myocardium except in the apex and
Echocardiography contrast agents significantly improve
apical anterior wall. At peak stress, no new wall motion abnor-
malities were observed with persistent akinesis, which is consist- the blood–endocardial border visualization at rest and
ent with nonviable tissue. This study is consistent with scar in the stress.16 Contrast-enhanced echocardiography improves
apex and apical anterior wall without any significant ischemia. images in patients with poor acoustic windows and thus
(LV: Left ventricle; RV: Right ventricle). (Movie clip 61.1). improves inter- and intraobserver variability and diagn
ostic accuracy.15 (Fig. 61.3 and Movie clip 61.1)
to the flow-limiting stenosis, resulting in ischemia and

subsequent wall motion abnormalities.13 Dipyridamole
Safety of Stress Echocardiography
is administered intravenously over 6 minutes at a rate of Absolute contraindications for stress echocardiography
0.14 mg/kg/min. Peak stress images are obtained at 4- and are: (a) unstable angina, (b) decompensated congestive
6-minute intervals from the start of infusion. Adenosine heart failure, (c) uncontrolled hypertension (> 210/110
is infused starting at 140 μg/kg/min over a 4- to 6-minute mm Hg), (d) uncontrolled cardiac arrhythmias (causing
period. Peak stress images are obtained at 3 minutes and symptoms or hemodynamic compromise), and (e) severe
Fig. 61.4: A baseline echocardiogram showing aortic dissection in Fig. 61.5: Interpretation of response to stress echocardiography
a patient hospitalized for chest pain. The stress echocardiogram for each of the 17 segments. During exercise stress, the thicken-
was cancelled and the patient underwent a computed tomography ing and excursion of segments at rest, peak, and recovery are
of the chest, which was consistent with ascending aortic dissection compared. During dobutamine stress, segments are compared
(Type A). The patient underwent subsequent surgery for repair at rest, low dose, peak dose, and recovery. Row 1/biphasic: de-
of the aortic dissection. (LA: Left atrium; LV: Left ventricle) scribes an ischemic response in both exercise and dobutamine.
(Movie clip 61.2). Row 2/monophasic: describes a normal response to either exer-
cise or dobutamine stress with inotropic contractile reserve. Row
3/nonphasic: describes scar or nonviable myocardium.
symptomatic aortic stenosis. The relative safety of stress
echocardiography is similar to other forms of stress testing ventricular end-systolic volume. Figure 61.5 describes the
modalities. In a study of 85,000 patients, the event rate various wall motion responses and their interpretation. The
was 1 in 6,574 for exercise, 1 in 557 for dobutamine, and left ventricle is divided into a 17-segment model at rest and
1 in 1,294 for dipyridamole.17,18 Minor arrhythmias such stress. The 17-segment model with the distribution of the
as premature ventricular contractions, atrial arrhythmia, respective coronary arteries is shown in Figure 61.6. Each
nonsustained ventricular tachycardia (3%) are common segment is scored as follows: 1 = normal, 2 = hypokinesis
with dobutamine stress and are not necessarily indicative (reduced wall thickening and excursion), 3 = akinesis (no
of CAD. Adenosine is well tolerated in the majority wall thickening and excursion), 4 = dyskinesis (paradoxical
(> 80%) of patients. In the Adenoscan Multicenter Trial, the motion away from the center of the left ventricle during
infusion was prematurely terminated in < 0.01% of patients, systole) and 5 = Aneurysmal.22 It is important to remember
only 0.8% received aminophylline, and no sustained that the normal myocardial response postexercise is
episodes of atrioventricular block were observed.19 thickening of the myocardium, marked excursion of the
endocardium, and almost complete obliteration of the left
INTERPRETATION OF STRESS ventricular cavity.23 With dobutamine stress in patients
with rest wall motion abnormality, ischemia is defined as
ECHOCARDIOGRAPHY improvement of wall motion at low dose and deterioration
Among expert readers, interpretation of stress echocar of wall motion at peak dose (biphasic response). Therefore,
diography has been highly reproducible.20 Interobserver an abnormal (ischemic) response to stress is defined as:
variability is mostly due to suboptimal image quality and (a) a deterioration in left ventricle wall segment thickening
subtle degrees of wall motion abnormality.21 The resting and excursion during stress (increase in wall motion of
echocardiogram may reveal important diagnosis such as ≥ 1 grade) or (b) a biphasic response with dobutamine
aortic dissection, critical aortic stenosis, and obstructive stress during which wall motion abnormality improves
hypertrophic cardiomyopathy (HCM) (Fig. 61.4 and Movie at baseline (viable myocardium) and then deteriorates
clip 61.2). at peak stress (ischemia). Furthermore, an abnormal
A normal response during either exercise or pharma increase in end-systolic left ventricle volume [transient
cological stress echocardiogram is an increase in wall ischemic dilatation (TID)] is associated with multivessel
thickening and endocardial excursion with decrease in left CAD.24,25
Fig. 61.6: The American Society of Echocardiography (ASE) has divided the left ventricle into 17 segments. The schematic
demonstrates the relationship between the coronary artery distribution and the corresponding ASE 17 left ventricular segments.
Analyses of stress echocardiogram should provide the quantitative result according to this model. The four standard echocardiography
views provide evaluation of territories of each of the three main coronary arteries. (Ao: Aorta valve; LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle). Source: Reproduced with permission from Vidhun R Echocardiography pocketcard
set, second edition. Börm Bruckmeier Publishing; 2010.
wall segments divided by the number of visualized

segments. A normal study has a WMSI of 1.0 at both rest
and stress (Fig. 61.7 and Movie clip 61.3) A WMSI of >1.0 at
rest and stress is abnormal (Fig. 61.8 and Movie clip 61.4).
Diagnostic Accuracy to Detect CAD

Using angiography as the gold standard for comparison
for a > 50% stenosis by quantitative coronary angiography
and 70% visually, the overall sensitivity for stress
echocardiography is 75–85% and specificity is 80–90%
(Table 61.1).26–29 Sensitivity and specificity are comparable
in both men and women and among different stress
modalities (exercise, dobutamine, and vasodilator).29
Studies comparing the accuracy of single photon emission
Fig. 61.7: An example of a normal treadmill exercise echocar- computed tomography myocardial perfusion imaging
diogram demonstrating a hyperdynamic response to stress. The (SPECT-MPI) and stress echocardiography in the same
standard format to display exercise stress echocardiography
patient population have shown similar sensitivities for the
images. It demonstrates side-by-side rest-stress images. The
heart rate and time of image acquisition postexercise are displayed detection of CAD, with stress echocardiography having a
for each image. The resting study is on the left and postexercise higher specificity (Table 61.2).30
study is on the right. The patient had 1-mm ST depressions, which Factors that can cause false-positive or false-negative
are likely false-positive ECG changes (Movie clip 61.3). (ECG:
Electrocardiogram; LV: Left ventricle; RV: Right ventricle).
results are listed in Table 61.3.
A semiquantitative approach for image interpretation

Risk Stratification and Prognosis
is done by calculation of the wall motion score index The goal of prognostic testing is based on the premise
(WMSI). WMSI is a cumulative sum score of 17 left ventricle that patients identified as being at highest risk for adverse
Fig. 61.8: This treadmill exercise echocardiogram demonstrates ischemia. The resting study is normal while the
postexercise images reveal anteroapical wall motion abnormalities. The distribution is consistent with obstructive
coronary artery disease involving left anterior descending artery. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle) (Movie clip 61.4).
outcomes can be intervened upon to alter the natural wall motion abnormalities in a designated segment, both
history of their disease process, thereby reducing future being quantified at peak stress. Ischemic extent reflects the
cardiac risk. number of new stress-induced wall motion abnormalities
Stress echocardiography results can risk stratify and corresponds roughly to the number of stenosed
and prognosticate patients into a low-risk group (< 1%), coronary arteries. Maximal severity reflects the magnitude
intermediate (1–5%), and high (> 5%) risk for major cardiac of ischemia within a designated myocardial segment and
mortality.31 Table 61.4 summarizes the different variables reflects the severity of a subtending coronary stenosis
important in identifying risk and predicting prognosis. within a given coronary artery territory. Estimation of both
Patients with mild-to-moderate wall motion abnormalities ischemic extent and maximal severity variables by stress
(peak WMSI = 1.1–1.7) have an intermediate risk of cardiac echocardiography provides a functional depiction of a
events and if stable, may be initially managed medically.32 noninvasive coronary angiogram and accurate prognostic
Patients with peak WMSI > 1.7 and especially those assessment of the amount of jeopardized myocardium.
with ejection fraction ≤ 45% are at high risk of cardiac The extent and severity of wall motion abnormalities
events (Fig. 61.9). Such patients should be appropriately by stress echocardiography are independent and cumu
referred to catheterization and consideration of coronary lative predictors of prognosis.34 Thus, prognostic risk
revascularization in order to decrease future cardio stratification by stress echocardiography is both a separate
vascular risk.33 and combined function of the extent and severity of
wall motion abnormalities (Fig. 61.10). This proposed
Extent and Severity of Myocardial Wall model extends the use of stress echocardiography from a
Motion Abnormality as Predictors simple diagnostic tool, toward establishing its utilization
in precise risk stratification, prognosis, and direction of
of Prognosis patient management decisions.
The prognostic value of stress echocardiography is
based on its ability to quantify the severity and extent of Prediction of Myocardial Infarction Versus
jeopardized (ischemic) myocardium with exercise or
pharmacologic stress. The ischemic extent reflects the areas
Cardiac Death by Stress Echocardiography
of myocardium (number of segments) that are abnormal, Identifying patients at high risk for ischemic events versus
and maximal severity reflects the degree or magnitude of high risk for sudden cardiac death is important in deciding
Table 61.1: Studies with Diagnostic Accuracy of Treadmill, Dobutamine, and Vasodilator Stress Echocardiography Compared to
Coronary Angiographya
Studies Study Size Sensitivity (%) NPV(%) Specificity (%) PPV (%) Accuracy (%)
Peteiro et al. 201258 116 patients
Peak supine bicycle SE 84 63 77
Peak treadmill exercise SE 75 80 77
Post treadmill exercise SE 60 78 66

Jang et al. 201159 1,287 Treadmill Exercise SE
68 61 78 83 72
(Korean population)
Aggeli et al. 201160 60 patients dobutamine stress
Two-dimensional 80 82
Real time, three-dimensional
82 64
with perfusion
Meta-analyses:
Gargiulo et al. 201161 11 studies (701 hypertensive
77 89
patients)
Mahajan et al. 201027 23 studies (15 SPECT, 14 SE) 94 40
Geleijnse et al. 62 studies (6,881 patients)
200962
With prior myocardial infarction 83 81
Without prior myocardial
74 85
infarction
Patients without RWMA 76 88
Patients with RWMA 82 81

Picano et al. 200829 5 studies (435 patients)
Dipyridamole 85 89 87
Dobutamine 86 86 84
de Albuquerque 8 studies (533 patients)
Fonseca et al. 200163
Dipyridamole 72 82 77
Treadmill 79 92 80
a
Obstructive disease considered > 50% stenosis on coronary angiography.
(NPV: Negative predictive value; PPV: Positive predictive value; RWMA: Rest wall motion abnormality; SE: Stress echocardiography;
SPECT: Single photon emission positron computed tomography).
on the optimal clinical management strategy. Patients death and nonfatal myocardial infarction.33 Patients with
at high risk for ischemic events but low risk for cardiac ejection fraction < 30% are at high risk of cardiac death
death benefit more from medical therapy, but patients at (> 4%/year), and these patients should be aggressively
intermediate to high risk of cardiac death benefit more managed with optimal medical therapy, consideration of
from early revascularization.35 Stress echocardiography revascularization, device, and cardiac resynchronization
is an effective modality at differential risk stratification therapy (Fig. 61.11). In patients with ejection fraction
of patients for the outcome-specific end points of cardiac ≥ 30%, peak WMSI can further risk stratify into a low-
Table 61.2: Comparative Accuracy of Stress Echocardiography Versus Nuclear SPECT Imaging
Echocardiography SPECT
Studies Study size Sensitivity (%) Specificity (%) Sensitivity (%) Specificity (%)
Meta-analyses:
Heijenbrok-Kal et al.28 351 patients 79 87 88 73
Imran et al. 2003a 64 10 studies (651 patients) 70 90 88 67
Fleischmann et al.30 EXSE 24 studies (2,637 patients); 85 77 87 64
EXSPECT 27 studies (3,237 patients)
O’Keefe et al.65 SE 12 studies (913 patients); 81 89 90 72
SPECT 12 studies (2,626 patients)
Pharmacologic SE 14 studies 81 83 87a 75a
(1,049 patients);
Pharmacologic SPECT 14 studies 89b 83b
a
Dipyridamole stress echocardiography versus SPECT.
b
Adenosine SPECT. (EXSE: Exercise stress echocardiography; EXSPECT: Exercise SPECT; SE: Stress echocardiography; SPECT: Single
photon emission computed tomography).
Table 61.3: Factors that Affect Accuracy of Stress Echocardiography for Detecting Hemodynamic Obstructive Coronary Artery
Disease Compared with Coronary Angiography
False Positive False Negative
Hypertensive response to stress Submaximal stress (< 85% maximal predicted heart rate)
Microvascular disease: e.g. diabetes, left ventricular Poor image quality
hypertrophy, syndrome X, hypertrophic cardiomyopathy
Cardiomyopathies Delayed poststress image acquisition
Paradoxical septal motion, e.g. postcardiac surgery, left bundle Very mild ischemia, circumflex coronary stenosis, branch
branch block or distal stenosis
Coronary spasm, endothelial dysfunction Good coronary reserve (collateral circulation), potent
endothelial function
Localized basal inferior wall motion abnormalities Antianginal drug therapy during testing (calcium channel
blockers, b-blockers, nitrates)
Fig. 61.9: Cardiac event rate per year as a function of wall motion Fig. 61.10: Cumulative effect of ischemic extent and maximal
score index. Worse cardiac event rate is observed with higher severity (jeopardized myocardium) of wall motion abnormalities
peak wall motion score index. on event rate per year. The event rate increases as a curvilinear
Source: Reprinted with permission of Elsevier from Yao S, Qureshi function of both extent and severity combined.
E, Sherrid MV, Chaudhry FA. Practical applications in stress Source: Reprinted with permission of Elsevier from Yao S, Qureshi
echocardiography: risk stratification and prognosis in patients E, Syed A, Chaudhry FA. Novel stress echocardiographic model
with known or suspected ischemic heart disease. Journal of the incorporating the extent and severity of wall motion abnormality for
American College of Cardiology. 2003;42(6):1084-90. risk stratification and prognosis. American Journal of Cardiology.
2004;94(6):715-9.
Table 61.4: Stress Echocardiography Predictors of Risk

Very Low Risk < 0.5 to 1%/year Low Risk < 2%/year Intermediate Risk High Risk (> 4%/year) Incremental Risk/Other
2% to 4%/year Independent Predictors
Normal exercise stress Normal pharmaco- pWMSI 1.0–1.7 Extensive resting wall Age
echocardiography logical stress test Resting ejection motion abnormalities Male gender
fraction > 50% (4–7 segments) Prior history of CHF or MI
> 6 METs achieved Submaximal stress Single vessel Resting ejection > 3 CAD risk factors
test (< 85% MPHR) disease fraction < 45% Left atrial size
(LCx or RCA)
Achieved > 85% MPHR Off anti-ischemic Extensive ischemia Multivessel coronary artery
therapy (4–5 segments) disease
Transient ischemic left
ventricular cavity dilatation
pWMSI > 1.7 Abnormal Right ventricular
Ischemia induced at wall motion
lower workload Limited exercise capacity.
Inability to exercise
On anti-ischemic Ischemic ECG or symptoms
therapy on stress
High pretest likelihood
(CAD: Coronary artery disease; CHF: Congestive heart failure; ECG: Electrocardiogram; LCx: Left circumflex; METs: Metabolic equiv-
alent of tasks; MI: Myocardial infarction; MPHR: Maximal predicted heart rate; pWMSI: Peak wall motion score index; RCA: Right
coronary artery).
Source: Modified with permission of Elsevier from Pellikka PA, Nagueh SF, Elhendy AA, et al. Echocardiography recommendations
for performance, interpretation and application of stress echocardiography. Journal of American Society of Echocardiography.
2007;20(9):1021-41.
Fig. 61.11: Schematic for the risk stratification of patients undergoing stress echocardiography. (CAD: Coronary artery disease; CD:
Cardiac death; CRT: Cardiac re-synchronization therapy; EF: Ejection fraction; WMSI: Wall motion score index).
Source: Reprinted with permission of Elsevier from Bangalore S, Yao S, Chaudhry FA. Prediction of myocardial infarction versus cardiac
death by stress echocardiography. Journal of the American Society of Echocardiography. 2009;22(3):261-7).
Fig. 61.12: This figure demonstrates an abnormal left ventricular Fig. 61.13: Cardiac event rate per year as a function of stress
volume response to stress. The resting study is normal. echocardiogram results and transient ischemic dilatation (TID). A
Postexercise, there is evidence of anteroseptal, apical, and lateral 15-fold increase in cardiac event rate is observed with TID.
ischemia, resulting in left ventricular dilatation or transient ischemic Source: Reprinted with permission of Elsevier from Yao S, Shah
dilatation (TID). Cardiac catheterization was consistent with A, Bangalore S, Chaudhry FA. Transient ischemic left ventricular
severe multivessel coronary artery disease. (LV: Left ventricle). cavity dilation is a significant predictor of severe and extensive
coronary artery disease and adverse outcome in patients under-
(Movie clip 61.5).
going stress echocardiography. Journal of the American Society of
Echocardiography. 2007;20(4):352-8).
risk group (pWMSI = 1.0; cardiac death rate < 1.0%/year) WMSI, and a worse prognosis than patients without TID
managed with risk factor modification alone, a low-inter (Fig. 61.13). TID during stress echocardiography is a
mediate risk group (pWMSI = 1.1–1.7; cardiac death rate sensitive marker of severe and extensive angiographic
1.0–2.5%/year) managed with optimal medical therapy CAD and is associated with a very high risk of cardiac
and consideration of revascularization for symptom relief events (19.7%/year event rate).25 These patients should be
only, and a high-intermediate risk group (WMSI >1.7; referred for consideration of catheterization and coronary
cardiac death rate 2.5–4%/year) managed with optimal revascularization as the best means to modify and reduce
medical therapy and consideration of revascularization future cardiac risk.24,25
(to decrease future cardiac risk).36 Table 61.5 summarizes
the list of studies reporting the prognostic value of stress Role of Right Ventricular Wall Motion
echocardiography. Abnormalities in Risk Stratification
and Prognosis
Transient Ischemic Left Ventricular The right ventricle is often termed the “forgotten cham
Cavity Dilatation ber.” Evidence from clinical studies emphasizes the
importance of evaluating right ventricular function during
Transient ischemic left ventricular cavity dilatation (TID)
routine echocardiography. Right ventricular function has
signifies an increase in left ventricular cavity size after a prognostic implications in patients with CAD and heart
wall motion abnormality induced with exercise or dobu failure.37
tamine stress. Plausible clinical explanations for TID With stress echocardiography, abnormal right vent
include: subendocardial ischemia, systolic left ventricular ricle function (ischemia or infarction) can further risk
dysfunction, and actual physical left ventricular dilatation stratify and add prognostic value to left ventricular
in end-diastole (Fig. 61.12 and Movie clip 61.5). TID has functional parameters.38 Patients with both an abnormal
a high sensitivity (100%) and moderate specificity (54%) right ventricle and left ventricle have a worse prognosis.
for the detection of severe and extensive angiographic Right ventricular wall motion analysis should be routinely
CAD.25 Patients with TID have a greater extent and severity assessed in patients referred for stress echocardiography
of stress-induced wall motion abnormality, higher peak for more effective and complete risk stratification.
Table 61.5: Studies Reporting the Prognostic Value of Stress Echocardiography

Studies No. of Patients Follow-Up Duration Normal Abnormal
Wever Pinzon et al.66 311 HIV patients 2.9 ± 1.9 years 0.6%/year MI/CD 11.8%/year MI/CD
Bangalore et al. 67
1,002 LVH patients 2.6 ± 1.1 years 1.1%/year MI/CD 4.9%/year MI/CD
Wake et al.68 890 patients 30 ± 17 months 88% 2-year (men) 74% 2-year (men)
(contrast-enhanced DSE)
91% 2-year (women) 80% 2-year
(women)
Metz et al.69 Meta-analyses 33 months 0.54%/year-MI/CD —
3,021 patients
Chaowalit et al.70 3,014 patients (DSE) Median 6.3 years 93% 5-year MI/ —
revascularization
78% 5-year all-cause mortality
D’Andrea et al. 71
607 patients (supine bicycle SE) Mean 46 months 96% 5-year 84% 5-year
Biagini et al. 72
3,381 patients (DSE) 7 ± 3.4 years 2.5%/year (men) MI/CD 5.9%/year (men)
1.2%/year (women) MI/CD 4.6%/year
(women)
Sozzi et al.73 401 patients (DSE) 5 ± 1.7 years 0.8%/year (first 3 years)
MI/CD
1.7%/year (between 4th and
6th year of follow-up)
Yao et al.31 1,500 patients 2.7 ± 1.0 years 0.9%/year 4.2%/year overall;
1.4%/year TME,
4.7% DSE
Hoque et al.74 206 patients EXSE 8.8 ± 2.9 years 0.8%/year all-cause mortality Moderate to large
< 0.5%/year CD ischemia: 5.5%/
year CD
Sicari et al.75 7,333 patients Dipy or DSE 2.6 ± 3 years 0.9%/year or 71.2% at ~16 years
92% at ~16 years CD
Elhendy et al.76 4,347 patients EXSE Median 3 years 97.5% at 5 years (< 1%/year) 89.7% at 5 years
CD
Marwick et al.77,78 1,581 patients DSE 3.8 years ± 1.9 years 1.2%/year —
5,375 patients EXSE 5.5 ± 1.9 years 1.0%/year 2–4%/year CD
Krivokapich et al.79 558 patients DSE 12 months 10% all cardiac events 34% all cardiac
3% hard events events
10% hard events
Poldermans et al.80 1,734 patients DSE Median 3 years 1.3%/year —
(range 6–96
months)
McCully et al.81 1,325 patients EXSE Median 2 years 0.9%/year —
(range 5–65
months)
(ACM: All-cause mortality; CD: Cardiac death; Dipy: Dipyridamole; DSE: Dobutamine stress echocardiography; EXSE: Exercise stress
echocardiography; HIV: Human immunodeficiency virus; LVH: Left ventricular hypertrophy; MI: Myocardial infarction; TME: Tread-
mill exercise stress echocardiography).
Role of Left Atrial Size in Risk self-referral incentives for coronary angiography, stress
echocardiography is an effective gatekeeper for an
Stratification and Prognosis invasive management strategy. Patients with normal stress
An increase in left atrial dimension is a risk factor for atrial echocardiography studies (pWMSI = 1.0) have uniformly
fibrillation, stroke, and death and is closely related to low referral rates for early coronary angiography (1.7% at
general cardiovascular mortality.39 Left atrial size reflects 30 days) and late revascularization (2.8% percutaneous
the chronicity and magnitude of increased left ventricular coronary intervention, 1.1% coronary artery bypass graft
filling pressure. Left atrial size is a marker of the severity surgery at 2 years). The frequency of referral to coronary
and duration of left ventricular diastolic dysfunction in angiography and revascularization increased in proportion
patients without significant mitral valve disease or systolic to magnitude of the extent and severity of abnormal stress
heart failure. echocardiography. The fact that only a minority of patients
Left atrial size has been found to further risk stratify with abnormal stress echocardiography were referred for
patients with both normal and abnormal stress echocar coronary angiography and revascularization implies that
diography results.40 A normal stress echocar diography such decisions are often complex and incorporate other
in the setting of a normal left atrial size confers a benign comorbidities into the decision on whether to refer for
prognosis (< 1% year). Left atrial size alone provides invasive testing. These findings are also consistent with a
independent and incremental prognostic value, indep low referral for coronary angiography and revascularization
endent of traditional risk factors, ejection fraction, and following abnormal nuclear scintigraphy studies.43
stress echocardiographic variables. Left atrial size should
be routinely incorporated in prognostic interpretation of
Cost-Effectiveness of Stress
stress echocardiography.
Echocardiography in Postmyocardial
“Warranty Time” of a Normal Infarction Patients
Stress Echocardiogram Cost-effectiveness of the four testing strategies in patients
with prior myocardial infarction was previously evaluated.44
A normal stress echocardiogram confers a benign prog
A primary cardiac catheterization strategy (Strategy 1)
nosis (< 1%/year) in most subgroups of patients. However,
was found to be 23% more expensive, a primary stress
it is unclear at what time a stress echocardiogram should
electrocardiogram (ECG)/exercise treadmill test strategy
be repeated for risk stratification and prognosis if clinically (Strategy 2 and Strategy 3) was 82% more expensive
warranted (change in chest pain or clinical symptom when compared to a primary stress echocardiography
characteristics). strategy (Strategy 4). In patients undergoing stress
In patients with a normal stress echocardiogram, the echocardiography followed by cardiac catheterization
event rate at the end of 6, 12, and 18 months was <1%/year.41 (Strategy 4), the total cost savings was $57,293/patient
After 18 months, the event rate in patients with a normal compared to a primary cardiac catheterization strategy,
stress echocardiogram increases to >1%/year. Thus, a which translated into cost savings of $217/patient correctly
normal stress echocardiogram has a benign prognosis identified. Given the high-risk nature of postmyocardial
(< 1%/year) for up to 18 months and may be repeated infarction patients, it is no surprise that a primary stress
after that if clinically warranted for effective risk ECG/exercise treadmill test strategy was not cost-effective.
stratification. The cost-effectiveness analysis of the postmyocardial
infarction patient study showed that a strategy based on
Impact of Stress Echocardiography initial stress testing and cardiac catheterization only in
patients with abnormal stress echocardiogram is ~$217/
on Patient Outcome
patient correctly identified, more economical than a
The ultimate maturity of an imaging modality is confi primary invasive strategy. With a projected incidence of
rmed by patient outcome data. There has been a demo 1.1 million new, recurrent, or silent myocardial infarctions
nstrated parallel between the degree of abnormal stress for 2013 by American Heart Association (AHA),45 the above
echocardiography results and referral to coronary data could translate into cost savings of billions of dollars
angiography and revascularization.42 Despite physician each year. This is in concordance with previous studies by
Shaw and colleagues who showed that patients with stable ACC/AHA recommends stress echocardiography as a
angina who went directly to coronary angiography had a Class I indication for symptom assessment and severity of
much higher utilization of subsequent revascularization mitral stenosis. An increase in mean transmitral pressure
but with a similar rate of death and myocardial infarction, gradient > 15 mm Hg or an increase in peak pulmonary
and much higher attendant costs compared to those who pressure > 60 mm Hg is considered significant to warrant
were tested first with perfusion imaging and referred to invasive strategy. In asymptomatic patients with severe
catheterization dependent on the findings.46 In patients mitral regurgitation, left ventricular dysfunction can be
with high-risk features (ST changes, unstable angina, evaluated with exercise stress echocardiography.50 In
elevated cardiac enzymes), a primary invasive strategy asymptomatic patients with severe mitral regurgitation,
may be more appropriate. pulmonary artery systolic pressure > 60 mm Hg during
exercise may lower threshold for valve surgery.50
Doppler Hemodynamic
with Stress Echocardiography Hypertrophic Cardiomyopathy
The most important indication for surgical intervention Asymmetric hypertrophic cardiomyopathy (HCM) is a
in patients with hemodynamically significant aortic or common cause of dyspnea and is associated with sudden
mitral valve disease is the development of symptoms, as cardiac death. HCM may be present with or without left
emphasized in the recent guidelines.47 As valvular heart ventricular outflow tract (LVOT) gradient. ACC/AHA
disease progresses, the patient may be unaware of these guidelines for the diagnosis and treatment of HCM assign
chronic changes in effort tolerance but instead adapt to exercise echocardiography as Class IIa for the detection
the symptoms by reducing physical activity and lifestyle and quantification of exercise-induced dynamic LVOT
modifications. Therefore, exercise stress echocardiography obstruction in patients who do not have significant
provides objective evidence in assessing symptoms, gradients at rest or provocation.51
exercise capacity, and hemodynamic changes of the valve
during stress that may be absent at rest.
Latent Diastolic Dysfunction
Aortic Valve Disease Many patients with diastolic dysfunction have symptoms
of dyspnea on exertion. The symptoms are due to rise in
Exercise stress echocardiography is contraindicated in left ventricular filling pressures that is needed to maintain
severe symptomatic aortic stenosis. In patients with asymp adequate left ventricular filling and stroke volume during
tomatic aortic stenosis, stress echocardiography can provide
exercise. Stress echocardiography can assess latent dias
prognostic information by unmasking symptoms.48
tolic dysfunction not apparent at rest but only at stress.
An important role of stress echocardiography is in
When relaxation is normal, the mitral annulus velocity
patients with left ventricular dysfunction and “low-flow” or
(e') and mitral inflow velocity (E) increase proportionally
low-gradient aortic stenosis. Low-dose dobutamine is used
(~25%), whereas E/e' ratio remains unchanged or is
to increase inotropic contractility and increasing stroke
reduced (< 8). In patients with impaired myocardial
volume differentiating pseudosevere aortic stenosis due to
relaxation, the increase in e' with exercise is much less than
left ventricular dysfunction from true aortic stenosis. The
that of mitral E velocity, such that the E/e' ratio increases
American College of Cardiology (ACC)/American Heart
(> 15).52 Therefore, E/e' ratio increases with exercise
Association (ACH) recommends stress echocardiography
compared with rest in patients with latent diastolic
as Class IIa for low-flow, low-gradient aortic stenosis and
dysfunction.
Class IIb for symptom assessment.49
Dynamic Pulmonary Hypertension

Mitral Valve Disease
In normal subjects, exercise increases stroke volume while
In some patients with severe asymptomatic mitral stenosis pulmonary vascular resistance decreases. Normal values
and symptomatic moderate stenosis, exercise stress are defined by systolic pulmonary artery pressure < 43
echocardiography may precipitate or reproduce symptom. mm Hg during exercise.53 Exercise-induced pulmonary
Microbubble image-enhancing agents such as

perflutren lipid microspheres (Optison/Definity)
microbubbles have been used to assess myocardial
perfusion. The perfusion information in conjunction
with the corresponding regional wall motion improves
sensitivity to detect flow-limiting CAD.56
Major technological advances have led to the
development of real time, 3D stress echocardiography.
It allows for numerous tomographic interrogations,
eliminates off-axis acquisition and analysis, avoids
foreshortening of the apex, thus allowing multiple cross-
sectional views of the left ventricle, and more precise
comparison of similar segments to improve the detection
of localized ischemia.57 3D stress echocardiography
Fig. 61.14: An example of a nonischemic treadmill exercise echo overcomes the difficulties in acquiring multiple views
cardiogram demonstrating dynamic pulmonary hypertension. The immediately postexercise. Furthermore, multiple
resting study is on the left that shows normal right ventricular size tomography views can be evaluated for better assessment
and function. The postexercise images on the right show right
of wall motion abnormality.
ventricular dilatation and compression of the left ventricular cavity
with paradoxical septal motion. The abnormal septal motion is due
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CHAPTER 62
Squatting Stress
Echocardiography
Premindra AN Chandraratna, Dilbahar S Mohar, Peter Sidarous
Snapshot
Squatting Echocardiography
¾¾
INTRODUCTION moderate stenosis.3 Furthermore, WMA in the immediate

postexercise period may rapidly resolve in some patients
Regional mismatch between coronary oxygen supply resulting in false-negative studies.4 Patients with
and myocardial demand results in myocardial ischemia.
comorbid conditions such as intermittent claudication,
In such ischemic settings, wall motion abnormalities
chronic obstructive pulmonary disease (COPD), and
(WMAs) detectable by echocardiography manifest early
musculoskeletal or joint abnormalities may not be able to
as diastolic and subsequently systolic changes. Moreover,
achieve the levels of exercise sufficient for the detection
functional wall abnormalities are early changes in the
of myocardial ischemia. Occasionally, ventricular tachy
well-described ischemic cascade, which concludes with
cardia, ventricular fibrillation, myocardial infarction, or
later surrogates including electrocardiography (ECG)
death may occur during exercise testing.5
changes and subsequently overt chest pain.1 As such,
stress echocardiography (SE) has an established utility for Pharmacological stress testing is performed when exercise
the detection of significant coronary artery disease (CAD) testing is not feasible or it may be the preferred method of SE
with a notable accuracy of 80–90%, which is superior to that in some laboratories. Moreover, the 2011 American Society
of exercise electrocardiographic testing and comparable to of Echocardiography (ASE) Appropriate Use Guidelines for
that of nuclear stress imaging.2 Echocardiography recommend dobut amine as the first-
Stress echocardiography is performed either with line agent for pharmacologic SE.6 Minor side effects such as
exercise on a treadmill or bicycle or by infusion of a chest pain, tremor, palpitations, and dizziness are frequently
pharmacological agent such as dobutamine, adenosine, noted.5 Less commonly, more serious complications such as
dipyridamole, or transes ophageal atrial pacing. Each ventricular or supraventricular tachyarrhythmia, myocardial
of these techniques has advantages and disadvantages. infarction, or death may occur.7 Although both exercise and
Treadmill or bicycle exercise echocardiography permits pharmacological SE are reliable methods of detecting CAD,
assessment of both myocardial ischemia and functional the potential for serious side effects, cost, and the time factor
capacity. However, this technique may have limited are disadvantages of these techniques. Thus, there is the
echocardiographic utility in patients in whom peak need for a safe, rapid, and inexpensive echocardiographic
exercise is not attainable or in patients with single vessel or stress test.
Fig. 62.1: End-systolic frames during standing (left) and squat- Fig. 62.2: Echocardiogram of a patient with left ventricular (LV) func-
ting (right) in a normal subject. Note the triangular shape of the tion normal in the standing position. The LV apex had a triangular
apex during standing and squatting. (LV: Left ventricle). (Movie appearance at end-systole. An extensive wall motion abnormality
clip 62.1). developed during squatting (arrows). The distal posterior septum,
Source: Reproduced with permission from Ref. 10. apex, and distal posterolateral wall became akinetic, and the distal
half of the LV became dilated. The wall motion abnormalities and the
acute left ventricular remodeling (AVLRM) normalized on standing.
(Movie clip 62.2).
SQUATTING ECHOCARDIOGRAPHY Source: Reproduced with permission from Ref. 10.
Squatting echocardiography has emerged as a promising

was used for analysis of echocardiographic images. A
modality which augments afterload and preload with little
squatting stress echocardiogram was considered positive
or no change in cardiac contractility. Moreover, as afterload
if there was a new or worsening WMA during squatting. An
and preload are major determinants of myocardial oxygen
consumption, patients with significant CAD develop acute isolated fixed WMA was not considered a positive result.
WMAs during squatting, which reversibly resolve upon
arising. Lewis et al. studied the effects of squatting on
Squatting Echocardiography:
hemodynamic parameters and left ventricular (LV) dime Results/Observations
nsions in normal subjects.8 They observed that squatting
produced increased LV cavity dimensions and increase Squatting Induces LV WMA in Areas
in mean blood pressure. There was an increase in stroke Subtended by Stenotic Coronary Arteries
index and cardiac index.
The study populations consisted of 15 normal male
Chandraratna and colleagues have extended previous
subjects (Group 1) and 42 males subjects (Group 2) who
observations on squatting echocardiography and demon
had coronary angiography.9 Each patient underwent
strated that squatting produces LV WMA in patients with
squatting echocardiography testing as per protocol, and
CAD.
standard echocardiographic views were attained and
interpreted by an expert echocardiography reader blinded
Squatting Echocardiography Protocol to angiographic results.
The squatting protocol included recording of the standing Group 1 subjects had normal LV global and regional
(3 minutes of quiet standing) heart rate and blood pressure. function while standing. There were no WMA while
Standard parasternal long- and short-axis views and apical squatting (Fig. 62.1 and Movie clip 62.1). In Group 2, five
two-, three-, and four-chamber views were obtained in patients had a baseline WMA. New or worsening WMA
the standing position. The positions of the transducer occurred during squatting in 35 patients. Twelve patients
were marked and used for the squatting and dobutamine developed a WMA in the left anterior descending coronary
studies. The subjects were asked to squat for 2 minutes. The artery territory, five had WMA in the circumflex coronary
body weight was positioned over the heels and the torso artery territory, seven had WMA in the right coronary
maintained in a nearly vertical position. The subjects were artery territory, and seven had normal wall motion. Eleven
instructed to maintain a normal breathing pattern, and had WMA in multiple territories (Fig. 62.2 and Movie
the blood pressure, heart rate, and echocardiogram were clip 62.2). All WMA resolved on standing within 1 min.
recorded. The subjects were then asked to stand up and None of these patients developed chest pain, arrhythmias,
the above parameters were repeated. A 16-segment model or hypotension.
Chapter 62: Squatting Stress Echocardiography 1325
(≥ 90% stenosis). Six subjects in Group 2 had LMCAS

and none had severe 3-vessel disease (p < 0.05 vs Group
1 for LMCAS and/or 3-vessel disease). In Group 3, eight
had LMCAS and none had severe 3-vessel disease
(p < 0.0001 vs Group 1).
These observations suggest that patients who develop
ALVRM during squatting have severe CAD and should be
considered for urgent revascularization therapy.
Comparison of Squatting SE and

A B Dobutamine SE for the Diagnosis of CAD11
Figs 62.3A and B: (A) Apical four-chamber, end-systolic frame in
a patient with severe 3-vessel disease obtained in the standing Thirty-nine patients scheduled to have coronary angio
position. Note that the apex is triangular; (B) Apical four-chamber, graphy for the evaluation of chest pain were included
end-systolic frame during squatting. There is marked dilatation of in the study. Each patient had squatting SE followed by
the distal half of the left ventricle (LV). The arrows indicate the dobutamine SE. For squatting SE, the echocardiogram in
extent of the wall motion abnormality. (Movie clip 62.3).
Source: Reproduced with permission from Ref. 10. standard views was recorded in the standing position. The
procedure was repeated during squatting for 2 minutes.
Dobutamine echocardiography was performed using
The sensitivity, specificity, and accuracy of squatting standard protocol.
Hemodynamic response to both squatting and dobut
echocardiography for diagnosis of the CAD were 92%,
amine are compared in Table 62.1.
80%, and 91%, respectively.
During squatting, new or worsening WMA developed in
In summary, the study showed that squatting induces
20 patients. Six patients developed WMA in the left anterior
LV WMA in areas subtended by stenotic coronary arteries.
descending (LAD) territory, three in the circumflex territory,
three in the RCA territory, and eight in multiple coronary
Patients Who Exhibit Acute LV Remodeling territories (Movies clips 62.4 to 62.6). The sensitivity,
(ALVRM) on Squatting Have Severe CAD specificity, and accuracy of squatting echocardiography for
the diagnosis of CAD were 95%, 94%, and 94%, respectively.
It was previously demonstrated that squatting induces LV
For dobutamine SE, the sensitivity, specificity, and
WMA in areas subtended by stenotic coronary arteries.9,10
accuracy for the diagnosis of CAD were 85%, 94%, and 90%,
In addition, it was observed that some subjects developed
respectively. There was no significant difference between
acute changes in LV shape (ALVRM) during squatting. squatting and dobutamine SE for the diagnosis of CAD
Ninety-six subjects were divided into three groups. (p = .702).
Group 1 consisted of 12 subjects who developed squatting- These data indicate that squatting and dobutamine
induced ALVRM with apical and distal posterior septal echocardiography are equally useful in the diagnosis of
akinesis, dilation of the apex, and marked LV shape CAD.
change at end-systole (Figs 62.2 and 62.3; Movie clips 62.2
and 62.3). Group 2 consisted of 20 subjects with distal
posterior septal and apical akinesis without ALVRM,
Mechanism of Squatting-Induced WMA
during squatting. Group 3 consisted of 64 subjects who Although the mechanism by which squatting induces WMA is
developed WMA in areas other than the apex (n = 49), or uncertain, a potential mechanism is unmasking of subclinical
normal wall motion (n = 15) during squatting. segmental dysfunction in normokinetic segments subtended
Coronary angiography in Group 1 revealed that six by stenotic coronary arteries. Yuda et al.12 have demonstrated
subjects had left main coronary artery stenosis (LMCAS subclinical LV dysfunction in patients with CAD and normal
≥ 50%), two had severe 3-vessel disease (≥ 90% stenosis), ejection fraction. A second more likely mechanism is the
and one had 100% left anterior descending coronary artery induction of myocardial ischemia as squatting increases
occlusion. Severe CAD was defined for purpose of this myocardial oxygen consumption by increasing stroke
study as the presence of LMCAS, or severe 3-vessel disease volume via augmentation of preload and afterload.
Table 62.1: Heart Rate and Blood Pressure Response to Squatting versus Dobutamine11
Total Cohorts Standing Squatting p-value Dobutamine p-value
Standing versus Squatting versus
Squatting Dobutamine
Heart rate (beats/min) 64 ± 10 73 ± 10 <0.0001 123 ± 18 <0.0001
Systolic blood pressure (mm Hg) 121 ± 11 136 ± 11 <0.0001 159 ± 23 <0.0001
Diastolic blood pressure (mm Hg) 79 ± 6 86 ± 7 <0.001 88 ± 10 <0.001
Source: Reprinted with permission from Chandraratna PA, Kuznetsov VA, Mohar DS, et al. Comparison of squatting stress echocardi-
ography and dobutamine stress echocardiography for the diagnosis of coronary artery disease. Echocardiography. 2012; 29(6):695–9.
Advantages may be used as an adjunct to or independent of current

noninvasive stress techniques to risk stratify patients
Furthermore, there are numerous advantages to squatting
with CAD. Moreover, squatting SE has been identified
as a stress test. The absence of marked changes in heart
as an inexpensive, simple, and rapid noninvasive CAD
rate during squatting makes comparison with the baseline
risk assessment modality with fewer complications than
echocardiogram and interpretation of stress-induced
current stress imaging techniques.
WMA easier than when tachycardia is present. Ventricular
dilatation that occurs with squatting facilitates analysis
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Squatting SE possesses similar limitations as com stress echocardiography. Experience in 1118 patients. Cir
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orthopedics. However, unless patients have morbid obesity 6. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/
AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011
or advanced orthopedic restrictions, squat imaging is still
Appropriate Use Criteria for Echocardiography. A Report
attainable with limited effort as squatting is essentially
of the American College of Cardiology Foundation
a static position. The same cannot be said for treadmill Appropriate Use Criteria Task Force, American Society of
testing in which certain heart rate and blood pressure end Echocardiography, American Heart Association, American
point parameters need to be achieved for proper treadmill Society of Nuclear Cardiology, Heart Failure Society of
assessment. America, Heart Rhythm Society, Society for Cardiovascular
Angiography and Interventions, Society of Critical Care
CONCLUSION Medicine, Society of Cardiovascular Computed Tomo
graphy, Society for Cardiovascular Magnetic Resonance
In patients with significant CAD, squatting echocar American College of Chest Physicians. J Am Soc Echo
diography has emerged as a unique stress modality, which cardiogr. 2011;24(3):229–67.
Chapter 62: Squatting Stress Echocardiography 1327
7. Picano E, Mathias W Jr, Pingitore A, et al. Safety and 10. Chandraratna PA, Mohar DS, Sidarous PF, et al. Implications
tolerability of dobutamine-atropine stress echocar diog of acute left ventricular remodeling during squatting stress
raphy: a prospective, multicentre study. Echo Dobutamine echocardiography. Echocardiography. 2012;29(6):700–5.
International Cooperative Study Group. Lancet. 1994; 11. Chandraratna PA, Kuznetsov VA, Mohar DS, et al.
344(8931):1190–2. Comparison of squatting stress echocardiography and
8. Lewis BS, Lewis N, Gotsman MS. Effect of standing and dobutamine stress echocardiography for the diagnosis of
squatting on echocardiographic left ventricular function. coronary artery disease. Echocardiography. 2012;29(6):
Eur J Cardiol. 1980;11(6):405–12. 695–9.
9. Chandraratna PA, Maraj R, Tabel G, et al. Left ventricular 12. Yuda S, Fang ZY, Marwick TH. Association of severe coronary
wall motion abnormalities induced by squatting: a new stenosis with subclinical left ventricular dysfunction
echocardiographic stress test for the diagnosis of coronary in the absence of infarction. J Am Soc Echocardiogr.
artery disease. J Am Coll Cardiol. 2005;46(5):931–3. 2003;16(11):1163–70.
CHAPTER 63
Three-Dimensional Stress
Echocardiography
Rajesh Ramineni, Masood Ahmad
Snapshot
Two-Dimensional Stress Echocardiography
¾¾ Differences Between 2DSE and 3DSE in Wall
¾¾
Three-Dimensional Transducers
¾¾ Visualization
Advantages of Three-Dimensional in Stress Imaging
¾¾ Parametric Imaging in Three-Dimensional Stress
¾¾
Three-Dimensional Image Acquisition
¾¾ Echocardiography
Three-Dimensional Stress Protocol
¾¾ Role of Contraction Front Mapping in RT3DSE
¾¾
Postacquisition Analysis
¾¾ Contrast in Three-Dimensional Stress Testing
¾¾
Review of Studies Comparing Three-Dimensional Stress
¾¾ Future Directions
¾¾
Echocardiography to Current Standards
INTRODUCTION two-dimensional (2D) images from multiple windows can

be challenging. The likelihood of capture at peak stress is
Stress testing has been in use for a very long time in the
decreased in some patients, thereby reducing sensitivity
diagnosis of coronary artery disease (CAD), in testing
in the detection of ischemia.8 This creates a role for the
functional capacity, and in assessing prognosis. Stress-
application of three-dimensional (3D) imaging during
induced changes in electrocardiogram were initially
stress.9 3D images can be acquired rapidly from a single
used to evaluate ischemia until the introduction of
acquisition and multiple views of the myocardial segments
imaging techniques, which offered higher sensitivity and
can be derived from the data set. Thus, three-dimensional
specificity. We reported the use of isometric exercise stress echocardiography (3DSE) can be performed with a
during M-mode echocardiography in the detection of left shorter scan time, and the technique does not require a
anterior descending (LAD) artery disease as far back as high level of operator skill. In this chapter, we will discuss
1976.1 The value of treadmill exercise echocardiography in the evolving technology of 3DSE, describe its current
the diagnosis of CAD was subsequently demonstrated.2 A applications, and review major clinical studies to date.10
plethora of reports on the use of stress echocardiography
followed.2–6 Ever since, two-dimensional stress echocardi
TWO-DIMENSIONAL STRESS
ography (2DSE) has become an established technique
in assessing patients with CAD. Since the stress-induced
ECHOCARDIOGRAPHY
transient left ventricular (LV) wall motion abnormality As an established technique in the assessment of
needs to be obtained rapidly,7 the technique of acquiring patients with CAD, 2DSE is commonly used in many
Chapter 63: Three-Dimensional Stress Echocardiography 1329
these transducers was extremely time consuming, thereby

precl
uding their use during stress. The introduction
of matrix array transducers revolutionized the image
acquisition in 3D. For the first time, the images could be
obtained in a true 3D format.
The first version of these transducers was designed and
described by von Ramm and Smith.18 These transducers
had 256 nonsimultaneous firing elements with an achieved
frequency of 2.5/3/5 Hz. A pyramidal volume data set was
obtained in a heartbeat with a sector angle of 60° × 60°.
We had the opportunity to use this transducer during
stress and described, for the first time, the application of
3D during stress.8 All of the data could be obtained in a
single heartbeat and the images were cropped to display
A B
the orthogonal and short-axis views of the LV. Due to the
Figs 63.1A and B: 3D transducers. (A) Current three-dimensional small number of elements in the matrix, the resolution of
(3D) transducer with a smaller footprint; (B) First-generation 3D
transducer with a larger footprint.
the images generated with this transducer was somewhat
low and the sector angle of 60° × 60° did not accommodate
dilated LVs.
echocardiography laboratories. It has an estimated The latest version of matrix array transducers uses
sensitivity of 71%, a specificity of 85.7%, and a negative 3,000 to 4,000 elements. This change significantly impr
predictive value of 95% for any vessel disease.11 The oved the image resolution, penetrability, and side lobe
sensitivity of stress echo is higher for multivessel CAD12 suppression. Moreover, the new transducers are smaller,
and lower for single vessel disease.13 However, 2DSE has making it possible to fit the transducer in the rib spaces and
a number of limitations. In addition to the difficulties acquire images at wider sector angles. These transducers
in obtaining images in a very short time frame, the can be used comprehensively for combined 2D and 3D
technique has some intrinsic limitations. 2D planes are imaging.
not always aligned precisely for accurate comparison of
segmental wall motion from baseline to stress. Moreover, ADVANTAGES OF THREE-
apical foreshortening is a major concern, increasing false DIMENSIONAL IN STRESS IMAGING
negatives. With the advent of 3D echocardiography (3DE),
many of the limitations of two-dimensional echocardi With advances in transducer technology and the arrival
of matrix array transducers, real time 3D acquisitions
ography (2DE) are put to rest.14–17 Simultaneous assessment
(RT3D) became possible and the role of 3DE in stress
of the LV in multiple planes from one acquisition removes
testing surfaced (Table 63.1).9 Multiple studies have
temporal assumptions and improves diagnostic accuracy.
successfully documented the feasibility of performing 3DE
A shorter scan time means greater possibility of image
during dobutamine and exercise stress testing. Although
acquisition at the target heart rate. Moreover, cropping there are variations from vendor to vendor, generally, full-
of the full volume images provides unlimited number of volume acquisitions of the LV are used for stress testing
conventional and unconventional views of the LV that that are obtained over a span of three to four cardiac
cannot be obtained by the standard 2D technique. cycles. Data can be obtained with a single cardiac cycle
as well; however, the volume rate may be limited at high
THREE-DIMENSIONAL heart rates. With one of the major limitations of 2DE being
TRANSDUCERS the inability to obtain images in multiple planes in a very
short time period at peak stress, acquisition of full-volume
Initial attempts to obtain 3D images were based on image with a maximum of four cardiac cycles places RT3D
reconstruction of multiple 2D images acquired by at an advantage. In our laboratory, we demonstrated that
manually tilting the transducers at various angles around a RT3D data during dobutamine stress echocardiography
fixed plane (Figs 63.1A and B). However, the acquisition by (DSE) can be obtained in less than half the time needed
Table 63.1: Advantages and Limitations of RT3DSE

Advantages:
Shorter acquisition time
User-friendly and less operator dependent
Good interobserver agreement. Reproducible
Precise alignment/anatomically correct tomographic section. More accurate comparison of matched rest and stress images
Full-volume acquisition of entire true LV. Multiple sections of the LV and apical region essentially eliminate problems related to
off-axis image acquisition or LV foreshortening
Quantitative. Assessment of LV volume and EF comparable to CMR
Limitations:
Lower spatial and temporal (frame/volume rate) resolution especially at peak stress
Influenced by respiration, patient motion, and significant variation in heart rate causing image artifacts
Suboptimal anterior and lateral wall visualization related to larger transducer footprint
Longer offline data analysis time
(CMR: Cardiac magnetic resonance; EF: Ejection fraction; LV: Left ventricle).
for 2DE with greater sensitivity validated by cardiac (multiplane) or in short-axis (multislice) views from a
catheterization.8 These data are replicated in multiple single data set was truly impressive and obviated the need
other studies.19–27 The 3D data set obtained during the brief for multiple acquisitions from multiple windows. The new
acquisition period can be cropped and processed to assess matrix array transducers have 3,000 to 4,000 elements in
wall motion at rest and peak stress. the matrix, resulting in higher imaging quality.
Misaligned 2D images can lead to erroneous estimation Using Philips iE33 (Philips Medical Systems, Bothell,
of LV wall motion. This is overcome in RT3D by properly WA), RT3D images can be obtained in three different
aligning the LV along its long axis so that the true apex of modes. They are live 3D, zoom, and wide angle with their
the LV is displayed. Even though, at this time, the image respective angles being 50° × 30°, 30° × 30°, and 90° × 90°.
resolution in 2DE is superior to 3DE, the current advances Live 3D images can be obtained within a single heartbeat.
in transducers make it possible to acquire full volume This the most common mode of RT3D used for structural
data set at a high volume rate (40 volumes/s) resulting in assessment of the heart. When a more focused zone needs
diagnostic images in the vast majority of patients. further detailed exam such as valvular abnormalities,
3D imaging is quantitative and can assess LV volumes the zoom mode is used. This mode has greater detail at
as well as LV ejection fraction with accuracy comparable the expense of providing information about a smaller
to cardiac magnetic resonance imaging, which is an area. A wide angle image is obtained by merging four
additional benefit in stress imaging to identify transient small pyramids. These separate pyramids are obtained in
ischemic dilatation.28,29 consecutive beats by ECG gating. Due to a wider sector
angle, more volume is captured in this mode making it the
THREE-DIMENSIONAL mode of choice in stress imaging. However, intrinsic to the
IMAGE ACQUISITION use of information from multiple beats, arrhythmias, stitch
artifacts, and motion artifacts pose a limitation to this
The introduction of matrix array transducers and parallel mode, some of which can be overcome by holding breath
processing allowed real time acquisition of 3D images as during acquisition. In patients with arrhythmias, the
opposed to reconstruction from spatially oriented and artifacts can be further reduced by decreasing the number
sequentially obtained 2D images. Initially, the matrix array of beats in the full-volume acquisition. The 3D data set
had 256 elements that generated a 60° × 60° pyramidal can be cropped to display any number of conventional
volume. Although the stress images had limited resolution, and nonconventional views of the LV for a more thorough
the ability to obtain multiple views in either orthogonal assessment of wall motion. The images can be aligned
along the true long axis of the LV for a superior assessment that is, if a wall motion abnormality is suspected in 2D
of the true LV apex. With recent developments in 3D images but is not entirely clear, 3D images are reviewed
software, high-quality, single-beat 3D acquisitions can be for confirmation or exclusion of abnormal wall motion.
obtained at good volume rates of 30–40 frames per second. This integrated approach is very useful in evaluating
questionable wall motion changes. In some patients,
when the LV is foreshortened in 2D images, 3D images are
THREE-DIMENSIONAL
aligned along the true long-axis of the LV and the apex is
STRESS PROTOCOL carefully examined for wall motion abnormalities (WMA)
(Movie clips 63.5 and 63.6). Quantitative data regarding
The early versions of the 3D equipment lacked combined
LV volumes and LV ejection fraction are available from
2D and 3D imaging. Therefore, in order to obtain 2D and
the 3D images. Improvements in software used in postac
3D images at baseline and at peak stress, the transducers
quisition analysis are making it possible to perform a side-
had to be switched to obtain both sets of images. With
by-side synchronized analysis of anatomically aligned 3D
the newer systems, the transducers have both 2D and DSE data sets resulting in better interobserver agreement.26
3D imaging capabilities. Both sets of images can also be In selected patients, we have used contraction front
obtained by simply switching the acquisition modes. mapping (parametric imaging) in assessing dyssyn
Alternatively, all 2D images can be derived from the chronous LV wall motion induced by ischemia.30 This
3D data set. The protocol for imaging 3D data sets with technique is still evolving and needs further study.
routinely performed 2D stress imaging remains variable
from laboratory to laboratory. Our approach is to use an REVIEW OF STUDIES COMPARING
integrated 2D/3D stress protocol that includes 3D images
in the standard 2D stress imaging protocol. Essentially,
THREE-DIMENSIONAL STRESS
3D full-volume acquisitions are obtained at baseline after ECHOCARDIOGRAPHY TO CURRENT
capturing standard 2D images from multiple windows. At STANDARDS
this time, we obtain 3D acquisitions from the apical and
parasternal windows. These steps are repeated at peak Dobutamine Stress Test
stress. Given the concern about obtaining good images when
patients are hyperventilating postexercise, initial studies
POSTACQUISITION ANALYSIS comparing the use of 3DE over 2DE for stress testing
started with DSE (Table 63.2). Using the first-generation
A single, full-volume acquisition of RT3D can be matrix array transducers, we tested for feasibility of RT3DE
analyzed in various formats. A simultaneous display of during DSE and compared its performance to 2DE in
major orthogonal views can be created. This is called 253 patients.8 A subanalysis of correlation with coronary
the multiplane view. We are able to obtain parasternal angiograms was done in 90 patients. It was noted that
long- or short-axis or apical four-, two- or three-chamber LV wall motion scores were similar with both techniques
views by this technique. Multiple short-axis 2D images at rest as well as peak stress, but acquisition of RT3DE
(nine equidistant images in general) can also be obtained was quicker (27.4 ± 10.7 s vs 62.4 ± 20.1 s by 2D with a
from apex to base. This is called the multislice view. P < 0.0001). Real time 3D echocardiography was also noted
(Movie clips 63.1 to 63.4). Studies have shown that to have higher interobserver agreement for detection of
specificity of detection of CAD and accuracy of identifying ischemia at peak stress and higher sensitivity in detection
right coronary lesions were significantly greater with the of CAD in patients who underwent coronary angiograms.
use of multislice view.26 This advantage was attributed to The next major study in this field was unveiled in
better visualization of basal wall motion abnormalities 2005 when Matsumura et al., compared 2DE to RT3DE
and the acquisition of correct short-axis views. This view during dobutamine stress in 56 patients who previously
is also associated with greater reduction in interobserver underwent SPECT due to suspicion of ischemia.24 Similar
variances.26 Analysis of the LV wall motion can be done by to our findings they also noted that RT3DE is significantly
the 16- or 17-segment model as recommended by the ASE. quicker than 2DE with a success rate comparable to 2DE
The images are analyzed in a complementary fashion; (92% at rest and 89% at peak stress in RT3DE vs 94% and
Table 63.2: Diagnostic Sensitivity and Specificity of Three-Dimensional (3D) Versus Two-Dimensional (2D) in Detecting Coronary
Artery Disease (CAD)
Study (Author, Year, Ref.) No. Stress Test (Type) Validation 2D Sen. Sp. 3D Sen. Sp.
Ahmad et al. (2008) 8
58 DSE Coronary angiography 79 — 88 —
Matsumura et al. (2005) 24
56 DSE Thalium201-SPECT 86 83 6 80
Takeuchi et al. (2006)31 78 DSE None — — 58 75
Aggeli et al. (2007)22
56 DSE Coronary angiography 73 78 93 89
Yoshitani et al. (2009)26 71 DSE-3D multiplane Coronary angiography — — 72 72
DSE-3D multislice Coronary angiography — — 77 95
Jenkins et al. (2009) 32
90 Treadmill exercise-2D Coronary angiography 83 65 — —
Treadmill exercise-3D Coronary angiography — — 40 84
Treadmill exercise-3D+ Coronary angiography — — 55 78
CFM
Badano et al. (2010)21 107 Dipyridamole None 78 91 80 87
Abdelmoneim et al. (2010) 33
30 Adenosine 2D Tc 99m Sestamibi 92 75 — —
SPECT
Adenosine Live 3D Tc 99m Sestamibi — — 91 69
SPECT
Adenosine full volume 3D Tc 99m Sestamibi — — 90 79
SPECT
(CFM: Contraction front mapping; DSE: Dobutamine stress echocardiography; No.: Number of subjects; Sen.: Sensitivity;
Sp.: Specificity; SPECT: Single-photon electron-computed tomography; Tc: Technetium).
90%, respectively, in 2DE). The sensitivity, specificity, and inferior, mid-inferior, and basal interventricular septum)
accuracy of these two modalities were very similar without were assigned to the right coronary artery territory, and
any statistical difference when the results of SPECT were four segments (basal lateral, basal posterior, mid-lateral,
used as the reference standard. and mid-posterior) were assigned to the left circumflex
In the following year (2006), Takeuchi et al. used coronary artery territory. It was noted that a large
contrast to enhance the endocardial borders and number of segments were uninterpretable with 3D DSE
compared RT3DE with 2DE during DSE for the assessment in comparison to 2D DSE, the majority of which were in
of WMA in 78 patients with known or suggested history of anterior and lateral walls. Despite a significant correlation
CAD.31 This study was limited by the fact that it used an of WMSI between these two modalities, the concordance
earlier generation of bulky transducers with a narrow rates were only moderate. The bulk of the transducer
angle. Levovist (Schering AG, Berlin, Germany) or Optison limiting visualization of the anterolateral segments
(Mallinckrodt Inc., St. Louis, MO) /Definity® (Bristol-Myers and the low frame rate causing erroneous diagnosis of
Squibb, N. Billerica, MA) were used as contrast material in dyssynchrony were the likely limitations of this study.
Japan and the United States, respectively, for the included Using 2D DSE results as the gold standard in this study, the
patients. This study used the concept of worsening stress- sensitivity and specificity for detecting WMA by 3D DSE
induced increase in segmental wall motion score index were 58% and 75%, respectively. Sensitivity and specificity
(WMSI) as being suggestive of ischemia. Moreover, they values were 67% and 94% for the right coronary artery,
assessed for regional wall motion in each coronary artery 53% and 81% for the LAD, and 88% and 100% for the left
territory by predefining segments into major coronary circumflex coronary artery territory, respectively.
arterial territories. Nine segments (basal anteroseptal, In 2007, Aggeli et al. designed a study in close comp
basal anterior, mid-interventricular septum, mid- arison to ours but with newer generation 3D transducers.
anteroseptal, mid-anterior, and four apical) were assigned Real time 3D echocardiography with 2DE during DSE
to the LAD coronary artery territory, three segments (basal were compared in 56 patients and validated with results
from coronary angiography.22 They also showed that with the multislice mode. On a patient basis, sensitivity
acquisition time for RT3DE was significantly less (< 50%) was not different, but specificity was significantly higher in
when compared to 2DE. Both the modalities showed the multislice mode (95%) compared with the multiplane
excellent agreement in WMSI at rest, but at peak stress mode (77%, P < 0.05). Diagnostic accuracy for detecting
RT3DE showed significantly higher values. A regional wall right CAD was also significantly higher in the multislice
motion score of the apical segments (apWMS) was done mode (93% vs 80%, P < 0.05).
to delineate differences between the modalities, since
2DE is known to have limitations in accurate evaluation Treadmill Exercise Stress Test
of the apex. RT3DE showed significantly higher wall In 2009, Jenkins et al studied the feasibility of treadmill
motion scores at peak stress and higher sensitivity in the exercise stress testing and compared 2DE against 3DE.32
LAD territory. The study concluded that the diagnostic Initially starting the study with 110 patients, the final
value of RT3DE is at least equivalent to 2DE during DSE analysis was done on 90 patients with 20 excluded
and, in addition, serves the advantage of markedly shorter (12 for nondiagnostic stress and 8 for poor quality 3DE).
acquisition times. Better assessment of apical segments is Unlike the studies done using dobutamine, there was no
an added advantage. significant benefit in the acquisition time for 3DE in this
Overall, the studies using dobutamine as the stress study. This study also involved an arm which uses 3DE
agent showed that 3DSE significantly reduces the time with contraction front mapping (CFM), which is discussed
needed for imaging and the WMSI was higher. There was in detail later in the chapter. The sensitivity of wall motion
also better interobserver agreement with 3DSE. assessment with 3DE (40%) was noted to be lower than
2DE (83%, P < 0.01) at comparable levels of specificity
Dobutamine Stress Test with (65% and 78%). However, the combined use of 3DE along
Multiplane versus Multislice Imaging with CFM added to the sensitivity and overall accuracy.
This study was noted to be different on many fronts. The
Yoshitani et al., in a study which included 71 patients average time taken to acquire 3D images was longer
with known or suspected CAD, compared the use of than the previous studies with a possible impact on the
multiplane versus multislice modes in the assessment of outcomes. 3D echocardiography images were obtained on
wall motion from data obtained using RT3DE.26 Data sets an average of 58 (± 25) seconds after the patient was off
were acquired using a wide-angle (60° × 60°) acquisition the treadmill, which brings into question the ability to get
mode, in which four wedge-shaped subvolumes (60° × images at target heart rate. Moreover, contrast agents were
15° each) were obtained from four consecutive cardiac not used to enhance the endocardium. These limitations,
cycles during held breath. Multiplane mode provided in addition to this being the only study available using
simultaneous visualization of parasternal long- and short- exercise as stress technique, make it difficult to draw
axis views or apical four-, two-, and three-chamber views conclusions.
whereas nine equidistant 2D short-axis images from LV
base to apex were extracted and simultaneously displayed
comprising the multislice mode. Wall motion score was
Adenosine Stress Test
obtained by visual assessment of these views, which Abdelmoneim et al., in a study involving 30 patients,
were then compared against the findings obtained from compared 3DE (full volume and live 3D) to 2DE and the
a coronary angiogram performed the next day in which a standard SPECT imaging to evaluate for CAD.33 Sensiti
luminal narrowing of greater than 50% was considered as vities and specificities were corelated between these three
significant stenosis. It was noted that, unlike the biplane modalities. Adenosine was administered as the stress
mode where there was no change in the number of agent and contrast (Definity®) was used for all echocardio
uninterpretable segments from rest to peak stress, these graphic studies. It was noted that live 3D identified
numbers significantly dropped in the multislice mode. The abnormalities in areas with reversible defects in SPECT
majority of these uninterpretable segments were noted in in 88% versus 63% with full-volume 3D images. This
the anterior and lateral walls in both modes. In contrast was the first attempt comparing 3D and 2D contrast
to absence of differences at rest, WMSI was significantly echocardiography in the evaluation of perfusion defects
lower at peak stress in the multiplane mode compared with SPECT as the diagnostic standard. However, a
et al. the use of 3DSE was also associated with greater

sensitivity and specificity in assessing the lateral LV wall,
which plays a role in its detection of isolated left circumflex
lesions.31 However, due to the greater resolution of 2DE,
good views of the basal structures were obtained with 2DE
during stress testing.
PARAMETRIC IMAGING IN THREE-

A B
DIMENSIONAL STRESS
Figs 63.2A and B: Segmental time–volume curves of a patient
who underwent three-dimensional stress echocardiography ECHOCARDIOGRAPHY
(3DSE) and contraction front mapping (CFM). (A) At baseline,
synchronous time–volume curves are seen suggesting uniform
As an alternative to wall motion assessment, parametric
myocardial segmental contraction; (B) At peak stress, time–vol- imaging can be applied in analysis of 3D stress data
ume curves are dyssynchronous with nonuniform segmental (Figs 63.2A and B). Segmental time–volume curves and
contraction suggesting reversible ischemia. contraction front maps can be generated from the
acquired 3D data sets. Temporal heterogeneity of myo
limitation of this study was a lack of comparison with cardial contraction induced by stress can be measured
coronary angiography in patients with reversible perfusion by the dyssynchrony index similar to the evaluation of
defects. mechanical dyssynchrony in patients with LV dysfunction
for cardiac resynchronization therapy. Segmental time
to minimal systolic volume can be displayed at baseline
Dipyridamole Stress Test and at peak stress. Stress-induced ischemia will result in
Badano et al., with the newer high-volume rate scanners delayed contraction in ischemic segments thus resulting
with higher temporal resolution and the possibility of in dyssynchrony.
displaying cropped images side by side, compared RT3DE
with 2DE during dipyridamole-induced stress (DipSE) ROLE OF CONTRACTION FRONT
in 84 patients.21 The results of this study were consistent MAPPING IN RT3DSE
with the other dobutamine-based studies reviewed earlier,
with RT3DE displaying significantly lower acquisition Contraction front mapping (TomTec Imaging Systems,
times, higher WMSI at peak stress (more so in the apical Munich, Germany) analyzes temporal and spatial activ
regions), and better sensitivity in the LAD territory as ation of LV contraction and displays a bull’s-eye plot of the
noted in patients who underwent coronary angiograms. contraction wave front of the myocardial segments that
Interestingly, the postacquisition analysis time, which reach peak contraction every 25 milliseconds (Movie clips
63.7 and 63.8). Color-coded maps show the contraction
in general was assumed to be longer in RT3DE, was
wave in blue and the noncontracting segments in shades of
also significantly shorter in RT3DE than in 2DE. However,
red. Thus, increasing degrees of dyssynchrony induced by
the temporal resolution was significantly better in
ischemia are displayed in shades of red. Contraction front
2DE (75 ± 5 frames/s vs 41 ± 5 volumes/s, respectively;
mapping also has the potential to provide quantitative
P < 0.0001). analysis of LV contraction through assessment of time
to minimal volume, aiding in the diagnosis of segm
DIFFERENCES BETWEEN 2DSE AND ental ischemia. Contraction front mapping might be
3DSE IN WALL VISUALIZATION applicable as a good qualitative and quantitative tool
in the assessment of ischemia as noted in its initial
One of the major limitations of 2DSE is its inability to open application described from our laboratory.30 Jenkins
up the entire LV apex (LV foreshortening), thus generating et al., in their study done on treadmill exercise stress test,
a possibility of error in assessment of wall motion. Aggeli compared 2DE against 3DE as well as 3DE with CFM.32
et al. showed that 3DSE predicted a higher wall motion CFM was done offline from the 3DE full-volume data.
score predominantly in the LV apex, which corresponded After defining normal cutoff ranges for contraction
with ischemia in LAD territory,22 as seen in the reference delay based on receiver-operating characteristics, they
standard (coronary angiography). As shown by Takeuchi noted that the concordance between angiography and
qualitative CFM (63%) was similar to quantitative CFM allow assessment of the LV with 3D during stress without
(70%). Moreover, it was noted that the combined use the need for concurrent conventional 2D imaging.
of 3DE along with CFM added to the overall sensitivity Automated fused stress image technique is another
(improved from 40% to 55%), specificity (improved from modality which might prove to be beneficial in the future.
65% to 84%), and accuracy of 3DSE. The limitations of this This method incorporates RT3D echocardiography with
study were discussed earlier. myocardial perfusion SPECT imaging to create a fused
image which had excellent specificity and sensitivity in
CONTRAST IN THREE-DIMENSIONAL detecting myocardial ischemia with low interobserver
variability in a study by Walimbe et al.35 They studied
STRESS TESTING
20 patients with 36 angiographically evaluated coronary
Given the limited resolution of 3D when compared to 2D, arteries. This was a pilot study with a small number of
use of contrast agents to improve the visualization of the subjects. More studies with greater power are needed to
endocardial border makes theoretical sense. Pulerwitz, evaluate its feasibility and confirm these findings.
in an initial study of 14 patients, noted that ultrasound
contrast significantly increased the proportion of segments CONCLUSION
adequately visualized during rest and peak dobutamine
3DSE is an exciting technology that is rapidly evolving and
infusion (91%–98%, P = 0.001, and 87%–99%, P = 0.001,
currently applied as an adjunctive technique to 2DSE.
respectively).16 There was almost complete concordance
The availability of 3D images is tremendously useful
between observers (96.9% at rest and 98.2% at peak
in evaluating questionable areas of abnormality seen
stress with almost no interobserver variability), whereas
in 2DSE and in more accurately assessing the changes
noncontrast studies had much lower agreement (84.4%
induced by ischemia.36 In the near future, 3DSE may be
at rest and 79.9% at peak stress with kappa values less
applied independent of the traditional 2DSE resulting in a
than 0.4).
comprehensive diagnostic stress test that is more efficient,
Nemes et al. in 2007, studied the use of contrast in
quantitative, and reproducible in the diagnosis of CAD.
36 patients undergoing routine stress test for stable chest
pain.34 The images obtained with and without contrast
were compared for image quality index, and wall motion REFERENCES
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CHAPTER 64
Coronary Arteries—Morphology and
Coronary Flow Reserve
Karina Wierzbowska-Drabik, Jarosław D Kasprzak
Snapshot

The Assessment of Coronary Morphology and Flow in 
Distal Coronary Flow and Coronary Flow Reserve
Transthoracic and Transesophageal Studies 
Congenital AbnormaliƟes of the Coronary Arteries

VisualizaƟon of Coronary Arteries
INTRODUCTION anomalies of their origin by two-dimensional and

color Doppler studies
Coronary arteries are visible during standard echocar- • Assessment of flow in the main coronary arteries
diographic examination. However, small size and vigorous (left main coronary artery (LMCA), circumflex (Cx),
motion of epicardial coronary segments during respiratory left anterior descending (LAD), and right coronary
and cardiac cycle pose a significant challenge for all nonin- artery (RCA) with possible detection of stenotic flow
vasive imaging methods. Therefore, direct evaluation of acceleration and luminal narrowing or aneurysms, for
coronary arteries has not become a part of routine trans- example, in Kawasaki disease in pediatric population
thoracic echocardiographic (TTE) examination despite • Evaluation of accessible parts of mid and distal
significant technical improvements leading to improved coronary arteries and the assessment of coronary
quality of imaging. These limitations are less evident in by-pass grafts (usually internal mammary artery)
transesophageal echocardiogram (TEE). Recent progress with assessment of coronary flow velocity reserve
(CFVR) based on Doppler recording at rest and under
in Doppler sensitivity encouraged routine use of distal
vasodilator stress (with highest feasibility in LAD but
coronary flow velocity and vasodilator-induced velocity
examined also in RCA)
reserve measurements.
• Diagnosis of coronary fistulas draining to heart
chambers and pulmonary artery, which may be
THE ASSESSMENT OF CORONARY often clearly visualized by TTE color Doppler study
MORPHOLOGY AND FLOW IN (Fig. 64.1).
TRANSTHORACIC AND
VISUALIZATION OF
TRANSESOPHAGEAL STUDIES
CORONARY ARTERIES
Current TTE and TEE allows addressing the following
clinical aspects of coronary anatomy and physiology:
Transthoracic Echocardiography
• Identification of ostia and proximal segments of left Visualization of proximal segments of coronary arteries
and right coronary arteries and diagnosis of congenital is possible in the majority of TTE examinations. Good-
Fig. 64.1: A typical image of proximal coronary arteries in para- Fig. 64.2: Additional image of the proximal right coronary artery
sternal short-axis view. (Ao: Aorta, LCA: Left coronary artery; orifice in parasternal long-axis view. (Ao: Aorta; LV: Left ventricle;
RCA: Right coronary artery; RV: Right ventricle). RCA: Right coronary artery; RV: Right ventricle).
quality color flow recording in LMCA including proximal flow in the locations where normally no color of spectral
Cx and RCA may be obtainable in approximately 55% of coronary flow signal could be recorded. Twofold increase
cases.1 However, more recent publications report much in coronary flow velocity or acceleration of diastolic flow
higher feasibility values2—antegrade LAD flow may be above 2 m/s are specific signs of a significant coronary
completely seen in >90% of patients, with lower values stenosis or restenosis.3,4
for the proximal, middle, and distal segments of Cx (88%, Unfortunately, there are no direct specific signs for the
61%, and 3%, respectively) or RCA (40%, 28%, and 54% of color flow detection of coronary occlusion. In such cases,
patients, respectively). reversed diastolic flow in coronary arteries depending
Typically, the coronary ostia are seen in parasternal from retrograde filling by collateral circulation could
short-axis views at the level of the sinuses of Valsalva be recorded. This finding is very specific but offers low
(Fig. 64.1; Movie clip 64.1), with additional views of the sensitivity since collaterals may also provide anterograde
right coronary ostium seen in parasternal long-axis view perfusion.5 Two-dimensional detection of coronary
(Fig. 64.2). The perpendicular direction of LMCA in short- luminal stenosis is usually not reliable in TTE.
axis view in TTE may cause suboptimal detection of flow
with underestimation of recorded flow velocity, thus
Transesophageal Echocardiography
prompting a search for images from additional window—
modified apical five-chamber view (Fig. 64.3). Higher ultrasound beam frequency (thus higher resol-
The possibility of transthoracic detection of coronary ution) and anatomical proximity create opportunities for
stenosis was underestimated for years. Recent studies improved imaging of proximal coronary arteries (especially
demonstrate that with a careful technique, many major left) from the transesophageal window, including better
coronary segments can be visualized and a strategy of high anatomical definition of possible lesions. TEE enables
sensitivity Doppler detection of stenosis can be adopted in visualization of proximal coronary arteries in more than
experienced hands.3 90% of studies and offers interpretable recordings of flow
Due to anatomical relationships, the easiest recording in 85%, 65%, and 58% of LAD, Cx, and RCA , respectively.
of coronary flow is that obtained for the proximal LAD and As opposed to TTE, TEE allows the assessment of vessel
both interventricular branches. The most specific sign of morphology including the identification of luminal
coronary stenosis is flow acceleration and turbulence— narrowing.6 Importantly, from the clinical point of
thus color Doppler can serve as a roadmap for more view, LMCA is evaluable in nearly in 100% for two-
detailed spectral flow interrogation both in TTE and dimensional and 88% for flow imaging by experienced
TEE studies (Movie clip 64.2). The presence of stenosis observer during TEE study.7 The technique of TEE
with rapid, multidirectional flow may “lighten up” the assessment of coronary ostia is based on the systematic
Chapter 64: Echocardiographic Assessment of Coronary Arteries—Morphology and Coronary Flow Reserve 1339
Fig. 64.3: Additional image of the proximal left coronary artery in Fig. 64.4: Normal left main coronary artery with bifurcation visual-
modified apical five-chamber view; note red-coded LCA flow with ized in transesophageal echocardiography (TEE). (Ao: Aorta; LA:
focal turbulence (stenotic site—arrow). (Ao: Aorta; LCA: Left coro- Left atrium; RVOT: Right ventricle outflow tract).
nary artery; RV: Right ventricle; LV: Left ventricle).
Fig. 64.5: Normal proximal right coronary artery visualized in two- Fig. 64.6: Normal left main coronary artery flow visualized in
and three-dimensional echocardiography including ostial en-face transesophageal echocardiography (TEE). (Ao: Aorta; RVOT:
views and flow recording. (Ao: Aorta; RA: Right atrium; RCA: Right Right ventricle outflow tract; LCA: Left coronary artery; LA: Left
coronary artery; RVOT: Right ventricle outflow tract; LA: Left atrium). atrium).
visualization of left and right sinus of Valsalva in high attempt to record flow with spectral Doppler should be
esophageal short-axis cross-sectional views (Movie clip done, especially when abnormal color pattern is seen
64.3) leading to the identification of LMCA dividing into (Movie clip 64.5), with LMCA and LAD being most feasible.
LAD and Cx (Fig. 64.4), and, usually in separate view, RCA Measuring diameters is feasible mainly for LMCA and Cx
(Fig. 64.5). This approach allows the exclusion of coronary due to the perpendicular course versus TEE ultrasound
artery anomalies. Usually coronary arteries visualization beam of LMCA to ultrasound beam in both TTE and TEE
is accomplished in color flow mode (Fig. 64.6; Movie examination allows its measurement in optimal linear
clip 64.4) with filter setting kept low to enhance low velocity resolution.8 Beyond the search of stenosis, significant
diastolic flow of the coronary arteries. Detection of focal dilatation of proximal segment of the coronary artery
acceleration and aliasing is the typical, highly specific sign may be diagnostic for the presence of fistula, aneurysm
of significant luminal stenosis (Figs 64.7 and 64.8). Nyquist (atherosclerotic or Kawasaki disease), or anomalous
limit should be kept high at this stage to avoid false-positive origin of the coronary artery from the pulmonary artery
findings of flow acceleration coded by aliased signal. An (e.g. White–Bland–Garland syndrome). The progress in
A B
Figs 64.7A and B: Examples of stenosis in circumflex (Cx; A) and left anterior descending (LAD; B) detected by transesophageal echo-
cardiography (TEE) color Doppler. (Ao: Aorta; RVOT: Right ventricle outflow tract; LCA: Left coronary artery; Cx: Circumflex artery; LAD:
Left anterior descending; LA: Left atrium).
color Doppler is close to 100% for LMCA, 95% for LAD,

75% for Cx with much less sensitivity for RCA .11
DISTAL CORONARY FLOW

AND CORONARY FLOW RESERVE
Due to improving Doppler technology, routine detection
of distal coronary flow (apical segments of major epicardial
coronary arteries) has become routinely feasible.13 Intra-
venous contrast agent injection may be useful to enhance
low-intensity flow signals.14
Usually, the detection of flow in distal LAD is achieved
in modified apical three-chamber view, usually in the
range of 20 to 50 cm/s (Fig. 64.10). Increased velocities
Fig. 64.8: Stenotic distal left main artery (LMA) with accelerated may occur if a stenotic situs is directly sampled, whereas
flow spectrum recorded by transesophageal echocardiography very low distal velocities (around 10 cm/s) with slow
(TEE). (Ao: Aorta; RVOT: Right ventricle outflow tract; LA: Left
atrium; LCA: Left coronary artery). deceleration were reported distally to critical stenoses
in LAD. Feasibility of distal LAD Doppler flow recording
three-dimensional echocardiography allows registration
is higher (90–95%) than in RCA (60–70%) or marginal
of high-resolution data sets with unique ostial views
branches of Cx (main Cx flow registration is not feasible).15
and improved tracking of the spatial course of proximal
Recording of coronary sinus flow allows insight in the
coronary arteries9 (Figs 64.5 and 64.9). Multiplane features
total coronary flow return and TEE approach has been
of matrix probes are also valuable tools for imaging of
rapidly moving objects such as coronary arteries including suggested for the diagnosis of LAD stenosis (abnormal
flow (Movie clip 64.3). value < 2.0)16 or evaluation of microvascular dysfunction in
Twofold increase in coronary flow velocity (maximal- patients with diabetes with proposed cut-off values < 1.7.17
to-prestenotic flow velocity ratio)10 or maximal flow Distal post-stenotic diastolic-to-systolic velocity ratio
velocity above 1.5 to 2.0 m/s represents a specific threshold has been reported as an accurate approach to define
for diagnosing significant coronary stenosis, consistent significant LAD or marginal branch stenosis (abnormal
with TTE. Specific velocity thresholds for TEE have also cutoff < 1.68).18 Early postinfarction resting flow pattern in
been proposed (Table 64.1). The accuracy for detection of distal LAD bed was described to predict remodeling and
proximal stenosis based on combined two-dimensional, transmural necrosis. Short deceleration time of diastolic
Fig. 64.9: Proximal left coronary artery analyzed in three-dimensional echocardiography including ostial en-face views and multislice
imaging. (Ao: Aorta; AV: Aortic valve; RV: Right ventricle; LA: Left atrium; LCA: Left coronary artery).
Table 64.1: The Optimal Views for Proximal Segments of Coronary Arteries and Proposed Cut-Off Values for the Assessment of
Significant Stenosis
Coronary Artery TTE-Optimal View TEE-Optimal View Proposed Cut-Off Proposed Cut-Off
Values11 Values12
LMCA High parasternal short-axis, Short axis, 0° or 180°, 1–2 cm > 1.23 m/s > 1.40 m/s
apical five-chamber above aortic valve
LAD High short-axis proximal and Short-axis distal and directed > 1.10 m/s > 0.9 m/s
directed toward the probe, from the probe—near parallel
apical five-chamber to Doppler beam
Cx High short-axis distal and Short-axis proximal and > 1.40 m/s > 1.10 m/s
directed from the probe directed toward the probe
RCA Parasternal long-axis, Short axis, slightly higher than > 0.53 m/s —
parasternal short-axis LMCA ostium and in the aortic
long-axis view
(Cx: Circumflex; LAD: Left anterior descending; LMCA: Left main coronary artery; RCA: Right coronary artery; TEE: Transesophageal
echocardiography; TTE: Transthoracic echocardiography).
coronary flow (≤600 ms) recorded 2 days after percutaneous provide false-negative results because of the presence
coronary intervention (PCI) in the distal part of LAD and of normal side branches between the sampling site and
intramyocardial arteries predicted the lack of myocardial the stenosis. In clinical practice, coronary flow reserve
viability after anterior wall infarction19 and worse clinical (CFVR) is usually assessed in LAD after flow detection
outcomes.20 Finally, calculating distal to proximal diastolic in dedicated color flow mapping protocols from apical
velocity ratio was attempted to improve the detection of foreshortened three-chamber view (with probe located in
left coronary artery (LCA) stenosis (normal value < 0.5).21 higher intercostal space). The evaluation of coronary flow
Measuring resting values alone is subject to many reserve (CFVR) in RCA requires recording of flow in distal
technical inaccuracies and thus velocity ratio approach posterior interventricular artery from a modified apical
has become more popular. Technically, sample volume two-chamber view (Fig. 64.11).
of pulsed wave Doppler should be located distally Noninvasive evaluation of CFVR in echocardiography
from the investigated stenosis (which can be localized allows the assessment of functional significance of the
anywhere proximal to the measurement). The localization stenosis in epicardial parts of coronary arteries and the
of the sample volume in part proximal to stenosis may status of the microcirculation.22 Distal coronary flow
Fig. 64.10: Distal left coronary artery (LCA) flow recorded in color Fig. 64.11: Distal right coronary artery (RCA) flow recorded in
and spectral Doppler, modified apical four-chamber view. (Ant IVS: color and spectral Doppler, modified apical two-chamber view.
Anterior interventricular septum; LCA: Left coronary artery; LV: Left (RCA: Right coronary artery; LA: Left atrium).
ventricle).
Table 64.2: The Conditions Decreasing CFR in the Absence of Significant Stenoses in Epicardial Arteries
Hypertrophic cardiomyopathy
Hypertrophy in arterial hypertension
Aortic stenosis
Aortic insufficiency
Dilated cardiomyopathy
Diabetes
Syndrome X
Increased blood viscosity: policythemia, macroglobulinemia
Hypercholesterolemia
For some of these conditions (e.g. aortic valve disease, hypertension, hypercholesterolemia) surgical or medical treatment could
reverse coronary flow reserve (CFR) impairment.10
becomes abnormal in cardiac vascular or myocardial exercise) to the resting or baseline flow velocity. In practice,
disease and resting values have been recently proposed. the assessment of CFVR is more difficult in the setting of
Relationship of peak diastolic flow velocity during exercise or during dobutamine test (because of tachycardia
vasodilatory challenge (in practice—usually intravenous and increased respiratory motion), and the two methods
dipyridamole or adenosine, which act on precapillary of choice are studies with dipirydamole (0.84 mg/kg, iv)
resistance vessels to maximize flow) to resting flow velocity or adenosine (0.14 mg/kg/min, iv) infusions. Normal
allows to calculate CFVR, closely corresponding with the values for CFVR range from 3 to 5 and values below
values provided with intracoronary Doppler measure- 2 confirm the physiological significance of coronary
ments and useful to define physiologically meaningful artery stenosis, usually with luminal narrowing > 70%
stenosis in LAD and RCA.23 coronary artery stenosis or indicate other conditions
The conditions impairing CFVR values despite normal impairing CFVR24,25 (Figs 64.12 and 64.13). The assessment
epicardial arteries in invasive angiography are listed in of CFR may help in classification of intermediate stenosis
Table 64.2. (40–70%) to the invasive treatment.
CFVR is defined as the ratio of maximal (or mean) CFVR decreases with age, but may exceed 5 in selected
velocity of coronary flow measured during vasodilatation groups of patients such as young healthy athletes.26
(which may be induced medically by dipirydamole, CFVR also show transmural dispersion, related to greater
adenosine, dobutamine, or papaverine infusion or by extravascular component of microcirculatory resistance
to impair CFVR34 and in long-term, an improvement in

CFVR was recorded (invasively by intracoronary Doppler)
4 months after stem cell therapy in reperfused acute
myocardial infarction comes from REPAIR-AMI trial.35 In
the group of 30 patients treated by intracoronary bone
marrow cells infusion, coronary flow reserve improved
from 2.0 ± 0.1 to 3.8 ± 0.2, as P < 0.001 as compared to
controls (change 1.9 ± 0.1 to 2.8 ± 0.2).
CONGENITAL ABNORMALITIES OF
THE CORONARY ARTERIES
The prevalence of coronary arteries anomalies ranges
from about 1 to 5.6% and the most frequent anomaly is the
Cx branch originating from the RCA or the right sinus of
Fig. 64.12: Normal coronary velocity flow reserve measured in 36–39
distal left anterior descending (LAD)—coronary flow velocity Valsalva, found in 0.48% of the invasive angiographies.
reserve (CFVR) = 3.0. The clinical presentation may be silent or they may cause
angina, arrhythmia, syncope, myocardial infarction, and
40
in subendocardial layer, with higher CFVR values in sudden cardiac death. Some anomalies of coronary
subepicardial than subendocardial regions.27 The value arteries origin including the LMCA arising from the right
of adding CFVR (usually LAD) to wall motion assessment sinus of Valsalva (although less common than the origin of
lies in improved sensitivity without specificity loss.28 RCA from the left sinus of Valsalva (Fig. 64.14), may have
CFVR is recommended as a component of state-of-the- fatal consequences related to slit-like orifice narrowing,
art standard stress echo protocol with dipyridamole and sharp angulation, and risk of intra-arterial course between
is most practical when clinical questions regarding the the aorta and pulmonary artery, with threatened sudden
specific anatomical locations arise. Recently published cardiac death during exercise. The diagnosis of coronary
study has shown high accuracy of coronary flow reserve anomalies by echocardiography has been overshadowed
< 2 assessed by TTE for the detection of significant by magnetic resonance or computed echocardiography;
restenosis after stent implantation, again with cutoff value however, it remains a useful option, is radiation-free,
< 2. 0 for three major coronary arteries.29 The diagnostic and allows real-time assessment of coronary anatomy
value of TTE coronary flow reserve is high and similar to and flow. While TTE remains challenging in some cases,
320-row computed tomography.30 TEE images usually unveil realistic coronary anatomy.
The impairment of coronary flow reserve with cut- Such composite diagnostic approach, including invasive
off value < 1.7 assessed during the 24-hour period after coronary angiography or intracoronary ultrasound as
primary coronary intervention was also documented necessary, may be necessary to define the exact course of
as the predictor of left ventricular remodeling early coronary arteries, elucidate possible pathomechanisms
after anterior myocardial infarction.31 Decreased aiding in therapeutic decision-making. This may be
coronary flow reserve < 2.6 with shortened < 840 ms especially valuable when overlap of congenital and
diastolic deceleration time were also related to higher acquired atherosclerotic coronary disease comes into
incidence of cardiac events in patient after heart play.41 Identification of aberrant left coronary artery with
transplantation, defined as cardiac death, heart failure, interarterial course alone represents an indication for
and stent implantation.32 Reduced long-term survival surgical intervention, whereas in anomalous origin of the
was also described in patients with coronary flow reserve RCA more percutaneous interventions may be considered.
< 2 in dilated cardiomyopathy together with such known Finally, echocardiography may provide the valuable
predictors as increased wall motion score index (WMSI) noninvasive and radiation-free tool for the follow-up and
and mitral regurgitation.33 monitoring of coronary intervention results.42
In the context of intracoronary stem cell therapy, early Coronary fistulas are uncommon coronary pathology.
injection in the infarct-related coronary bed does not seem “Pediatric” type features low-resistance connection of the
Fig. 64.13: Low coronary velocity flow reserve measured in distal left anterior descending (LAD)—coronary flow velocity reserve (CFVR)
= 1.7 in a hypertensive diabetic patient free of epicardial coronary disease.
Fig. 64.14: Coronary anomaly—right coronary artery (RCA) originating from the left sinus of Valsalva with interarterial course. Mild flow
abnormality (turbulence) visualized in transesophageal echocardiography (TEE). (RCA: Right coronary artery; RVOT: Right ventricle
outflow tract).
dilated coronary artery with cardiac chamber, which can SUMMARY

be imaged using color Doppler, usually in the right side
of the heart. TTE and TEE offer diagnosis by visualization The evaluation of coronary arteries by modern echocar-
of marked arterial dilatation and tortuosity, with multiple diography enables routine noninvasive detection of
cross-sections of a winding vessel in select views with congenital anomalies and significant proximal stenosis,
detectable diastolic flow pattern (Fig. 64.15). In adults, especially in LMCA, which underscore the role of this
tiny fistulous connections (usually LAD—pulmonary method as noninvasive and valuable screening tool in
trunk) are accidentally detected in coronary angiography these frequently dangerous setting. Functional assessment
and can be validated by detecting flow using color of coronary stenosis by noninvasive CFVR contributes to
Doppler (Fig. 64.16) The echocardiographic visualization physiological stratification of luminal coronary narrowing,
of coronary aneurysms and fistulas has been reported and echocardiogram may support monitoring of coronary
also in iatrogenic complications related to percutaneous artery interventional treatment, offering additional
coronary interventions.43 prognostic information.
Fig. 64.15: Pediatric type coronary fistula from the right coronary
artery to the right atrium. (RV: Right ventricle; RCA: Right coronary
artery; LV: Left ventricle).
Fig. 64.16: Minor left anterior descending (LAD) fistulous connection to the proximal pulmonary trunk (diastolic color flow visible) with
corresponding angiogram. (Ao: Aorta; RVOT: Right ventricle outflow tract; MPA: Major pulmonary artery; LA: Left atrium; LAD: Left
anterior descending; LCA: Left coronary artery).
Thus, TEE can detect coronary lesion in prognostically artery segments: a feasibility study. Cardiovasc Ultrasound.
critical proximal locations at no added cost. Incorporation 2009;7:58.
3. Krzanowski M, Bodzon W, Brzostek T, et al. Value of
of assessment of coronary arteries in routine protocol of
transthoracic echocardiography for the detection of high-
TEE study can be therefore recommended, and the field of grade coronary artery stenosis: prospective evaluation in 50
diagnostic approaches based on transthoracic imaging is consecutive patients scheduled for coronary angiography.
expanding. with evidence of clinical benefits, for example, J Am Soc Echocardiogr. 2000; 13(12):1091–99.
in patients studied due to cryptogenic embolism.44 4. Hozumi T, Yoshida K, Akasaka T, et al. Value of acceleration
flow and the prestenotic to stenotic coronary flow velocity
ratio by transthoracic color Doppler echocardiography in
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after intracoronary administration of bone marrow stem Nonatherosclerotic ostial stenosis of left main coronary
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35. Erbs S, Linke A, Schächinger V, et al. Restoration of micro- 43. Lipiec P, Peruga JZ, Krzeminska-Pakula M, et al. Right
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CHAPTER 65
Echocardiography in Hypertrophic
Cardiomyopathy
Dan G Halpern, Mark V Sherrid
Snapshot

Defini ons and Loca ons of Hypertrophy 
Treatment Strategies in Hypertrophic Cardiomyopathy

Le Ventricular Ou low Tract Obstruc on 
Surgical Septal Myectomy

Differen al Diagnosis 
Dynamic Systolic Dysfunc on
INTRODUCTION such as uncontrolled hypertension or aortic valve stenosis.

As discussed below, elite young athletes may have mild
Hypertrophic cardiomyopathy (HCM) is a genetic disorder degrees of hypertrophy that must be distinguished from
with clinically unexplained myocardial hypertrophy (most HCM. All TTE modalities are employed: M-mode and
commonly of the interventricular septum) that occurs in two-dimensional (2D) imaging, spectral, continuous
the absence of a hemodynamic cause. HCM predisposes wave (CW), and tissue Doppler to evaluate: (a) location
to symptoms, dynamic left ventricle obstruction, and of hypertrophy, quantitative estimation of wall thickness;
infrequently, to life-threatening arrhythmias.1 It is the most (b) detection and, if necessary, provocation of systolic
common inherited disorder among cardiovascular diseases anterior motion (SAM) of the mitral valve, anatomy of
(1:500) and is the leading cause of sudden cardiac death the mitral apparatus, and degree of mitral regurgitation
(SCD) in young adults.1 Among its previous names are (MR); (c) Doppler of left ventricular outflow tract (LVOT)
idiopathic hypertrophic subaortic stenosis and muscular velocities or mid-LV gradients at rest and after provocation;
subaortic stenosis. The preferred nomenclature is HCM, (d) evaluation of diastolic dysfunction; and (e) pulmonary
either obstructive, or nonobstructive. HCM is inherited with arterial pressure.
autosomal dominant transmission; currently mutations in Echo also impacts the family of an HCM patient. Since
11 genes coding for various cardiac sarcomeric proteins are HCM is a genetic disease, family members of diagnosed
associated with HCM.2,3 Varying phenotypic expressions HCM patient should be screened. Annual echocar-
and marked heterogeneity is a hallmark of HCM (Figs 65.1A diographic surveillance should include all first degree
to D). Microscopically, HCM is characterized by myocyte relatives of the patient until the age of 21, and afterward
hypertrophy and myocytic disarray interlaced with fibrosis. every 5 years. Screening under the age of 12 years is
Transthoracic echocardiography (TTE) is the most optional unless there are suspicious symptoms of HCM or
powerful tool for the diagnosis, management, and follow- malignant family history of premature death.1 Genotype
up of HCM.4 TTE demonstrates the site and extent of analysis for screening or confirmation of HCM is most
hypertrophy, and delineates and quantifies obstruction. beneficial when there is a positive family history; here, 50%
Before making the diagnosis, it is imperative to rule out have an HCM-associated mutation, whereas in sporadic
more common secondary causes of concentric hypertrophy cases only 30–40% have an HCM-associated gene.3
Chapter 65: Echocardiography in Hypertrophic Cardiomyopathy 1349
A B
C D
Figs 65.1A to D: Major patterns of hypertrophy in hypertrophic cardiomyopathy (HCM). Schematic (left panel) versus echocardiographic
images (right panel). (A) Anterior septal hypertrophy; (B) Subaortic septal bulge; (C) Apical; (D) Mid–left ventricular with obstruction.
Source: Reproduced in part with permission from Shah A et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy.
Echocardiography. 2009;26:922–33.
is ≥ 15 mm in the absence of hemodynamic cause for

the hypertrophy observed. There is great phenotypic
variation in the location and magnitude of hypertrophy.
Several common patterns of hypertrophy in HCM are
depicted in Figures 65.1A to D. The most common pattern
of hypertrophy is of the anterior and posterior septum,
and often of the anterior wall. Other distributions are
thickening restricted to the proximal portion of the septum
A B C D
that is referred to as discrete subaortic septal bulge;
Figs 65.2A to D: Spectrum of sub-basal hypertrophic
cardiomyopathy (HCM). (A) shows pure apical HCM; (B) shows HCM that spares the base but only involves the mid and
apical HCM with some extension to the mid-LV; (C) shows apical apical segments has been referred to as sub-basal HCM.5
and mid-HCM with severe encroachment of the LV cavity resulting Varieties of sub-basal HCM are shown in Figures 65.2A
in a small slit-like left ventricle (LV) cavity in diastole, but no LV to D. Among these are apical HCM, mid–left ventricular
obstruction and no apical akinetic chamber; (D) shows mid-LV
HCM with mid-LV obstruction and an apical akinetic chamber.
thickening with severe encroachment of the LV cavity,
Source: Reproduced with permission from Shah A, et al. Severe and mid-LV obstruction with an apical akinetic chamber.
symptoms in mid and apical hypertrophic cardiomyopathy. Rarely, thickening is restricted to the anterior, posterior,
Echocardiography. 2009;26:922–33. or lateral walls.6 On occasion, the right ventricle (RV)
may be thickened, and rarely obstructs the subvalvular
DEFINITIONS AND TYPES OF RV outflow tract.7 The pattern of hypertrophy may be
useful to predict if an individual patient will have positive
HYPERTROPHY testing for a HCM-related mutation. Patients with septal
Hypertrophy is defined to as end diastolic wall thickness hypertrophy that extends all the way to the apex with a
≥ 12 mm and HCM may be considered when wall thickness resulting crescent-shaped ventricular cavity with reversal
after physiological provocation, including exercise.12–18

LVOT pressure gradients ≥ 30 mm Hg at rest are associated
with decreased survival.17,19 Patients who are not obstructed
at rest but who develop LVOT obstruction after provocation
are referred to as having latent obstruction.13 LVOT
dynamic obstruction causes increased LV work, decreased
diastolic aortic perfusion pressure, increased supply–
demand ischemia, load-related impairment in diastolic
relaxation, and a midsystolic drop in instantaneous LV
ejection flow velocities and volumetric flow.12,18,20,21
The most common cause of LVOT obstruction is SAM
Fig. 65.3: Systolic anterior motion (SAM) of the mitral valve. SAM of the mitral valve with mitral–septal contact (Figs 65.3
of the mitral valve, drawn from an apical five-chamber view, as it to 65.5, Movie clips 65.1 to 65.3). The first demonstration
proceeds in early systole. of SAM was made with M-mode through the mitral valve
Source: Reproduced with permission from Sherrid MV, et al. An
by Shah et al. in 1969, revolutionizing understanding
echocardiographic study of the fluid mechanics of obstruction
in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1993:22; of the obstruction mechanism.22 Previously, surgical
816–25. observations and cardiac catheterization suggested that
LVOT obstruction was caused by a subaortic sphincter
of the normal apical concavity had a 79% probability of an similar to that found in the right ventricular outflow tract
HCM-associated mutation, as compared to 8% of those in congenital heart disease. There are several anatomical
with a discrete subaortic septal bulge.8 features in HCM that permit and predispose to SAM—
The maximum wall thickness, most commonly found septal hypertrophy, anterior displacement of the mitral
in the septum, is a prognosticator for SCD in HCM, as a papillary muscles, and elongation of the mitral leaflets
septal thickness of <19 mm denotes a lower incidence of and chordae.12,16,23,24 These geometric–anatomical features
sudden death, whereas ≥30 mm alone indicates nearly allow ejection flow to get behind the mitral leaflets, and
a 2% per year risk of SCD.9 In patients with high risk for sweep them into the septum. Flow drag, the pushing force
SCD the physician should discuss the benefits and risks of flow, plays the dominant role causing SAM,12,16,25–27 while
of implantable cardioverter defibrillator implantation and the Venturi effect, suctioning from the LVOT tunnel has
consider implantation of patients at high risk.10 Maximum a minor contribution. The septal bulge redirects ejection
wall thickness is thus an important variable in HCM and flow so that, in the apical 3-chamber view, it comes from
should be reported in every patient. The septum is best a relatively posterior direction and catches the anteriorly
visualized in the parasternal long-axis and short-axis views positioned mitral leaflets (Figs 65.4 and 65.5). The anterior
and attention should be given on the short axis to not mitral leaflet is most often elongated.23 In most patients,
include the RV moderator band or anomalous papillary both leaflets move anteriorly and participate in SAM.
muscles. In patients with inadequate windows due to However, in some patients it is the posterior leaflet that
body habitus, pulmonary disease, or atypical pattern of extends beyond the coaptation point and obstructs.28 SAM
distribution of hypertrophy, cardiac magnetic resonance often begins during isovolumetric systole but may begin
(CMR) imaging may aid in delineating wall thickness.11 It later after the aortic valve opens.16,25
is also invaluable when the magnitude of wall thickening is As systole progresses, pushing forces displace the
ambiguous and there is doubt whether 30 mm thickening mitral valve anteriorly with increase in drag as the angle of
is present or not. attack between the leaflet and the ejection flow increases.26
With mitral–septal contact, which may occur from early to
Left Ventricular Outflow Obstruction and late systole, a dynamic gradient is created across the LVOT.
Systolic Anterior Motion of the Mitral The pressure difference across the mitral valve pushes the
valve leaflets further into the septum, further decreasing
Valve the orifice and creating higher pressure gradients.13,25
While one quarter of HCM patients have LVOT obstruction Obstruction begets more obstruction. The narrowing
at rest, two thirds of patients exhibit obstruction at rest or orifice raises the pressure difference; the rising pressure
A B
Figs 65.4A and B: The pushing force of flow. (A) The dark blue, low-velocity flow behind the mitral valve pushes the leaflets
into the septum, before high-velocity flow has occurred in the outflow tract; (B) The spinnaker is pushed by the wind that strikes
its undersurface. Image of Stanley Rosenfeld reproduced with permission, The Rosenfeld Collection, Mystic Seaport Museum.
Source: Reproduced with permission from Sherrid MV, et al. Pathophysiology and treatment of hypertrophic cardiomyopathy. Prog
Cardiovasc Dis. 2006;49:123–51. (LA: Left atrium; LV: Left ventricle; MV: Mitral valve; LVOT: Left ventricular outflow tract.
difference further narrows the orifice. The longer in systole

that the mitral valve touches the septum, the higher the
gradient29 (Figs 65.6 and 65.7).
Obstruction may be thought of as a tug-of-war
between the anteriorly displacing force of flow and
posterior restraint by the chordae and papillary muscles.
Pharmacological treatment with negative inotropes
decrease LV ejection acceleration and thus the pushing
force on the mitral valve.30 Flow drag is related to the square
of the flow velocity, so even small changes in acceleration
and velocity will have a large change in the pushing force.
A B With negative inotropes and a decrease of force on the
valve, the equilibrium is moved toward posterior restraint,
mitral–septal contact is delayed, and pressure gradients
Figs 65.5A and B: Early in systole flow drag is the dominant
hydrodynamic force on the mitral leaflets (A). After mitral–septal are reduced or eliminated.
contact, the pressure difference (gradient) is the force that pushes Echocardiographic evaluation of SAM and LVOT
the mitral leaflet further into the septum (B). Source: Figure obstruction includes M-mode 2D, and Doppler imaging.
modified and reproduced with permission from Sherrid MV, et al.
Systolic anterior motion begins at low left ventricular outflow tract Because of its high temporal resolution, M-mode through
velocity in obstructive hypertrophic cardiomyopathy. J Am Coll the mitral valve is very useful for diagnosing and timing
Cardiol. 2000;36:1344–54.
of SAM and observing the duration of mitral–septal
Fig. 65.6: Four separate cardiac cycles from a patient, displaying Fig. 65.7: Top: Continuous wave (CW) Doppler echocardiographic
simultaneous echocardiographic and hemodynamic tracings during tracing through the obstructing orifice in the left ventricular outflow
pharmacological manipulation of the pressure gradient by increasing tract (LVOT) of a patient with a gradient of 64 mm Hg. The contour
doses of isoproterenol. Systolic anterior motion (SAM) without septal after the inflection point is concave to the left because of the
contact in the first systole is not associated with a significant pressure progressive decrease in the size of the orifice formed as the mitral
gradient. When SAM–septal contact first develops late in systole, valve is pushed by the rising pressure gradient into the septum.
it is brief and the pressure gradient is low (second systole). When Reproduced with permission from Sherrid MV, et al. Reflections
SAM–septal contact develops early in systole, it is prolonged and the of inflections in hypertrophic cardiomyopathy. J Am Coll Cardiol.
pressure gradient is high (fourth systole with higher dose isoproterenol). 1993;54:212–9. Bottom: Comparison of the Doppler velocity tracings
Source: Reprinted with permission from Pollick C, et al. Muscular
of the high-velocity jets of aortic stenosis, mitral regurgitation (MR),
subaortic stenosis: the quantitative relationship between systolic
and obstructive hypertrophic cardiomyopathy (HCM). In aortic
anterior motion and the pressure gradient. Circulation. 1984;69:43–9.
stenosis and MR, as velocity increases, acceleration decreases. In
contrast, in obstructive HCM, as velocity increases, acceleration also
contact (see Fig. 65.6). In addition, M-mode through the increases. In obstructive HCM, the rising pressure difference forces
the mitral leaflet against the septum, which decreases the orifice
aortic valve displays midsystolic closure or notching that
size and further increases the pressure difference. This amplifying
correlates with the aortic bisferiens pulse. Partial closure feedback loop explains the concave contour seen in obstructive
of the aortic valve in midsystole occurs due to a transient HCM. The orifice size changes as an inverse function of the pressure
fall of flow from the obstruction (Fig. 65.8). difference across the stenosis, with the pressure difference itself
The best 2D view to assess SAM and measure LVOT causing an increase in narrowing. Progressive orifice narrowing also
gradients is the apical 3-chamber view where the Doppler explains why the jet peaks late in systole in obstructive HCM.
Source: Reprinted with permission from Sherrid M. Mid-systolic
beam is the most parallel to the LVOT blood flow and
drop in left ventricular ejection velocity in obstructive hypertrophic
can be directed anteriorly and medially away from cardiomyopathy—the lobster claw abnormality. J Am Soc
the left atrium to avoid confusion with MR (Figs 65.7 Echocardiogr. 1997;10:707–12.
and 65.9). The LVOT jet is “narrower” (of shorter duration)
than MR because it does not include isovolumetric It is important to determine what is the mechanism
systole and isovulumetric diastole. Color Doppler shows producing obstruction for accurate surgical planning.
aliasing in the LVOT and CW Doppler shows typical late Although the SAM of the leaflets of the mitral valve is most
peaking velocities. The contour of the Doppler CW jet is commonly the obstructing culprit, the papillary muscles
concave to the left (“dagger-shaped”) because the LVOT may obstruct instead (Figs 65.10A to C).
orifice continues to narrow in systole. This continuing In patients without resting gradients across the LVOT,
acceleration pattern is distinct from the aortic stenosis provocative maneuvers should be performed in order
where the jet is convex to the left with decreasing to unmask obstruction because treatment of symptoms
acceleration, because the orifice is fixed in systole in HCM is heavily dependent on finding a gradient
(see Fig. 65.7). Careful differentiation of the LVOT jet and (Fig. 65.11). While there are many advanced treatments
the MR jet cannot be overemphasized. “Contaminated” jets for gradient that relieve symptoms, there are very few, if
are frequent, and lead to overestimation of the true gradient. any, treatments to improve symptoms in nonobstructed
Fig. 65.8: M-mode through the aortic valve depicting midsystolic Fig. 65.9: Differentiating between the continuous wave (CW) left
closure of the aortic valve, correlating with midsystolic fall in ventricular outflow tract (LVOT) jet and mitral regurgitation (MR)
ventricular ejection velocity and flow. jet in obstructive hypertrophic cardiomyopathy (HCM). The panel
on the left is the outflow jet alone showing the typical concave-
to-the-left contour. The panel on the far right shows the MR jet
patients. Any decrease in preload, or afterload, or increase that has a longer duration, and is continuous with the mitral inflow
velocities shown above the baseline. The MR has higher velocity
in contractility increases obstruction; thus, standing,
than the LVOT jet. The middle panel shows an overlapping
Valsalva maneuver, the postprandial state, or imaging LVOT/MR signal obtained with beam in an intermediate position.
after exercise may reveal latent obstruction.13,31–34 Amyl (Ac: Aortic closure; Ao: Aortic opening; Mc: Mitral closure).
nitrite and dobutamine are nonphysiological means to Source: Reproduced with permission from Yock PG, et al. Patterns
provoke gradient and thus must be viewed as suspect and timing of Doppler-detected intracavitary and aortic flow in
hypertrophic cardiomyopathy. J Am Coll Cardiol. 1986;8:1047–58.
since the change in load they produce may not exist in
daily life. Medications commonly used in cardiology that
decrease preload such as nitrates, high-dose diuretics, or around 1 hour after a moderate meal. The mesenteric
that decrease afterload such as angiotensin-converting vasodilatation creates a decrease in afterload.39
enzyme blockers and dihydropyridine calcium channel It is crucial to understand the pathophysiology of SAM
blockers promote LVOT obstruction. These agents can be when preparing for surgical correction of the obstruction
thought of as provocative of gradient.35 as the operative technique should be tailored individually
Different modalities of provocation should be used from patient to patient. The extent of septal hypertrophy,
in order to ascertain LVOT obstruction. Standing and the the position of the papillary muscles, and slack of the
straining phase of the Valsalva maneuver, both decrease anterior or posterior mitral valve leaflets may significantly
venous return to the heart, reduce preload, and promote vary in their contribution to SAM.12,40 As mentioned
obstruction. In one third of patients, standing produces above, a hypertrophied or anomalous papillary muscle
higher gradients than Valsalva.13 Inability to demonstrate inserting into the midanterior mitral valve leaflet without
obstruction by these simple maneuvers warrants exercising intervening chordae may be the reason for obstruction—
the patient.13-15,36–38 Exercise echocardiography may provoke inadequate depth of septal resection or failure to partially
SAM and obstruction, and provides functional information or completely resect the anomalous papillary muscle may
including exercise tolerance, blood pressure response, leave the patient with persistent obstruction (Fig. 65.10).41
symptoms, and ischemia detection. Microvascular disease Conversely, dynamic LVOT obstruction has also been
in HCM may provoke wall motion abnormalities that are reported in conditions other than HCM such as acute
not distinguishable from large epicardial coronary disease coronary syndrome (ACS) with apical ballooning due to left
without coronary angiography. Dobutamine promotes anterior descending infarct, Takotsubo syndrome, post-
obstruction but is nonspecific and should not be used to aortic valve replacement, concentric hypertrophy with
diagnose obstruction in HCM. Another useful modality is hypovolemia, mitral valve apparatus abnormalities, and
postprandial exercise testing, where the patient is imaged during positive inotropes use.42–46 Careful history taking,
A A
B B
C C B
Figs 65.10A to C: Anomalous papillary muscle inserting in the middle of the anterior mitral valve leaflet and causing left ventricular
outflow tract (LVOT) obstruction. Left panels show the obstructing papillary muscle on parasternal (A) and short-axis views (B)
and the anatomical specimen. Center (A,B,C) and right panels (A,B) show inadequate resections in patients with this pathology
(center C and right B). Because the septal resections have not been carried far enough down the septum, and because the papillary
muscle abnormalities have not been addressed, there is still severe residual obstruction shown by the arrowheads in both cases.
Source: Reproduced with permission from Klues HG, et al. Anomalous insertion of papillary muscle directly into anterior mitral leaflet in
hypertrophic cardiomyopathy. Significance in producing left ventricular outflow obstruction. Circulation. 1991;84:1188–97, and Maron
BJ, et al. Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction
due to anomalous papillary muscle. Circulation. 1998;98:2505–8.
electrocardiography, and echocardiographic assessment MR is commonly seen during LVOT obstruction due to
are required in order to rule out these pathologies where malcoaptation of the mitral valve leaflets. The MR jet is
the treatment strategy is fundamentally different. directed posteriorly; its severity varies and is maximal
during peak SAM. The MR velocity is the highest velocity
recorded by CW Doppler and its tracing is rounded
Mitral Apparatus and Regurgitation
and symmetrical; in contrast, the LVOT obstruction
In HCM patients, the papillary muscles are anteriorly Doppler contour is late peaking and concave to the left
displaced in the LV and the mitral leaflets or chordae are (see Figs 65.7 and 65.9). MR velocity traces begin in
elongated.16,23,47 This prepositioning predisposes to SAM. isovolumetric systole and extend into the isovolemic
Fig. 65.11: Patient with obstructive hypertrophic cardiomyopathy and latent obstruction. There is no left ventricle (LV) out-
flow gradient at rest. Gradient rises to 49 mm Hg after Valsalva, to 74 mm Hg after standing, and to 144 mm Hg after treadmill
exercise. In 30% of cases, we found a higher standing than Valsalva gradient, as in this patient. Of the 56 patients (57%)
with resting gradients < 30 mm Hg, standing provoked a gradient 30 mm Hg in 23 patients (41%), thus placing them in the
domain of obstructive HCM. Standing gradient is recommended on the index echocardiogram in every patient with HCM or SAM.
Source: Reproduced with permission from Joshi S, et al. Standing and exercise Doppler echocardiography in obstructive hypertrophic
cardiomyopathy: the range of gradients with upright activity. J Am Soc Echocardiogr. 2011;24:75–82.
relaxation period and thus are “wider” than LVOT gradient techniques recognize the importance of the distortion
velocities. The LVOT gradient is usually quantified directly of the mitral valve apparatus contributing to the SAM,
from the CW jet (4V2) and this modality is most often LVOT obstruction, and secondary MR. Thus, to thoroughly
employed.48 However, it is also possible to calculate it eliminate SAM in selected patients, in addition to septal
from the MR velocity and the patient’s systolic blood myectomy, further procedures are directed at the mitral
pressure (SBP) by using the modified Bernoulli equation: valve: (a) papillary muscle release or reposition49,50 and
LVOTgradient(mm Hg) = [4(MRvelocity)2 + 10] − SBP, where the (b) mitral anterior leaflet plication that shortens and stiffens
left atrial pressure is assumed to be 10 mm Hg. the anterior leaflet.51–53 Thus, mitral valve replacement
Operations or pharmacological therapy that relieve is uncommonly necessary for pure obstructive HCM.
SAM and LVOT obstruction reliably improve or completely However, it is important to recognize that MR can be due
eliminate MR when it is due to SAM. Current surgical to structural degenerative non-HCM abnormalities of the
valve such as calcification or prolapse, and not to SAM. A of supply–demand ischemia and afterload mismatch.5
clinical pearl is that the MR in these cases is often central This pathology is best imaged by the administration of
or anteriorly directed. In these patients if severe MR is intravenous echo contrast; in the United States, Definity or
present, mitral valve replacement is usually necessary. Optison are used, whereas in India, Definity or Sonovue are
used. Consequently, we administer intravenous contrast
Diastolic Dysfunction in all patients with apical or apical-mid HCM. CMR also
may aid in diagnosing apical aneurysm.65
Impaired relaxation is widely present in HCM as well as
impaired compliance from myocardial fibrosis.54,55 Global
and segmental assessment of diastolic function should
MID–LEFT VENTRICULAR
be assessed by measurement of the transmitral inflow HYPERTROPHIC CARDIOMYOPATHY
velocities, tissue Doppler velocities and pulmonary vein
An uncommon but clinically important form of HCM has
flow velocities, as well as left atrial size. Tissue Doppler
a unique midventricular distribution of hypertrophy,
velocities are decreased in HCM.56 However, the E/e' ratio
and in many patients subsequent midcavity obstruction
has limited application in HCM and does not correlate well
(see Figs 65.2A to D).5,66,67 Mid-LV hypertrophy may be
with LV filling pressures.54 The thickened septum usually
recognized when the extent of the hypertrophy is
has the greatest segmental diastolic dysfunction.57 The
greatest at the level of the papillary muscles. The
presence of a transmitral A-wave should also be noted. Its
highest midventricular gradients are usually recorded
absence could denote atrial fibrillation or atrial stunning,
at the level of the hypertrophied papillary muscles.
which might be an important source of symptoms in
The triad of mid-LV hypertrophy, small LV cavity, and
patients with restrictive physiology. Furthermore, tissue
hypertrophic papillary muscles predispose obstruction
Doppler imaging in a gene-positive HCM relative with
as the LV comes into apposition with opposite wall
normal wall thickness might denote latent subclinical
and the papillary muscles.66 Mid-LV obstruction may
cardiomyopathy by demonstrating reduced systolic
occur on its own without development of an apical
(S') and early filling (e') velocities.58 Strain imaging may
akinetic chamber; many investigators believe that mid-LV
provide additional information on segmental dysfunction
obstruction leads over time to apical aneurysm through
and latent myopathy.59 In patients with LVOT obstruction,
the mechanisms of afterload mismatch and supply–
the relief of the obstruction improves diastolic relaxation.60
demand ischemia, ballooning, scar formation, and
Otherwise, no pharmacological therapy has been shown
thinning (see Figs 65.12 and 65.13). Contrast imaging is
convincingly to directly improve diastolic function in
helpful for demonstrating the obstruction together with
HCM.61,62
the secondary apical chamber.68 An invaluable clue in
diagnosing midventricular HCM and an apical akinetic
Apical Hypertrophic Cardiomyopathy chamber is the detection of diastolic paradoxical jet flow
Apical HCM, first described by the Japanese, is more (Fig. 65.13).69 The jet is paradoxical because it originates
prevalent in the Asian population and consists of significant from the apex in early diastole and travels backward
hypertrophy at the apex; the base of the LV is spared.5,63 toward the LV chamber—normally diastolic flow travels
This variant has a typical electrocardiogram exhibiting toward the apex from the mitral valve. As with apical HCM,
deep symmetrical T-wave inversion in leads V2-6 with contrast is essential for diagnosis and should be given in
apical hypertrophy and a “spade-shaped” LV cavity every case, and CMR is also useful for the detection of the
(Figs 65.2A to D). Because of the distribution of the apical chamber.
hypertrophy, there is far less incidence of obstruction.
Its prognosis is better than classic septal HCM but is still Systolic Dysfunction and Hypertrophic
infrequently associated with arrhythmias and SCD.64
Cardiomyopathy—Chronic Irreversible
Previously, apical aneurysms were thought to accompany
apical HCM; however, aneurysms are most often a product of Verus Dynamic
mid-LV obstruction in which chronic trapping of blood “Burnt out” HCM with severe irreversible systolic dys-
at the apex, and high systolic and diastolic pressures function is seen in a small minority (approximately 2%)
there, lead to aneurysm formation via the mechanisms of patients and is associated with increased morbidity
Fig. 65.12: Two hypertrophic cardiomyopathy (HCM) patients with mid–left ventricle (LV) obstruction and apical akinetic
chambers. In both cases, diastole is on the left and systole is on the right. The thin long arrows show the mid-LV hypertrophy.
The arrowheads show the mid-LV obstruction. The thicker long arrow shows the apical akinetic chambers. In the patient below,
echocardiographic contrast has been given to enhance visualization of the apical akinetic chamber. (LA: Left atrium; LV: Left ventricle).
Source: Reproduced with permission from Shah A et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy.
Fig. 65.13: Paradoxical jet flow in mid–left ventricular (LV)

obstruction with an apical akinetic chamber. Five-chamber color
Doppler view of the LV in early to mid diastole. Jet from the apex
is termed paradoxical because it courses from the apex toward the
mitral valve, at the same time as transmitral filling, below, the red
flow. There are multiple aliasing shells in the apical flow, indicating
it is high velocity. Blood is trapped in the apex during systole due
to the mid-LV obstruction, only to emerge upon relaxation of the
obstructing neck in diastole. Paradoxical jet flow should be looked
for in every patient with apical LV thickening, because it is an
important sign of a hidden apical akinetic chamber. When such flow
is detected, the routine administration of echocardiographic contrast
will readily show the apical akinetic chamber. (AC: Apical chamber;
LA: Left atrium; LV: Left ventricle. Source: Reproduced with
permission from Shah A, et al. Severe symptoms in mid and apical
hypertrophic cardiomyopathy. Echocardiography. 2009;26:922–33.
A B
Figs 65.14A and B: (A) The Lobster Claw Abnormality is the midsystolic drop in left ventricle (LV) ejection velocities, here seen with
pulsed Doppler echocardiography at the entrance to the left ventricular outflow tract (LVOT). The midsystolic drop is due to the sud-
den imposition of afterload from mitral–septal contact. It is the cause of the midsystolic closure of the aortic valve and the “spike and
dome” pattern seen on aortic pressure tracings in patients with hypertrophic cardiomyopathy (HCM) and LVOT gradients > 60 mm Hg.
It is direct evidence of the corrosive effect of gradient in obstructive HCM; (B) The same patient after disopyramide and abolition of the
gradient. Note that the midsystolic drop in ejection velocities is no longer present. Note also the decrease in initial LV ejection accelera-
tion, which is the mechanism of benefit of disopyramide. A decrease in ejection acceleration decreases the pushing force on the mitral
valve leaflets.
and premature death.70 Its pathophysiology is related to during midsystole due to premature termination of
the progression of ventricular fibrosis, and it may occur in contraction at the apex with flow re-established in late
both obstructed and nonobstructed patients. The extent of systole.
myocardial fibrosis is best visualized by CMR gadolinium- In contrast to the irreversible type systolic dysfunction,
delayed hyperenhancement. alleviation of the LVOT obstruction improves systolic
More common than fixed LV systolic dysfunction, function in patients with dynamic obstruction.76,77 This is best
LVOT obstruction may result in dynamic systolic illustrated in reported cases with sudden catastrophic LV
dysfunction due to afterload-mismatch and supply– ballooning with severe LV systolic dysfunction following
demand ischemia.71-73 During midsystole when the flow the sudden transition from latent to overt high-resting
velocity and gradient is maximal through the LVOT, there
gradient LVOT obstruction. Patients present with obstructive
is a transient fall in LV ejection flow with partial closure
cardiogenic shock that mimics ACS. The coronary angiogram
of the aortic valve.73 M-mode through the aortic valve
shows no significant stenoses. As treatment strategies
shows the classic midsystolic closure of the aortic valve
are completely different, echocardiographic imaging is
while pulsed Doppler interrogation at the entrance of the
LVOT shows a midsystolic drop in velocities in the shape crucial to show that the obstruction is the cause of the LV
of a “lobster claw” (Figs 65.14 to 65.17).73-75 The midsystolic dysfunction. Treatments focus on reversing precipitating
drop in Doppler LV ejection velocities occurs due to a causes, intravenous fluids, intravenous beta-blockers,
premature termination of LV contraction caused by the and in resistant cases emergency surgical relief of LVOT
sudden imposition of afterload due to the obstruction.71,72 obstruction; in contrast, ACS requires coronary reperfusion.77
Obstruction worsens myocardial function in midsystole Positive inotropes such as dobutamine or dopamine
on top of the inherently myopathic process. As mentioned augment obstruction in HCM and are disastrous. Such cases
earlier, the “lobster claw” abnormality is also seen in mid- require expert echocardiography continuously. Repeat
LV obstruction. Doppler tracing at the neck of the apical echocardiograms the day after relief of obstruction either by
akinetic chamber, near the beginning of the mid-LV negative inotropic therapy or surgery shows improvement in
narrowing universally shows the lobster claw abnormality systolic function.
DIFFERENTIAL DIAGNOSIS
Other etiologies that predispose to ventricular hyper-
trophy may be mistakenly diagnosed as HCM. This has
been termed pseudo-HCM. The most common etiology
A of hypertrophy is chronic hypertension but others
include discrete subaortic membrane, athlete’s heart, LV
noncompaction, amyloidosis, Anderson–Fabry disease,
and Friedreich’s ataxia.
Congenital subaortic membrane may cause high
A B gradients in the LVOT without the dynamic obstructive
pattern—the Doppler spectra is similar to aortic stenosis
with midsystolic peak gradient and it is convex to the left.
In symptomatic patients, it is treated by surgical resection
of the membrane and replacement of the aortic valve if
C there is more than mild aortic insufficiency.
LV noncompaction may superficially appear to be
hypertrophied myocardium. But left heart contrast echo-
cardiography or CMR differentiates the compacted myo-
cardium from the noncompacted, trabeculated myo-
B D cardium.68
Figs 65.15A to D: The midsystolic drop in left ventricle (LV) ejection Thickened myocardium is the hallmark of cardiac
velocities. Left panel: Doppler echocardiographic tracings in a patient amyloidosis. The myocardium may have a “ground glass
with systolic anterior motion (SAM) of the mitral valve, mitral–septal
appearance”, but this finding is not specific and can be
contact, and a left ventricular outflow (LVOT) gradient of > 100 mm Hg.
(A) Pulsed wave (PW) tracing with the cursor at the entrance of the seen in renal failure and HCM. Moreover, with harmonic
LVOT, upstream from the mitral valve. The midsystolic drop in left imaging it is less prominent. A most valuable clue in making
ventricular ejection velocities begins at the inflection point (arrows). the diagnosis of amyloidosis is the demonstration of low
It is caused by afterload-mismatch. The LV is unable to maintain
instantaneous ejection against the sudden rise in afterload; (B) CW voltage on the electrocardiogram, which is the reverse of any
tracing through both the orifice and also through the entrance of the other type of hypertrophy. Biopsy either from abdominal
LVOT that is apical of the mitral valve. After the inflection point (white fat, gastrointestinal tract, or the right ventricle makes this
arrow), the contour of the jet velocity becomes concave to the left.
The superimposed midsystolic drop that occurs at the entrance of
diagnosis. The recent advent of transthyretin genetic analysis
the LVOT is shown with the yellow arrow. The midsystolic drop also is also useful in the rare patient with mutant transthyretin.
begins at the same point (white arrow). Right panel: four tracings Anderson–Fabry disease, alpha-galactosidase A defi-
from a patient with LVOT systolic gradient of 120 mm Hg due to SAM ciency, a rare X-linked recessive lysosomal storage disease
and mitral–septal contact. Tracings were obtained during the same
examination. (A) M-mode echocardiogram shows midsystolic closure may present with hypertrophy and infrequently with
of the aortic valve leaflets. The arrow points to midsystolic closure; (B) SAM.78 It can be diagnosed with galactosidase levels in men,
The midsystolic drop in left ventricular ejection velocities are shown and better by genetic testing in both genders. Treatment with
on pulsed Doppler tracing obtained from the apex. The pulsed cursor
is in the body of the LV, at the entrance of the left ventricular outflow
intravenous enzyme replacement is now available. Lastly,
tract (LVOT). Velocity drops from 0.8 to 0.5 m/s. The arrow indicates Friedreich’s ataxia is an autosomal recessive spinocerebellar
the nadir of the midsystolic drop; (C) Tissue Doppler echocardiogram neuromyelopathy that may rarely present with severe hyper-
of the interventricular septum as measured from the apex of the LV.
trophy in childhood, but more commonly presents as a
Premature termination of systolic septal shortening is shown (arrow).
Scale in cm/s; (D) Continuous wave LVOT jet velocities are shown dilated cardiomyopathy.
from the left ventricular apex. LVOT gradient is 120 mm Hg. Scale
in m/s. Symmetry of events in early and midsystole is shown. The
midsystolic closure of aortic valve correlates with the midsystolic The Athlete’s Heart
drop in the left ventricular ejection velocities at the entrance of the
outflow tract, with premature termination of the septal shortening,
Highly trained athletes may develop a physiological
and with the peak of the gradient, afterload. Source: Reproduced hypertrophic adaptation by proportionally increasing
with permission from Sherrid M, et al. Reflections of inflections in the heart size and wall thickness resembling HCM.79 The
hypertrophic cardiomyopathy. J Am Coll Cardiol. 2009;54:212–19.
condition is not found with amateurs but rather with
A B
Figs 65.16A and B: Midsystolic drop in ejection velocities in mid–left ventricle (LV) obstruction. (A) Pulsed wave (PW) spectral
Doppler with cursor located in the apical akinetic chamber at the neck of the mid–left ventricular obstruction showing prominent
midsystolic drop in ejection velocity (thin arrow). There is an initial rise in velocities during the unobstructed phase (thick arrow),
followed by the marked decrease in midsystolic velocities and a second peak in early diastole. The drop in velocities corresponds
to the attenuation of the flow signal of the systolic jet in the obstructing neck during mid- and late systole; (B) Marked midsystolic
drop in LV ejection velocities in a patient with severe mid-LV obstruction. The pulsed Doppler is in the apical chamber at the
entrance of the neck of the mid-LV obstruction. Note the complete cessation of forward flow in this patient in midsystole and the
robust emptying of the chamber in late systole and early diastole. The midsystolic drop in ejection velocities is due to afterload
mismatch and provides persuasive evidence that the apical akinetic chamber is due to obstruction and supply–demand ischemia.
Source: Reproduced with permission from Sherrid M, et al. Reflections of inflections in hypertrophic cardiomyopathy. J Am Coll Cardiol.
2009;54:212–9.
elite or professionals who train for more than 5 days a

week with hours of work-out a day. The physiological
adaptation of the heart to intense training depends on
the type of training. Repetitive pressure load training
such as weight lifting produces increased afterload with
subsequent concentric hypertrophy, whereas endurance
training such as jogging or cycling combines pressure and
volume load, an increase that leads to a balanced increase
in ventricular mass and chamber dilatation. Interestingly,
the largest extent of hypertrophy has been reported in
US football players, upper limit 16 mm (Fig. 65.18).80 The
“gray zone” between athlete’s heart and HCM occurs in
patients with wall-thickening between 13 and 16 mm.81
Other echocardiographic parameters that can be applied
Fig. 65.17: Midsystolic drop in tissue Doppler velocities in in gray zone patients for differentiation favoring athlete’s
obstructive hypertrophic cardiomyopathy (HCM). The electro- heart include: uniform hypertrophy pattern with an upper
cardiogram, invasively measured left ventricular outflow tract
(LVOT) pressure gradient, and the tissue Doppler imaging (TDI)
limit of 16 mm, LV cavity size > 55 mm, and a proportional
velocity trace from the basal septum. In the post PVC beat, note increase in all chamber sizes with normal atria size indexed
the simultaneous development of the LVOT gradient (red arrow) to body surface area and normal LV diastolic function.
and the midsystolic septal deceleration notch (black arrow). CMR may detect myocardial scarring in HCM that does
Source: Reproduced with permission from Breithardt OA, et al.
Mid systolic septal deceleration in hypertrophic cardiomyopathy: not occur in athletes. In ambiguous cases, athlete’s heart
clinical value and insights into the pathophysiology of outflow may be distinguished from HCM by halting training for a
tract obstruction by tissue Doppler echocardiography. Heart. period of approximately 3 months to allow regression of
2005;91:379–80.
hypertrophy in the athlete.82
physiological provocations that are refractory to medical

therapy. In the modern era, more extended myectomies
are performed that are explicitly designed to relieve
drag forces on the mitral valve.27,53,85,86 Contemporary
HCM surgical techniques also aim to both reduce septal
thickness and restore more normal mitral apparatus
architecture. TTE thus plays a pivotal role for planning
the extent of myectomy performed and to assess the
contribution of abnormal mitral anatomy to the SAM
(Figs 65.19A to D). Three planes are interrogated in order
to establish an effective septal resection: (a) Transverse
thickness of the proximal and midseptum. Adequate
myectomy redirects flow anteriorly and medially away
from the mitral valve. Over-resection may result in a
disastrous ventricular septal defect (VSD) while under-
Fig. 65.18: Maximal wall thickness of professional football
players. The mean value was 11.2 mm (± 0.2 mm). Six percent of resection results in the persistence of SAM and MR. (b)
players had a wall thickness > 14 mm. Septal long-axis plane determines the length of resection
Source: Reproduced with permission from Abernethy et al. toward the apex; routinely the resection must extend 4.0
Echocardiographic characteristics of professional football players.
cm from the insertion of the right coronary cusp, 1.5 cm
J Am Coll Cardiol. 2003;41:280–4.
past the point of mitral septal contact, down to the level of
the base of the papillary muscles. The most common cause
TREATMENT STRATEGIES IN of persistent SAM is inadequate resection in this plane, just
in the subaortic area. Our surgical colleagues specifically
HYPERTROPHIC CARDIOMYOPATHY
leave the subaortic thickening because it has nothing to do
Pharmacologic Therapy with the cause of obstruction and because resection here
is fraught with the above mentioned complications. (c)
Pharmacologic therapy is first-line treatment for sympto-
Medial–lateral septal plane that determines the transverse
matic patients with obstructive HCM and adequate drug
extent of the resection. While the classic Morrow
trials should be administered before invasive measures
myectomy was a narrow trough 1 to 1.5 cm in diameter,
are contemplated. In patients with obstruction, the
wider resection is now performed, particularly deeper
pharmacologic treatment of HCM is based on negative
in the ventricle. It is the subaortic medial border of this
inotropes to relieve the dynamic obstruction by decreasing
resection that is at a high risk for iatrogenic damage of the
anteriorly displacing drag forces on the mitral valve,
and thus potentiating normal posterior restraint of the conduction system and for VSD.
mitral apparatus by the papillary muscles and chordae. Additional corrective measures are added to the
Beta blockade is first-line negative inotropic therapy for myectomy when abnormal mitral anatomy contributes
obstruction. Disopyramide in adequate dose is added to obstruction. Papillary muscle release is added to
to patients who do not respond to beta blockade.83,84 The myectomy in selected cases that have anterior positioning
goal of therapy in obstructed patients is improvement of the papillary muscles in the LV cavity. Papillary release
in symptoms and functional status and reduction in was first introduced by Messmer, Schoendube, and others
gradient. In patients without obstruction, beta blockers and divides muscular–fibrotic tissue connections between
and verapamil are used empirically for symptoms. The end the papillary muscles and the LV free wall.85,87 The release
point here is improvement in functional status. allows the mitral apparatus to drop posteriorly into a
more normal position separating the inflow and outflow
Surgical Myectomy and “Resect– tracts of the LV. Horizontal anterior leaflet plication was
first introduced by Swistel et al. It shortens and stiffens
Plicate–Release” the anterior leaflet when it is elongated and billows into
Septal myectomy is indicated for HCM patients who the outflow tract—when the anterior leaflet ≥ 30 mm
have symptoms and gradients ≥ 50 mm Hg at rest or after (measured from mitral leaflet tip to the insertion of the
A B
C D
Figs 65.19A to D: Surgical separation of left ventricular inflow from outflow in obstructive hypertrophic cardiomyopathy (HCM):
extended myectomy and papillary muscle mobilization. (A) Line drawing of outflow relative to the mitral valve in early systole. Note
the anterior position of the mitral valve coaptation. The prominent midseptal bulge redirects outflow so that it comes from a relatively
posterior direction, catching the anteriorly positioned mitral valve and pushing it into the septum; (B) After subaortic septal resection.
The subaortic septum has been resected, but only down to the tips of the mitral leaflets. Flow is still redirected by the remaining septal
bulge so that it comes from a posterior direction. It still catches the mitral valve; SAM persists, as does obstruction; (C) The septal bulge
below the mitral leaflet tips has been resected, an extended myectomy. Now, flow tracks more anteriorly and medially, away from the
mitral leaflets; (D) Mobilization and partial excision of the papillary muscles is added to extended myectomy. The mitral coaptation plane
is now more posterior, explicitly out of the flow stream. (See Movie Clips 65.3 and 65.4).
Source: Reprinted with permission from Sherrid MV. Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and
the continuing evolution of surgery for obstruction. Ann Thorac Surg. 2003;75:620–32.
noncoronary aortic cusp in the 3 chamber view).52,88,89 As should assure absence of significant SAM, more than mild
with reefing of a sail, the reduction of leaflet area reduces MR or VSD. 2D imaging should be used to measure the
the billowing of the valve and stiffens it. Significant new septal transverse thickness and length of the plicated
calcifications of the leaflet are a contraindication for anterior mitral leaflet; CW and color Doppler imaging of
the procedure. Horizontal plication is preferred over the LVOT and septum should rule out residual obstruction
the vertical plication introduced earlier by McIntosh or VSD, respectively. Then, the patient is given intravenous
as the horizontal plication does not interfere with the dobutamine adequate to raise the heart rate to provoke
coapting surfaces.90 At St. Luke’s-Roosevelt, NYC, the obstruction and to assess MR. If the spectral Doppler in the
combined operation is referred to as the Resect–Plicate– LVOT persistently shows a gradient more than 30 mm Hg
Release operation (RPR) (Fig. 65.20 and Movie clips 65.3 or more than mild MR, the surgeon should initiate a second
and 65.4).52,53 Alternate successful approaches to mitral pump run for additional corrective surgery.
valve pathology have been developed elsewhere.50,86,91 In
any given patient, preoperative planning with TTE and ALCOHOL SEPTAL ABLATION
transesophageal echocardiogram (TEE) determines which
aspects of the operation are applied. A second option for septal reduction to alleviate obstructive
The role of TEE both before and after cardiopulmonary symptoms in HCM patients resistant to pharmacologic
bypass is pivotal (Movie clips 65.3 and 65.4).27,92,93 TEE per- therapy is alcohol septal ablation.94,95 Ablation is recomm-
formed before decannulation allows complete assessment ended for patients who are not candidates for surgical septal
of the new hemodynamics and extent of septal resection. It myectomy. At cardiac catheterization, a catheter is introduced
Dual Chamber Pacing with Short AV Delay

Here by shortening the atrioventricular (AV) delay
complete capture of the ventricles is accomplished.
Depending on the native PR interval, AV delays of
60–100 milliseconds may be required (longer for patients
with intrinsic AV delay). It is uncertain how ventricular
pacing improves obstruction, but it is most likely related
to the ventricular dyssynchrony that is produced by
ventricular pacing. Dual chamber (DDD) pacing with
short AV delay is only effective in half of patients and it is
not considered a primary therapy for LVOT obstruction.
However, guidelines support its use in patients who are
elderly or frail, or in patients who have devices for other
indications like implantable cardioverter defibrillator for
SCD prevention or for symptomatic bradycardia.1 Formal
AV optimization with echocardiography is required for all
patients who have DDD pacing for obstructive HCM.97,98
Fig. 65.20: Schematic representation of the hypertrophied heart in
hypertrophic cardiomyopathy (HCM) depicting the morphological The AV delay is gradually reduced by 20 milliseconds
variations leading to obstruction and the potential surgical options intervals until the QRS is maximally widened (complete
for management including resection (extended myectomy), capture). At this point, duration of the diastolic transmitral
plication (horizontal mitral plication), and release (manipulation of A-wave is measured, as well as its Doppler time velocity
the subvalvular structures). Also see Movie Clips 65.3 and 65.4.
integral (TVI), and the TVI of the whole transmitral
Source: Reproduced with permission from Swistel DG, Balaram
SK. Surgical myectomy for hypertrophic cardiomyopathy in the diastolic flow tracing. This is compared to baseline measu-
21st century, the evolution of the “RPR” repair: resection, plication, rements before ventricular capture to assure that there is
and release. Prog Cardiovasc Dis. 2012:498–502. no significant (<10%) decrement in A-duration and TVI.
Lastly, the LVOT gradient pre and post ventricular pacing
into the first septal branch of the left anterior descending are compared to assess gradient decrease. It can sometimes
coronary artery. Then, careful assessment of the perforator take months for the full effect of DDD pacing to take effect.
distribution is accomplished by injecting radiographic Disopyramide and DDD pacing have a synergistic effect
contrast dye or dilute echocardiographic contrast into the on LVOT gradient.99 At least yearly echocardiography is
septal branch before the alcohol. The ablation procedure mandatory whenever DDD pacing is used, to avoid over
creates a locally controlled myocardial infarction in the therapy and marked reduction of LV systolic function due
septal region (Figs 65.21A to H). Variability in vessel to chronic dyssynchrony.
anatomy exists, so the operator must be careful not to
induce infarction of the distal septum, RV, anterior, lateral ENDOCARDITIS PROPHYLAXIS
or apical walls, or papillary muscles.94,96 If the pre-alcohol
contrast extends to any of these unexpected locales, Endocarditis antibiotic prophylaxis is no longer
another septal perforator must be interrogated before recommended for obstructive HCM. This is a relatively
alcohol is injected. Once the operator is convinced that rare complication of HCM with a prevalence of <1% that
the contrast distribution is limited to the proximal septum, mainly affects patients with resting obstruction.100 The
alcohol 100% is injected beyond an inflated balloon. mitral valve apparatus and site of septal contact are most
As with surgery, the echocardiogram at the end of the common locations of vegetations. TEE is the modality of
procedure should evaluate the extent of the infarcted area, choice to diagnose endocarditis and distinguish between
presence of SAM, MR, and VSD. vegetations and redundant leaflets from HCM.
A B C D
E F G H
Figs 65.21A to H: Variety of left and right ventricular structures at risk for alcohol-induced necrosis as detected by intraprocedural echo-
cardiography. Opacification of the medial papillary muscle of the left ventricle (LV; A, arrow), the basal segment of the posterolateral wall
(B), the entire posterolateral wall in the apical four-chamber view (C, arrows), the entire interventricular septum (D), a papillary muscle
(arrow) of the right ventricle (RV) via a moderator band in association with a small contrast depot within the interventricular septum (E),
a small segment of the basal inferior portion (arrows) of LV free wall (parasternal short-axis view) (F), the entire posterolateral wall (G)
in the apical long-axis view (same patient as C; arrows), and a larger septal portion together with a right ventricular papillary muscle
(H, arrow). (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Faber L, et al. Targeting percutaneous transluminal septal ablation for hypertrophic obstruc-
tive cardiomyopathy by intraprocedural echocardiographic monitoring. J Am Soc Echocardiogr. 2000;13:1074–9.
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Force on Practice Guidelines. Developed in collaboration hypertrophic cardiomyopathy: Endorsed by the American
with the American Association for Thoracic Surgery, Society of Nuclear Cardiology, Society for Cardiovascular
American Society of Echocardiography, American Society Magnetic Resonance, and Society of Cardiovascular
of Nuclear Cardiology, Heart Failure Society of America, Computed Tomography. J Am Soc Echocardiogr. 2011;
24(5):473–98.
Heart Rhythm Society, Society for Cardiovascular
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CHAPTER 66
Nonobstructive Cardiomyopathies
Rohit Gokhale, Manreet Basra, Victor Vacanti, Steven J Horn, Aylin Sungur, Robert P Gatewood Jr, Navin C Nanda
Snapshot

Cardiomyopathies 
Findings in Dilated Cardiomyopathy Based on EƟology

Dilated Cardiomyopathy (DCM) 
RestricƟve Cardiomyopathy

Secondary Findings in Dilated Cardiomyopathy 
Other InfiltraƟve Cardiomyopathies

The Role of Echocardiography in OpƟmizing Heart Failure 
InfecƟous and Metabolic Cardiomyopathies

Echocardiography in Assessing Ventricular Remodeling 
Carcinoid Heart Disease
CARDIOMYOPATHIES d. Immune-mediated
e. Toxic/alcoholic
The diagnosis of a cardiomyopathy encompasses a wide
2. Hypertrophic cardiomyopathy (HCM)
spectrum of cardiac diseases, all of which are characterized
3. RCM
by cardiac myocyte dysfunction, and therefore a myopathy.
4. ARVC/D
While systolic dysfunction impairs end-organ perfusion,
5. Unclassified cardiomyopathies—for example, left
diastolic dysfunction disrupts the perfusion to the cardiac
ventricle (LV) noncompaction
myocyte itself. This results in the propagation of a vicious
It also included specific cardiomyopathies such as the
cycle whereby systolic dysfunction can ensue.
following:
The World Health Organization (WHO) defined cardio-
• Ischemic
myopathy as a “disease of the myocardium associated
with cardiac dysfunction” in 1995.1 The cardiomyopathies • Valvular
were classified according to anatomy and physiology • Hypertensive
into five major types. The inclusion of arrhythmogenic • Inflammatory: Idiopathic, autoimmune, and infectious
right ventricular cardiomyopathy/dysplasia (ARVC/D) • Metabolic: Endocrine-related; storage diseases hemo-
and primary restrictive cardiomyopathy (RCM) in the chromatosis/glycogen storage
classification for the first time broadened the definition, • Muscular dystrophies: Duchenne, Becker, and myo-
characterizing the breadth of its presentation: tonic
1. Dilated cardiomyopathy (DCM) • Peripartum
a. Idiopathic In 2006, the American Heart Association (AHA)
b. Familial/genetic scientific statement proposed a more contemporary defi-
c. Viral nition and classification of the cardiomyopathies.2
The expert consensus panel proposed the following DILATED CARDIOMYOPATHY (DCM)
definition:
The primary features of all dilated cardiomyopathies include
“Cardiomyopathies are a heterogeneous group of
decreased cardiac output and left ventricular contractility
diseases of the myocardium associated with mechanical
with significantly enlarged left ventricular dimensions.
and/or electrical dysfunction that usually (but not invariably)
The etiology of DCM is variable and encompasses a
exhibit inappropriate ventricular hypertrophy or dilatation
wide spectrum of diseases. These are as noted below:
and are due to a variety of causes that frequently are genetic. • Ischemic cardiomyopathy
Cardiomyopathies are either confined to the heart or are • Idiopathic cardiomyopathy
a part of generalized systemic disorders, often leading to • Familial cardiomyopathy
cardiovascular death or progressive heart failure-related • Noncompacted myocardium
disability”. • Peripartum cardiomyopathy
Cardiomyopathies are categorized into two groups: • Hemochromatosis
1. Primary cardiomyopathies (predominantly involving • Infectious
the heart) – Postviral myocarditis
a. Genetic: – HIV
• Hypertrophic – Legionella
• ARVC/D. – Sepsis
• Left ventricular noncompaction • Toxic cardiomyopathy
• PRKAG2 and Danon glycogen storage diseases – Alcohol
– Adriamycin
• Conduction defects, mitochondrial myopathies,
– Other chemotherapy-induced
and ion channel disorders.
Patients with DCM may initially present with
b. Mixed:
symptoms of a low cardiac output state such as fatigue or
• Dilated effort intolerance. In more progressive states, they may
• Restrictive. also note orthopnea or paroxysmal nocturnal dyspnea
c. Acquired: and if untreated often present with acute congestive heart
• Myocarditis failure (CHF). Echocardiography is a valuable tool in the
• Stress-induced (Takotsubo) management of such patients and has a Class I indication
• Peripartum according to the ACC/AHA guidelines for the management
• Tachycardia-induced of CHF. Patients with a DCM have a constellation of
• Infants of insulin-dependent diabetic mothers. diagnostic features that characterize this condition.
2. Secondary cardiomyopathies (accompanied by other Characterized by chamber enlargement, DCM initially
organ system involvement) affects the LV. Measuring the left ventricular dimension by
In 2008, the European Society of Cardiology’s working echocardiography forms a cornerstone in the diagnosis
group on myocardial and pericardial diseases presented of DCM. According to the ASE guidelines,4 the normal
an update to the WHO/ISFC classification in which reference range for LV diastolic dimension for women is
cardiomyopathy was defined as: “A myocardial disorder 3.9–5.3 cm and for men is 4.2–5.9 cm. When indexed to
in which the heart muscle is structurally and functionally body surface area, the normal range would be 2.4–3.2 cm/m2
for women and 2.2–3.1 cm/m2 for men. LV dimensions
abnormal in the absence of coronary artery disease (CAD),
in excess of 6.2 cm in women and 6.9 cm in men,
hypertension, valvular disease, and congenital heart
suggest severely enlarged and abnormal ventricular size
disease sufficient to explain the observed myocardial
(Figs 66.1A to D; Movie clips 66.1 A to C).4
abnormality”.3
In summary, the definition and classification of
Assessment of Left Ventricular
cardiomyopathies continues to evolve with better under-
standing of the diverse disease states of the myocardium. Contractility
It is important that the sonographer and cardiologist Visual estimation of left ventricular functional can be per-
keep abreast of the developments in cardiomyopathies, formed with two-dimensional (2D), three-dimensional
since it will impact the acquisition and interpretation of (3D), and M-mode echocardiography. With M-mode
echocardiographic studies. measurements of left ventricular size and thickening, a
Chapter 66: Echocardiographic Assessment of Nonobstructive Cardiomyopathies 1371
A B
C D
Figs 66.1A to D: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A), parasternal
short-axis (B), and apical four-chamber (C) views showing marked global left ventricle (LV) hypokinesis. Arrow points to a catheter in
the right heart. The echogenic tricuspid valve (TV) annulus probably represents fatty infiltration; (D) M-mode also showing poor LV func-
tion with increased mitral E point to interventricular septum separation. (Ao: Aorta; DA: Descending thoracic aorta; IVS: Interventricular
septum; LA: Left atrium; MV: Mitral valve; PW: Posterior wall; RV: Right ventricle) (Movie clips 66.1A to C).
reliable estimate of fractional shortening can be made, cardiomyopathies usually present with a global reduction
which in turn determines contractility and ejection frac- in contractility, although regional differences may be seen.
tion. Measurement of the ejection fraction by Simpson’s Regional wall motion abnormalities involving the
method using estimates of the left ventricular volume dur- septum are also seen in the presence of bundle branch
ing systole and diastole provides an objective assessment blocks, postoperatively after coronary artery bypass
of left ventricular contractility (see Chapter 41). grafting surgery and with chronic right ventricular pacing.
The decrease in systolic function can be segmental Bundle branch blocks lead to mechanical dyssynchrony
or global depending on the underlying cause of the during ventricular systole, which further compromises
cardiomyopathy. Segmental wall motion abnormalities contractility and cardiac output. The presence of a
usually suggest an ischemic etiology, corresponding to prolonged QRS duration, particularly left sided, in patients
CAD in the distribution to the affected segments. With with heart failure is associated with more advanced
time and progression of ischemic cardiomyopathy, a myocardial disease, greater LV dysfunction, a poorer
global decrease in contractility may be seen. Nonischemic prognosis and a higher all-cause mortality.5 In the presence
of bundle branch blocks, there is evidence to suggest index is helpful in this regard. The sphericity index of the
that cardiac resynchronization therapy (CRT) decreases LV is the ratio of the long-axis dimension to the minor-axis
all-cause mortality, reduces heart failure hospitalizations, dimension (diameter) of the LV. The normal ratio is > 1.6. A
and improves left ventricular ejection fraction (LVEF) sphericity index of < 1.5 implies pathological remodeling.
in NYHA functional Class I/II heart failure patients.6 As the ventricle enlarges and becomes more spheri-
Abnormal septal motion can also result from right ventricular cal, there also occurs a displacement of the papillary
pressure or volume overload with the onset of pulmonary muscles apically and laterally. Consequently, there
is a change in mitral valve leaflet coaptation as the
hypertension and/or right ventricular failure (Figs 66.2
length of the mitral apparatus is decreased, resulting in
to 66.4; Movie clips 66.2—Part 1 and 2; 66.3A to C).
functional mitral regurgitation (FMR). Besides mitral
regurgitation, dilatation of the LV often results in left atrial
SECONDARY FINDINGS IN DILATED enlargement with subsequent pulmonary hypertension
and right ventricle (RV) enlargement. Right ventricular
CARDIOMYOPATHY
contractility can be primarily affected by the etiology of
the cardiomyopathy or can result from progression of left
Cardiac Dimensions ventricular failure.
The increase in left ventricular dimensions is accompanied In patients with DCM, wall thickness is variable but
by a change in LV shape, with remodeling resulting in a is typically within normal limits. The LV mass though, is
more spherical structure. Measurement of the sphericity uniformly increased.
A B
C D
Figs 66.2A to D
Figs 66.2A to E: Dilated cardiomyopathy. Live/real time three-

dimensional transthoracic echocardiography (3D TTE). (A to C)
Systematic and sequential anteroposterior cropping of right ventricle
(RV) demonstrates the moderator band (arrowhead) as well as other
trabeculations and papillary muscles (arrow) in the right ventricle
(RV); (D and E) Further cropping shows only a few trabeculations in
the RV apex. There is no evidence of noncompaction. Also, there is
no evidence of a mass or clot in the RV apex, which was suspected
on the two-dimensional (2D) study. A prominent trabeculation in the
RV apex very clearly delineated by 3D TTE (arrows in the Movie
clip 66.2, Part 1) was most likely the culprit for the mass-like lesion
suspected on the 2D echocardiogram. Both ventricles are dilated and
show globally poor motion typical of dilated cardiomyopathy. Arrow in
A, B, D, and E points to a false tendon in left ventricle (LV). Movie clip
66.2, Part 2 shows another patient with dilated cardiomyopathy and
globally poor biventricular function. The arrowhead shows a large
clot in the LV which when further cropped demonstrates a central
echolucency consistent with clot lysis. A few trabeculations are also
seen. (LA: Left atrium; RA: Right atrium) (Movie clips 66.2 Parts 1
and 2). Source: Reproduced with permission from Bodiwala K, et
al. Live three-dimensional transthoracic echocardiographic assess-
ment of ventricular noncompaction. Echocardiography. 2005;22:
E 611–20.
A B
Figs 66.3A and B: Dilated cardiomyopathy. (A) The left ventricle (LV) end-diastolic dimension in the parasternal long axis has decreased
in the follow-up study (right side) as compared to baseline examination (left side). Movie clips 66.3A (parasternal long-axis view), 3B
(parasternal short-axis view), and 3C (apical four-chamber view) also show improved LV function 1 year after antifailure regimen.
(Ao: Aorta; DA: Descending aorta; LA: Left atrium; PW: Posterior wall; RA: Right atrium; RV: Right ventricle; VS: Ventricular septum).
Chamber dimensions as assessed by echocardiography With increasing right ventricular size and pressure/
also aid prognostication in patients with DCM. Significant volume overload, the inferior vena cava (IVC) is also
dilatation of the left atrium as noted when the left dilated. It can be imaged in the subcostal views of the heart
atrial dimension indexed to body surface area exceeds about 1–2 cm from its point of entry into the right atrium
26 mm/m2; has been shown to predict mortality and (RA). The diameter of the IVC and the percent decrease in
rehospitalization due to heart failure exacerbation.7 In the diameter during inspiration correlate with RA pressure.
patients above 70 years of age, increased indexed left atrial Evaluation of the inspiratory response with a brief sniff
size was the single independent predictor of death from (the sniff test) is more reliable than normal inspiration
cardiac causes and the most important indicator of cardiac alone. The normal IVC diameter is < 1.7 cm. There is a
events. 50% decrease in the diameter when the right atrial (RA)
A B
Figs 66.4A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical four-
chamber; (B) views demonstrate LV and left atrial (LA) dilatation. (RA: Right atrium; RV: Right ventricle).
volume overload. In particular, the presence of bilateral

right and left pleural effusions are often noted in poorly
compensated cardiomyopathies.
The presence of an isolated pericardial effusion
can provide diagnostic data regarding the etiology
of the cardiomyopathy, especially when the etiology
is postinfectious such as with viral myocarditis. The
pericardial effusion can be tapped with echocardiographic
guidance to help in the diagnostic work-up. However, in the
absence of infectious or metabolic causes such as uremia,
a circumferential effusion may also be accompanied by
anasarca.
In patients with decompensated DCM, the distribution
is typically circumferential. However, with postinfectious
Fig. 66.5: Dilated cardiomyopathy. Two-dimensional transthoracic
echocardiography. Subcostal view adjusted to demonstrate the DCM, the effusion may be localized. The detection of
long axis of the inferior vena cava (IVC) in a patient with dilated stranding within the fluid suggests an inflammatory or
cardiomyopathy and severe pulmonary hypertension. (RA: Right a hemorrhagic etiology of the effusion. Although rare in
atrium).
cardiomyopathies, when large pericardial effusions occur,
the concern for tamponade becomes a determining factor
pressure is normal (0–5 mm Hg). A dilated IVC (> 1.7 cm)
in the management. Evidence of tamponade physiology as
with normal inspiratory collapse (≥ 50%) is suggestive of
noted by right ventricular collapse, abnormal inspiratory
a mildly elevated RA pressure (6–10 mm Hg). A dilated
increase of blood flow velocity through the tricuspid
IVC with an inspiratory collapse < 50% suggests that the
valve and abnormal inspiratory decrease of mitral valve
RA pressure is moderately elevated (10–15 mm Hg).
flow velocity, phasic variation in right ventricular outflow
Finally, a dilated IVC without any collapse suggests a
markedly increased RA pressure of >15 mm Hg (Fig. 66.5).8 tract (RVOT) or left ventricular outflow tract flow, and
exaggerated respiratory variation in IVC flow suggest
significant hemodynamic compromise. The left ventricular
Evidence of Effusion function is further diminished, resulting in decreased
Pericardial and pleural effusions are readily imaged cardiac output. These suggest the need for emergent
on echocardiography and can be signs of significant evacuation of the effusion.
A B
Figs 66.6A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Mitral valve M-mode echocardiography
showing a B bump (A) predictive of significant left ventricular end-diastolic pressure (LVEDP) and an enlarged E-point septal separation
(EPSS; B) in a patient with dilated cardiomyopathy due to a reduced stroke volume with poor systolic function. A normal EPSS should
generally be < 1 cm.
Two-Dimensional and M-Mode Findings outflow tract (LVOT area) gives an estimate of the stroke
volume and thus the cardiac output (stroke volume × heart
In the evaluation of patients with cardiomyopathy, rate). This is significantly reduced in patients with DCM.
helpful M-mode echocardiographic findings include The use of the mitral inflow velocity as well as mitral
E-point septal separation (EPSS) and the mitral M-mode annular excursion by tissue Doppler imaging provides
evaluation showing a prominent “b”-bump.
valuable information for the evaluation of diastolic
The distance (in millimeters) from the anterior septum
function. Diastolic dysfunction accompanies systolic
to the maximal early opening (E-point) of the mitral valve
dysfunction and is a determinant of the prognosis of
is defined as the EPSS. The internal diameter of the LV is
patients with cardiomyopathy. The spectrum of diastolic
proportional to diastolic LV dimension and the maximal
dysfunction ranges from delayed relaxation to irreversible
systolic excursion of the mitral valve is proportional to
end-stage restrictive physiology. The diastolic function
the mitral stroke volume. Therefore, the ratio of these two
correlates with LVEDP. The progression to restrictive
dimensions would be proportional to the ejection fraction.
patterns of diastolic dysfunction is suggestive of volume
The normal EPSS is approximately 6 mm. It is significantly
overload and poorly compensated heart failure.
elevated in patients with advanced cardiomyopathy, often
The poor stroke volume results in elevated LV
exceeding 15 mm. M-mode imaging also may reveal a
end-diastolic pressures and decreased deceleration time.
b-bump at the end of the “a”-wave, which is also consistent
The rapid deceleration time and an early to late diastolic
with an elevated left ventricular end diastolic pressure
peak velocity ratio of mitral flow > 1.0 were associated with a
(LVEDP) with advanced cardiomyopathies (Figs 66.6A
poorer prognosis among patients with an ejection fraction
and B).
< 40% after a recent myocardial infarction (MI).9 Patients
with reversible restrictive filling patterns have a more
Doppler Echocardiography favorable long-term prognosis10 with lesser hospitalizations
Doppler echocardiography assists in assessing cardiac for heart failure. The evidence of a pseudonormal pattern of
output, cardiac index, stroke volume, stroke volume index mitral inflow velocities (early—E-wave, and late—A-wave)
(stroke volume indexed to body surface area), ventricular can be seen when patients progress from mild diastolic
diastolic function, pulmonary arterial pressures, and right dysfunction to more severe stages. It can be unmasked
atrial pressure. The product of the transaortic velocity time by imaging with the use of the Valsalva maneuver. During
integral with the cross sectional area of the left ventricular Valsalva, the flow into the left heart is reduced and therefore,
ventricular remodeling and therefore, consideration of

pharmacological and nonpharmacological treatment to
correct it might be reasonable (Fig. 66.7).
Evaluation of the tricuspid valve with Doppler
echocardiography permits assessment of tricuspid
regurgitation. A measurement of the flow across the
tricuspid valve allows an estimate of the transvalvular
pressure gradient. This in turn provides a noninvasive
assessment of RV systolic and pulmonary artery pressures.
The presence of pulmonary hypertension among patients
with DCM is a poor prognostic factor and suggestive
of poorly compensated cardiomyopathy.13 Therefore,
Doppler assessment is a valuable tool in the follow-up and
prognostication of patients with DCM.
Fig. 66.7: Dilated cardiomyopathy. Two-dimensional transthoracic
echocardiography. An apical four-chamber view showing functional
mitral regurgitation (FMR). (LA: Left atrium; LV: Left ventricle; RA:
Additional Findings
Right atrium; RV: Right ventricle). With long-standing DCM, the mitral regurgitation and
elevated left ventricular diastolic pressures result in left
atrial enlargement. This in turn predispose patients to
the left atrial and ventricular diastolic pressure is reduced
atrial fibrillation, which can result in further decrement
thus resulting in reduced E-wave velocities and thus an
of mechanical atrial function. With slow flow within the
abnormal E/A ratio of < 1.
atrium, spontaneous echocardiographic contrast can
The mitral inflow filling pattern enables assessment of
be noted. Transesophageal echocardiography can often
diastolic filling (by determining deceleration time of E and
demonstrate spontaneous echocardiographic contrast
evaluating for mitral annular early diastolic velocity E').
within the left atrium indicative of poor systolic function
In addition, mitral annulus systolic velocity (S') and early
among patients with atrial fibrillation. Sometimes,
diastolic velocity (E') are reduced in patients with DCM.
spontaneous echocardiographic contrast may be also
It also provides useful indices of the volume status of
seen in the LV. Although mural thrombi in the LV are more
patients. Studies have shown that the E/E' ratio correlates
frequent in ischemic cardiomyopathy with significant wall
well with pulmonary capillary wedge pressure, which
motion abnormalities, they can occur in nonischemic
helps in prognosticating patients with cardiomyopathy.11
DCM. They may be laminar, pedunculated, or mobile
However, the LV inflow pattern is a less reliable estimate
based on the duration of onset of systolic dysfunction
of LV diastolic function when there is moderate to severe
(Figs 66.8 and 66.9; Movie clips 66.8A and B; 66.9—Part 1
mitral regurgitation.
and 2).14
Doppler evaluation of mitral regurgitation is another
valuable tool in the assessment of patients with DCM. As
previously mentioned, with a more spherical architecture THE ROLE OF ECHOCARDIOGRAPHY
of the LV, the papillary muscles are displaced laterally. This IN OPTIMIZING HEART FAILURE
in turn causes tenting of the mitral valve leaflets, which can
worsen mitral regurgitation. Recently, a study12 suggested Patients with advanced conduction system disease
that the severity of FMR is an independent predictor of necessitating pacemaker placement benefit from opti-
adverse events in patients with heart failure due to DCM. mizing the pulmonary regurgitation (PR) interval with
Severe FMR, defined as regurgitant volume (RV) > 30 mL echocardiographic guidance. Using Doppler echocardio-
or effective regurgitant orifice > 0.2 cm2 or vena contracta graphy to assess mitral inflow, the optimal atrioventricular
(VC) > 0.4 cm, was associated with a two-fold increased risk delay can be identified as the interval that produces
of adverse events after adjustment for LVEF and restrictive nonfused E and A velocities without truncating the
mitral filling pattern in patients with HF due to DCM. As duration of A velocity. This has been known to help
a corollary, perhaps FMR is not simply a consequence of improve cardiac output and the patient’s symptoms.15
A B
Figs 66.8A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Apical four- (A) and three- (B) chamber
views. Spontaneous echo contrast (arrowhead) is noted within the left ventricle (LV) cavity in B. Arrow points to a pacemaker in the right
heart. (Ao: Aorta; DA: Descending aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle) (Movie clips 66.8A and B).
A B
Figs 66.9A and B: Multiple thrombi in a patient with poor ventricular function. Two-dimensional transthoracic echocardiography. The
movie clip on day 1 shows prominent thrombi (arrows), some of them mobile, in both atria and in the left ventricle (LV). A few show
central echolucencies of varying sizes consistent with clot lysis. Repeat examination on day 2 shows the clots to be smaller and less
prominent, most likely related to significant resorption. There was no clinical evidence for embolization. A and B are representative
frames from the movie clip. (Ao: Aorta; LA: Left atrium; RA: Right atrium; RV: Right atrium) (Movie clips 66.9 Parts 1 and 2).
Among patients with left bundle branch block (LBBB), the severe dyssynchrony or poor contractile function. This
degree of intraventricular dyssynchrony can be estimated suggested that the degree of mechanical dyssynchrony
by echocardiographic evaluation. Doppler imaging, strain and contractile function may be important determinants
imaging, tissue tracking, and tissue synchronization of benefit in CRT and that these measures may provide
imaging are useful in quantification of this dyssynchrony. incremental value over traditional characteristics such as
Dyssynchrony, assessed as the standard deviation of time- LVEF, QRS duration, and LBBB (Figs 66.10A to G; Movie
to-peak transverse strain, and contractile function assessed clip 66.10A to G).
by global longitudinal strain predicted response to CRT.16 More recently, Doppler tissue imaging (DTI) has been
Patients with mild to moderate dyssynchrony and good shown to add incremental information to the clinical
contractile function derived significant improvement with exam of patients with ischemic cardiomyopathy.17 Among
CRT in the presence of LBBB compared with patients with patients who demonstrate a high degree of heterogeneity
A B
C D
E F
Figs 66.10A to F
Figs 66.10A to G: Biventricular pacing. Two-dimensional trans-

thoracic echocardiography. Before removal of coronary sinus lead.
Parasternal long-axis (A), short-axis (B), apical four-chamber
(C), and subcostal (D) views. Left ventricle (LV) shows mild to
moderate dysfunction with an estimated ejection fraction of 37%.
The arrow points to a pacemaker lead in the right ventricle (RV).
RV function is normal. After removal of coronary sinus lead due to
symptomatic diaphragmatic stimulation. Parasternal long-axis (E),
short-axis (F), and apical four-chamber (G) views show the marked
deterioration of LV function with an estimated ejection fraction of
10–15%. RV function is unchanged. (Ao: Aorta; DA: Descending
thoracic aorta; L: Liver; LA: Left atrium; MV: Mitral valve; RA: Right
G atrium) (Movie clips 66.10A to G).
assessed on DTI, it was noted to be an independent extensively remodeled ventricles might be beyond
predictor of ischemic cardiomyopathy (ICM) and cardiac recovery with no significant benefit from CRT. Thus,
mortality in patients with advanced LV dysfunction. echocardiographic evaluations complement clinical and
elctrocardiographic parameters in determining the favora-
ECHOCARDIOGRAPHY IN ASSESSING bility of CRT. However, there remain a few limitations as
VENTRICULAR REMODELING echocardiography cannot always predict response to CRT
with absolute certainty and this continues to be an area of
Association between increased LV diameters at baseline evolving research.
and poor outcome after CRT implant has been described The rapid evolution of ventricular assist devices has
in some studies.18,19 LV diameter measurements by given patients with end stage heart failure, another avenue
M-mode echocardiography allow acceptable estimation
of augmenting ventricular function. Echocardiography has
of LVEF and correlate with LV volumes, but are hindered
a useful role in evaluating the valvular function among
by a wide margin of error when it comes to accurate LV
patients with left ventricular assist devices (LVADs). Pati-
size assessment, especially for enlarged ventricles.20 The
ents with LVAD often have significant aortic regurgitation
addition of parameters such as sphericity index and end-
accompanying poor systolic excursion of the aortic valve
systolic volume enable a more valid and reliable estimate
of the extent of LV dilatation. due to the impaired cardiac output. Echocardiography
Patients with extensive remodeling [end-systolic can be used to assess cavity size in the optimization of
volume index (ESVI) > 103 mL/m2] and only one para- LVAD settings to achieve clinical improvement (Figs 66.12
meter of intraventricular dyssynchrony at baseline and 66.13; Movie clips 66.12 and 66.13).
showed no significant changes in ejection fraction after
CRT.21 Therefore, patients with low likelihood to improve FINDINGS IN DILATED CARDIO-
in LV function after CRT were more likely to be those with MYOPATHY BASED ON ETIOLOGY
larger LVs. Large ESVI at the time of implant was also an
independent and strong predictor of poor outcome during Familial
long-term follow-up (Figs 66.11A and B and Movie clip It is often an unrecognized entity, with prevalence in
66.11). epidemiological studies, of 20–50% among patients with
Furthermore, increased end-systolic volume limits idiopathic DCM.23,24 Most familial DCM is transmitted
improvement in LVEF after revascularization in patients as an autosomal dominant condition, although all
with chronic ischemic cardiomyopathy, despite presence inheritance patterns have been identified (autosomal
of viability.22 Therefore, it has also been proposed that recessive, X-linked, and mitochondrial).
A B
Figs 66.11A and B: Cardiac resynchronization therapy (CRT). Biventricular pacing. Two-dimensional transthoracic echocardiography.
Apical four-chamber view. (A) Patient with cardiomyopathy with poor left ventricle (LV) ejection fraction; (B) Marked improvement in LV
function is noted following biventricular pacing. Arrowhead points to a pacing wire. (LA: Left atrium; MV: Mitral valve; RA: Right atrium;
RV, right ventricle). These are representative frames from the movie clip (Movie clip 66.11).
A B
Figs 66.12A and B: Left ventricular assist device in a patient with cardiomyopathy. Two-dimensional transthoracic echocardiography.
Parasternal long-axis view. (A) Note very poor function of both ventricles. The aortic valve (arrow) does not open and remains in the
closed position throughout because all the ventricular flow is routed by the left ventricle (LV) assist device into the proximal ascending
aorta (Ao). There is no direct ejection of flow into the AO through the aortic valve; (B) Color Doppler examination shows significant
aortic regurgitation (AR). (LA: Left atrium; RV: Right ventricle) (Movie clips 66.12A and B).
Fig. 66.13: Left ventricular assist device. Two-dimensional

transthoracic echocardiography. Parasternal long-axis view. The
ventricular septum is dyskinetic. Arrowhead points to an assist
device in the left ventricle (LV). (Ao: Aorta; RV: Right ventricle)
(Movie clip 66.13).
The diagnosis requires the typical features of a DCM, After the diagnosis has been established, a follow-up
that is LV dilatation and impaired systolic function echocardiogram is recommended in the postpartum
(i.e. LVEF < 40–50% or fractional shortening < 25%). period at 6 weeks, 6 months, and yearly thereafter.28
Familial cardiomyopathy is diagnosed in the setting of It has been shown that women who did not recover
a family history of two or more closely related family ventricular function completely had higher left ventricular
members with idiopathic dilated cardiomyopathies. end-diastolic dimension and a lower fractional shortening
at the time of diagnosis as compared to those women who
Peripartum did have complete recovery.29
Most recently, TAPSE (tricuspid annular plane systolic
Peripartum cardiomyopathy is a fairly uncommon but
excursion) has been used to study RV systolic function in
serious condition with an incidence of 1 in 3,000 to 4,000
peripartum cardiomyopathy patients as compared to DCM
live births in the United States. It is seen most commonly
patients.30 Mean TAPSE was significantly less in postpartum
in the African American population, advanced age
cardiomyopathy as compared with DCM patients, and a
pregnancies, and high-risk pregnancies such as twins and
TAPSE ≤ 14 mm was found in the majority of postpartum
toxemia.25
cardiomyopathy patients and in about one-third of DCM
Traditionally, it is defined as a cardiomyopathy
patients. Therefore, the RV systolic function (measured
developing between the last month of pregnancy and
by TAPSE) is worse in peripartum cardiomyopathy
the first 5 months postpartum, in the absence of any
other determinable etiology for heart failure or any patients as compared to DCM patients. Like other dilated
other demonstrable heart disease prior to the last month cardiomyopathies, peripartum cardiomyopathy may
of pregnancy.26 This definition has been modified to present with a thrombus inside the dilated LV cavity, which
include the echocardiographic criteria of left ventricular can be seen on 2D or 3D echocardiography (Figs 66.14A
dysfunction with ejection fraction < 45%, or M-mode to R; Movie clip 66.14).
fractional shortening < 30%, or both and left ventricular Dobutamine echocardiography has been used
end-diastolic dimension indexed to body surface area of to challenge the LV in women who have recovered
> 2.7 cm/m2.27,28 Various etiologies postulated include viral from peripartum cardiomyopathy. This permits the
infection, autoimmune mechanisms, hormonal changes, assessment of contractile reserve, which is a measure
genetic disorders, and toxemia.26 of the enhanced contractility with the infusion of an
Echocardiography has been the diagnostic procedure inotropic agent over baseline values. Patients with
of choice because it involves no radiation exposure, is recovered peripartum cardiomyopathy, when compared
noninvasive, and still provides all the required information. with age and parity matched controls, have a lower
A B
Figs 66.14A and B
C D
E F
G H
Figs 66.14C to H
I J
K L
M N
Figs 66.14I to N
O P
Q R
Figs 66.14A to R: Peripartum cardiomyopathy. Morphological assessment of left ventricular thrombus by live three-dimensional tran-
sthoracic echocardiography. (A) Arrowhead points to the thrombus attached to left ventricle (LV) apex; (B and C) Transverse plane
(TP, horizontal plane or short axis) section at the attachment point of the thrombus (arrowhead) shows it to be highly echogenic (viewed
en-face, arrow in C); (D and E) TP and longitudinal plane (LP, vertical plane or long axis) sections through the thrombus (arrowhead)
showing the echogenic attachment (arrow) and a large echolucency within the thrombus consistent with lysis; (F to H) TP and both
TP and LP sections at midthrombus level showing clot lysis. (I and J) TP section at thrombus tip level showing a solid rim (viewed
en-face); (K and L) LP section through thrombus showing clot lysis. (M and N) Frontal plane (FP) section through the thrombus viewed
en-face (M) and after tilting (N); (O) TP and LP sections through the thrombus. The data set has been tilted and rotated to show the
position of the FP; (P to R) Oblique sections through the thrombus. The arrow in R points to residual fibrin strands within the lytic area
of the thrombus. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clip 66.14). Source: Reproduced with
permission from Sinha A, et al. Morphological assessment of left ventricular thrombus by live three-dimensional transthoracic echocar-
diography. Echocardiography. 2004;21:649–55.
contractile reserve.31 This might explain why patients with those at risk of relapse, with a higher incidence of relapse
peripartum cardiomyopathy are at greater risk for relapse in patients with lower cardiac reserve.
in subsequent pregnancies. This was further clarified
by Fett et al. who showed that patients with a history of
Left Ventricular Noncompaction (LVNC)
peripartum cardiomyopathy and recovery had a lower risk
of relapse when they demonstrated adequate contractile Noncompaction of the ventricular myocardium is a
reserve as assessed by exercise echocardiography.32 Thus, cardiomyopathy thought to be caused by the arrest
the assessment of cardiac reserve also helps to identify of normal embryogenesis of the endocardium and
presence of a two-layered structure and the Chin criteria,

which focus on the depth of recesses compared to the
height of the trabeculae.35,37
The Jenni criteria include:
• Ratio of thick noncompacted layer to thin compacted
> 2 and
• Perfused intertrabecular recesses are supplied by
intraventricular blood on color Doppler analysis.
The Chin criterion is based on:
• Ratio of distance from epicardial surface to the trough
of the trabecular recesses and distance from epicardial
surface to peak of trabeculation < 5.
In both of these, it is important that no other cardiac
structural abnormalities such as valvular obstruction or
Fig. 66.15: Left ventricular noncompaction. Two-dimensional
coronary anomalies are present. The parasternal short-
transthoracic echocardiography. Contrast study using definity was
performed for better assessment of left ventricle (LV) endocardial axis view of the apex is particularly helpful in visualizing
border. Arrows point to multiple trabeculations in the LV apex the recesses (Figs 66.15 to 66.18 and Movie clips 66.15
indicative of noncompaction. Note very poor LV function often and 66.17A and B).
noted in this entity. (RV: Right ventricle) (Movie clip 66.15). Color Doppler helps to differentiate noncompacted
ventricular myocardium from prominent normal myo-
myocardium. This abnormality may be associated with cardial trabeculations, false tendons, aberrant bands,
other congenital cardiac defects, although it is also seen cardiac tumors, and left ventricular apical thrombus by
in the absence of other cardiac anomalies. Clinical demonstrating direct blood flow from ventricular cavity
manifestations are highly variable, ranging from patients into deep intertrabecular recesses in noncompacted
who remain asymptomatic to those with profound CHF, myocardium.35
arrhythmias, and systemic thromboemboli. The incremental value of live 3D transthoracic
The prevalence of noncompaction cardiomyopathy is echocardiography (3D TTE) has been illustrated by
estimated to be at 0.05% in the general population based Bodiwala et al. and Baker et al.38,39 This modality allows for
on echocardiographic studies.33 more detailed and accurate diagnosis as the intracavitary
In normal fetal ontogenesis, the myocardium conde- echo densities suspicious for trabeculations can be
nses and intertrabecular recesses are reduced to capillaries. tracked in multiple directions from the base to the apex.
Deep intertrabecular recesses communicating with the Besides viewing the entire trabeculation in detail, there
ventricular myocardium may evolve in some patients
is a well-demonstrated distinction between prominent
because of arrest of compaction of the loosely interwoven
noncompacted and thin compact layer of LV myocardium.
network of myocardial fibers during intrauterine life.34
The ability to quantify the depth of penetration of the
Ventricular noncompaction affects the apical and
intertrabecular recesses in addition to localizing the exact
midventricular segments of both the inferior and lateral
dimensions and severity of noncompaction provides
wall in > 80% of the patients35 and generally spares the basal
prognostic value. Evidence of extensive left ventricular
segments. Segmental appearance is an important feature,
involvement is associated with a poorer prognosis and
since a more diffuse prominent trabecular pattern may be
prompt referral for transplantation, or prophylactic
present in hypertrophied hearts.36 It is identified by the
defibrillator implantation has been suggested to be useful
presence of more than three prominent trabeculations in
in this situation.40
the LV found at autopsy or on various imaging techniques
Further, it also enables distinguishing LVNC from
including echocardiography and magnetic resonance
imaging (MRI). other differential diagnoses such as left or right ventricular
hypertrophy, apical hypertrophic cardiomyopathy (HCM),
right or left ventricular dysplasia, endocardial fibroelastosis,
Echocardiographic Features of LVNC and intracardiac masses such as thrombus and tumors.
There are two separate echocardiographic criteria for the Differentiation from these entities is enhanced by full
diagnosis of LVNC—the Jenni criteria, which stress the characterization of segmental involvement, especially of
A B
C D
Figs 66.16A to E: Left ventricular noncompaction. Two-dimen-

sional transthoracic echocardiography. Parasternal long-axis (A),
apical three-chamber (B), apical four-chamber (C and D) views
showing the trabeculations of the LV wall and deep intertrabecular
recesses (arrowheads) characteristic of left ventricle (LV)
noncompaction. (E) Apical two-chamber view with color Doppler
showing direct blood flow from the ventricular cavity into deep
intertrabecular recesses in LV noncompaction. (Ao: Aorta; LA: Left
E atrium; RA: Right atrium; RV: Right ventricle).
A B
Figs 66.17A and B: Left ventricular noncompaction. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and
apical two-chamber (B) views with Doppler evidence of flow in the recesses (B). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; MR: Mitral
regurgitation; MV: Mitral valve; RV: Right ventricle) (Movie clips 66.17A and B).
A B
Figs 66.18A to C: Isolated left ventricular noncompaction.

Two-dimensional transthoracic echocardiography. (A) Arrow points
to prominent trabeculations involving the whole extent of left
ventricular (LV) posterolateral wall consistent with noncompaction;
(B) Myocardial perfusion study using perflutren lipid microspheres
demonstrated apical hypoperfusion (arrowhead); (C) Echo contrast
time–intensity curves show diminished rate of filling as well as peak
filling in the LV apex as compared to the ventricular septum (VS).
(LA: Left atrium; MV: Mitral valve; RA: Right atrium; RV: Right
ventricle).
Source: Reproduced with permission from Bodiwala K, et al. Live
three-dimensional transthoracic echocardiographic assessment of
C ventricular noncompaction. Echocardiography. 2005;22:611–20.
the right ventricular apex and by correct measurement cardiomyopathy, characterized by basal and midventricular
of the noncompacted/compacted ratio (N/C ratio). This segmental akinesis. At times, right ventricular dysfunction
is best performed in cross-sectional views obtained can accompany left ventricular dysfunction (Figs 66.26A
perpendicular to the heart’s axis,38 and is superior with 3D and B Movie clips 66.26A and B).48
TTE. The left ventricular dysfunction is reversible with
Therefore, the full characterization of noncompaction recovery of ventricular function anticipated with medical
with 3D TTE is of clinical value. In addition, 3D TTE management as outlined for DCM.46
may potentially offer a better modality for following
patient response to therapy, including use of -blocking Tachycardia-Induced Cardiomyopathy
agents that have been associated with improvements in
and Other High-Output States
ventricular function (Figs 66.19 to 66.25; Movie clips 66.19
[parts 1 to 5], 66.20, 66.22A to C, 66.23A to C, and 66.24 Tachycardia-induced cardiomyopathy is a well-recognized
[parts 1 and 2]). entity leading to DCM, but its exact incidence is still
When conventional images are of poor quality or unknown. It should be suspected in patients with atrial as
nondiagnostic, contrast agents can be used to enhance well as ventricular arrhythmias including atrial fibrillation,
endocardial border delineation.41,42 The opacification atrial tachycardias, re-entrant arrhythmias, ventricular
within the deep recesses can help confirm the diagnosis. tachycardias, and premature ventricular contractions
Contrast can also be used to determine the associated (PVCs) resulting in persistently high heart rates.
presence of thrombi with and without clot lysis. It has all the features of DCM on echocardiography
with the exception that LV end-diastolic dimension (EDD)
tends to be smaller in patients with tachycardia-mediated
Takotsubo Cardiomyopathy/Left
cardiomyopathy.49 Follow-up of the ventricular function
Ventricle Apical Ballooning Syndrome after a period of 3–6 months with satisfactory rate control
Takotsubo cardiomyopathy, also called stress-induced will often demonstrate significant recovery of function
cardiomyopathy or broken heart syndrome, is an (Fig. 66.27A and B; Movie clips 27A and B).
increasingly reported syndrome characterized by transient
systolic dysfunction of the apical and/or mid segments Ischemic Cardiomyopathy (ICM)
of the LV that occurs in the setting of acute emotional
Ischemic cardiomyopathy is a form of DCM characterized
stress. The initial presentation often mimics that of an
by systolic dysfunction due to CAD. The prevalence of
acute MI with concomitant rise in cardiac biomarkers,
ischemic cardiomyopathy in the United States is nearly
electrocardiographic abnormalities, and in severe two-thirds of all cases of chronic systolic dysfunction.50
cases with features of acute CHF.43 However, coronary Patients with CAD have concomitant risk factors such as
angiography does not reveal a significant obstruction hypertension, which can independently cause a decline in
that can explain the nature or magnitude of systolic systolic function.
dysfunction noted. The etiology is thought to be related to The echocardiographic features are typical of DCM.
the hyperadrenergic state associated with emotional stress An important finding is that of significant wall motion
or psychological trauma. abnormalities independent of conduction system abnor-
The classical finding of apical ballooning (typical malities, consistent with a territory of ischemia on prior
variant) and/or midventricular hypokinesis is usually infarction.
seen on left ventriculography or echocardiography.43–45 In Revascularization can result in improvement of
a minority of cases, the transient left ventricular hypokinesis ventricular function. However, with long-standing cardio-
is restricted to the midventricular segments (“atypical myopathy, evidence of increased end-systolic volume
variant” or “apical sparing variant”) without involvement limits improvement in LVEF after revascularization in
of the apex.46 As reported by Kurowski et al. the atypical patients with chronic ischemic cardiomyopathy despite
variant may account for nearly 40% of patients with the presence of viability. Further, secondary features
Takotsubo cardiomyopathy.46 More recently, Manzanal on echocardiography such as myocardial thinning with
et al.47 also described a case series of patients with increased echogenicity and akinesis or dyskinesia of the
the atypical variant, the so-called inverted Takotsubo left ventricular segment suggest that the systolic function
A B
C D
Figs 66.19A to E: Combined left and right ventricular non-
compaction. Live/real time three-dimensional transthoracic echo-
cardiography. (A and B) Arrows point to prominent trabeculations in
both ventricles; (C) Arrows show multiple prominent trabeculations
in RV; (D) Transverse cropping of left ventricle (LV) apical
area shows a honeycomb-like appearance (arrow) typical of
noncompaction; (E) Echo contrast study using perflutren lipid
microspheres shows filling of intertrabecular recesses with
the contrast agent (arrows). See Movie clip 66.19, Part 1 to 5.
Systemic cropping of the three-dimensional data set demonstrates
extensive trabecular involvement of the LV (arrowheads). (AV:
Aortic valve; LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
RA: Right atrium; RV: Right ventricle) (Movie clips 66.19 Parts 1
to 5). Source: Reproduced with permission from Bodiwala K, et al.
Live three-dimensional transthoracic echocardiographic asse-
ssment of ventricular noncompaction. Echocardiography. 2005;
E 22:611–20.
A B
C D
E F
Figs 66.20A to F
G H
I J
Figs 66.20A to K: Combined left and right ventricular noncompaction.
Live/real time three-dimensional transthoracic echocardiography.
(A and B) Arrows point to a cauliflower-like mass in right ventricle
(RV) mimicking a tumor. Magnetic resonance imaging in this patient
erroneously suggested a ventricular septal tumor; (C to E) Arrows
point to massive trabeculations in both left ventricle (LV) and RV
consistent with noncompaction; (F and G) Transverse (F) and
anteroposterior (G) cropping of LV apical area show a honeycomb-
like appearance (arrow) typical of noncompaction; (H) Arrowheads
point to multiple trabeculations occupying over 80% of RV cavity; (I
and J) Arrow points to massive trabeculations in the ventricular septal
area; (K) Arrows point to multiple trabeculations crossing the RV
cavity transversely. (Ao: Aorta; AV: Aortic valve; LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; RA: Right atrium; RCA: Right coronary
artery; RV: Right ventricle; TV: Tricuspid valve; # 1, anterior leaflet of
tricuspid valve, # 2, septal leaflet of tricuspid valve) (Movie clip 66.20).
Source: Reproduced with permission from Bodiwala K, et al. Live
three-dimensional transthoracic echocardiographic assessment of
K ventricular noncompaction. Echocardiography. 2005;22:611–20.
Fig. 66.21: Isolated right ventricular noncompaction. Live/real time

three-dimensional echocardiography. In this patient, prominent
trabeculations (arrowhead) occupy over 80% of the right ventricular
(RV) cavity.
A B
Figs 66.22A to C: Isolated right ventricular noncompaction. Two-

dimensional and live/real time three-dimensional transthoracic
echocardiography. (A) Two-dimensional study. Arrowheads point
to a few muscle bands in the right ventricular apex but there is
no clear-cut evidence for noncompaction; (B) Three-dimensional
study. Cropping of the image reveals a honeycomb-like appear-
ance typical of RV noncompaction. Trabeculations (arrowhead) fill
the distal 40% of RV almost completely; (C) Intracardiac echocardi-
ography. Shows RV noncompaction (arrowhead). (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie
clips 66.22A to C).
Source: Reproduced with permission from Reddy VK, et al.
Incremental value of live/real time three dimensional transthoracic
echocardiography in the assessment of right ventricular masses.
C Echocardiography. 2009;26:598–609.
A B
Figs 66.23A to C: Isolated left ventricular noncompaction and thrombi

in an adult patient. (A) Two-dimensional transthoracic echocardio-
graphy. Arrowheads show echodensities in the LV suggestive of thrombi
with areas of echolucency consistent with clot lysis. The arrow points
to adjacent trabeculations which are relatively less echogenic; (B and
C) Live/real time three-dimensional transthoracic echocardiography.
Arrows point to multiple trabeculations in left ventricle (LV). In C,
one of the echodensities (arrowhead) was cropped revealing a
prominent echolucency, typical of clot lysis (Movie clips 66.23A to C).
Source: Reproduced with permission from Yelamanchili P, Nanda NC,
Patel V, et al. Live three-dimensional echocardiographic demonstration
of left ventricular noncompaction and thrombi. Echocardiography.
C 2006;23:704–6.
Fig. 66.24: Isolated left ventricular noncompaction and thrombus

in another patient. Live/real time three-dimensional transthoracic
echocardiography. Arrowhead points to a clot in the left ventricle
(LV). Clot hypermobility (arrowhead) is demonstrated in the Movie
clip 66.24, Part 1. Movie clip 66.24, Part 2 is from another patient
with isolated left ventricular noncompaction with thrombi in the LV
cavity. The thrombi (T) are easily differentiated from trabeculations
(arrowheads) by their much higher echogenicity. Thrombi are
denoted by arrowheads in sections of the movie containing no
trabeculations. (RV: Right ventricle) (Movie clips 66.24 Parts 1 and 2).
Source: Reproduced with permission from Yelamanchili P, Nanda NC,
Patel V, et al. Live three-dimensional echocardiographic demonstration
of left ventricular noncompaction and thrombi. Echocardiography.
2006;23:704–6.
Fig. 66.25: Left ventricular noncompaction. Shows the

technique of estimating left ventricle (LV) mass in by live/real
time three-dimensional transthoracic echocardiography.
Source: Reproduced with permission from Rajdev S, Nanda
NC, Singh A, Baysan O, Hsiung MC. Comparison of two-
and three-dimensional transthoracic echocardiography
in the assessment of trabeculations and trabecular mass in
left ventricular non-compaction. Echocardiography. 2007;24:
760–7.
B
Figs 66.26A and B: Takotsubo cardiomyopathy. Apical four-chamber view. Velocity vector imaging. The individual arrows point
to the direction of endocardial motion, while the lengths are proportional to velocity. (A) Compared to baseline (left figure), the
velocity of motion has significantly increased in the left ventricle (LV) mid segments, while the apex is still hypokinetic during follow
up (right figure); (B) No change is noted by visual inspection in the left atrial (LA) wall motion during follow-up (right figure) as com-
pared to baseline (left figure). This patient belonged to a series of five patients with Takotsubo cardiomyopathy, in whom the left
atrium also appeared to be involved by velocity vector imaging with a statistical tendency toward improvement during follow-up.
(RA: Right atrium; RV: Right ventricle). These are representative frames from the movie clips. (Movie clips 66.26A and B).
A B
Figs 66.27A and B: “Tachycardia-induced cardiomyopathy.” Two-dimensional transthoracic echocardiography. (A) Parasternal long-
axis view. The left ventricle (LV) is dilated with poor function. The heart rate was 133/min; (B) Apical four-chamber view. The patient was
placed on a -blocker, which resulted in heart rate reduction and improvement in LV function. (Ao: Aorta; DA: Descending aorta; LA: Left
atrium; MV: Mitral valve; RA: Right atrium; RV: Right ventricle) (Movie clips 66.27A and B).
A B
Figs 66.28A and B: Ischemic cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical
four-chamber (B) views. All the chambers are markedly dilated with extremely poor function of both ventricles. The septum is thin and
echogenic, consistent with scarring and fibrosis indicating ischemic heart disease. Arrowhead points to a pacemaker wire. (Ao: Aorta;
LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right ventricle) (Movie clips 66.28A and B).
is unlikely to benefit from revascularization (Figs. 66.28A individuals. Its prevalence is estimated between 1:2,000
and B; Movie clips 66.28 A and B). and 1:5,000.51 It is characterized by fibrofatty infiltration
Therefore, it has also been proposed that extensively of right ventricular myocardium, which often leads to
remodeled ventricles might be beyond recovery with no ventricular arrhythmias, RV dilation, and dysfunction,
significant benefit from revascularization. Thus, echocar- ultimately leading to RV failure.52,53 Criteria for the clinical
diographic evaluations complement clinical parameters diagnosis of ARVC/D were proposed in 1994 by the
in determining the favorability of revascularization in International Task Force, based on structural, histological,
ischemic cardiomyopathy.
ECG, arrhythmic, and familial features of the disease.54
Various echocardiographic findings reported in
Arrhythmogenic Right Ventricular
ARVC/D patients include:
Cardiomyopathy/Dysplasia (ARVC/D) 1. Abnormalities of RV morphology (trabecular derange-
ARVC/D is a genetically predetermined disorder, often ments, hyper-reflective moderator band, focal RV
causing sudden death among young, apparently healthy aneurysms, and sacculations);55
an abnormal trabecular pattern, a highly reflective and

irregularly shaped moderator band, or an isolated dilation
of the RVOT.55
The use of Doppler tissue imaging and 2D strain-
derived parameters to assess regional contractility to
identify ARVC/D has been reported,59 but this is not yet a
standard criterion. In addition, there is an increased use
of 3D echocardiographic measurements of RV volume
and ejection fraction, which have been shown to correlate
with MRI values and hence can be used in prognostication
as well as in follow-up of patients (Fig. 66.29; Movie
clip 66.29).60,61
Toxic Cardiomyopathies
Fig. 66.29: Arrhythmogenic right ventricular dysplasia. Two-
dimensional transthoracic echocardiography. Apical four-chamber
Chemotherapy-Induced Cardiomyopathy
view shows diminished right ventricular (RV) function as well Chemotherapy with adriamycin or other anthracyclines is
as presence of small, localized berry-like aneurysms involving
often adopted for a variety of hematological malignancies.
the free wall (arrows) typical of this entity. The left ventricle (LV)
function is normal. (LA: Left atrium; MV: Mitral valve; RA: Right It, however, is associated with cardiotoxicity and can be
atrium) (Movie clip 66.29A). an issue among adult patients receiving chemotherapy
as well as survivors of childhood cancers.62 The toxicity is
likely due to a combination of factors including reactive
2. Abnormal RV structure (increased end-diastolic and
oxygen species generation and myocardial cellular
end-systolic RV inflow tract dimensions and RVOT
toxicity due to adriamycin itself. While acute toxicity can
dimension in long- and short-axis views);56 and result in cardiomyopathy that can in part be reversed
3. Abnormal RV function (global hypokinesis or regional by discontinuation of the chemotherapy, late onset of a
wall motion abnormalities).57 cardiomyopathy is irreversible.
It has been reported that RVOT > 30 mm is the most Late onset chronic progressive cardiomyopathy occurs
common abnormality associated with Task Force Criteria more than 1 year after anthracycline treatment.63,64 With
diagnosis of ARVC/D, followed by RV dysfunction.57 the loss of cardiomyocytes, cardiac function begins to
Over the past years, advancement in technology and deteriorate with resultant LV wall thinning and in some
imaging including cardiac MRI, 3D echo, and contrast cases, progressive LV dilation.65,66
echo have led to the addition of more quantitative imaging Echocardiographic abnormalities may include
parameters to improve diagnostic sensitivity while main- decreased LV fractional shortening, end-diastolic posterior
taining the diagnostic specificity.58 wall thickness, and mass. Associated would be decreased
These include: LV contractility and increased LV afterload. Left ventricular
dimension may be increased, normal, or decreased
Major criterion:
(Figs 66.30A and B; Movie clip 66.30). Strain rate imaging
1. Regional RV akinesia/dyskinesia/aneurysm
is a new technique to evaluate myocardial mechanics
2. Parasternal long-axis RVOT (end-diastole) > 32 mm
and can detect early changes prior to a decrement in
or parasternal short-axis RVOT > 36 mm or fractional
the ejection fraction. This technique will enable early
area change < 33% detection of patients at risk of cardiomyopathy and would
Minor criterion: be useful in managing chemotherapy regimens.
1. Regional akinesia/dyskinesia Although adults typically develop chronic DCM
2. Parasternal long-axis RVOT > 29 to < 32 mm or para- after anthracycline chemotherapy, children at the end
sternal short-axis RVOT > 32 to < 36 mm or fractional of anthracycline treatment have a DCM, which may
area change > 33% to < 40% then progress to a RCM.66 Afterload increases, in spite of
Echocardiographic findings reported in asymptomatic normal-to-reduced blood pressure caused by decreased
individuals, who were family members of patients with LV wall thickness and mass. Therefore, the elevated LV
ARVC/D included—inferobasal localized diastolic bulge, afterload reduces the cardiac output to a greater extent
A B
Figs 66.30A and B: Adriamycin-induced cardiomyopathy. Two-dimensional transesophageal echocardiography. Parasternal long (A)
and short; (B) axis views. Left ventricle (LV) is markedly enlarged and severely hypokinetic. The right ventricle (RV) is also very hypoki-
netic. Arrow points to a catheter in the right heart. (LA: Left atrium; RA: Right atrium) (Movie clip 66.30).
Long-term heavy alcohol consumption, estimated to be

>90 g of alcohol a day for more than 5 years is associated
with a higher risk to develop ACM.
It often occurs in stages, beginning with a preclinical
or asymptomatic stage, and then progressing to a sympto-
matic stage. The early signs of ACM appear to be LV
dilation, with an increase in EDD and increased systolic
dimension. It also results in increased LV mass, and
modestly increased posterior and septal wall thickening.
Associated is an increase in isovolumic relaxation time and
deceleration time.68 Diastolic dysfunction thus, appears
to be an early finding. However, patients may have both
diastolic and/or systolic dysfunction with progression
Fig. 66.31: Dilated cardiomyopathy. Two-dimensional transtho-
racic echocardiography. Left ventricle (LV) short-axis view at the of the condition. Patients may also have some degree of
level of the papillary muscles shows decreased rotation of the wall thickening which when coupled with LV dilation will
ventricle in systole and unrotation in diastole. This is a repre- serve to offset wall tension. Therefore, many patients may
sentative frame from the movie clip demonstrating a dilated LV.
(RV: Right ventricle). (Movie clip 66.31). indeed remain in a compensated and asymptomatic state
even with LV dysfunction.
than the reduced LV systolic performance alone. While As the condition progresses, more classical features of
adriamycin is among the more commonly used agents with DCM are seen with severely impaired systolic function and
significant cardiotoxicity resulting in cardiomyopathy, progressive LV dilation (Fig. 66.31 and Movie clip 66.31).
all chemotherapeutic agents can potentially result in
varying degrees of cardiomyopathy too due to their toxicity RESTRICTIVE CARDIOMYOPATHY
profile.
The distinct morphological and hemodynamic characte-
ristics that separate restrictive from the more common
Alcohol-Induced Cardiomyopathy dilated and hypertrophic cardiomyopathies are:
Alcohol-induced cardiomyopathy (ACM) is among the 1. Nondilated ventricle (<40 mL/m2) with normal wall
leading causes of nonischemic DCM in the United States.67 thickness (<1–1.2 cm)
2. Rigid ventricular walls with severe LV diastolic Amyloid Cardiomyopathy

dysfunction with restrictive filling leading to elevated
filling pressures The annual incidence of systemic amyloidosis is approxi-
mately 6–10 cases per million of the general population.
3. Biatrial enlargement
Evidence suggests that the incidence of cardiac amyloi-
4. Normal systolic function in majority of patients
dosis is increasing as the mean life expectancy rises. This
5. Normal pericardium
may also be due, in part, to improvements in diagnostic
These features are identified with 2D echocardio-
modalities that have led to earlier identification of this
graphy as the initial diagnostic test using the Doppler
condition.71
inflow velocities and tissue Doppler techniques.
The development of a cardiomyopathy results from the
RCM is classified based on the etiology69 as follows:
deposition of amyloid within the myocardium. It presents
1. Noninfiltrative disorders—idiopathic RCM, familial
predominantly as a RCM characterized initially by diastolic
cardiomyopathy, HCM, scleroderma, pseudoxanthoma dysfunction, progressing to profound biventricular systolic
elasticum, and diabetic cardiomyopathy. Familial dysfunction and arrhythmias. The two most common forms
disease (unrelated to amyloidosis) may overlap with involve deposition of monoclonal light chain fragments
familial HCM and may be caused by some of the same in the myocardium (termed primary amyloidosis—AL),
gene mutations. or fragments of serum amyloid in secondary amyloidosis
2. Infiltrative disorders—amyloidosis, sarcoidosis, Gaucher (secondary amyloidosis—AA). Endomyocardial biopsy
disease, Hurler syndrome, and fatty infiltration shows interstitial prominence with massive amyloid
3. Storage diseases—hemochromatosis, Fabry disease deposits and varying size myocardial cells often containing
(FD), and glycogen storage disease vacuoles.
4. Endomyocardial diseases—for example, endomyo- In cardiac amyloidosis, echocardiography demon-
cardial fibrosis (EMF) and hypereosinophilic syndrome strates symmetric left ventricular wall thickening typically
5. Radiation and/or drug (e.g. anthracycline) toxicity can involving the interventricular septum, small ventricular
cause an RCM or DCM. chambers, thickening of the atrial septum, pericardial
effusion, and dilated atria.72,73 Right ventricular diastolic
Doppler Findings dysfunction may also be present in association with
increased right ventricular wall thickness.73 The typical
Evaluation by Doppler echocardiography is crucial to findings of biventricular wall thickness usually precede
the diagnosis of RCM. With advanced disease, often an multichamber dilation seen in the later stages. Intracardiac
elevated peak mitral inflow velocity, rapid early mitral thrombi may be present in up to 35% of patients with the
inflow deceleration and reduced early annular velocity AL type and up to 15% in other types74 of amyloidosis
on tissue Doppler imaging is noted. Pertinent values to be (Figs 66.32A to C; Movie clips 66.32A and B).
considered in the diagnosis include: With progression of amyloidosis, decreased systolic
1. The E/A ratio of mitral valve inflow is pathologically thickening of the ventricular septum and globally
elevated typically > 2.070 and the deceleration time is decreased LVEF ensues. The ventricular walls show a
shortened to < 160 milliseconds. granular and brightly reflective sparkling appearance,
2. Doppler tissue imaging of the mitral annulus also corresponding to amyloid particle deposition (Figs 66.33A
reveals abnormally low diastolic Doppler annular to H; Movie clip 66.33).
velocities typically < 10 cm/s. The cardiac valves typically have a thickened
3. The high mitral inflow velocities with low annular appearance and although their movement is usually
velocity also result in a particularly high E/E' ratio > 25. normal, valvular regurgitant flow is often detected.72
4. Since restrictive cardiomyopathies are usually global, Interestingly, it has been suggested that the characteristic
the RV may also be affected. Hypertrophy can be seen speckle pattern might not be as obvious when using
in the right ventricle due to pressure overload and also harmonics on later-generation echocardiography
abnormal tricuspid valve inflow velocities. These can machines.71 The use of echocardiography to differentiate
lead to abnormal hepatic vein flow as well. cardiac constriction from RCM, while not definitive,
The many different etiologies of RCM also show is certainly helpful. The measure of the transvalvular
characteristic patterns on echocardiography. diastolic velocities across the atrioventricular valves is
A B
Figs 66.32A to C: Amyloid. Two-dimensional transthoracic

echocardiography. Parasternal long-axis (A) and apical four-
chamber (B) views in a 86-year-old female patient with amyloidosis.
All chamber walls are echogenic and markedly hypokinetic due
to the infiltrative process. The ventricular walls appear thickened
because of amyloid deposition, not hypertrophy; (C) Tissue Doppler
examination shows diminished mitral E’- and A’-waves (arrow).
(Ao: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA:
Right atrium; RV: Right ventricle; TV: Tricuspid valve) (Movie clips
C 66.32A and B).
A B
Figs 66.33A and B
C D
E F
G H
Figs 66.33A to H: Amyloidosis. Two-dimensional transthoracic echocardiography. Both the ventricular septum (IVS) and left ventricle
(LV) posterior wall (PW) are echogenic and markedly thickened because of amyloid deposition, not hypertrophy. These are visualized
in parasternal long- (A systole, B diastole) and short- (C systole, D diastole) axis as well as apical four-chamber (E systole, F diastole)
views. LV function is diminished; (G) M-mode examination showing similar findings; (H) Tissue Doppler imaging of the medial mitral
annulus shows a low E' velocity of 3.8 cm/s (arrow) consistent with poor longitudinal shortening of LV. (Ao: Aorta; DA: Descending aorta;
MV: Mitral valve; RA: Right atrium; RV: Right ventricle; RVW: Right ventricular wall) (Movie clips 66.33A to C).
particularly useful. With constriction, there is a > 25% Males with specific gene mutations77 and heterozygous
variation in the mitral and tricuspid velocities during females with low enzyme activity78 can present with a
inspiration, while with amyloidosis only the tricuspid cardiac variant of the disease. It is characterized by progr-
velocity changes with little change in the mitral velocity. essive concentric LVH, often symmetric and occasionally
In addition, in amyloidosis, the change in velocities is asymmetric, which correlates with disease severity.79,80
concordant between both valves—that is, they increase The predominant finding on echocardiography is usually
and decrease at the same time, whereas in constriction the wall thickening without cavity dilatation, with excessive LV
opposite is the rule. mass (up to 240 g/m2) in some male patients.78
These findings are valuable especially in identifying While systolic function is usually preserved early
patients who can present in the advanced stages of the on, a pseudonormal filling pattern can be detected by
disease when systolic function is depressed. These signs of careful Doppler transmitral and pulmonary venous flow
a mixed picture of dilated and restrictive cardiomyopathy analysis, which suggests increased LV filling pressures. A
are more classical with advanced cardiac amyloidosis. prolongation of the deceleration time is often noted, and
Linhart et al. also demonstrated that up to 25% of patients
had an impaired LV relaxation pattern, indicating less
Fabry Disease
severe diastolic dysfunction.78,80
Fabry disease (FD) is a lysosomal storage disease, The basis of the cardiac hypertrophy in FD is different
caused by mutations in the gene encoding the enzyme from that seen in other infiltrative cardiomyopathies where
-galactosidase A, resulting in a deficit in enzyme activity. predominantly interstitial infiltration is encountered. The
It is X-linked and is characterized by the progressive deposits in FD are lysosomal and represent only a small
accumulation of glycosphingolipids. Multiple organs can part of the increase in LV mass,77 with true ventricular
be affected with the heart, kidneys, and the neurological hypertrophy as a result of neurohormonal influences.
system in particular being common targets.75 Valvular changes are seen and are likely related to the
Cardiac involvement is quite frequent and is one of the glycosphingolipid deposits and fibrosis of valvular tissue.
main causes of death in patients with advanced FD. In the In the case of the mitral valve, papillary muscle thickening
heart, glycosphingolipids’ deposition causes progressive can be found accompanying ventricular hypertrophy.
left ventricular hypertrophy (LVH) that can at times mimic These result in valvular regurgitation that is usually of mild
the morphological and clinical characteristics of HCM.76 to moderate grade. Among patients with asymmetrical
septal hypertrophy, mimicking hypertrophic obstructive
cardiomyopathy, the typical systolic anterior motion of
the anterior mitral leaflet, can contribute to mitral valve
dysfunction. The mitral valve appears to be affected in
relatively young subjects, whereas aortic abnormalities
appear later. With progressive LVH, aortic root dilatation
can be seen. This may be accompanied by the development
of aortic valvular regurgitation, which is seldom severe
and usually mild (Fig. 66.34; Movie clip 66.34).80
With progression of the disease, myocardial fibrosis
ensues and is accompanied by left ventricular systolic
dysfunction.81 Strain imaging is particularly useful in
estimating myocardial fibrosis. The extent of myocardial
fibrosis correlates with the loss in peak systolic strain.81
Therapeutic options include the use of enzyme
Fig. 66.34: Fabry disease. Two-dimensional transthoracic echo- replacement therapy (ERT) to prevent the extensive
cardiography. Arrow shows an echolucency within the myocardium deposition of glycosphingolipids. ERT is effective in
of the ventricular septum, which has been reported in patients with
this entity. This finding reflects glycosphingolipids compartmen-
reversing the microvascular changes in FD by catabolizing
talization in which the subendocardial layer is spared resulting in the lipid deposits and improving cardiac function in
an echolucent area. Note that left ventricle (LV) function is poor. patients with cardiac involvement.82
Arrowhead in the Movie clip 66.34 points to an intracardiac defibrilla- A recent study has also shown the benefit of emerging
tor lead. (LA: Left atrium; RA: Right atrium; RV: Right ventricle).
echocardiographic techniques in the early diagnosis of
cardiac involvement in FD.83 Through the use of speckle of the chordae tendineae can occur with progressive
tracking to evaluate myocardial longitudinal strain, the scarring, which also results in mitral and/or tricuspid
study found that the presence of at least one strain value valve regurgitation.
≥ −15% demonstrates subclinical myocardial dysfunction
in patients with preclinical FD without myocardial Findings on Transthoracic
hypertrophy. Therefore, this provides evidence of ventri- Echocardiography
cular dysfunction even with the absence of LVH and prior
to extensive deposition within the LV. Echocardiographic evaluation during the necrosis stage
Weidemann et al. have shown that among patients is usually normal. The hallmark echocardiographic
receiving ERT, the maximal benefit occurred in those finding is the persistent obliteration of the apex of the
without myocardial fibrosis.81 Given the emphasis for early left or right ventricle, or both, by laminar thrombus.91 The
diagnosis and institution of treatment, echocardiography common differential diagnosis of LV apical infiltration and
obliteration include LV apical thrombus and apical HCM,
arguably has an important role in the management of FD.
which can be differentiated by their echocardiographic
Especially with the use of emerging techniques to guide
characteristics. A LV apical thrombus is associated with
therapy, ERT may be able to prevent complications such as
underlying LV dysfunction and wall motion abnormality. In
LVH, irreversible myocardial fibrosis, lethal arrhythmias,
apical cardiomyopathy, the LV apex is visualized in diastole
and coronary heart disease.
with obliteration in systole, thus producing the peculiar
“ace of spades” configuration. HES, however, shows
Hypereosinophilic Cardiomyopathy persistent apical obliteration, mitral valve involvement,
The WHO published a recent update that outlined the and progressive features of a RCM. Resolution of the
apical infiltration may sometimes be observed if treated
diagnosis of hypereosinophilic syndrome (HES).84 These
appropriately by medical and surgical intervention.
conditions are characterized by:
Tricuspid and pulmonary valvular thickening may
1. Persistent marked eosinophilia (>1,500 eosinophils/
accompany right ventricular involvement with thrombus
mm3);
formation in the RV apex. Doppler evaluation of the valves
2. The absence of a primary cause of eosinophilia (such enables the estimation of valvular regurgitation and the
as parasitic or allergic disease); and determination of restrictive physiology (Figs 66.35A to H;
3. Evidence of eosinophil-mediated end organ damage. Movie clips 66.35A to E).
Cardiac involvement unrelated to hypertension,
atherosclerosis, or rheumatic disease is identified in 20% Contrast Echocardiography
of patients (only 6% at the time of initial presentation).85
Typical cardiac findings include endocardial fibrosis and Contrast echocardiography may be invaluable in the
mural thrombus, which is most frequent in the apices of diagnosis of hypereosinophilic cardiomyopathy enabling
both ventricles and is also characterized by progressive it to be differentiated from a thrombus or apical HCM.
heart failure. Specifically since it delineates the shape of LV, it
The pathophysiology involves eosinophil infiltration distinguishes between hypertrophy and complete persis-
of cardiac tissue and release of toxic mediators resulting tent obliteration in these conditions.92
in endocardial damage and formation of platelet thrombi.
These mural thrombi pose a high risk for embolization.
Later with disease progression, there is fibrous thickening When the transthoracic echo images are poor or limited,
of the endocardial lining leading to a RCM.86,87 transesophageal echocardiography is useful. The deep
Hence, three stages of pathophysiology—necrosis, transgastric views enable visualization of the LV apex and
thrombosis, and fibrosis are usually identified. Endomy- can demonstrate the obliteration of the LV cavity thus
ocardial fibrosis (EMF), which was initially described in confirming the diagnosis.
1936 by Loeffler, is the most characteristic cardiovascular
abnormality.88 Valvular insufficiency can result from mural Endomyocardial Fibrosis
endocardial thrombosis and fibrosis involving leaflets of In the third stage of HES, significant EMF ensues, resulting
the mitral or tricuspid valves.89,90 In addition, entrapment in a RCM. It is considered a particularly devastating
A B
C D
E F
Figs 66.35A to F
G H
Figs 66.35A to H: Loeffler endocarditis. Two-dimensional transthoracic echocardiography. Baseline. (A and B) Modified four-chamber
view. (A) Arrowhead shows marked thickening of the tricuspid valve (TV). The arrow points to a large mass in the right ventricular
(RV) apex. A small pericardial effusion (PE) is also noted; (B) Color Doppler examination. Arrowhead shows moderate to severe TV
regurgitation; (C and D) Aortic (AO) short-axis view; (C) Shows marked thickening of the pulmonary valve (PV); (D) Color Doppler-
guided continuous wave Doppler interrogation shows a peak gradient of 48.50 mm Hg consistent with moderate PV stenosis (arrow).
After therapy; (E and F) Modified four-chamber view. Show complete normalization of TV and RV apex. Arrowhead points to moderator
band. PE is absent; (G and H) Aortic short-axis view; (G) Thickening involving the PV has completely regressed and the valve appears
structurally normal (arrowhead); (H) Color Doppler-guided continuous wave Doppler examination shows normal flow velocities across
the PV (arrow). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium) (Movie clips 66.35A to E).
Source: Reproduced with permission from Garg A, Nanda NC, Sungur A, et al. Transthoracic echocardiographic detection of pulmonary
valve involvement in Loeffler’s endocarditis. Echocardiography (2013, in press).
disease in the tropical region with an estimated 10 million

people affected by it.93 The association between this condi-
tion and parasitic infestation has been established by
multiple studies such as those of Rashwan et al. and
Mocumbi et al. 94,95
Echocardiography is particularly useful in identifying
the condition. It is characterized by obliteration of the
ventricular apices with progression of the fibrocalcific
process, spontaneous echo contrast in the ventricles
without significant systolic dysfunction, and significant
atrioventricular valve dysfunction due to adhesion of
the valve apparatus to the ventricular wall. These are
considered the major criteria used to assess the severity of
EMF. The minor criteria include severely dilated atria with
Fig. 66.36: Endomyocardial fibrosis in a 62-year-old female with normal ventricular size, restrictive flow pattern across the
a previous history of mansoni schistosomiasis. Two-dimensional mitral or tricuspid valve, and thickening of the anterior
transthoracic echocardiography. Apical four-chamber view mitral leaflet.95 As the condition progresses, more and
shows endocardial thickening and cavity obliteration of both
more of the left ventricular cavity is obliterated, leading
ventricular apices with greater involvement of the left ventricle
(LV). The atria are enlarged, which is typical of restrictive to a progressively restrictive physiology.96,97 Evidence
cardiomyopathy. The patient improved after heart failure treatment. of the severest form carries a very poor prognosis,
Source: Reproduced with permission from Carneiro Rde C et al. with an estimated survival of 2 years after diagnosis
Endomyocardial fibrosis associated with mansoni schistosomiasis. with progressive heart failure being the predominant
Rev Soc Bras Med Trop. 2011;44(5):644–5.
presentation (Fig. 66.36).
associated with scarring. Specifically, the thinning of the

basal anterior septum, while relatively uncommon, is very
characteristic of cardiac sarcoidosis.99 Interestingly, the
finding of anterior septal thinning does not correlate with
conduction system abnormalities, namely varying degrees
of AV block, which are commonly seen in this condition
(Fig. 66.37; Movie clip 66.37).99
In the absence of LV thinning, ventricular wall
thickness is usually preserved. In fact, Matsumori et al.
have described a presentation similar to HCM in some
patients with sarcoidosis.100 This is, however, a relatively
rare finding.
A nonspecific, but commonly found, feature on
echocardiography is diastolic dysfunction. This may
Fig. 66.37: Sarcoidosis. Two-dimensional transthoracic echo- be found early on with initial interstitial inflammation,
cardiography. Parasternal long-axis view. Both the ventricular when systolic function may still be normal.101 Valvular
septum (VS) and posterior wall (PW) are echogenic, consistent involvement is rare and might be seen as sequelae of DCM
with myocardial fibrosis. (Ao: Aorta; LA: Left atrium; MV: Mitral
when present.
valve; RV: Right ventricle) (Movie clip 66.37).
Hemochromatosis
OTHER INFILTRATIVE Hemochromatosis represents an iron overload disorder
CARDIOMYOPATHIES characterized by the accumulation of iron within various
cells, including cardiac myocytes. Cardiac manifestations
Sarcoidosis of hemochromatosis are characterized by systolic
Sarcoidosis is a systemic disease characterized by the dysfunction, and cardiac MRI can detect and quantify
formation of noncaseating granulomas that can infiltrate myocardial iron infiltration using T2 weighted imaging.
the myocardium. Cardiac involvement only occurs in Liver biopsy is the definitive test for iron overload.102 Serum
approximately 5% of patients with systemic sarcoidosis. transferrin saturation is typically > 45% and elevated serum
Myocardial granulomas with central areas displaying ferritin levels are seen, which help confirm the diagnosis
low signal intensity characteristic of fibrosis and a high of hemochromatosis.
peripheral signal intensity corresponding to edema are Although cardiac MRI is a superior imaging modality
typically seen on cardiac MRI.98 for the diagnosis of cardiac hemochromatosis, TTE is
Patients with cardiac sarcoidosis may manifest with a useful for following disease response to chelation therapy
variety of clinical scenarios varying from cardiomyopathy and/or phlebotomy. The echocardiographic features
and heart failure to conduction system abnormalities and of hemochromatosis include mild LV dilatation, LV
ventricular tachyarrhythmias. systolic dysfunction, normal wall thickness, and biatrial
While contemporary diagnosis often entails the use enlargement.103 The degree of iron deposition in the
of cardiac MRI, traditional echocardiographic findings myocardium correlates with the degree of LV dysfunction.
are useful in identifying cardiac sarcoidosis. The usual
echocardiographic appearance is that of a DCM. The INFECTIOUS AND METABOLIC
ventricle may be globally hypokinetic or the patchy nature CARDIOMYOPATHIES
of sarcoid infiltration of the heart may result in regional
wall motion abnormalities. With edema or infiltration, Infectious Cardiomyopathy
mild wall thickening may also be present. This is noted
on echocardiography by the presence of bright shadows Septic Cardiomyopathy
consistent with infiltration. Acute and reversible cardiac dysfunction commonly
Typically with progression, areas of wall thinning occurs in patients with septic shock. In the absence of
are seen, most commonly in the ventricular septum, other causes of cardiomyopathy, there are two main
A B
Figs 66.38A to C: Septicemic myocarditis. Two-dimensional tran-

sthoracic echocardiography. Parasternal long-axis (A) and apical four-
chamber (B) views. This patient with streptococcal pneumonia devel-
oped poor biventricular function consistent with myocarditis; (C) Apical
four-chamber view. Further deterioration of ventricular function was
noted on the next day and the patient succumbed in the next few days
from multiorgan failure. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
PW: Posterior wall; RA: Right atrium; RV: Right ventricle; VS: Ventricu-
C lar septum) (Movie clips 66.38A to C).
factors that contribute to cardiac depression in the setting Echocardiographic Features of Septic
of sepsis. Decreased RV function, presumably related
to both acute pulmonary hypertension from acute lung
Cardiomyopathy
injury and reduced RV contractility, act to decrease the
Unlike cardiogenic shock, which typically manifests
LV filling pressures, and hence decrease cardiac output.
as globally reduced LV function with pronounced LV
In addition, a direct depression in LV contractility due
to circulating cytokines also decreases cardiac output dilatation and a restrictive pattern of LV inflow (pulsed
in sepsis. Exposure of myocardial cells to inflammatory Doppler of the mitral valve showing high E-wave velocities
cytokines, mainly circulating tumor necrosis factor alpha and low A-wave velocities suggestive of high LV filling
(TNF-) and interleukin-1B (IL-1B), have a direct negative pressures), septic cardiomyopathy manifests as global LV
inotropic effect on cardiac myocytes mainly through dysfunction with minimal LV dilatation and mitral inflow
increases in intracellular cGMP and nitric oxide (NO). velocities suggesting near-normal LV filling pressures.
Mitochondrial dysfunction and decreased myofilament There is often pronounced yet reversible RV dysfunction
response to Ca2+ secondary to troponin I phosphorylation and RV dilatation (Figs 66.38A to C; Movie clips 66.38A
have also been implicated.104 to C).104
Septic Cardiomyopathy Cardiogenic Shock of complex carbohydrate metabolism, storage disorders,

LV function Globally reduced Often globally
neuromuscular diseases, organic acidemias, and other
reduced diseases such as congenital disorders of glycosylation
LV dilatation Mild Severe (CDG) and disorders of metal and pigment metabolism
Mitral inflow Mostly normal Restrictive pattern— (Wilson disease, hemochromatosis, Dubin–Johnson
velocities high E-wave veloci- syndrome).109 Children and young adults who develop
ties, low A-wave cardiomyopathy should be evaluated for underlying
velocities inherited metabolic disorders if no obvious other sources
RV Often decreased Less common of cardiomyopathy are found.
involvement RV systolic function
and RV dilatation Echocardiographic Features of Metabolic
Reversibility Yes (days) Depends on etiology
Cardiomyopathies
The degree of global dysfunction in septic cardio- Cardiomyopathies in pediatric patients with inborn
myopathy may be masked by severe peripheral vaso- errors in metabolism can manifest as hypertrophic or
dilatation causing significantly reduced afterload, thereby dilated ventricles with globally reduced systolic function.
appearing as if cardiac output is not significantly reduced. Restrictive patterns are less common in this patient
However, once patients receive restoration of normal population. However, patients with cardiomyopathies due
afterload with fluid resuscitation and/or vasopressor to disorders of metal metabolism often have a restrictive
support, the degree of sepsis-induced LV dysfunction pattern. The typical features of RCM, namely biatrial
is often unmasked. Once the diagnosis of septic enlargement, severe diastolic dysfunction of the LV, and
consequent right ventricular hypertrophy have been
cardiomyopathy is made, serial bedside transthoracic
discussed previously.
echocardiograms may be obtained to assess the degree of
LV and RV dysfunction in response to treatment.
Diabetic Cardiomyopathy
Diabetic cardiomyopathy is diagnosed when ventricular
HIV-Associated Cardiomyopathy dysfunction develops in diabetic patients, in the absence
Cardiac abnormalities can be found in up to 44% of of coronary atherosclerosis and hypertension.110,111
patients with HIV.105 HIV is associated with LV systolic There has been a reported increased risk of heart
dysfunction in addition to DCM, although additional failure in diabetic patients after matching them for age,
cardiac abnormalities may also be present. Specifically, blood pressure, weight, cholesterol, and CAD.112 There
pericardial effusion, pulmonary arterial hypertension, has also been a significant association between diabetes
infective endocarditis, and intracardiac masses due to and diastolic dysfunction leading to CHF with preserved
lymphoma and Kaposi sarcoma have all been described in systolic function.113
HIV patients. DCM and LV dysfunction are often associated
with myocarditis with various viral (including HIV), fungal,
and atypical mycobacterial organisms. Toxoplasma gondii
Two-Dimensional Echo
has also been implicated.106-108 In addition, ischemic heart Echocardiography can be used to ascertain the systolic
disease and the development of LV diastolic abnormalities and diastolic function. Diastolic dysfunction is depicted
are associated with highly active antiretroviral therapy by reduced early peak mitral inflow velocities at annular
(HAART).106 septal and lateral levels in early diastole, as noted on
Doppler echo.
Metabolic Cardiomyopathy Increased myocardial reflectivity in patients with
diabetes-related heart disease has also been reported
Metabolic cardiomyopathies encompass a wide range
(Figs 39A to C).114
of inherited metabolic disorders and a wide spectrum
of other pathological conditions. Inherited metabolic
disorders often present in childhood and include defects
CARCINOID HEART DISEASE
in mitochondrial long-chain fatty acid oxidation, carnitine The carcinoid syndrome is a constellation of signs and
deficiency disorders, respiratory chain defects, disorders symptoms that are seen with neuroendocrine tumors,
A B
Figs 66.39A to C: Diabetic cardiomyopathy. Doppler mitral inflow

and Doppler tissue imaging in a 79-year-old female patient. (A)
Peak early mitral inflow diastolic velocity (E) measured 86.2 cm/s
in this patient; (B and C) Peak mitral annular septal (Es) and
lateral (El) velocities in early diastole measured 4.07 cm/s (B) and
4.47 cm/s (C), respectively. The ratio of E to Es and E to El
calculates to be 21.17 (normal < 15) and 19.28 (normal < 12),
respectively. The ratio of E to average of Es and El is 20.18 (normal
< 13). These findings are indicative of left ventricle (LV) diastolic
C dysfunction with increased left-sided filling pressures.
arising from the enterochromaffin cells most often in the sided valvular cusps and leaflets as well as the RA and
gastrointestinal tract. These tumors are malignant with RV.121 The involvement of the tricuspid valve typically
an incidence of around 1.2–2.1 per 100,000 of the general results in hemodynamically significant regurgitation and,
population.115 They are characterized by the production less frequently, in valvular stenosis. The pulmonary valve,
of vasoactive substances such as serotonin, 5-hydroxy- which is also commonly affected, develops a combination
tryptamine, bradykinin, tachykinin, and prostaglandins, of stenosis and regurgitation. Hemodynamically relevant
which result in the typical clinical features of flushing, pulmonic valve stenosis is more frequently noted than
diarrhea, bronchospasm, and hypotension.115,116
tricuspid stenosis. This is because, the orifice of the
Carcinoid heart disease is seen in > 50% of patients with
pulmonic valve is much smaller and consequently,
carcinoid syndrome.117 In approximately 20% of patients,
plaque deposition on the pulmonary valve and within the
carcinoid heart disease is the primary presentation of
metastatic carcinoid disease. Historically, the association pulmonic annulus and sinuses results in narrowing of the
between carcinoid tumors and specific cardiac disease pulmonic root. Left-sided involvement occurs in < 10%
was described in the 1950s.118 The echocardiographic and is characterized by valve thickening and regurgitation
features of carcinoid heart disease were well described in without concurrent stenosis.122 Hepatic metastases are
the 1980s and used reliably in its diagnosis.119,120 often associated with carcinoid heart disease. Tumor
Cardiac involvement is predominantly right-sided as growth within the liver allows large quantities of humoral
the lungs filter the tumor products before they reach the tumor products to reach the RV without being inactivated
left atrium. It is characterized by plaque-like deposits of by the first-pass metabolism in the liver, which is thought
fibrous tissue, typically on the endocardium of the right- to be responsible for cardiac plaque formation.123,124
If carcinoid valvular heart disease involves the Chronic tricuspid and pulmonic valve regurgitation
mitral or aortic valve, a right-to-left shunt or a primary results in progressive volume overload of the right-sided
bronchial carcinoid is frequently found. Alternatively, chambers. The hemodynamic situation can be further
left-sided valve disease may occur in the absence of these compromised by pressure overload due to pulmonic
conditions if the carcinoid syndrome is severe and poorly stenosis. As a consequence, the RA and ventricle become
controlled.125 A recent multimodality imaging study of increasingly dilated. Furthermore, hypokinesis of the RV
52 patients with carcinoid heart disease showed the may be apparent in advanced stages of the disease. Conse-
presence of a patent foramen ovale in 13 of 15 (87%) quently, patients present with right ventricular failure and
patients with left-sided carcinoid involvement further signs of volume overload characterized by pedal edema,
giving credence to the theory of right-to-left shunts being pulsatile liver, and effort intolerance. While isolated left
responsible for left heart involvement.126 ventricular dysfunction is not seen per se, the presence
of right ventricular failure results in a low systemic output
Echocardiographic Features state with diminution of the cardiac output.
Left-sided valvular involvement is infrequent. It is
On 2D echocardiography, the tricuspid valve leaflets are characterized by diffuse valve thickening and retraction
typically thickened and shortened. The leaflets become with reduced mobility and regurgitation, but without
increasingly retracted with progression of the disease, significant stenosis (Figs 66.40A to C, Movies 66.40A to C).
resulting in reduced mobility. The septal and the anterior Dumaswala et al have elegantly illustrated the
leaflets are usually predominantly affected, whereas the incremental value of 3D echocardiography in carcinoid
posterior leaflet may exhibit relatively preserved mobility. heart disease.128 Three-dimensional echocardiography
Severe tricuspid regurgitation results in advanced stages can allow for better assessment of (a) valvular structure,
of tricuspid valve disease as the leaflets become fixed (b) severity of valvular disease, (c) extent of cardiac
in a semi-open position. There is also some degree of involvement, and (d) metastatic lesions. It is particularly
concomitant stenosis due to a fixed orifice. Color flow useful as it permits evaluation of all three leaflets of the
Doppler assessment of the hepatic veins may show tricuspid and pulmonary valves. This is not so with 2D
systolic flow reversal, consistent with severe tricuspid echocardiography wherein only the anterior and septal
regurgitation. On continuous wave Doppler tracings, leaflets of the tricuspid valve and the anterior (left anterior)
severe tricuspid regurgitation is characterized by a dagger- and left (posterior) leaflets of the pulmonary valve can be
shaped profile with an early peak velocity and a rapid visualized. Estimation of the effective regurgitant orifice
decline, indicating rapid pressure equalization between area is made using the proximal isovelocity surface area
the right-sided cardiac chambers. The peak regurgitant (PISA) method by 2D transthoracic echocardiography
velocity may be increased due to coexistent pulmonic (2D TTE), which depends on the assumption that flow
convergence is hemispheric, although this does not
stenosis. The presence of a prolonged pressure half-time
hold true at most times.129 The vena contracta method,
of the tricuspid inflow indicates associated tricuspid valve
which is also used to find the area of regurgitation by
stenosis. Increases in tricuspid inflow velocities and the
2D TTE, depends on the assumption that the area of
mean gradient across the tricuspid valve result from a
regurgitation is circular or elliptical, which is also not
combination of valvular stenosis and increased blood flow
the case in most circumstances. The measurement of the
through the valve owing to the regurgitant volume.127
vena contracta is also more accurate with 3D imaging
The pulmonary valve cusps appear thickened with since it permits visualization of the entire regurgitant jet.
retraction and reduced mobility. The cusps may be difficult Thus, the quantification of valvular regurgitation,
to visualize if they are severely retracted. Constriction of particularly tricuspid and pulmonary, which are more
the pulmonary annulus as a result of plaque deposition common in carcinoid heart disease, is more reliable with
may also be observed. Doppler echocardiographic 3D echocardiography.
assessment of the pulmonary valve is particularly helpful Further, the visualization of endocardial deposits is
because demonstration of the anatomical changes may be superior with 3D echocardiography as is the evaluation
challenging. Increased systolic velocities on continuous of hepatic metastasis in the subcostal views. These help
wave Doppler examination are consistent with pulmonary improve the diagnostic accuracy while also identifying
stenosis, whereas a dense regurgitant spectral profile with the anatomical extent of endocardial deposits (Figs 66.41A
a short deceleration time is typical for severe regurgitation. to K; Movie clips 66.41A to F).
The pulmonary valve appeared thickened, retracted, Enlargement of the RA and the RV was seen in
and immobile in 36 patients (49%) and could not be 67 (91%) and 65 (88%) patients, respectively. Four patients
visualized in an additional 29 patients (39%). Pulmonic (5%) exhibited impaired right ventricular systolic function.
stenosis was identified in 25 patients (53%) by Doppler Left-sided valve lesions were only found in five patients
echocardiography. (7%). Presence of myocardial carcinoid metastases was
A B
Figs 66.40A to C: Carcinoid disease. Two-dimensional trans-

thoracic echocardiography. (A) Apical four-chamber view shows
systolic noncoaptation of thickened anterior (1) and septal
(2) leaflets of tricuspid valve (TV); (B) Color Doppler examination
shows severe tricuspid regurgitation (arrowhead) resulting from
systolic noncoaptation of TV leaflets. Tricuspid regurgitation (TR)
jet practically fills the right atrium (RA); (C) Aortic (Ao) short-axis
view shows severe pulmonary regurgitation (PR) with the PR jet
extending all the way to the TV level. (LA: Left atrium; LV: Left
ventricle; PA: Pulmonary artery; RV: Right ventricle) (Movie clips
66.40A to C).
Source: Reproduced with permission from Ref. 128. 66.40A
C to C).
A B
Figs 66.41A and B
C D
E F
G H
Figs 66.41C to H
I J
K
Figs 66.41A to K: Carcinoid disease. Live/real time three-dimensional transthoracic echocardiography. (A) 1, 2, and 3 represent
thickened anterior, septal, and inferior (posterior) leaflets of the tricuspid valve (TV), respectively, showing a very large area of
noncoaptation in systole (Movie clip 66.41A, part 1). Movie clip 66.41A part 2 shows QLAB images. Movie clip 66.41A part 3 shows color
Doppler assessment of TV regurgitation (TR). The vena contracta (VC; arrowhead) is very large, measuring 2.51 cm2, consistent with
torrential TR; (B) 1, 2, and 3 represent thickened anterior (left anterior), left (posterior), and right (right anterior) leaflets of the pulmonary
valve (PV), respectively, (Movie clip 66.41B part 1). In Movie clip 66.41B part 2 the arrowhead points to noncoaptation of the PV leaflets
in diastole. Movie clip 66.41B part 3 shows color Doppler assessment of pulmonary regurgitation (PR). The VC (arrowhead) is large,
measuring 0.7 cm2, consistent with severe PR; (C to E) Arrowhead shows a prominent, localized echogenic carcinoid deposit involving
the interatrial septum (IAS); (F) The arrowhead demonstrates a carcinoid deposit lining the wall of the inferior vena cava (IVC); (G and H)
QLAB. Upper arrowheads point to the carcinoid deposit involving the right atrium (RA) superior wall. Lower arrowhead views the same
plaque en-face. It measured 2.02 × 0.85 cm, area = 1.36 cm2; (I to K) Subcostal examination; (I) A large carcinoid metastasis, bounded
by dots, is noted in the liver (L). QLAB sections taken at two different levels show the extent of hemorrhage/necrosis (arrowheads) in
the lesion; (J) Another liver metastasis (M, dots) is seen encroaching the RA and IVC; (K) Examination of another patient with known
simple L cysts. Arrowheads point to some of the cysts, which are generally completely echolucent with thin, well-defined borders. These
features distinguish them from a carcinoid lesion, where the echolucencies do not involve the whole cyst and the borders are less well
defined and are thickened. (Ao: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; PA: Pulmonary artery; RV: Right ventricle)
(Movie clips 66.41A parts 1 to 3, 66.41B parts 1 to 3, 66.41C to F, 66.41I parts 1 and 2, 66.41J and 66.41K).
Pellikka et al. have described the spectrum of the regurgitation was the predominant finding present in
echocardiographic changes and the frequency of their 72 patients (97%) by 2D criteria and all 69 patients who
detection in an elegant series of 74 patients with carcinoid underwent Doppler examination. In 62 patients (90%),
heart disease treated at the Mayo Clinic.122 Tricuspid the degree of regurgitation was moderate or severe.
demonstrated in three patients (4%) and small pericardial 7. Dini FL, Cortigiani L, Baldini U, et al. Prognostic value of
effusions without hemodynamic significance were seen in left atrial enlargement in patients with idiopathic dilated
cardiomyopathy and ischemic cardiomyopathy. Am J
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CHAPTER 67
Echocardiographic Differentiation
of Ischemic and Nonischemic
Cardiomyopathy: Comparison with
Other Noninvasive Modalities
Sula Mazimba, Arshad Kamel, Navin C Nanda, Maximiliano German Amado Escanuela, Kunal Bhagatwala, Nidhi M Karia
Snapshot
Echocardiographic Assessment of Ischemic and
¾¾ Echocardiographic Distinction between Ischemic
¾¾
Nonischemic Cardiomyopathy Cardiomyopathy and Nonischemic Dilated
M-Mode Echocardiography
¾¾ Cardiomyopathy
Two-Dimensional/Three-Dimensional/Doppler
¾¾ Other Noninvasive Imaging Modalities
¾¾
Echocardiography
INTRODUCTION the identification of significant coronary artery disease

(CAD) as the primary mechanism for the LV dysfunction
The left ventricle (LV) may be enlarged and show (< 45%). Definition of ICM requires the identification of
dysfunction in both ischemic heart disease and dilated significant CAD in the presence of depressed LV ejection
cardiomyopathy. It is therefore important to distinguish fraction (EF). Significant CAD for the ICM has been
ischemic cardiomyopathy (ICM) from nonischemic dilated proposed as stenosis of any of the epicardial vessels
cardiomyopathy (NICM) as management may be different. > 75%, or a history of myocardial infarction or previous
The distinction between the two types of conditions may revascularization of the coronaries.5 Coronary angiography
have therapeutic and prognostic implications. Patients remains the gold standard method of evaluating CAD.5–9
with ICM may benefit from a revascularization treatment Nonetheless, coronary angiography is not without risks.
strategy.1–3 Furthermore, patients with ICM have worse It is invasive, operator-dependent, and may be associated
prognosis than NICM patients.4 In clinical practice, with adverse events.9,10 A noninvasive imaging modality,
distinguishing between the two types of conditions can particularly in patients with low to intermediate pretest
be very challenging. In some situations, a diagnosis probability for CAD is often recommended. Coronary
can be inferred from history and physical examination angiography is reserved for patients with a high pretest
(e.g. postpartum or chemotherapy-induced NICM). In probability for CAD, who may also benefit from revascul
general, distinction of the two cardiomyopathies lies in arization.9
Chapter 67: Echocardiographic Differentiation of Ischemic and Nonischemic Cardiomyopathy 1419
Fig. 67.1: Parasternal long-axis view showing a severely Fig. 67.2: Nonischemic cardiomyopathy. M-mode echocardi-
dilated left ventricular cavity. There is malcoaptation of the ante- ography shows a large mitral–septal separation, E-point septal
rior and posterior mitral valve leaflets. (AO: Ascending aorta; DA: separation (EPSS) of 45 mm is noted on the M-mode. (AO: Aorta;
Descending aorta; LA: Left atrium; LV: Left ventricle; RA: Right LA: Left atrium; LV: Left ventricle).
atrium; RV: Right ventricle).
ECHOCARDIOGRAPHIC ASSESSMENT LV systolic function is strongly related to functional status

and prognosis in patients with cardio myopathy.22 The
OF ISCHEMIC AND NONISCHEMIC predictive impact of LVEF on mortality is much more
CARDIOMYOPATHY pronounced in ICM than NICM.23
Doppler measures of contractility are reduced in both
Two-dimensional (2D) echocardiography is a low-cost and
ICM and NICM. The left ventricular outflow tract flow
widely available diagnostic tool used in the initial evaluation
velocity or velocity time integral is usually decreased to
of patients with left ventricular systolic dysfunction.11 Left
<18 cm. Another measure of ventricular contractility is
ventricular dilatation has been implicated in the initial
the dP/dt (i.e. the change in left ventricular pressure over
development and progression of systolic dysfunction with
time). This is measured from the mitral regurgitant jet.
or without CAD (Figs 67.1 to 67.3. Also Figs 66.1C and 66.2B
A value of <600 indicates significant impairment of the
in Chapter 66)12–14 it is thought that ventricular dilatation
LV contractility. A low dP/dt is associated with adverse
is the initial adaptive response of a failing heart.15,16 As
cardiovascular outcomes and mortality.24,25 The myo
the LV chamber further enlarges, a remodeling process cardial performance index (MPI), or the Tei index is
that is regulated by mechanical, neurohormonal, and another Doppler-derived measure of systolic and diastolic
genetic factors ensue.12,17–19 The end result is a change in ventricular function.26 A calculated MPI value of > 0.60 is
the geometric configuration of the heart muscle from an correlated with adverse outcomes in both patients with
ellipsoid to a spherical shape.20 A spherically shaped heart nonischemic and ischemic cardiomyopathies.27–29
has ineffectual muscle contractions, larger ventricular
volumes, and conformational changes in the mitral
annular apparatus. NICM on echo is characterized by an
M-MODE ECHOCARDIOGRAPHY
increase in ventricular chamber sizes with reduced indices The sphericity index (SI) is a surrogate marker of LV
of systolic function (left ventricular ejection fraction remodeling. It is the ratio of left ventricular long-axis
[LVEF] < 45%, or fractional shortening < 25% and reduced internal dimension to the LV diameter at end-systole.30,31
fractional area change). The enlarged LV chambers occur A SI of <1.5 underscores a very severely dilated LV cavity.
in the setting of normal or reduced LV thickness.21 Another M-mode parameter, which is related to LV cavity
Systolic function is often reduced in dilated cardio dilatation and reduced LV systolic excursion, is the E-point
myopathy (Fig. 67.1). The degree of reduction of the LVEF septal separation (EPSS). This is measured as the distance
is well correlated to the severity of the cardiomyopathy.4 from the tip of the open anterior mitral leaflet in diastole
A B
C D
E F
Figs 67.3A to F
Figs 67.3A to G: Ischemic cardiomyopathy in a 38-year-old female

patient. M-mode/two-dimensional transthoracic echocardiography.
(A and B) Parasternal long-axis views demonstrating a huge left
ventricle (LV) measuring 90 mm and severe mitral regurgitation
(arrow); (C and D) M-mode examination showing a very large
LV with severely reduced function. Also note markedly increased
E-point septal separation (EPSS) measuring 32 mm in D; (E to G)
Apical views; (E) The coaptation point of the mitral leaflets is dis-
placed into the LV and directed eccentrically toward the LV free wall,
suggesting ischemic origin of cardiomyopathy; (F) Shows dyski-
nesis (arrow) of the distal LV inferior wall and septum; (G) Severe
mitral regurgitation (arrow) resulting from reduced coaptation of the
displaced mitral leaflets. Movie clip H shows hypokinesis (arrow) of
the distal right ventricular (RV) free wall. (AO: Aorta; LA: Left atrium;
G RA: Right atrium) (Movie clips 67.3A to 67.3H).
to the ventricular septal wall. A normal value is < 6 mm.32 (DCM). Even though linearly derived measurements are
EPSS of >10 mm reflects severe LV dilatation and systolic used to calculate chamber sizes, these measurements
dysfunction (Fig. 67.2).32–34 The utility of EPSS is limited in are based on geometric assumptions of the LV cavity in
aortic regurgitation and mitral stenosis. The aortic valve the form of a truncated ellipsoid.21 These measurements,
may demonstrate reduced leaflet excursions coupled however, may grossly underestimate the true LV sizes
with early closure. Both these features are a reflection and volumes in CAD patients with distorted LV cavities.
of a diminished cardiac output. Another feature on This is because of the regional wall motion abnormalities
M-mode that signifies low cardiac output is the reduced (WMAs) often present in CAD (presence of aneurysms,
wall motion of the aortic root. One of the advantages WMAs, asymmetrical and foreshortened ventricles).
Therefore, volumetric measurements for accurate cardiac
of M-Mode echo is its high temporal resolution (1000–
chamber size quantification are preferred to linear
3000 Hz compared to 20–129 Hz with 2D echo). Two-
measurements. More recently, three-dimensional (3D)
dimensional echocardiography, on the other hand, has
echo has been shown to have an advantage over 2D echo-
a higher spatial resolution compared to M-mode. Thus, derived measurements. Three-dimensional transthoracic
combining the two modalities can be helpful in guiding echocardiography (3D TTE) can overcome the limitation
accurate alignment of the M-mode beam for reliable of quantifying volumes in ICM patients with distorted
quantification of the chamber sizes.35 Severe NICM is LVs. Volumetric measurements by 3D echo yield more
suggested by left ventricular end-diastolic dimension accurate and reproducible measurements [comparable
> 56 mm in males and 53 mm in females. A dilated LV to radionuclide or cardiac magnetic resonance imaging
chamber in the setting of normal or reduced LV wall (CMR)].37–40 One of the challenges of obtaining volumetric
thickness (end-diastolic wall thickness of <6 mm) that is measurements in 3D echo arises when the cardiac
global in nature is highly indicative of NICM. In ICM, there chambers are foreshortened. This situation can lead
may be regional variation in thickness of the myocardium. to an underestimation of LV volumes.41 Automated
Myocardial thickness of <6 mm at end diastole is highly endocardial tracings have been recommended as one way
suggestive of nonviable myocardium.36 of overcoming this limitation.42 In the difficult situation
with poor endocardial visualization, the use of contrast
TWO-DIMENSIONAL/THREE- agents can enhance the accuracy and reproducibility of
measurements.43
DIMENSIONAL/DOPPLER
ECHOCARDIOGRAPHY Left Ventricular End-Systolic
Left Ventricular Volumes Volume Index
Correct quantification of the cardiac chambers is necessary End-systolic volume index (ESVI) provides important
in assessing the severity of the dilated cardiomyopathy information regarding the severity and prognosis of
NICM.44–46 ESVI increases progressively with the advan Left Atrium

cement of disease. It has been shown conclusively that
adverse outcomes increase with an ESVI of > 25 mL/m2 Left atrial (LA) size has been shown to have prognostic
(normal value, < 20 mL/m2).45 An ESVI > 60 mL/m2 is value in patients with NICM. LA volume is determined by
associated with increased adverse outcomes in patients the degree of LV dilatation, diastolic dysfunction as well
as the severity of mitral regurgitation (MR).64,65 LA size
with ICM undergoing surgical ventricular reconstruction.47
correlates well with the severity of diastolic dysfunction,
which in turn has prognostic information for patients
Two-dimensional Echo/Doppler-Derived with both ICM and NICM.66–72 In patients with NICM with
Left Ventricular Diastolic Dysfunction functional MR, LA size may reflect the duration of the MR.73
The LA size was a more powerful predictive variable than
Diastolic function assessment in NICM and ICM provides the severity of MR jet in determining adverse outcomes
useful information regarding the severity and overall in NICM patients.65 The current American Society of
prognosis. Patients with restrictive diastolic physiology Echocardiography guidelines recommends obtaining
on two-dimensional transthoracic echocardio graphy LA volumes rather than linear dimensions, which do
(2D TTE) have increased adverse outcomes. 48,49
A short not take into account the asymmetric remodeling of
deceleration E time is associated with severe symptoms the chamber.21,74,75 The upper limit of normal LA-ESVI is
and is also a powerful and independent prognostic 28 mL/m2.21
indicator of poor outcomes in patients with NICM.22,49–
51
Grade II diastolic dysfunction has also been shown Mitral Regurgitation: Mechanisms
to predict adverse outcomes and increased hospital
re-admissions in patients with NICM.52 Functional MR is a common feature in patients with
NICM or ICM. The presence of MR is associated with
poor prognosis even in patients with prior repair of the
Right Ventricle mitral valve (MV).76–79 Functional MR is characterized
Right ventricular (RV) dysfunction is a powerful predictor by malcoaptation of the two mitral leaflets despite a
for exercise capacity as well as mortality in patients with structurally normal MV (Figs 67.3B, 67.3G and 67.4). In
NICM.53–55 Assessment of RV function is often challenging an occasional patient with ICM, the “seagull” sign may be
because of the complexity of the 3D structure that is visualized as explained in Figure 67.4.80 The mechanism
less amenable to geometric assumptions like the LV. In related to the malcoaptation of the leaflets is thought
addition, the RV is in a substernal position, which renders to be due to the remodeling of the LV cavity.81–83 Factors
imaging more challenging. The numerous trabeculations thought to contribute to MR include dilatation of the
also renders volumetric assessment of the RV function mitral annulus, tethering of the leaflets (due to apical or
challenging.56,57 3D TTE, however, compares favorably posterior displacement of the papillary muscles), and
with CMR in the assessment of RV function.58 A 2D TTE reduced LV function (geometric changes occurring in a
parameter that has been validated in the assessment of RV remodeled ventricle).84–94 The degree of conformational
function is the tricuspid annular plane systolic excursion changes that occur in the MV apparatus in functional MR
(TAPSE).59,60 Right ventricular function is measured by the can be quantitatively assessed. These geometric variables
for MV deformation are discussed in the following section.
degree of excursion of the tricuspid annulus from the base
to the apex. A TAPSE of <15 mm is associated with poor
RV systolic function. Decreased RV systolic function as Tenting Area
estimated by TAPSE is associated with increased mortality This is the area enclosed by the annular plane and the two
in patients admitted for heart failure. Among patients with mitral leaflets (Fig. 67.5). It has been shown to accurately
LV systolic dysfunction, the involvement of RV dilatation is reflect the degree of functional mitral regurgitation
often suggestive of a NICM. The RV is often spared in ICM (FMR). It is also an independent predictor of mortality
unless there is accompanying right ventricular infarction and hospitalization in patients with NICM.95 Patients
in which case the right ventricle will be affected. RV with a tenting area > 3.4 cm2 had higher brain naturetic
involvement portends worse adverse outcomes in both peptide (BNP) levels, worse functional status, more
NICM and ICM.55,61–63 hospitalizations, and higher death rates.
Fig. 67.5: The shaded region illustrates the tenting area of the
mitral valve. (H: Tenting height; LA: Left atrium; LV: Left ventricle;
MV: Mitral valve).
Fig. 67.4: Transesophageal apical four-chamber view showing annular plane. On the other hand, tethering at the distal AL
severe mitral regurgitation by color Doppler flow imaging
(arrow). Arrowhead shows a kink in the middle of the anterior leaflet
tip (ALa tip) is calculated by measuring the angle between
mimicking a “seagull.” This “seagull” sign results from tethering the annular plane and a line that joins the anterior annulus
produced by a strut chord and is considered indicative of ischem- and the coaptation point of the two leaflets.94,97 Lee et al.
ic origin of cardiomyopathy. (LA: Left atrium; LV: Left ventricle; have proposed a classification of AL tethering into three
RV: Right ventricle) (Movie clip 67.4). subtypes based on the morphology and site of maximal AL
tenting.97
Type I AL: Tethering involves minimally tethered AL
along its long-axis dimension.
Type II AL: Tethering is characterized by posteriorly
directed tethering by the basal chordae. The
morphology of AL has a very prominent bend
on 2D echo imaging.
Type III AL: Consists of a pronounced apical tethering of
both the base and the distal tip of the AL. It
is often recognized on echo by a large AL tip
angle.
Fig. 67.6: A section of mitral valve from apical four-chamber view. It has been proposed that this classification should
1 = anterior leaflet angle (ALα) which measured 21°. 2 = posterior help guide treatment decisions in patients with functional
leaflet angle (PLα) which measured 62° (LA: Left atrium; LV: Left MR. For instance, mitral annuloplasty is more likely to be
ventricle). successful with type I and II subtypes.97
Coaptation depth, otherwise known as MV tenting Both the PLa and ALa have been shown to offer
height, is the shortest distance between the leaflet coap diagnostic and prognostic information in patients with
tation point and the mitral annular plane (Fig. 67.5).96 ischemic MR.98–101 One of the distinguishing features of
The degree of leaflet tethering is measured by the angle ICM from NICM is in the pattern of MV deformation
at which each of the two mitral leaflets joins the mitral (measured as a change in the AL and PL angles). The
annular plane. These are the anterior leaflet angle (ALa) MV deformation is geometrically asymmetrical in ICM
and the posterior leaflet angle (PLa; Fig. 67.6). Because compared to NICM.101,102 In the NICM, the conformational
there is differential insertion of fibers on the mitral leaflets changes of the MV are the same in all the planes. It appears
(fine marginal chordae fibers insert at the leaflet tips and that regional LV remodeling from ischemia results in
the thicker chordae insert at the leaflet base), further asymmetrical papillary muscle displacement, which then
categorization of the AL angles can be made. Tethering of leads to asymmetrical leaflet tethering, thereby affecting
the anterior MV leaflet at the base (ALa base) is measured the coaptation of the two mitral leaflets. Two separate
by obtaining the angle at AL base as it intersects with the regurgitant jets often characterize ICM MR.101,103,104 These
jets correspond to the deformational changes in the baseline and at end of the study. A cumulative score
MV leaflets. There is a funnel-shaped conformational of all the segments is then divided by the number of
deformity in the medial side of the MV and a prolapsed segments. A numerical score of 1 is normal while that
deformity in the lateral portion of the valve. The medial above 1 is abnormal. Higher scores predict higher severity
deformity is responsible for the centrally directed jet, of obstructive disease or the presence of more extensive
while the lateral prolapsed deformity is responsible for the disease. Dobutamine stress echo can also help identify
eccentric jet. Interestingly, it is the asymmetrical apically patients with ICM that can benefit from revascularization.
oriented displacement of the papillary muscles rather Patients who have an initial augmentation of LVEF and
than the asymmetrical medial lateral orientation of the contractility at low dose dobutamine followed by a decline
papillary muscle displacement that accounts for the MV
with high dose (biphasic response) are candidates for
deformational changes seen in ICM.102
revascularization. A biphasic response indicates viable
The coaptation depth and tenting area both correlate
myocardium that could be salvaged with revascularization.
with the severity of MR in patients with systolic dysfu
nction.93 Furthermore, these parameters have been shown
to confer prognostic information in patients undergoing Coronary Echocardiography
functional MV intervention. Magne et al noted that in 2D TTE has been studied as a means of detecting CAD
ICM patients undergoing functional MV annuloplasty, the and by extension, potentially differentiating ICM from
presence of a PLa of more than 45° had increased rates NICM.110 2D TTE was able to detect proximal CAD in
of recurrent MR and other adverse events.100 There is a
93% of the study patients. Interestingly, visualization of
growing body of evidence to suggest that the geometric
the coronaries in dilated LV was much easier in patients
differences in the patterns of tethering seen in ICM
with NICM than ICM. This is primarily because NICM
and NICM can help guide the choice of therapy for
patients generally have much larger chamber sizes than
functional MR.92,97,101,105
ICM patients and, therefore, the curvature of the coronary
arteries is less steep and easily visualized. Although the
success rate of visualizing CAD lesions using 2D TTE was
Stress echocardiography has emerged as a useful tool high in this study, other researchers have recorded much
for the evaluation of selected patients with suspected or lower success rates.111–113
known ischemic heart disease.106 The high specificity of
stress echocardiography compared to other modalities
Myocardial Contrast Echocardiography
contributes to its utility as a cost-effective diagnostic tool
for CAD. The sensitivity and specificity of new WMAs There is an emerging role of myocardial contrast echocar
induced by dobutamine for detection of CAD is 89% and diography (MCE) in assessing myocardial perfusion status
85%, respectively. The sensitivity in those with multivessel in patients suspected of having CAD.114–117 MCE uses
or left main disease is 100% compared to 81% in those contrast agents to improve the visualization of blood–
with a single vessel disease.107 Dobutamine stress echo endocardial interface. This enables the assessment
can help differentiate between ICM and NICM. In the of ventricular wall motion, wall thickness, LVEF, and
absence of CAD, the normal response to dobutamine qualitative and quantitative evaluation of myocardial
stress test is augmentation of systolic function and
and coronary blood flow.43 One of the advantages of MCE
contractility with increasing doses of dobutamine. In the
is that it not only provides microcirculatory blood flow
presence of significant disease, an initial augmentation
information, but also transmural blood flow information.
of LVEF and contractility is followed by a decline in
LVEF and/or contractility or emergence of new WMA Using a vasodilator pharmacological stress test, myocardial
at higher doses of dobutamine.106,108 The segmental wall perfusion may be assessed both at rest and stress. With this
motions are graded by dividing the heart into 16 segments information, myocardial blood flow reserve is measured
(17 segments if echo is being compared with another as the difference between the peak flow during stress and
imaging modality such as nuclear perfusion) and each baseline flow at rest. Endomyocardial flow impairment has
segment is then graded on a scale of 1–4 on the basis of been shown to be directly associated with the progression
wall motion (1 = normal, 2 = hypokinesis, 3 = akinesis, of LV dysfunction. 7,118 The application of MCE in clinical
4 = dyskinesis).109 A cumulative score is obtained at practice is still limited and remains to be standardized.
ECHOCARDIOGRAPHIC DISTINCTION situation is made worse if there is a concomitant LBBB.

Duncan et al used quantified stress echocardiography
BETWEEN ISCHEMIC CARDIOMYO- to assess changes in the long-axis systolic amplitude to
PATHY AND NONISCHEMIC DILATED discriminate between ICM and NICM. In the presence
CARDIOMYOPATHY of LBBB, inability to increase septal systolic amplitude
by >1.5 mm was highly indicative of CAD. This method
There are several features on 2D TTE that help distinguish was better than visual assessment of wall motion score
ICM from dilated NICM (Table 67.1). Traditionally, WMAs index (WMSI; sensitivity and specificity of 94% and
have been used to discriminate between NICM and ICM. 100%, respectively).133 RV dilatation can be suggestive
In ICM, the WMA tend to be regional and correspond to of NICM. The right ventricle is often spared in ICM
specific coronary artery distribution.119,120 The presence unless, as mentioned previously, there is accompanying
of WMA has more diagnostic value in normal sized RV infarction in which case one would expect to see a
ventricles.121–125 Medina et al.121 studied 60 patients with hypocontractile, dilated RV.62,63 Involvement of the RV
dilated LV and LV dysfunction using 2D TTE for the is indicative of an advanced disease stage and is a poor
detection of regional WMA so as to differentiate between prognostic sign in either ICM or NICM.134 Because of the
ICM and NICM. They reported a sensitivity, specificity, and complex 3D structure, quantification of the RV is rendered
predictive accuracy of 83%, 57%, and 77%, respectively, in difficult with 2D TTE, but can be assessed more accurately
differentiating ICM from NICM based on the presence of with 3D TTE.135
WMA. In patients with normal LV size but LV dysfunction,
the sensitivity, specificity, and predictive accuracy OTHER NONINVASIVE
were found to be 95%, 100%, and 95%, respectively, in
detecting ICM.120 Besides WMA, identification of regional
IMAGING MODALITIES
thinning of myocardial wall (< 6 mm), or aneurysmal Single-Photon Emission
myocardial segment corresponding to coronary blood
flow area increases the likelihood of the diagnosis of ICM. Computed Tomography
Aneurysms and scar on the LV surface were found in fewer Myocardial perfusion imaging (MPI) using single-
than 15% of NICM on necropsies conducted by Roberts photon emission computed tomography (SPECT) is
et al.126 Chen et al. used semiquantitative echocardiographic a well-validated, noninvasive imaging modality used
segmental wall motion scoring to predict CAD. Myocardial in the diagnosis, treatment and prognostication of
wall motion was scored according to the scoring described CAD.136–139 SPECT MPI has high sensitivity and specificity
by Heger et al.127 (i.e. hyperkinesia = −1, normal = 0, hypoki that approaches 90% in detecting significant CAD (with
nesia = 1, akinesia = 2, and dyskinesis = 3). Patients with a at least 50% luminal diameter stenosis).140–142 In broad
LVEF < 50% had a mean score of 6.9, while those with LVEF terms, SPECT MPI classifies patients in three categories—
above 50% had a mean score of 1.1.122 normal study (normal perfusion defects at stress and
The above criteria are, however, not entirely exhaustive rest), reversible ischemia (perfusion defects with stress
in defining ICM because WMA may also be seen in up to and normal perfusion at rest), and a fixed scar (perfusion
two-thirds of patients with NICM.107,128–130 These WMA in abnormality at rest and stress). It is known that on SPECT
patients with NICM have been attributed to abnormal MPI, NICM is characterized by homogenous tracer
microcirculatory perfusion despite normal epicardial uptake, whereas ICM has extensive perfusion defects
blood vessels.131,132 Wallis et al. showed that up to 64% of that are often regional.138,143–145 Patients with reversible
patient with NICM had WMAs especially when conduction perfusion defects usually benefit from revascularization
abnormalities were present (i.e. left bundle branch block treatment strategies. The limitation of SPECT with
[LBBB]). When they excluded patients with LBBB, WMA thallium is that patients with NICM can still present
were found in 59% of the study population with NICM.128 with perfusion defects even in the absence of significant
In the same study, Wallis et al. found that WMA were more CAD.146 The combination of perfusion abnormalities that
commonly associated with older age. The younger patients occur in myocardial territories consistent with areas of
with NICM had more diffuse involvement, were more WMA overcomes the problem of WMA alone in NICM
symptomatic, and generally had a poor prognosis. Because undergoing thallium imaging alone.147 Large perfusion
resting WMA is not sensitive at discriminating CAD, the defects, however, are more predictive of significant ICM
Table 67.1: Comparison of Noninvasive Findings in Ischemic Cardiomyopathy and Nonischemic Dilated Cardiomyopathy
Modality ICM with Dilated LV NICM with Dilated LV
Clinical evaluation • Older patients • May be younger
• H
istory of ischemic heart disease such as angina • May not have history of ischemic heart disease
and myocardial infarction
• May have history of viral infections, septicemia,
alcoholism, metabolic, or infiltrative diseases
Echocardiography • S
egmental WMA/thinning (<6 mm in end diastole) • Less common. Defects when present may not
in coronary artery distribution. Sensitivity 65% to relate to coronary artery distribution
81%, and specificity 56% to 99%
• E
ndocardial brightening/scarring more common • Less common
due to infarction
• B
iphasic response with dobutamine stress test • Less common
indicative of hibernating viable myocardium is
more common
• P
erfusion defects with contrast echo more • Less common
common
• 2 D TTE/Doppler may show stenosis in proximal or • Coronary arteries may show no stenosis, may
other coronary arteries, which may be small be larger
• Spherical LV less common • Spherical LV more common
• M
ay show prominent plaques in larger vessels • May be normal
such as ascending aorta, arch, and abdominal
aorta.
• A
symmetric closure of mitral valve leaflets with • Symmetric closure with central jets more com-
eccentric regurgitation jet(s) more common. mon. “Seagull” sign absent
“Seagull” sign (Fig. 67.4) may be present
• LV dilatation less severe • Severe spherical LV dilatation with malcoapta-
tion of mitral valve leaflets
• R
ight ventricle usually not enlarged and function • RV enlargement and/or dysfunction more
normal except when RCA is stenotic/occluded or frequent
RV infarction present
Single-photon • P erfusion defects in coronary artery distribution. • Perfusion defects less common and if present
emission computed More accurate if combined with WMA do not relate to coronary artery distribution
tomography (SPECT) • Normal sized RV with good function more • Large RV with reduced function more common
common
Coronary computed • C
oronary artery calcification more common.Coro- • Less common
tomographic angio- nary stenosis, both obstructive and
gram (CCTA) nonobstructive may be identified
Cardiac magnetic • I ncreased gadolinium uptake or enhancement in- • Less common
resonance (CMR) dicating scar formation in the previously infarcted
areas more common
• Regional WMA/thinning and perfusion defects • Regional WMA/thinning and perfusion defects
similar to echocardiography above similar to echocardiography above
Positron emission • Large mismatch defects more common • Less common
tomography (PET)
(2D TTE: Two-dimensional transthoracic echocardiography; ICM: Ischemic cardiomyopathy; LV: Left ventricle; NICM: Nonischemic
cardiomyopathy; RV: Right ventricle; WMAs: Wall motion abnormalities).
than smaller defects. When a large perfusion defect is Cardiac Magnetic Resonance Imaging
present, the sensitivity of SPECT to accurately identify
ICM was as high as 97%. Conversely, in the absence of a CMR has become an important noninvasive diagnostic
large perfusion defect, SPECT MPI was able to accurately tool in the evaluation of various cardiac conditions.161 It
discriminate NICM in 94% of the study group.144 More has become the reference, if not the gold standard, for
recently, an approach that incorporates the severity of evaluation of cardiac chamber sizes, mass, volumetric
the defect with the extent of WMA was shown to increase measurements, and EF. In clinical situations that require
the likelihood of detecting ICM. Defect severity ratio was serial cardiac measurements, CMR has an added
computed by measuring the ratio of the count density of advantage of being highly reproducible with low inter-
the most severe perfusion defect over the count density and intra-study variability. Other advantages of CMR
of the most normal area of myocardium. A stress defect include absence of ionizing radiation and relatively
ratio < 45% was predictive of ICM (with a sensitivity of high spatial and temporal resolution.161,162 The clinical
60% and specificity of 91%). Most patients with NICM had applications of CMR continue to evolve and expand,
higher stress defect ratio.148 Electrocardiographically gated and now include noninvasive evaluation of the proximal
radionuclide ventriculography (RNV) has been shown to coronaries.163,164 The sensitivity and specificity of CMR
have incremental value in differentiating between NICM for the detection of CAD is 91% and 81%, respectively.165
and ICM. Given that WMA are not confined to ICM, the CMR has been studied in the differentiation of ICM from
EF information provided by RNV can further serve as a NICM. Furthermore, the use of gadolinium enhancement
pointer to the diagnosis. NICM patients have lower LVEF, helps with characterizing scar and other secondary
which often involves the RV as well.149 causes of NICM (i.e. myocarditis, sarcoidosis).164,166,167
In patients with ICM, CMR identifies subendocardial or
Coronary Computed transmural enhancement indicative of scar formation in
virtually 100% of the patients.167,168 In the NICM patients,
Tomographic Angiogram three patterns are identified. The first pattern shows no
Coronary computed tomographic angiogram (CCTA) enhancement with gadolinium, the second subset shows
has emerged to be an important noninvasive diagnostic subendocardial/transmural enhancement similar to the
modality in the evaluation of CAD.150–152 The diagnostic ICM, and the third subset shows longitudinal patchy
utility of CCTA is in patients with low to intermediate risk midwall enhancement. Enhancement with gadolinium
for CAD. There are several published studies that have highlights areas of the myocardium with previous
looked at the role of CCTA in differentiating between infarction.169 The most common type of CMR pattern (58%)
NICM and ICM.153–157 Two primary avenues by which CCTA is no enhancement, a finding consistent with a clinical
stratifies patients for the underlying CAD is the calculation picture of no previous areas of infarction. The second
of calcium score and by anatomical definition of coronary
subtype shows enhancement similar to that of ICM (13%)
arteries for presence of a plaque or stenosis. Calcium score
but without obstructive coronary lesions on angiography.
is calculated using electron-beam computed tomography
These findings represent myocardial infarction from
(EBCT) and multislice computed tomography (MSCT). A
high burden of coronary calcium (calcium score of > 80) an embolic plaque or a coronary artery source that has
assessed by EBCT was associated with a 99% sensitivity recanalized. The third subtype (28%) involves patchy
and an 83% specificity in accurately identifying patients midwall enhancement that has been attributed to
with ICM.154 Presence of calcium is a known marker fibrosis of the midventricular wall. The pathogenesis
for atherosclerosis. In another imaging modality using of this type of fibrosis has been linked to a whole host
ultrasound of the carotids, carotid calcification in patients of potential etiologies such as genetic factors, toxins,
with dilated cardiomyopathy had a sensitivity of 96% and infections, microvascular ischemia, neurohormonal, and
specificity of 89% of accurately identifying ICM.158 CCTA immunological factors.170–173 The pattern of enhancement
is more sensitive in identifying patients with ICM than of the scar has prognostic information. Improvement of
2D TTE (sensitivity of 68% and specificity of 73%).159,160 contractility after revascularization is low in patients with
However, the sensitivity of CCTA to accurately diagnose extensive transmural extent of the scar; for instance, if there
ICM decreases in patients with a high calcium burden is >50% delayed enhancement on CMR, the likelihood of
(to 73%).156 response to revascularization was <10%.164 More recently,
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CHAPTER 68
Pericardial Disease
Trevor Jenkins, Brian D Hoit
Snapshot

Acute Pericardi s 
Constric ve Pericardi s

Pericardial Effusion 
Effusive-Constric ve Pericardi s

M-Mode and Two-Dimensional Echocardiography 
Congenital Anomalies

Pericardial Tamponade 
Mul modality Imaging of the Pericardium
INTRODUCTION Anatomy and Echocardiographic

Pericardial heart disease represents a spectrum of Appearance
conditions with significant mortality and morbidity The pericardium is an avascular tissue comprising two
commonly encountered in cardiovascular medicine. histologically distinct layers—the visceral pericardium, a
Normal pericardial anatomy was first described in serosal single cell layer adherent to the epicardium and
antiquity, while descriptions of pericardial pathology great vessels; and the parietal pericardium, a thick fibrous
appeared during the 17th to 18th centuries.1 Ultrasound outer layer. A potential space is enclosed between these
visualization of the pericardium has advanced rapidly two layers, which normally contains 15–35 mL of serous
during recent decades and has become an essential fluid distributed mostly over the atrial–ventricular and
tool for diagnosis and management of pericardial heart interventricular grooves.5 This fluid serves as a lubricant
disease. The value of echocardiography in this regard is during cardiac motion. Pericardial reflections around the
the ability to display both structure and physiology at proximal ascending aorta, central pulmonary arteries,
high spatial and temporal resolution. Efforts to visualize pulmonary veins, and venae cavae form the oblique and
pericardial disease originate with the early days of cardiac transverse sinuses. The fibrous parietal pericardium is less
ultrasound. Inge Edler first published images of an distensible than myocardium, a property that functionally
anterior pericardial effusion in 1961.2 Harvey Feigenbaum limits myocardial chamber distention.6,7
published several early manuscripts documenting the The normal pericardium is usually not well visualized
power of echocardiography to display pericardial effusions by two-dimensional (2D) echocardiography. The typical
and published the relation between the “swinging heart” appearance is that of a thin, bright, highly echogenic line
in cardiac tamponade by M-mode with electrical alternans denoting the intersection of parietal pericardium and lung
by electrocardiography (ECG).3 Such early observations tissue. The normal pericardium by computed tomography
helped expand the scope of echocardiography beyond (CT) is < 2 mm in diameter. Thickening of the pericardium
detection of mitral stenosis.4 (> 5 mm diameter) may allow for direct visualization.
Normally, a trace amount of pericardial fluid may be seen and congenital etiologies. Thus, pericardial disease
at end-systole. may present either as an isolated phenomenon or as a
complication of a variety of systemic disorders, trauma, or
Physiology and Pathophysiology certain drugs. In these settings, pericardial involvement
may be overshadowed by extracardiac manifestations
The pericardium is not essential for life as no adverse and difficult to recognize.8 This chapter will review the
consequences follow congenital absence or surgical remo- echocardiographic findings in each of these conditions.
val of the pericardium. However, the pericardium serves
many important functions (Table 68.1). Most relevant
to echocardiographic evaluation, it limits distension of
ACUTE PERICARDITIS
the cardiac chambers, and facilitates interaction and Acute pericarditis may be isolated or present as a
coupling of the ventricles and atria.8 Pericardial restraint manifestation of a systemic process. Although the etiology
of ventricular filling becomes significant when the is highly variable, most cases of acute pericarditis are
pericardial reserve volume (the normally small difference idiopathic or viral. Inflammation of the pericardium
between unstressed pericardial volume and cardiac is usually silent echocardiographically, as echogenic
volume) is exceeded. This may occur with rapid increases brightness of the pericardium lacks sufficient diagnostic
in blood volume and in disease states characterized by sensitivity and specificity.
rapid increases in heart size, such as acute mitral and Echocardiography is recommended as the initial
tricuspid regurgitation. In contrast, chronic stretching of noninvasive imaging test for acute pericarditis, because
the pericardium results in “stress relaxation” and “creep” it accurately detects pericardial effusion and tamponade,
(decreased pericardial pressure and increased in volume and ventricular dysfunction due to myopericarditis.9
with constant stretch, respectively, owing to viscoelastic Echocardiography estimates the volume of pericardial
properties of the pericardium) and cellular hypertrophy, fluid, identifies cardiac tamponade, suggests the basis of
which explains why large but slowly developing effusions pericarditis, and documents associated acute myocarditis.
do not produce tamponade.8 In addition, the presence of adhesions, fibrous strands,
In view of the pericardium’s simple structure, clinico- hemorrhage, and loculations may aid in the diagnosis of
pathological processes involving it are understandably morbid conditions such as purulent bacterial pericarditis
few and includes only pericarditis and its complications, that may require pericardiocentesis. Although patients
tamponade and constriction, and congenital lesions. with purely fibrinous acute pericarditis have a normal
However, the pericardium is affected by virtually every echocardiogram, the presence of a pericardial effusion is
category of disease, including infectious, neoplastic, consistent with acute pericarditis and is one of the criteria
immune-inflammatory, metabolic, iatrogenic, traumatic, for its diagnosis. A transthoracic echocardiogram (TTE) is
particularly critical in the setting of high-risk features of
Table 68.1: Functions of Pericardium
acute pericarditis associated with worse outcome including
fever > 38°C, subacute onset, an immunosupressed state,
Mechanical
trauma, or evidence of hemodynamic compromise.10
Effects on individual chambers:
The use of echocardiography for the evaluation of all
• Constrains chamber distention during cardiac cycle patients with suspected pericardial disease was given
• Modulates cardiac chamber interaction and coupling a Class I recommendation by a 2003 task force of the
• Maintains left ventricular geometric shape American College of Cardiology (ACC), the American
• Preserves pressure–volume relation of the cardiac Heart Association (AHA), and the American Society of
chambers Echocardiography (ASE).11 Additional imaging modalities
Effects on entire heart: may be necessary if the TTE is negative or inconclusive in
• Lubrication and friction reduction a patient with complex or atypical clinical presentations.
• Mechanical barrier to infection
• Balances inertial hydrostatic and gravitational forces PERICARDIAL EFFUSION
Miscellaneous (vasomotor, immunological, fibrinolytic, regula- Accumulation of transudative or exudative fluid in excess
tion of localized gene and protein expression) of 50 mL is abnormal and may be seen with pericarditis
Chapter 68: Pericardial Disease 1437
Table 68.2: Etiology of Pericarditis of cases. In the remainder, patients with evidence of an
Idiopathic:
inflammatory process were most likely to have acute
idiopathic pericarditis, while those without inflammatory
• Infectious (viral, bacterial, mycobacterial, fungal, or AIDS/HIV)
signs or tamponade were more likely to have a chronic
• Autoimmune (systemic lupus erythematosus, rheumatoid
idiopathic effusion, and those with cardiac tamponade
arthritis, systemic sclerosis, or ankylosing spondylitis)
but without inflammatory signs most commonly had a
• Neoplastic [primary (mesothelioma), secondary (breast,
lung, melanoma, lymphoma)]
malignant effusion.14
Radiotherapy:
• Metabolic (drugs, myxedema, amyloidosis)
M-MODE AND TWO-DIMENSIONAL
• Nephrogenic (uremic, dialytic) ECHOCARDIOGRAPHY
• Cardiac injury (surgery, interventional, trauma) Echo is the initial procedure of choice to detect the
• Myocardial infarction [acute or chronic (Dressler’s presence of a pericardial effusion because it is portable,
syndrome)] noninvasive, can be performed with minimal delay, and
attention to technical detail results in excellent sensitivity
and specificity. The diagnostic feature on M-mode
of any etiology (Table 68.2). Pericardial effusions are
echocardiography is the persistence of an echo-free space
very common after cardiac surgery. In 122 consecutive
between parietal and visceral pericardium throughout the
patients studied before and serially after cardiac surgery,
cardiac cycle. Separations that are observed only in systole
effusions were present in 103 patients; the majority
represent clinically insignificant accumulations. The
appeared by postoperative day 2, reached their maximum
superior spatial orientation of 2D echo allows delineation
size by postoperative day 10, and usually resolved without
of the size and distribution of pericardial effusion, as well
sequelae within the first postoperative month.12 However,
as detection of loculated fluid. As the amount of pericardial
large effusions or effusions causing pericardial tamponade
fluid increases, fluid distributes from the posterobasilar
are uncommon following cardiothoracic surgery. In one
left ventricle (LV) apically and anteriorly, and then laterally
retrospective survey of more than 4,500 postoperative
and posteriorly to the left atrium (LA). Fluid adjacent to the
patients, only 48 were found to have moderate or large
right atrium (RA) is an early sign of pericardial effusion. A
effusions by echocardiography; of those, 36 met diagnostic
left pleural effusion may mimic a pericardial effusion on
criteria for tamponade.13
M-mode, in which cases fluid anterior to the descending
Effusion should be suspected and an echocardiogram
aortic on a 2D echo parasternal long axis establishes the
obtained in all patients who present with chest pain
fluid as pericardial rather than pleural, which is posterior
consistent with pericarditis or aortic dissection, an
enlarged (typically “flask shaped”) cardiac silhouette to the aorta15 (Figs 68.1 and 68.2).
seen on chest radiogram, systemic disease associated The size of a pericardial effusion on 2D echo is
with pericardial effusion accompanied by jugular venous qualitatively described by the end-diastolic distance of
distension, after a myocardial infarction, or in patients the echo-free space between the parietal and visceral
who develop hypotension or hemodynamic instability in pericardium: trivial (seen only in systole), small (< 10
the setting of interventional cardiac procedures. However, mm), moderate (10–20 mm), large (> 20 mm), or very
asymptomatic pericardial effusions are often discovered large (> 2.5 cm); an even distribution of the effusion
during the evaluation of an unrelated medical complaint sampled from multiple 2D transducer positions increases
or disorder. the predictive accuracy of the estimate. 2D echo also
Chronic effusive pericarditis is an entity of unknown allows for the detection of fluid that may be loculated, or
etiology that may be associated with large, asymptomatic an echodensity more consistent with an exudate or clot
effusions. Many conditions that cause pericarditis (e.g. rather than a transudate. Exudative pericardial effusions
uremia, tuberculosis, neoplasia, connective tissue may show features such as stranding, adhesions, or an
disease) produce chronic pericardial effusions. In a series uneven distribution reflecting a more inflammatory
of 322 patients admitted to a tertiary care hospital with composition; finding these frond-like, band-like, or
at least a moderate-sized pericardial effusion, the cause shaggy intrapericardial echoes should alert one to the
was attributed to a preexisting medical condition in 60% possibility of a difficult and potentially less therapeutic
A B
C D
Figs 68.1A to D: Two-dimensional (2D) echocardiography of pericardial effusions of varying size. Anterior (*) and posterior (arrow) are
seen as echo-free spaces of increasing size including small, posterior (A), moderate circumferential (B), and large circumferential (C).
Figure D shows a large pleural effusion (curved arrow) posterior to a small pericardial effusion (arrow) with the pericardium visualized as
an echogenic linearity between both. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
pericardiocentesis and a more complicated course, but with obesity-related insulin resistance, and is a coronary
have little value in identifying the cause of the effusion risk factor16 (Fig. 68.4).
(Figs 68.3A and B). Pericardial effusions that contain clots
(e.g. after cardiac surgery), may be missed on a TTE and PERICARDIAL TAMPONADE
may require transesophageal echo (TEE), CT, or cardiac
magnetic resonance imaging (CMR). Cardiac tamponade is a life-threatening condition
Distinguishing epicardial fat from (particularly caused by fluid accumulation in the pericardial sac and
anterior) pericardial effusion may be difficult, but is characterized by elevation and equalization of cardiac
epicardial fat is slightly echogenic and moves in concert diastolic and pericardial pressures, a reduced cardiac
with the heart, whereas pericardial effusion is generally output, and an exaggerated inspiratory decrease in arterial
echolucent and motionless. Epicardial fat may appear systolic pressure (>10 mm Hg) referred to as pulsus
circumferentially (fat envelope) and be difficult to discern. paradoxus. Cardiac tamponade is poorly related to the size
In addition to its mimicry, pericardial fat accumulation is of the effusion, as it is the rapidity of fluid accumulation in
a source of bioactive molecules, is significantly associated the pericardial space and the eclipse of pericardial reserve
A B
C D
Figs 68.2A to D: M-mode echocardiography of pericardial effusions of varying size. Anterior (*) and posterior (arrow) are seen as
echo-free spaces of increasing size including small, posterior (A), moderate circumferential (B), and large circumferential (C). Concur-
rent pleural (curved arrow) and pericardial effusion are shown in Figure D. Note that parietal pericardium displays relatively flat motion
throughout the cardiac cycle best visualized in Figure C. (LV: Left ventricle; RV: Right ventricle).
A B
Figs 68.3A and B: Exudative pericardial effusions as seen by two-dimensional (2D) echocardiography apical views. Frond-like (A) and
band-like (B) adhesions are seen bridging a large pericardial effusion fluid, indicating an inflammatory component to the effusion. Note
the thickened pericardium in Figure A. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
tamponade, urgent pericardiocentesis is usually not

necessary, but careful monitoring is warranted.
Tamponade may also be regional. A loculated,
eccentric effusion, or localized hematoma can produce
regional tamponade in which only selected chambers
are compressed. As a result, the typical physical, hemo-
dynamic, and echocardiographic signs of tamponade are
often absent. Regional tamponade is most often seen after
pericardiotomy or myocardial infarction; clinical suspicion
should be heightened in these settings. Establishing
the diagnosis is challenging and may require additional
echocardiographic views (e.g. transesophageal) and other
advanced imaging techniques (e.g. CT, CMR).
When cardiac tamponade is suspected, a 2D echo with
Fig. 68.4: An echo-clear space (arrow) is seen anterior to the Doppler should be obtained emergently unless a delay
pericardium due to epicardial fat. (AO: Aorta; LA: Left atrium; LV: might prove life-threatening. CT and CMR are used only
Left ventricle; RA: Right atrium; RV: Right ventricle). for complicated cases such as postoperative or loculated
effusions. While there are many echo signs of tamponade,
the most important ones are the presence of a pericardial
volume that elevates pericardial pressure and interferes
effusion, dilated (plethoric) inferior vena cava (IVC)
with cardiac filling.
and hepatic veins (which indicate that systemic venous
Cardiac tamponade may be acute or subacute–chronic
pressures are elevated), and a LV with reduced end-
and should be viewed hemodynamically as a continuum
diastolic and systolic dimensions with Doppler evidence
ranging from mild (pericardial pressure lower than 10 mm
of reduced stroke volume and cardiac output.
Hg) to severe (pericardial pressure higher than 15–20 mm
In most cases of cardiac tamponade, other “classic”
Hg). Mild cardiac tamponade may cause few symptoms,
echo-Doppler findings are also present and confirm
whereas moderate tamponade and especially severe
the diagnosis. These signs include right heart diastolic
tamponade produce precordial discomfort, dyspnea, and
chamber collapses (when pericardial pressures exceed
a sense of doom.
intracardiac pressure), an inspiratory bulge or “bounce” of
Patients who are severely hypovolemic due to
the interventricular septum into the LV, and characteristic
hemorrhage, dialysis, or overdiuresis may have low
abnormal respiratory changes in Doppler flow velocity
pressure tamponade in which the intracardiac and peri-
recordings.
cardial diastolic pressures are < 10 mm Hg. In a series of
279 patients who underwent combined pericardiocentesis
and cardiac catheterization, 143 patients (51%) M-Mode and Two-Dimensional Echo
were diagnosed with cardiac tamponade defined as Two-dimensional echo imaging from standard transducer
intrapericardial pressures equal to RA pressure prior to positions establishes the qualitative size of the effusion, its
pericardiocentesis.17 Low pressure cardiac tamponade distribution, and to an extent, the nature of the effusion.
was diagnosed in 29 patients (10%) who had an initial When cardiac tamponade is present with a moderate
intrapericardial pressure of < 7 mm Hg and a RA pressure or large effusion, the LV cavity dimensions are reduced,
after pericardiocentesis of < 4 mm Hg. Clinical findings and because mass is conserved, the wall thickness is
commonly associated with cardiac tamponade, such increased (“pseudohypertrophy”)18 (Figs 68.5A and B).
as sinus tachycardia, jugular venous distention, and When the effusion is massive, the heart swings freely in
pulsus paradoxus were less common in the low pressure the pericardial space and displays a pendular motion
group. However, the hemodynamic significance of these that is associated with electrical alternans (Movie clip 1).
effusions could be demonstrated on echo by right heart Right ventricular end-diastolic diameter increases
chamber collapse and respiratory variations in Doppler during inspiration while reciprocally, LV end-diastolic
transvalvular flow velocities, despite the absence of diameter decreases; opposite changes are seen during
vena caval plethora. In the absence of clinical signs of expiration (see below). An important sign of tamponade
A B
Figs 68.5A and B: Pseudohypertrophy (A) is demonstrated in the setting of a large pericardial effusion with tamponade physiology with
normalization; (B) of left ventricular wall thickness after pericardiocentesis.
in cardiac tamponade and are particularly valuable in the

diagnosis of low pressure tamponade when IVC dilation
is minimal or absent. The subcostal view is often the best
to visualize RA and RV chamber collapse. The superior
temporal resolution of M-mode echo makes it ideal for
judging the timing and duration of collapse. Occasionally,
LA and LV chamber collapses are observed (Figs 68.7A
and B).
Chamber collapses indicate transient negative
transmural pressure and occur during their respective
relaxation phase when intracavitary pressure reaches its
nadir.20 Thus, atrial collapse begins at end-diastole near
the peak of the R-wave, while ventricular collapse begins
in early diastole after the end of the T-wave. In general,
Fig. 68.6: Plethora (dilation) of the inferior vena cava (IVC) indi- specificity of collapses is greater and tamponade more
cative of elevated central venous filling pressure in the setting of severe, the longer the duration of compression. Brief RA
tamponade. (L: Liver). collapse is sensitive but not specific, whereas RA collapse
that exceeds one-third of the cardiac cycle is nearly 100%
sensitive and specific for clinical cardiac tamponade.21
that should be carefully sought is IVC plethora, defined as RV diastolic collapse generally occurs when cardiac
dilation of the IVC and hepatic veins with < 50% reduction output has decreased about 20% from baseline but before
in diameter during inspiration. In one series, IVC plethora systemic blood pressure has fallen; initially, it is seen
was present in 92% of pericardial effusions that were only during inspiration, but as tamponade becomes
associated with pulsus paradoxus and which required more severe it occurs throughout the respiratory cycle22
pericardial drainage.19 Although IVC dilation is highly (Movie clip 2). Experimental studies indicate that right
sensitive for cardiac tamponade, it is a nonspecific sign heart chamber collapse occurs earlier than pulsus
indicating elevated right heart pressures and therefore is paradoxus, and that the sensitivity and specificity of
seen in heart diseases in which a pericardial effusion is chamber collapse improves as the severity of tamponade
absent (Fig. 68.6). increases. These studies also suggest that RA chamber
Diastolic RA and right ventricle (RV) chamber collapse may have a higher predictive value than RV
invagination or “collapses” on 2D echo are usually seen collapse.23
A B
Figs 68.7A and B: Two-dimensional (2D) echocardiograms in the apical four-chamber view demonstrating chamber collapse due to
tamponade. During late diastole, there is inversion of the right atrial lateral wall (A), and right ventricular free wall (B). (AO: Aorta; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).
Table 68.3: Sensitivity and Specificity of Right Heart

In cardiac tamponade, inspiration lowers right heart
Chamber Collapses in Cardiac Tamponade pressures and augments systemic venous return as it does
Sensitivity (%) Specificity (%) in normal individuals. However, unlike the minimal (< 5%)
change in left-sided filling during normal inspiration in
Any chamber 90 65
normal individuals, left heart filling decreases abnormally
Right atrium 68 66
in cardiac tamponade, resulting in a reduced stroke volume
Right ventricle 60 90
and the appearance of pulsus paradoxus. This phenomenon
Simultaneous right atrium/ 45 92 is due to enhanced ventricular interdependence, wherein an
right ventricle (RA/RV)
increase in filling on one side of the heart is associated with
Source: Modified from Reference 24. a decrease on the opposite side. Thus, during inspiration
there is an increase in RV dimension and a decrease in LV
dimension that is due to septal movement toward the LV free
Although the sensitivity and specificity of collapses are wall (and a decrease in the pulmonary venous to LA pressure
variable (Table 68.3), the absence of any cardiac chamber gradient). The result is a characteristic inspiratory bulge or
collapse has > 90% negative predictive value for clinical “bounce” of the interventricular septum into the LV. It should
cardiac tamponade.24 However, right heart diastolic be recognized that an inspiratory septal bulge or “bounce” is
collapse may occur only at higher levels of pericardial not specific for cardiac tamponade but may be seen in other
pressure, or may be absent in conditions in which right conditions associated with pulsus paradoxus, such as chronic
heart chamber pressures are elevated before the effusion obstructive pulmonary disease and pulmonary embolism.
accumulated, as may be seen with RV hypertrophy and In these instances, the clinical context and the absence of a
severe pulmonary hypertension. Conversely, collapse of pericardial effusion rule out cardiac tamponade as causal. On
the right heart chamber may occur earlier than normal the other hand, an inspiratory septal bulge may be absent in
when intracardiac pressures are low owing to hypovolemia cardiac tamponade when there is LV hypertrophy or marked
or with coexisting severe LV dysfunction.25,26 Posterior preexisting elevated LV filling pressures.27
loculated effusions after cardiac surgery and severe
pulmonary arterial hypertension may produce LA and LV
diastolic collapse. As indicated earlier, establishing the
Doppler Flow Velocity Recordings
diagnosis of regional tamponade is challenging and may Analogous to the changes seen on M-mode and 2D
require nontraditional echo views, TEE, CT, or CMR. echo, characteristic respiratory changes occur in pulsed
A B
Figs 68.8A and B: Pulsed wave Doppler in a patient with cardiac tamponade. Note the increased expiratory flow velocity of the mitral
valve (A) and increased inspiratory flow velocity of the tricuspid valve (B).
forward velocity or small reversals at atrial contraction

and end systole (venous reversals); with inspiration, both
peak systolic and diastolic flow velocities increase. In
mild cardiac tamponade, forward flow velocities decrease
and venous flow during systole predominates because
intrapericardial pressure decreases significantly only
during ventricular ejection. In moderate tamponade,
diastolic flow velocity is markedly reduced but still
augments with inspiration. When tamponade is severe,
forward flow occurs only during systole, and when hepatic
forward flow is observed only during inspiration, systemic
venous and intracardiac pressures are markedly elevated
and equalized at which time cardiac arrest is imminent
(Fig. 68.9).
Fig. 68.9: Hepatic vein flow reversals (arrow) during expiration. Patients with cardiac tamponade also display characte-
Note systolic forward venous flow predominates in moderate- ristic expiratory changes of hepatic venous flows. On the
severe tamponade with systolic inspiratory augmentation (*). first beat of expiration, diastolic flow velocity decreases
or reverses. High positive and negative predictive values
wave Doppler transvalvular velocities when compared for cardiac tamponade are reported using hepatic venous
with normal controls and patients with asymptomatic recordings (82% and 88%, respectively), but they are not
effusions, namely tricuspid and pulmonary flow velocities evaluable in about one-third of patients.24
increase with inspiration while simultaneously mitral
and aortic valve flow velocities decrease. The changes are Echo-Guided Pericardiocentesis
greatest on the first beat of inspiration and expiration (a Unless the situation is immediately life-threatening,
point which helps differentiate the respiratory variation experienced staff should perform pericardiocentesis in a
seen in obstructive lung disease). Respiratory variation facility equipped with monitoring to optimize the success
in the isovolumic relaxation and ejection times are also and safety of the procedure. Monitoring the cardiac rhythm
seen28,29 (Figs 68.8A and B). and systemic blood pressure is a minimum requirement.
Normal hepatic venous flow is biphasic, with systolic The advantages of needle pericardiocentesis include
velocity greater than diastolic velocity, and reduced the ability to perform careful hemodynamic measurements
A B
Figs 68.10A and B: Two-dimensional (2D) echocardiography-assisted pericardiocentesis from the apical four-chamber view. Note the
appearance of agitated saline bubbles (Figure A arrow) after successful needle entry into the pericardial space. Catheter advancement
(Figure B arrow) is visualized as the pericardial drain is passed into the effusion. (LV: Left ventricle; RV: Right ventricle).
and relatively simple logistic and personnel requirements. more common. Postoperative CP is an important cause of
The safety of the procedure has been increased by using constriction, with a reported incidence of 0.2%.8
2D echo guidance with a 1.2% major complication rate in Transient (acute) constriction may occur in
1,127 cases over 21 years.30 Echocardiographic guidance approximately 15% of patients with acute effusive
assists pericardial drainage using alternative sites on pericarditis. Doppler-detected constrictive physiology
the chest wall. Injection of agitated saline and imaging resolved without pericardiectomy in 36 of 212 patients
can confirm that the pericardial space was entered studied retrospectively after an average of approximately
(Figs 68.10A and B). 8 weeks at Mayo Clinic.32 The most common cause of
transient CP was caused by pericardial inflammation
CONSTRICTIVE PERICARDITIS after pericardiotomy in nine cases; infection, idiopathic,
collagen vascular disease, trauma, and malignancy
Constrictive pericarditis (CP) is a condition in which
accounted for the remaining cases. Treatment included
a thickened, scarred, inelastic, noncompliant, and
anti-inflammatory agents, antibiotics, chemotherapy, and
often calcified pericardium limits diastolic filling of the
angiotensin-converting enzyme inhibitors plus diuretics.
ventricles. The etiologies of CP are wide-ranging and
Five patients had resolution of constriction without any
include viral and idiopathic pericarditis, cardiac surgery
specific therapy.
(the most common antecedent in developed countries),
tuberculosis (common in underdeveloped countries), Localized CP is rare, but occasionally a localized band
collagen vascular disease, trauma, and chest radiation. constricts the inflow or outflow region of one or more of
CP may have a long latency period after the initial (and the cardiac chambers. The clinical picture then simulates
sometimes unrecognized) pericardial injury as might valve disease or venous obstruction.
occur in post-irradiation and post-traumatic pericarditis, The suspicion for CP is based on clinical history
becoming apparent only decades later. Although it is and examination, which require subsequent evaluation
commonly thought that a normal pericardial thickness and confirmation by imaging and hemodynamic data.
excludes the diagnosis of CP, 28% of 143 surgically Most patients with CP are referred to evaluate cardiac
confirmed cases had normal pericardial thickness on CT function, right heart failure, ascites, or edema. Typical
scan, and 18% had normal thickness on histopathological 2D and Doppler echo findings often arrive at the correct
examination.31 diagnosis and serve to differentiate CP from restrictive
Classic chronic CP is encountered less frequently cardiomyopathy and other conditions (e.g. severe
than it was in the past, whereas subacute CP, occurring tricuspid regurgitation, chronic liver disease) mimicking
weeks to months after the inciting injury, is becoming constriction.
Table 68.4: Echocardiographic Findings in Constrictive

Pericarditis
M-Mode:
• Thickened pericardium (dual pericardial echos)
• Septal shudder and bounce
• Atrial septal notch (immediately after atrial systole)
• Flattening of the posterior left ventricle (LV) wall in
mid-late diastole
• Premature opening of the pulmonic valve
Two-dimensional (2D) echo:
• Increased pericardial thickness (best with transesopha-
geal echo)
• Septal shudder and bounce
• Tubular appearance to ventricles with dilated atria
Fig. 68.11: M-mode echocardiogram from a patient with con-
• D’Cruz sign [abnormal angle formed by LV and left atrium strictive pericarditis. Note the echo-bright thickened posterior
(LA) posterior walls] pericardium and flat posterior left ventricular (LV) wall during mid
• Vena caval plethora to late diastole (arrow). An atrial systolic notch (*) is visualized
after atrial systole.
• Sharp diastolic filling halt
Doppler:
• Restrictive diastolic filling pattern on mitral and tricuspid
inflow flattening of the LV posterior wall endocardium, abrupt
• E/A velocity ratio > 1.5 with shortened deceleration time posterior motion of the ventricular septum in early
(< 150 ms) diastole with inspiration (septal shudder and bounce),
• Tricuspid inflow velocity increases on first beat after and occasionally, premature opening of the pulmonary
inspiration valve. These findings, which reflect abnormal filling of the
• Mitral inflow velocity decreases on first beat after inspira- ventricles, are insensitive and subtle and lack the specificity
tion to be clinically useful. Although no sign or combination
• Expiratory increase in pulmonary vein velocities of signs on M-mode echocardiography is diagnostic of
• Diastolic hepatic vein expiratory reversals CP, a normal study virtually rules out the diagnosis33
• Increased mitral propagation velocity (color M-mode)
(Fig. 68.11).
Two-dimensional echocardiography echo reveals
Tissue Doppler:
dilation and absent or diminished collapse of the IVC
• Elevated e' velocity
and hepatic veins indicative of elevated RA pressure,
• Annulus paradoxus
moderate biatrial enlargement, a sharp halt in ventri-
• Annulus reversus cular diastolic filling, and abnormal ventricular septal
motion that results from interventricular dependence
(Movie clip 3). LV systolic function as judged by the
M-Mode and Two-Dimensional Echo ejection fraction is typically normal but may be impaired
Echo is usually the initial diagnostic procedure in patients in mixed constrictive–restrictive disease, which may occur
with suspected CP (Table 68.4). Pericardial thickening and with radiation-induced disease or after cardiac surgery.
calcification, and abnormal ventricular filling produce Measurement of pericardial thickness by TEE correlates
characteristic changes on the M-mode echo. Increased strongly with that obtained by CT and has deserved an
pericardial thickness is suggested by parallel motion of the ACC/AHA/ASE Class IIb recommendation.34 However,
visceral and parietal pericardium, which is separated by a it is important to remember that demonstration of the
relatively echo-free space. Echocardiographic correlates characteristic “constrictive” hemodynamics is required to
of the hemodynamic abnormalities of CP include diastolic establish a firm diagnosis (Figs 68.12A and B).
A B
Figs 68.12A and B: Two-dimensional (2D) echocardiography findings in a patient with constrictive pericarditis. Note the tubular geometry
of left ventricle in parasternal long-axis (A) and apical four-chamber (B) views. Figure A demonstrates D’Cruz sign, an abnormal contour
of the posterior left ventricle and posterior left atrial walls giving rise to an angle < 150°. (LA: Left atrium; LV: Left ventricle; RA: Right
Doppler Flow Velocity Recordings

Doppler echo is essential for diagnosis and usually shows
restrictive LV and RV diastolic filling patterns, which
are characterized by high early (E) velocity, shortened
deceleration time (< 150 ms), and a reduced atrial (A)
wave. Mitral inflow velocity usually, but not always, falls
as much as 25–40% and tricuspid velocity greatly increases
in the first beat after inspiration35,36 (Fig. 68.13). These
respiration-induced phenomena are manifestations of
enhanced ventricular interaction and are not present
in either normal subjects or patients with restrictive
cardiomyopathy. Increased respiratory variation of mitral
inflow may be missing in patients with markedly elevated
left atrial pressure but can sometimes be brought out in Fig. 68.13: Transvalvular Doppler flow of the mitral valve in a
such patients by preload reduction with a head-up tilt patient with constrictive pericarditis. Mitral early inflow velocity falls
or diuretic administration.37 Similar to mitral flow, the by 40% in the first cardiac cycle after inspiration (arrow). There is
inversely, a simultaneous increase in tricuspid inflow velocity (not
respiratory variation in pulmonary venous (particularly shown).
diastolic) flow is often pronounced. Hepatic vein diastolic
flow reversal increases with expiration, reflecting the
Tissue Doppler imaging is particularly useful in
ventricular interaction and the dissociation of intracardiac
differentiating between CP and restrictive cardio-
and intrathoracic pressures, which is essential in the myopathy.40–43 Tissue Doppler shows a prominent early
diagnosis of constriction38,39 (Fig. 68.14). In contrast, diastolic velocity (e') from the medial mitral annulus,
inspiratory hepatic vein diastolic flow reversals suggest whereas the transmitral E is tall and narrow but the tissue
restrictive cardiomyopathy. The propagation velocity of e' is reduced (< 7 cm/s) in restrictive cardiomyopathy.
early diastolic transmitral flow on color M-mode is normal The usually positive linear relation between E/e' and left
or increased and is often >100 cm/s. atrial pressure is reversed in many patients with CP since
Fig. 68.14: Doppler echocardiography of the hepatic vein in a

patient with constrictive pericarditis. Hepatic vein diastolic flow
reversal increases with expiration (arrows), due to ventricular inter-
action and the dissociation of intracardiac and intrathoracic pres-
sures.
A B
Figs 68.15A and B: Tissue Doppler velocities in a patient with constrictive pericarditis. There are normal lateral (A) and supranormal
septal (B) early diastolic tissue velocities. Note the “annulus reversus” sign with higher septal than lateral early diastolic velocity. This
sign is likely due to tethering of the lateral atrioventricular groove to the thickened pericardium.
medial e' increases progressively as the severity of are preserved and longitudinal mechanics are reduced in
constriction becomes worse; this has been called “annulus restrictive cardiomyopathy. However, there could be reduced
paradoxus”.44 In addition, lateral mitral annulus e’ is regional longitudinal mechanics in CP due to tethering of the
usually lower than e' from the medial annulus in patients involved pericardium.46
with CP; this “annulus reversus” is thought to be due to In those situations where echo findings are equivocal,
the tethering of the lateral atrioventricular groove to the additional imaging testing (CT or MR) is needed to make
thickened pericardium45 (Figs 68.15A and B). Differences the diagnosis with greater confidence. In some patients,
in longitudinal and circumferential deformation may hemodynamic cardiac catheterization may be necessary
distinguish CP from restrictive cardiomyopathy. Thus, to establish the diagnosis. Even when the diagnosis of CP is
circumferential strain, torsion, and early diastolic untwisting certain after an echo, other imaging tests are often necessary
are reduced, and longitudinal strain, displacement, and early to evaluate pericardial inflammation, coexisting myocardial
diastolic tissue velocities are unchanged in constriction, disease, or comprehensive pericardial as well as cardio-
whereas circumferential strain and early diastolic untwisting vascular anatomy for subsequent management decisions.47
A B
Figs 68.16A and B: Two-dimensional (2D) echocardiogram (A) and M-mode of effusive constrictive pericarditis from the parasternal
view. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
EFFUSIVE-CONSTRICTIVE Metastatic neoplasia is the leading cause of pericardial

disease in hospitalized patients, most often in patients with
PERICARDITIS lung or breast cancer, melanoma, lymphoma, and acute
Effusive–CP is an uncommon syndrome of pericardial leukemia. Many cases are asymptomatic and are found only
constraint with clinical and pathophysiological features incidentally at autopsy, but others cause symptoms and may
of both cardiac tamponade and chronic CP. Idiopathic progress to cardiac tamponade. Primary cardiac tumors may
pericarditis, radiation, and neoplasia are the most invade the pericardium directly51,52 (Figs 68.17A and B).
frequent antecedents. Unlike the predominant parietal Primary mesothelioma of the pericardium is a rare and
involvement in chronic CP, involvement of the visceral as highly lethal tumor. Signs and symptoms are nonspecific,
well as the parietal pericardium (epicarditis) is involved in and echocardiography is insensitive for its detection; CT
effusive–CP with inflammation, fibrotic thickening, and and magnetic resonance imaging (MRI) are the most
variable degrees of myocardial adherence.48 In a series of promising diagnostic tests. Other primary tumors of the
190 patients undergoing pericardiocentesis for cardiac pericardium are quite rare.
tamponade, the disorder was diagnosed in 15 (8%).49 Pericardial tumors may appear as nodular echodensities
The diagnosis was defined by a failure of the right atrial or diffuse pericardial thickening, the latter more common
pressure to fall by 50% or to a level below 10 mm Hg after with malignant tumors. They are usually nonmobile
pericardiocentesis. In these patients, constriction was although they may contain mobile elements. Benign lesions
clinically suspect in 7; symptoms were usually present (teratomas, hemangiomas, and lymphangiomas) can appear
for less than 3 months, right-heart failure was evident in as cystic masses with septations. The pericardium may be
all, evidence of acute pericarditis was noted in 7, pulsus thickened and cause constriction; less commonly, effusive–
paradoxus was noted in 10, and pericardial calcification CP occurs. Echocardiography rapidly and accurately
was present in none. detects pericardial effusion, identifies metastatic lesions,
Noninvasive imaging is not very useful in the diagnosis and provides evidence for cardiac compression. Delayed-
of effusive–CP.50 The echo findings of effusive–CP depend enhancement contrast MRI is particularly useful in evaluating
on the stage of the disease, although most often the pericardial mass lesions.
M-mode, 2D, and Doppler features are consistent with
a moderate or large pericardial effusion and cardiac
tamponade. The pericardial effusion may become
CONGENITAL ANOMALIES
organized; echogenic and fibrinous strands may result in Pericardial cysts are usually rare remnants of defective
regions of loculation (Figs 68.16A and B). embryological development of the pericardium. In
A B
Figs 68.17A and B: A pericardial metastasis near the right ventricle (A) is seen in patient with widely metastatic lymphoma in the
subcostal window. Definity contrast enhancement (B) opacifies the right ventricle (RV) blood pool. (L: Liver).
A B
Figs 68.18A and B: A pericardial cyst (arrow) is seen adjacent to the right ventricle. Color Doppler flow (B) within the right atrium
demonstrates normal vena caval flow but a lack of flow within the pericardial cyst. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
contrast, hydatid cysts are infectious in nature. Cysts pericardial diverticula may communicate with the
vary greatly in size and are most commonly found in the pericardial space and change in size depending on body
right cardiophrenic angle. Cysts are benign and produce position and respiration.
no local or general symptoms; their importance lies in Congenital absence of the pericardium is a rare
differentiation from neoplasm. Although they can be condition that may be isolated or associated with other
demonstrated echocardiographically as echolucent cardiac and noncardiac abnormalities, and pericardium
spaces that do not communicate with the pericardial sac may be completely or partially absent. Complete absence
on echocardiography, the nature of the lesion usually is of the left pericardium is the most common variant.
confirmed by CT (Figs 68.18A and B). Echocardiographic and Doppler features of congenital
Pericardial diverticula are very rare and can be absence of the pericardium include unusual imaging
congenital or acquired malformations found most often windows, enlargement of the RV, excessive cardiac motion,
at the costophrenic angles. Unlike pericardial cysts, and abnormal motion of the interventricular septum.53
2. Edler I, Gustafson A, Karlefors T, et al. Ultrasound

cardiography. Acta Med Scand. 1961;370:68–74.
3. Feigenbaum H. Echocardiographic diagnosis of pericardial
effusion. Am J Cardiol. 1970;26(5):475–9.
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J Am Soc Echocardiogr. 2008;21(1):7–13.
5. Little WC, Freeman GL. Pericardial disease. Circulation.
2006;113(12):1622–32.
6. Spodick DH. Macrophysiology, microphysiology, and
anatomy of the pericardium: a synopsis. Am Heart J.
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7. Hoit BD, Lew WY, LeWinter M. Regional variation in
pericardial contract pressure in the canine ventricle. Am
J Physiol. 1988;255:H1370–1377.
8. Hoit BD. Diseases of the Pericardium. In: Fuster V, Walsh
RA, editors. Hurst’s The Heart. 13th ed. New York: McGraw-
Fig. 68.19: MRI-delayed enhancement sequence with gadolinium Hill; 2011: 1917–39.
contrast demonstrates a diffusely enhancing (arrows), thickened 9. Lange RA, Hillis LD. Clinical practice. Acute pericarditis. N
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10. Imazio M, Cecchi E, Demichelis B, et al. Indicators
of poor prognosis of acute pericarditis. Circulation.
MULTIMODALITY IMAGING OF THE 2007;115(21):2739–44.
PERICARDIUM 11. Cheitlin, MD, Armstrong, WF, Aurigemma, GP, et al.
ACC/AHA/ASE 2003 guideline for the clinical application
Although echo remains as usually the initial imaging of echocardiography. Available at: www.acc.org/
test for pericardial disorders because of its ease of use, qualityandscience/clinical/statements.htm. Accessed
wide availability, bedside availability, cost-effectiveness, February 2013.
12. Weitzman LB, Tinker WP, Kronzon I, et al. The incidence
and versatility, additional imaging modalities may be and natural history of pericardial effusion after cardiac
necessary. In general, CMR provides excellent anatomical, surgery–an echocardiographic study. Circulation. 1984;
tissue characteristics and some physiological data. 69(3):506–11.
Compared to echo, CMR provides superior anatomical 13. Kuvin JT, Harati NA, Pandian NG, et al. Postoperative
assessment with better spatial resolution and a larger field cardiac tamponade in the modern surgical era. Ann Thorac
Surg. 2002;74(4):1148–53.
of view, but physiological assessment is more limited. 14. Sagristà-Sauleda J, Mercé J, Permanyer-Miralda G, et al.
Specifically, CMR provides diagnostic identification of Clinical clues to the causes of large pericardial effusions.
pericardial edema from short tau inversion [T2 double Am J Med. 2000;109(2):95–101.
inversion recovery (DIR) sequence or short inversion time 15. Haaz WS, Mintz GS, Kotler MN, et al. Two dimensional
inversion recovery (STIR) sequence] and inflammation echocardiographic recognition of the descending thoracic
aorta: value in differentiating pericardial from pleural
from late gadolinium enhancement (LGE) sequences, effusions. Am J Cardiol. 1980;46(5):739–43.
which are not available with other imaging modalities54 16. Mahabadi AA, Massaro JM, Rosito GA, et al. Association
(Fig. 68.19) Cardiac CT is a predominantly anatomical of pericardial fat, intrathoracic fat, and visceral abdominal
modality with superior definition of pericardial fat with cardiovascular disease burden: the Framingham
anatomy relative to surrounding chest structures and Heart Study. Eur Heart J. 2009;30(7):8506.
17. Sagristà-Sauleda J, Angel J, Sambola A, et al. Low-pressure
pericardial calcification. It is, therefore, used in situations cardiac tamponade: clinical and hemodynamic profile.
where anatomy is incompletely defined by echo or a Circulation. 2006;114(9):945–52.
contraindication for CMR—for example, in a patient 18. Di Segni E, Feinberg MS, Sheinowitz M, et al. Left
with suspected absence of the pericardium or a patient ventricular pseudohypertrophy in cardiac tamponade: an
with a pacemaker. CT may also be useful for preoperative echocardiographic study in a canine model. J Am Coll
Cardiol. 1993;21(5):1286–94.
planning once pericardectomy is indicated especially in 19. Himelman RB, Kircher B, Rockey DC, et al. Inferior vena
patients with a previous sternotomy.47 cava plethora with blunted respiratory response: a sensitive
See Movie clips 1–3 under Chapter 68 in DVD. echocardiographic sign of cardiac tamponade. J Am Coll
Cardiol. 1988;12(6):1470–7.
20. Singh S, Wann LS, Klopfenstein HS, et al. Usefulness of
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28. Leeman DE, Levine MJ, Come PC. Doppler echo- of respiratory variation in mitral inflow velocity. J Am Soc
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CHAPTER 69
Three-Dimensional Echocardiographic
Assessment in Pericardial Disorders
O Julian Booker, Navin C Nanda
Snapshot

Two-Dimensional Transthoracic Echocardiography 
Two-Dimensional Transthoracic Echocardiography
Versus Three-Dimensional Transthoracic Versus Three-Dimensional Transthoracic
Echocardiography in Pericardial Effusion Echocardiography in Pericardial Masses

Two-Dimensional Transthoracic Echocardiography
Versus Three-Dimensional Transthoracic
Echocardiography in Constric on
INTRODUCTION to fix the heart within the chest. The nature and position of
the fibrous pericardium also make it an important barrier
The pericardium is a relatively avascular fibroserous sac. to infection. Pericardial constraint allows for greater
It is created by invagination of the heart within the serous isovolumic pressures at any given volume for both the
pericardium and surrounded by the fibrous pericardium. left and the right ventricles. These benefits of constraint
The serous pericardium is composed of a single layer are curbed by the absence of the pericardium.2–4 Further
of mesothelial cells. The layer adherent to the fibrous possible roles of pericardial constraint include preventing
pericardium is termed the parietal layer. The layer deep to acute increases in chamber volume as a response to
the fibrous pericardium is called the visceral pericardium. elevations in filling pressures. It also helps modulate
Those portions of the visceral pericardium that are the physical and hydrodynamic interaction between
adherent to the myocardium are specifically called the the four cardiac chambers5,6 and creates ventricular
epicardium. The fibrous pericardium is much sturdier interdependence.7,8
than the serous pericardium but is still relatively thin There are anatomical and hemodynamic consequences
(< 2 mm) and surrounds the heart and part of the great to pericardial disease. The cardiac chambers are physically
vessels.1 Between the serous layers, there is a potential connected within a confined space within the pericardium.
space that contains 15 to 50 mL of fluid that serves There is also hydrodynamic interdependence related to
as lubrication. Most of the fluid is located within the blood flow. Changes in the filling dynamics of one chamber
interventricular and atrioventricular grooves with a will impact the others. Tamponade and constriction are
predilection for more dependent areas. primarily hemodynamic entities resulting from abnormal
The pericardium has a unique interaction with heart. pericardial constraint. Echocardiography, with its ability
The fibrous pericardium attaches to the diaphragm, to not only evaluate structure but also hemodynamic
anterior mediastinum, and sternum. The pericardium assessment, is the cornerstone of the evaluation of
surrounds the proximal portions of the great vessels, which pericardial disorders. The standard evaluation involves
serve as additional anchor points. These attachments serve Doppler interrogation and two-dimensional (2D) imaging.
Chapter 69: Three-Dimensional Echocardiographic Assessment in Pericardial Disorders 1453
Over the past decade, more powerful computer pro- can help define potential structural abnormalities. Given
cessors have resulted in larger quantities of data that can the importance of echocardiography in the evaluation of
be processed quickly. Development of a full matrix array pericardial diseases, the potential incremental benefit of
has evolved echocardiography from an approximately additional 3D images cannot be overlooked. Some of the
5 mm slice to a nearly full volumetric acquisition of the potential diagnostic advantages of 3D echocardiography
heart and its immediately surrounding structures using a can be seen in Table 69.1 and Figures 69.1 to 69.11.
gated, multiple-beat acquisition. Three-dimensional (3D)
technology provides the ability to manipulate and crop the TWO-DIMENSIONAL TRANSTHO-
data set in an almost infinite number of orientations and
provides a more complete assessment of cardiac anatomy
RACIC ECHOCARDIOGRAPHY VERSUS
including the pericardium. With high-resolution images, THREE-DIMENSIONAL TRANSTHO-
the surfaces of the two layers can be viewed en face.9 This RACIC ECHOCARDIOGRAPHY IN
approach allows for a more complete structural assessment
than two-dimensional transthoracic echocardiography PERICARDIAL EFFUSION
(2D TTE) alone. 2D TTE is a sensitive, but not very specific method of
Publications regarding three-dimensional transthorac- identifying pericardial effusion.10 On 2D and M-mode
ic echocardiography (3D TTE) in the evaluation of pericar- imaging, pericardial fluid will appear as a hypoechoic
dial disease have been limited. Three-dimensional echo- space with hyperechoic structures, the epicardium and
cardiography’s more complete anatomical visualization pericardium, respectively, on either side. The planar
Table 69.1: Advantages of 3D TTE* in Evaluating Pericardial Diseases

• Anatomy of pericardial layers
– Parietal and visceral layers can be visualized en-face
– Identification of extent of fibrin deposits, exudative coating over pericardial layers
– Better visualization of fibrin strands—extension, attachments, mobility, and consistency
• Pericardial effusion
– Better identification of the echo reflectors within the fluid
– Ability to comprehensively assess effusion behind the atria and right ventricle
– More comprehensive identification of loculated effusions
– Increased accuracy of size estimation
• Pericardial hematomas
– Easy identification and differentiation from uncomplicated effusion
• Granulomatous disease
– Ability to identify the central necrosis and hence the pathological characterization as granuloma
– Ability to identify tethering of pericardium
• Tumor/mass
– More accurate identification of pericardial deposits
– Provides clue to identification of malignant nature by identifying the inhomogeneous nature
– Identification of extracardiac extension
• Constrictive pericarditis
– More comprehensive evaluation of extent and severity
– Identification of morphological type
Contd...
Contd...
• Differentiation of pericardial effusion from pleural effusion and ascites

– En-face viewing of the falciform ligament to better identify ascites
• Pericardiocentesis
– Better needle visualization with its pyramidal imaging plane
• Pericardial cyst
– Identification of loculations and trabeculations
*3D TTE, live/real time three-dimensional transthoracic echocardiography.

Source: Reproduced with permission from Sudhakar S, Nanda NC. Role of live/real time three-dimensional transthoracic echocardi-
ography in pericardial disease. Echocardiography. 2012;29:98–102.
A B
C D
Figs 69.1A to D: Live/real-time three-dimensional transthoracic echocardiography in pericardial effusion in a 64-year-old female with
renal failure. (A) The arrowhead points to fibrin deposition over the right ventricular pericardium giving a rugged appearance. Pericardial
effusion extends behind both atrial (see also Movie clip 69.1A). (B) Apical four-chamber view. Cropping from bottom displays a smooth
visceral pericardium over the basal left atrial (LA) wall (arrowhead; see also Movie clip 69.1B). (C) Subcostal examination. Arrowheads
show multiple fibrin deposits on the right atrial (RA) visceral pericardium resulting in a rugged appearance (see also Movie clip 69.1C).
(D) Arrowhead shows a flap-like fibrin mass (see Movie clip 69.1D parts 1 and 2). (IVC: Inferior vena cava; LV: Lleft ventricle; PE: Pe-
ricardial effusion; TV: Tricuspid valve).6
Source: Reproduced with permission from Ref. 10.
nature of standard 2D imaging can make distinguishing result is that pericardial effusions will be found anterior to
pericardial effusions from ascites or pleural effusion the descending aorta. 3D TTE can be used better to identify
difficult. The falciform ligament, which appears as a linear the pericardial space in relation to the aorta. Similarly,
band stretching from the liver to the abdominal wall, can visualization of atelectatic lung can help differentiate
be used to identify ascites. However, the linear nature of the pleural and pericardial effusions (Fig. 69.3).
ligament can make it difficult to distinguish from fibrinous 2D TTE’s ability to quantify pericardial effusions is
strands that can sometimes be seen within the pericardial unreliable. Estimation of pericardial volumes using the
space. With 3D TTE, the falciform ligament more closely posterior free space11 does not adequately account for
approximates its appearance in vivo and can be seen as a loculated effusions or asymmetry related to postural
sheet-like structure (Fig. 69.2C). shifts. 3D TTE can acquire the entire pericardial space in a
2D TTE’s ability to delineate pleural effusion can be single acquisition. By identifying the visceral and parietal
equally difficult. The parasternal long axis view is the most borders, pericardial fluid volumes can be quantified
frequently used to identify pleural effusion. The oblique without geometric assumptions. 3D TTE has proven
sinus represents the reflection of the pericardium. The itself to be more accurate and reproducible than 2D TTE
A B
Figs 69.2A to C: Live/real time three-dimensional transthoracic
echocardiography in pericardial effusion in an 85-year-old female
with congestive heart failure and ascites. (A) Upper and lower
arrowheads point to visceral and parietal layers of the pericardium,
respectively. Both the pericardial layers are smooth without fibrin
deposition (see also Movie clip 69.2A); (B) Right parasternal
examination. The arrowhead points to a smooth visceral pericardium
overlying the right (RA) and left (LA) atrium (see also Movie clip
2B); (C) The arrowhead shows a localized collection of fibrin over
the visceral pericardium of the left ventricular (LV) free wall (see
also Movie clip 69.2C). Movie clip 69.2D shows pericardial effusion
(arrowhead) extending behind the left atrium. The arrowhead
in Movie clip 69.2E points to three-dimensional images of the
falciform ligament which is a useful landmark in distinguishing
ascites from associated pericardial effusion. It appears as a sheet
of tissue connected to the liver rather than a linear echo mimicking
a fibrin strand seen with conventional two-dimensional imaging.
The arrowhead in Movie clip 69.2F points to the falciform ligament
viewed en-face in another patient with ascites. (D: Diaphragm; DA:
Descending thoracic aorta; IVC: Inferior vena cava; L: Liver; RV:
Right ventricle).
C Source: Reproduced with permission from Ref. 10.
Fig. 69.3: Live/real time three-dimensional transthoracic echo- Fig. 69.4: Live/real time three-dimensional transthoracic echo-
cardiography in an 84-year-old male with renal failure, and cardiography in a 62-year-old male with pericardial effusion
pericardial and left pleural effusions. Cropping of the three- developing following mitral valve replacement. Arrowheads point
dimensional data set using Qlab software analysis package to fibrinous strands connecting the visceral and parietal portions of
revealed a rugged visceral (1, 2, arrowhead) and parietal the pericardium. The accompanying Movie clips 69.4A and B from
(3) pericardium as well as a rugged visceral (4) and parietal the same patient show both mobile and nonmobile fibrin strands. In
(5) pleura, from fibrin deposits (see also Movie clip 69.3A). Movie Movie clip 69.4A, artifacts appeared when the instrument gain was
clips 69.3B and C show similar findings using regular cropping in decreased and disappeared with increase in gain, emphasizing
the same patient. Movie clips 69.3D and E are from a different the importance of optimizing gain settings when assessing three-
patient with pleural effusion, shown for comparison. These show dimensional images. Movie clip 69.4B shows cropping done using
cropping to more comprehensively assess the extent of pleural the Qlab software analysis package. Abbreviations as in previous
effusion (PLE) and the collapsed lung (arrowhead) and their figures.
relation to the heart (H). Movie clip 69.3F from another patient Source: Reproduced with permission from Ref. 10.
with chronic renal failure shows the attachment of collapsed lung
lobes (horizontal arrowheads) to the hilum (vertical arrowheads)
and its relationship to the heart (H). This patient had previously critically important in the evaluation of pericardial
undergone pericardiectomy for constriction. Both these patients
effusions. 3D TTE has shown to be superior to 2D TTE in
were studied from the back in the sitting position. (DA: Descending
thoracic aorta; PP: Parietal pericardium; VPL: Visceral pleura; identifying and characterizing the consistency, location,
VP: Visceral pericardium). and mobility of fibrinous material within the pericardial
Source: Reproduced with permission from Ref. 10. space (Figs 69.1 and 69.4). Fibrin stranding and other
echogenic pericardial materials are most often associated
in quantifying asymmetric pericardial effusions.12 The with inflammatory processes. 3D TTE’s ability to define
potential benefit for serial evaluations of effusion size these echo reflectors can help identify more complicated
cannot be overstated. effusions such as hematomas and purulent effusions
3D TTE has improved visualization of the inferior vena (Figs 69.5 and 69.6).9,16 Should pericardiocentesis be
cava, right ventricle, and right atrium. Classic changes required, the 3D TTE provides superior anatomical
often found in tamponade such as a plethoric inferior delineation to guide pericardiocentesis.17
vena cava diastolic collapse of the right ventricle as well
as inappropriate collapse of the atria can be seen by both TWO-DIMENSIONAL TRANSTHO-
2D and 3D TTE. However, these findings can be better RACIC ECHOCARDIOGRAPHY VERSUS
appreciated on 3D TEE providing incremental benefit in
the assessment of tamponade.13 THREE-DIMENSIONAL TRANSTHO-
Fibrin deposits have been documented in virtually RACIC ECHOCARDIOGRAPHY IN
every type of pericardial disease. Echogenic material seen
CONSTRICTION
within the pericardial space has been associated with
worse clinical outcomes like recurrence of pericardial Fibrinous adhesions are a result of the inflammatory
effusions and constrictive pericarditis.14,15 As such, process within the pericardial space. Fibrinous adhesions
identifying the presence of these strands becomes of the visceral and parietal pericardia can be found at the
Fig. 69.5: Live/real time three-dimensional transthoracic echo- Fig. 69.6: Live/real time three-dimensional transthoracic echocar-
cardiography in a 50-year-old male status post cardiac trans- diography in a 17-year-old male with a bullet injury and subse-
plantation and pericardial effusion compressing the right atrium. quent development of pericardial hematoma. The red dots outline
The arrowhead points to the compressed right atrium (RA). Crop- a loculated component of a very large pericardial hematoma (see
ping reveals no significant echo reflectors (“solid tissue”) in the also Movie clip 69.6A). Movie clip 69.6B shows a huge pericardial
effusion suggesting that it mainly comprises fluid or represents a hematoma (arrowhead) with large multiple echolucencies consist-
hematoma that is of uniform, homogenous consistency (see also ent with fluid collections. These were not well seen on two-dimen-
Movie clip 69.5). The patient improved after drainage of 1,000 cc sional imaging. (Abbreviations as in previous figures).
of yellowish fluid from the pericardial cavity using a pigtail catheter. Source: Reproduced with permission from Ref. 10.
(Abbreviations as in previous figures).
time of necropsy without any history of overt pericardial accurately delineate the thickness and calcification of the
disease.18 Constrictive pericarditis is the condition where involved pericardium.
the parietal and/or visceral pericardium become thickened Determining the extent of pericardial involvement
and sometimes calcified to the point where it inhibits by echocardiography can be unreliable with 2D imaging.
filling of the cardiac chambers. Constrictive pericarditis is 3D TTE has shown the ability to identify pericardial
a difficult-to-diagnose condition. The primary diagnosis of involvement along the entire extent of both ventricles.9,19
constriction is made by hemodynamic assessment, either An excellent example can be found in a woman with
by echocardiography or simultaneous right and left heart recurrent heart failure symptoms. Her 2D TTE showed
catheterizations. limited calcification of the posterior wall of the left ventricle
Unfortunately, there are no good medical treatments and anterior free wall of the right ventricle. 3D TTE showed
for chronic constrictive pericarditis as the mechanism extensive involvement of the regions in question and was
of the constrictive physiology is structural. Because suggestive of a severe constrictive process (Fig. 69.11).
pericardiectomy is difficult and associated with significant These findings were corroborated with cardiac MRI and
morbidity, it is paramount that the diagnosis is correct later confirmed at the time of surgery.10 Similar to MRI,
before proceeding. However, many clinicians prefer 3D echocardiography can demonstrate tethering of the
additional evidence in the form of imaging, such as affected pericardium to the adjacent myocardium. These
thickening or calcification of the pericardium to support characteristics of 3D TTE may make it an ideal third option
the diagnosis before committing the patient to surgery. for morphological assessment of pericardium in surgical
Historically, the imaging modalities of choice for planning. The fact that the 3D imaging can be acquired at
morphological assessment of the pericardium have the time of the standard 2D echocardiographic evaluation
been magnetic resonance imaging (MRI) or computed of constriction may someday make 3D TTE the preferred
tomography scan (CT). They have been shown to choice over MRI and CT.
TWO-DIMENSIONAL TRANSTHO- mass lesions. Manipulation of the 3D data set allows a

more complete visualization of pericardial-based masses.
RACIC ECHOCARDIOGRAPHY VERSUS Systematic cropping can provide information about the
THREE-DIMENSIONAL TRANSTHO- character of a mass. Identifying inhomogeneity within
RACIC ECHOCARDIOGRAPHY IN a mass can provide clues to its nature. Conversely, an
echolucent lesion without flow on Doppler is likely to be a
PERICARDIAL MASSES benign pericardial cyst (Fig. 69.10).
Perhaps the greatest benefit of 3D TTE over 2D TTE in the 3D TTE has been used successfully in a case series
evaluation of pericardial disease lies in the evaluation of to provide vital clues as to the pathology of several
A B
C D
Figs 69.7A to D: Live/real time three-dimensional transthoracic echocardiography in a 26-year-old male with tuberculous pericardial
effusion. (A) Cropping of the apical four-chamber data set shows no inward motion of the proximal right ventricular free wall (arrowhead)
during systole, probably due to fibrinous adhesions. The distal wall contracts well; (B) Examination of visceral pericardium (VP) over
the ventricles demonstrates a mildly rugged appearance. Movie clips 69.7A and B Parts 1 to 3 show the cropping technique used to
demonstrate the visceral pericardium of both ventricles; (C) A large mass (arrowhead) is seen involving the visceral pericardium of the
left ventricle (LV). The etiology is not clear but it could possibly represent a tuberculous granuloma; (D) The arrowhead points to a large
highly echogenic mass involving the right ventricular visceral pericardium consistent with a calcified granuloma in another patient with
tuberculosis. Movie clip 69.7C from the same patient shows a granuloma (arrowhead) involving the parietal pericardium. Movie clips
69.7D and E are from a different patient with purulent pericardial effusion due to methicillin-resistant Staphylococcus aureus. Movie clip
69.7D shows markedly thickened and echogenic parietal (upper arrowhead) and visceral (lower arrowhead) pericardium. Movie clip
69.7E shows an abnormal loculated appearance of the visceral pericardium (arrowhead) when viewed en-face by cropping from the
apex. (Abbreviations as in previous figures).
A B
Figs 69.8A and B: Live/real time three-dimensional transthoracic echocardiography in a 66-year-old male with pericardial metastasis
from a malignant thymoma. (A) The arrowhead shows a huge pericardial mass (bounded by red dots) measuring 9.2 × 6.3 cm. Movie
clips 69.8A to C show the full extent of this huge mass with three-dimensional imaging. Cutting open the tumor in its mid portion (arrow-
head in Movie clip 69.8B) revealed solid inhomogenous tissue. Arrowheads in Movie clip 69.8C show parietal involvement of the tumor
by multiple band-like extensions. The two-dimensional study in this patient (Movie clip 69.8D) shows a much smaller mass (arrowhead)
measuring 3.8 × 1.5 cm attached to the right ventricular outflow tract visceral pericardium; (B) Surgical specimen. (Other abbreviations
as in previous figures).
A B
Figs 69.9A and B: Live/real time three-dimensional transthoracic echocardiography in a 68-year-old male with lung carcinoma. (A and B)
The arrowhead points to an irregular mass in the parietal pericardium (see also Movie clips 69.9A and B). The etiology is not clear,
but it could possibly represent a pericardial metastasis. Movie clip 69.9C shows another mass (arrowhead) located over the visceral
pericardium (VP) in this patient. This type of mass (arrowhead) was also seen by two-dimensional imaging (Movie clip 69.9D). However,
the other mass seen involving parietal pericardium (PP) was not detected by this modality. Movie clip 69.9E is from a different patient
with a poorly differentiated lung adenocarcinoma. Arrowhead points to a mass in the parietal pericardium consistent with metastasis.
This was not visualized on two-dimensional imaging. Examination of pericardial fluid in this patient showed the presence of malignant
cells. (Other abbreviations as in previous figures).
masses. In one case, the echolucent core was suggestive a calcified granuloma (Fig. 69.7D). The utility of 3D TTE
of granuloma in a patient with confirmed tuberculosis is never more apparent than when evaluating masses in
(Fig. 69.7C). A second case of tuberculosis was found to the setting of metastatic disease. In a patient with a known
have a highly echogenic pericardial mass consistent with thymoma, a 3.8- × 1.5-cm was found to be adherent to the
A B
Figs 69.10A and B: Live/real time three-dimensional transthoracic echocardiography in a 36-year-old male with a pericardial cyst. (A)
The arrowhead points to a pericardial cyst confirmed by a CT scan of the chest (see Movie clip 69.10A). (B) Cropping of the three-
dimensional data set demonstrates multiple band-like tissue (arrowhead) within the cyst (see Movie clip 69.10B). Movie clips 69.10C
and D represent two-dimensional images which do not show multiple bands criss-crossing the cyst (arrowhead). (L: Liver). (Other
abbreviations as in previous figures).
A B
Figs 69.11A to C: Two-dimensional and live/real time three-
dimensional transthoracic echocardiography in a 53-year-
old female with constrictive pericarditis. (A) Two-dimensional
study. Arrowheads in the parasternal long-axis view point to
an echogenic left ventricular posterior wall consistent with
calcification (see also Movie clip 69.11A); (B) Represents a three-
dimensional study using Qlab software analysis package. The
arrow demonstrates a highly echogenic posterior pericardium
consistent with calcification. When this was cropped transversely
using an oblique cropping plane, widespread involvement of the
left ventricular posterior wall with calcification (arrowhead) was
evident. Highly echogenic calcification is visualized anteriorly also.
Top and bottom arrowheads in the left upper quadrant in Movie clip
69.11B point to anterior and posterior calcification, respectively.
The patient underwent pericardiectomy. (AO: Aorta; MV: Mitral
valve). (Other abbreviations as in previous figures).
C Source: Reproduced with permission from Ref. 10.
visceral pericardium. 3D TTE later demonstrated that the 6. Spodick DH. Threshold of pericardial constraint: the
mass was actually much larger, measuring 9.2 × 6.3 cm pericardial reserve volume and auxiliary pericardial
functions. JACC. 1985;6(2):296–7.
(Fig. 69.8). Moreover, the lesion was both solid and
7. Bove AA, Santamore WP. Ventricular interdependence.
inhomogeneous, consistent with tumor. These dimensions Prog Cardiovasc Dis. 1981;23:365–88.
correlated closely with the pathological specimen at the 8. Santamore WP, Shaffer T, Papa L. Theoretical model of
time of surgery.9 ventricular interdependence: pericardial effects. Am J
Physiol. 1990:259.
9. D’Cruz IA, Khouzam RN, Minderman D. Three-dimensional
CONCLUSION echocardiographic appearances of pericardial effusion
Pericardial diseases vary widely in their nature and with tamponade. Echocardiography. 2007;24(2):162–5.
10. Hernandez CM, Singh P, Hage FG, et al. Live/real time three
consequences. Although 2D TTE is the imaging modality of
dimensional transthoracic echocardiographic assessment
choice to evaluate pericardial conditions, its planar nature of pericardial disease. Echocardiography. 2009;26:1250–63.
can lead to incomplete visualization of the pericardium. 11. Horowitz MS, Schultz CS, Stinson EB, et al. Sensitivity and
3D TTE has shown benefit in both the diagnosis of specificity of echocardiographic diagnosis of pericardial
pericardial disease and guiding therapeutic intervention. effusion. Circulation. 1974;50:239–47.
12. Hsu FL, keefe D, Desiderio D, et al. Echocardiographic
Including a 3D data set should become part of the routine
and surgical correlation of pericardial effusion in patients
echocardiographic evaluation of pericardial disease where with malignant disease. J Thoracic Cardiovasc Surgery.
the diagnosis is in question or if intervention is required. 1998:1215–16.
13. Kim SH, Song JM, Jung IH, et al. Initial echocardiographic
characteristics of pericardial effusion determine the
REFERENCES pericardial complications. Int J Cardiol. 2009;136(2):151–5.
1. Choi HO, Song JM, Shim TS, et al. Prognostic value of initial 14. Lee SH, Kim WH, Ko JK. Fibrinous pericardial effusion in
echocardiographic features in patients with tuberculosis a three-dimensional echocarcardiography. QJM. 2013.
pericarditis. Korean Circ J. 2010;40(8):377–86. 15. Lewinter, M. Pericardial diseases. In: Libby, editor.
2. Shabetai R, Mangiardi L, Bhargava V, et al. The pericardium Braunwald’s Heart Disease. Philadelphia: Saunders; 2008.
and cardiac function. Prog Cardiovasc Dis. 1979;22: 16. Nanda CN, Hsiung MC, Miller AP, et al. Live/Real Time 3D
107–34. Echocardiography. Wiley-Blackwell, 2010.
3. Fowler NO, Gabel M, Holmes JC. Hemodynamic effects 17. Vazquez de Prada JA, Jiang L, Handschumacher MD,
of nitro-prusside and hydralazine in experimental cardiac et al. Quantification of pericardial effusions by three-
tamponade. Circulation. 1978;57:563–7. dimensional echocardiography. J Am College Cardiol.
4. Glantz SA, Misbach GA, Moores WY, et al. The pericardium 1994;24(1):254–9.
substantially affects the left ventricular diastolic pressure- 18. Waller BF, Taliercio CP, Howard J, et al. Morphologic
volume relationship in the dog. Circ Res. 1978;42:433–41. aspects of pericardial heart disease; Part 1. Clin Cardiol.
5. Maruyama Y, Ashikawa K, Isoyama S, et al. Mechanical 1992;15(3):203–9.
interactions between four heart chambers with and 19. Zagol B, Minderman D, Munir A, et al. Effusive constrictive
without the pericardium in canine hearts. Circ Res. 1982;50: pericarditis: 2D, 3D echocardiography and MRI imaging.
86–100. Echocardiography. 2007;24:1110–14.
CHAPTER 70
Cardiac Tumors and Masses
Leon Varjabedian, Jennifer K Lang, Abdallah Kamouh, Steven J Horn, Tuğba Kemaloğlu Öz, Aylin Sungur, Kruti Jayesh
Mehta, Kunal Bhagatwala, Nidhi M Karia, Maximiliano German Amado Escañuela, Robert P Gatewood Jr, Navin C Nanda
Snapshot
Echocardiographic Assessment of Cardiac Tumors and 
Secondary Cardiac Tumors
Masses

Normal Variants and Other Masses
Primary Benign Cardiac Tumors

MICE
Malignant Primary Cardiac Tumors

Extracardiac Masses
ECHOCARDIOGRAPHIC ASSESSMENT sessile, prolapsing, shimmering). (e) Description of the

relationship to adjacent structures (intra- or extracardiac).
OF CARDIAC TUMORS AND MASSES (f ) Identification of route of access to the heart [e.g. primary
Standard two-dimensional (2D) transthoracic echo- vs secondary through the wall, pulmonary veins, inferior
cardiography (TTE) with Doppler is the initial diagnostic vena cava (IVC)]. (g) Quantification of the hemodynamic
modality of choice in a patient with a cardiac tumor or consequences of the mass (e.g. flow or outflow tract
mass. Transesophageal echocardiography (TEE) is often obstruction, valvular stenosis or insufficiency, chamber
complementary to TTE in the full assessment of patients filling or ventricular function). (h) Calculation of cardiac
with a known or suspected cardiac mass. chamber dimensions, left ventricular volumes, and
A complete echocardiographic evaluation of a cardiac ejection fraction.
tumor or mass should include: (a) Characterization of The differential diagnosis of cardiac masses includes
the shape, dimensions, and volume of the mass (e.g. tumors, thrombi, nonbacterial thrombotic endocarditis,
round vs lobulated, small vs large).1 The size of an infective endocarditis, or normal variant intracardiac
intracardiac mass has important clinical implications or extracardiac structures. Imaging features that favor a
in predicting embolic events, congestive heart failure, diagnosis of tumor are a mobile, pedunculated appearance
and death, and as an efficacy assessment after treatment and an associated pericardial effusion. Masses that cross
(anticoagulation, antibiotics, and chemotherapy).2 Nanda anatomical planes, from myocardium to pericardium or
et al. reported that 2D measurements from a transthoracic endocardium, are likely to be tumors. The clinical setting
or a transesophageal study underestimate the true and associated echocardiographic findings are also crucial
maximum diameter of irregularly shaped structures. (b) in guiding the diagnosis of a cardiac mass. For example,
Identification of the location of the tumor and type of left atrial thrombi are associated with mitral valve
attachment to the heart (e.g. pedunculated vs sessile). stenosis, enlarged left atrium, and atrial fibrillation, while
(c) Description of the echogenicity (e.g. echolucent vs ventricular thrombi are associated with cardiomyopathies
echodense, homogenous vs heterogeneous, and pre- or regional wall-motion abnormalities. Right atrial (RA)
sence of calcification). (d) assessment of mobility (e.g. thrombi are seen in the setting of indwelling catheters or
Chapter 70: Echocardiographic Assessment of Cardiac Tumors and Masses 1463
pacemaker wires. Nonbacterial thrombotic endocarditis as well as very bright reflective echodense areas with or
is found in patients with malignancy or systemic lupus without acoustic shadowing due to calcifications pointing
erythematosus. to their chronicity.7 Hemangiomas are highly vascular
The sensitivities of transthoracic and Transesophageal tumors that demonstrate more extensive and closely
echocardiogram for detection of a cardiac mass are packed vascular (echolucent) areas that extend all the
93% and 97%, respectively.3 In general, the sensitivity way to the periphery with little solid tissue as compared
of both the TTE and TEE is highest for endocardial to myxomas.8 High-grade sarcomas may demonstrate
lesions because the mass is easily distinguished from the areas of necrosis with dilated vasculature (echolucencies)
echolucent chamber, while the sensitivity is slightly lower surrounded by dense hyperechoic band-like tissue
for intramyocardial lesions and lowest for pericardial consistent with fibrosis, thus giving the appearance of a
tumors. TTE offers a superior acoustic window for the left “doughnut”.9
ventricle (LV) and, therefore, is more sensitive in detecting On 3DE, papillary fibroelastomas are characterized
a left ventricular tumor. On the other hand, TEE has the by a central echodensity that corresponds to their
advantage of providing better resolution for valvular and fibrocollagenous core and finger-like projections, which
posterior structures that are distant from the anterior chest represent multiple fronds.10 The stalk by which this tumor
wall, such as the left and right atria, superior vena cava attaches to the valve can also be well seen. They may be
(SVC), and the descending thoracic aorta. Because of its difficult to differentiate from Lambl’s excrescences, which
superior image resolution and transesophageal approach, are also attached to the tip of the valve leaflets. Lambl’s
intraoperative TEE has proven extremely useful to the excrescences, however, usually present as multiple strands
surgeon for the guidance of tumor resection, particularly on multiple valves and tend not to be discreet rounded
in cardiac sarcoma. Increasingly, TEE is also used to aid lesions.
cardiac biopsy and guide surgical intervention, helping to When TTE and TEE cannot provide definitive answers
ensure that there is no residual tumor and that the repaired regarding the presence or absence of vegetations, a
structures are free of defects. three-dimensional transthoracic echocardiography (3D
A concerning limitation of assessing cardiac masses TTE) might prove useful.11 3DE is particularly helpful
with 2D echocardiography (2DE) is the possibility of in visualizing all three leaflets of the tricuspid valve, the
actually “missing” the mass during the evaluation due right atrium, the right ventricle (RV), and any indwelling
to complex geometric shapes. This can sometimes be catheters to assess for the presence of vegetations.
overcome by employing nonstandard views. Three- Three acquisition modes are used with real time 3D
dimensional echocardiography (3DE) has an important echocardiography (RT3DE) in the evaluation of cardiac
advantage over 2DE in its ability to show 3D structures tumors: (a) full volume, (b) live 3D, and (c) 3D zoom (a
rather than the need to conceptualize these complex smaller, magnified pyramidal data at a higher resolution).12
shapes from multiple cross-sectional 2D images.4 Full-volume acquisitions can be obtained from the
3DE has an additional advantage in evaluating parasternal, apical four-chamber, apical two-chamber,
cardiac tumors because of its ability to locate the precise and subcostal views. However, the availability of more
attachment of a mass to the myocardium, to section the data in these large pyramids of information comes at
mass and view it from any plane, to calculate the volume of the expense of lower image resolution. Hence, imaging
a mass and follow this volume over time, and to accurately with narrow angles (live 3D) is recommended if high-
define the spatial relationship of the mass to other resolution images of the cardiac mass are desired. Full-
structures.2,5,6 volume acquisition allows the echocardiographer to slice
Images obtained by 3DE can be sectioned and viewed and crop the heart in as many ways as required to obtain a
from any angle, providing the examiner a more detailed comprehensive tomographic evaluation of the mass. Full-
assessment of the mass even from within. Certain masses volume and live 3D acquisitions in the bicaval view are
have distinctive and characteristic appearances that can useful in characterizing masses in the SVC, IVC, interatrial
aid in making the diagnosis. For example, fibromas and septum, and right atrium.1
lipomas typically are dense, very bright homogenous RT3DE provides a more comprehensive assessment of
masses, which relate to the presence of fibrous the interior structure of the mass that correlates better with
tissue. Myxomas demonstrate localized areas of large pathological findings (necrosis, hemorrhage, cystic areas,
echolucencies consistent with necrosis or hemorrhage or fibrotic bands).9,13 RT3DE can also enhance the ability of
the echocardiographer to detect associated abnormalities majority of the remainder originating in the right atrium.22
and conditions that predispose to the development of a Other less common locations are the ventricles (RV more
mass, such as an LV apical aneurysm or rheumatic mitral than LV), valves [usually tricuspid valve (Figs 70.2A and B;
valve disease.14,15 It helps to characterize masses in the Movie clip 70.2) more than mitral valve], and IVC.23,24
right atrium, where the differential diagnosis includes On gross examination, myxomas have a soft, gelatinous
a normal structure (prominent IVC ridge or a crista appearance with some lobulation.25 They may contain
terminalis), embryonic remnants (prominent Eustachian areas of hemorrhage (Figs 70.3 to 70.5; Movie clip 70.3)5
valve or a Chiari network), thrombus, or a tumor arising and calcification (Figs 70.6A to C) and generally range
from the IVC.16,17 Therefore, full assessment of patients between 5 and 6 cm in dimension. Figure 70.6D explains
with a cardiac mass or tumor requires a comprehensive why, unlike 3DE, the size of a cardiac mass including
TTE that may need to be supplemented with TEE and or myxomas can be frequently underestimated by two-
3DE as well as cardiac computed tomography (CT) and dimensional TTE as well as multiplanar two-dimensional
magnetic resonance imaging (MRI). TEE.
3D TTE can assess the size of cardiac masses and The classic triad of symptoms relates to the obstructive,
describe the complex anatomy of the heart.18 If a cardiac embolic, and constitutional effects of the tumor.26,27
lesion is identified, chest CT with contrast enhancement However, myxomas are often asymptomatic or associated
and cardiac magnetic resonance imaging (CMR) with with no specific symptoms or clinical findings (e.g.
contrast are superior modalities for characterization incidental findings of a murmur on physical examination).
of the lesions and delineation of the extent of tumor Myxomas have female gender preponderance. No signi-
involvement. They can also help exclude the possibility ficant difference exists between male and female patients’
of direct cardiac extension of a tumor that originates age (32 ± 22.39 years, 43.75 ± 18.73 years, P = 0.1692).28
from adjacent mediastinal structures. CT and CMR are The most common origin of atrial myxoma is the
particularly good at depicting the pericardium and great interatrial septum (Figs 70.7A to F); Movie clips 70.7A and B)
vessels and evaluating the extent of disease, and CT near the fossa ovalis, accounting for 85% of the cases.29
can also detect calcification, which is important in the Other less common anatomical origins within the atria in
differential diagnosis.19 descending order of frequency are posterior and anterior
Echocardiographic contrast agents can also help walls and the atrial appendage.30,31 Atrial myxoma tends to
correctly classify intracardiac masses, differentiating be larger in size (Figs 70.8A and B),32 thereby obstructing
tumors from thrombi.20 Tissue characterization of the
mass with perfusion assessment can differentiate between
a contrast hyperenhanced (highly vascular or malignant
tumors), contrast hypoenhanced (with poor vascularity
such as myxomas), or with no enhancement (such as
avascular thrombi). Use of myocardial contrast has been
recommended by American Society of Echocardiography
Consensus statement to characterize masses.21 Other
methods including MRI can be used as complementary
techniques to evaluate intracardiac tumors.
PRIMARY BENIGN CARDIAC TUMORS

Cardiac Myxoma
Cardiac tumors are rare with an estimated incidence
Fig. 70.1: Transthoracic two-dimensional (2D) echocardiogram
of 1/100,000 per year. Albeit myxoma being the most
(apical four-chamber view) of a patient with a 5.0 × 5.6 cm
frequently encountered benign cardiac tumor. Myxomas spherical mass seen attached to the posterior roof of the left
commonly arise on the endocardial surface of all cardiac atrium representative of an atrial myxoma. (LA: Left atrium;
chambers and rarely the heart valves. Seventy-five percent LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Source: Dr Sachin Wadhawan, Buffalo Heart Group, Buffalo NY.
of myxomas occur in the left atrium (Fig. 70.1), with the
A B
Figs 70.2A and B: Transesophageal echocardiogram of a patient with a tricuspid valve myxoma. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle) (Movie clip 70.2.).
A B
C Figs 70.3A to C
D F
Figs 70.3A to G: Live/real time three-dimensional (3D) trans-

thoracic echocardiographic assessment of left atrial myxoma. (A
to C) Frontal plane sections taken at three different sequential
levels in the 3D data set demonstrate echolucencies (arrowhead)
within the tumor consistent with hemorrhage, which is largest in
the middle section (B); (D) Transverse plane sections of the tumor
viewed en face, also demonstrating hemorrhage (arrowhead);
(E) Frontal plane, transverse plane, and vertical plane sections of
the tumor showing extensive hemorrhage (arrowhead); (F) The
arrow points to the tumor stalk; (G) Resected specimen showing
a large hemorrhage. Arrows in the Movie clip 70.3 point to large
areas of hemorrhage that correspond with the surgical specimen.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral
valve; RA: Right atrium; RV: Right ventricle) (Movie clip 70.3).
Source: Reproduced with permission from Mehmood F, Nanda
NC, Vengala S, et al. Live three-dimensional transthoracic echo-
cardiographic assessment of left atrial tumors. Echocardiography.
G 2005;22(2):137–43.
A B
Figs 70.4A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of left atrial myxoma. (A) Arrowhead
points to a large echolucency consistent with a large hemorrhage. (B) Resected specimen showing a large hemorrhage. (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Mehmood F, Nanda NC, Vengala S, et al. Live three-dimensional transthoracic echocardio-
graphic assessment of left atrial tumors. Echocardiography. 2005;22(2):137–43.
A B
Figs 70.5A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of left atrial myxoma. (A) Arrowhead
points to a large echolucency, which corresponds closely to the hemorrhage seen in the resected specimen (B). (LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Mehmood F, Nanda NC, Vengala S, et al. Live three-dimensional transthoracic echocardio-
graphic assessment of left atrial tumors. Echocardiography. 2005;22(2):137–43.
A B
C D
Figs 70.6A to D: Two-dimensional (A) and live/real time three-dimensional (3D; B and C) transesophageal echocardiography.
(A) Arrow points to a myxoma in the left atrium with a broad attachment on the atrial septum. The dense areas represent calcification,
while the echolucent areas indicate the presence of hemorrhages. (B and C) The attachment of the tumor is much better delineated by
the 3D technique. (IAS: Interatrial septum; LA: Left atrium; RA: Right atrium); (D) Schematic diagram demonstrating that the maximum
dimension of an object (in this case, a cylinder) can be obtained only if the ultrasound beam cuts through its longest dimension (true
long axis) when using a multiplane probe. However, when the two-dimensional planes (dotted lines) are stacked together to obtain a 3D
image, the object (cylinder), including its long axis, can be viewed completely, even though it is not oriented parallel to the ultrasound
beam as it is rotated from 0° to 180° (Movie clips 70.6A to C).
Source: Reproduced with permission from Nanda NC et al. Incremental value of three-dimensional echocardiography over
transesophageal multiplane two-dimensional echocardiography in qualitative and quantitative assessment of cardiac masses and
defects. Echocardiography. 1995;12:619–28.
the valvular orifice. Myxomas originating from heart valves time 2DE. Some atrial myxomas can prolapse into the
are uncommon and tend to be small in size.33 mitral or tricuspid valve orifice and even into the ventricle
Atrial myxoma originating from the fossa ovalis during diastole.
protrude into the atrial cavity but remain attached to the Ventricular myxomas (Figs 70.11A to D; Movie clips
septum by a stalk (Figs 70.9 and 70.10; Movie clip 70.9). 70.11A, C Parts 1 to 3, D and E;34 Figs 70.12A to D; Movie
This stalk can be short, allowing little motion, or long, clips 70.12B–D)7 may originate on the ventricular free
therefore explaining the motion that can be seen on real wall or interventricular septum and may be sessile or
A B
C D
E F
Figs 70.7A to F: Transesophageal echocardiogram of a patient with a large left atrial septal myxoma (A to F) causing significant mitral
regurgitation (E and F). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clips 70.7A and B).
A B
Figs 70.8A and B: Two-dimensional transesophageal echocardiography in a patient with a large biatrial myxoma presenting for
operation. (A) Arrowheads point to areas of calcification within the tumor. (B) Arrowheads point to areas of hemorrhage within the tumor.
(AS: Atrial septum; AV: Aortic valve; H: Hemorrhage; LA: Left atrium; LVO: Left ventricular outflow tract; M1: Myxoma in right atrium; M2:
Myxoma in left atrium; RA: Right atrium; RVO: Right ventricular outflow tract).
Source: Reproduced with permission from Srivastava R, Hsiung MC, Fadel A, Nanda NC. Transesophageal echocardiographic
demonstration of biatrial myxoma. Echocardiography. 2004;21(2):187–8.
A B
Figs 70.9A and B: (A) Parasternal long-axis and (B) apical four-chamber views demonstrating a large right atrial myxoma attached
to the atrial wall by a stalk prolapsing through the tricuspid valve into the right ventricle. (RA: Right atrium; RV: Right ventricle) (Movie
clip 70.9).
pedunculated.35,36 Valvular myxomas are defined as geneous,40 homogeneous echogenic,38 multilobulated

a myxoma arising from different parts of the valvular mobile,41,42 well-circumscribed,42 or an irregular adherent
apparatus (valve leaflets, annulus, commissure, junction mass.
area, or subvalvular apparatus). A mitral valve myxoma, for Cardiac myxomas are generally first diagnosed by TTE,
example, has variable characteristics by echocardiography: and are often an unexpected finding. TEE is usually comple-
pedunculated,37 sessile,38 nonpedunculated,39 hetero- mentary by confirming and further defining the features.
Fig. 70.10: Live/real time three-dimensional transesophageal

echocardiography. Arrow shows a myxoma attached to the middle
portion of the atrial septum viewed from the left side. (AV: Aortic valve;
LA: Left atrium; RV: Right ventricle) (Movie clips 70.10).
C B
Figs 70.11A to C
Figs 70.11A to D: Two-dimensional (A and B) and live/real time

three-dimensional (C and D) transesophageal echocardiography
of right ventricular myxoma. (A) The tumor (T) is visualized in the
right ventricular (RV) outflow tract beneath the aortic valve (AV).
(B) Doppler studies. Color Doppler examination shows turbulent
signals (arrow in the upper panel) in RV and pulmonary artery (PA).
Continuous wave Doppler interrogation shows high velocity flow
signals consistent with obstruction. (C) Arrowheads show large
echolucencies consistent with hemorrhages in the tumor (T). Arrow
points to a linear area of calcification. (D) The dotted line shows
the echogenic area of attachment of the tumor viewed en face. It
measured 1.47 × 1.44 cm, area 1.04 cm2. The aortic (AO) wall
adjacent to the tumor attachment area is also echogenic. As shown
in the movie clips 70.11C (Parts 1–3), the three-dimensional data set
was cropped from the bottom to the tumor attachment just beneath
the AV. Then, the data set was rotated to fully delineate en face the
echogenic area of tumor attachment. In Movie clips 70.11C Part 3,
the AO wall was further cropped to reveal the vessel lumen and the
D mobile tumor tissue (red arrow) at the AO root level. Two other movie
clips 70.11D and E also show tumor attachment (arrows) beneath the
AO root. (RA: Right atrium; TV: Tricuspid valve) (Movie clips 70.11A,
Despite the individual variability of myxomas, TTE C parts 1 to 3, D and E).
Source: Reproduced with permission from Khairnar P, Hsiung MC,
typically reveals a homogenous echogenic masses arising Mishra S, et al. The ability of live three-dimensional transesophageal
within a cardiac chamber or from the valve leaflet with echocardiography to evaluate the attachment site of intracardiac
areas of echolucency correlating with hemorrhage or echo tumors. Echocardiography. 2011;28(9):1041–5.
bright areas correlating with necrosis and calcification
(Fig. 70.13, Movie clip 70.13). myxoma from thrombus or vegetation.44 Transducer
On M-mode, the tumor fills the left atrium in systole. angulations are a key in recognizing the tumor. Doppler
During diastole, the tumor prolapses into the mitral valve echocardiography can reveal mitral valve regurgitation
orifice. Because the tumor partially obstructs the mitral caused by tumor interfering with normal valve closure.
valve orifice, the mitral E-F slope is decreased, suggesting and also demonstrate an increased transvalvular gradient
a persistent gradient across the valve and impaired left produced by mitral orifice obstruction.
ventricular filling. The pattern can mimic M-mode pattern Myxomas must be differentiated from vegetations,
of mitral stenosis except the valves are not thickened. atrial thrombi, and other tumors or tumor-like structures
Left atrial myxomas on 2D echocardiogram generally (Fig. 70.15).31,45 The characteristic appearance, motion, and
appear as mobile, rounded, or ovoid echogenic masses site of origin of most myxomas distinguish them from the
that lie completely within the atrial cavity during systole majority of atrial and valvular masses. Myxomas arising
but may prolapse into or through the mitral valve during from locations other than the atria tend to have unusual
diastole (Fig. 70.14). These most commonly arise from shapes and be nonprolapsing, which make them more
the interatrial septum. Their point of attachment is best difficult to diagnose correctly. Valvular vegetations usually
demonstrated in an apical or subcostal four-chamber can be differentiated from left atrial or valvular myxomas
view. Large prolapsing myxomas generally interfere with by the clinical setting. The presence of valve disruption
left ventricular inflow and mitral valve diastolic motion.43 is a very strong evidence against a myxoma. A cardiac
The portion of the tumor protruding into the LV in mass in association with rheumatic mitral stenosis, atrial
diastole is very mobile, and because of this it has a greater fibrillation, or dilated cardiomyopathy is much more
potential to embolize as compared to the remaining of suggestive for a thrombus than a myxoma. Atrial thrombi
the tumor (Movie clip 70.13). Myxomas typically have tend to be located more in the atrial appendage compared
multiple internal reflective interfaces that give the tumor to atrial myxoma, which tend to originate from the septum
a finely speckled appearance and make the body of the and occupy the atrial chamber. Thrombi are laminated,
tumor as reflective as its margins.44 Bright echoes can be immobile, and have broad base attachment. Myxomas
due to localized calcifications. Visualization of echo-free are usually smooth, symmetrical, and frequently calcified.
region in a left atrial mass due to areas of hemorrhage Metastatic tumors generally have extension to other
or necrosis may be a useful feature in differentiating structures (e.g. pulmonary veins in lung malignancies).
A B
C D
Figs 70.12A to D: Real time two- (A) and three-dimensional (B to D) transthoracic echocardiography in right ventricular myxoma. (A)
The arrowhead points to a large myxoma in the right ventricle (RV) visualized in the apical four-chamber view. Tumor attachment is not
visualized. (B to D) The arrowhead in B points to the large myxoma. The arrowheads in C show attachments of the myxoma to the RV
inferior wall; D shows one of the attachments (arrow) of the tumor to the tricuspid valve using the QLab on the system. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium) (Movie clips 70.12 B–D Parts 1 and 2).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598–609.
RA myxomas can be visualized using subcostal and congenital variants, such as Chiari network or persistent
apical four-chamber views. Prior to the advent of 2DE, Eustachian valve, which appear as linear serpiginous
RA myxoma were difficult to diagnose with M-mode echoes in the right atrium and lack the globular shape
unless when present behind the tricuspid valve only when and bulk typical of myxoma.46 RA myxomas should also
protruding to the RV. As with left atrium, RA myxomas be differentiated from foreign bodies including right heart
on TTE are globular in shape with defined borders. catheters, and pacemaker wires that can mimic cardiac
Obstruction of the tricuspid valve can alter the leaflet masses, particularly when superimposed thrombus is
motion causing tricuspid stenosis or regurgitation. They present.47 Malignant tumors can be differentiated from
may be associated with RA and ventricular dilation, and myxomas when extension from the IVC is detected.
cause paradoxical motion of the interventricular septum. Right ventricular myxomas originate from the free
RA myxomas should be differentiated from normal wall or the interventricular septum. They appear as a
Fig. 70.13: Two-dimensional transesophageal echocardiography. Fig. 70.14: Parasternal long-axis view showing a left atrial
A huge myxoma is seen occupying almost the whole of the left myxoma prolapsing into the left ventricle. (Ao: Aorta; LA: Left
atrium (LA) with a broad attachment to the atrial septum. Highly atrium; LV: Left ventricle).
reflectile component of the tumor represents calcification. The por- Source: Dr Stephen Downing, Erie County Medical Center,
tion of the tumor protruding into the left ventricle (LV) in diastole Buffalo, NY.
is very mobile and because of this, it has a greater potential to
embolize as compared to the remaining of the tumor. (RA: Right
atrium; RV: Right ventricle) (Movie clip 70.13).
of other origin, vegetation, prominent moderator band,
and foreign bodies such as Swan–Ganz catheter and
transvenous pacemaker.
Left ventricular myxomas are extremely rare49 and
must be differentiated from other left ventricular tumors,
thrombi, false tendons, and prominent or calcified
papillary muscles. Left ventricular thrombi are generally
distinguished by associated LV wall akinesis or dyskinesis.
3DTTE may provide additional information in
assessing myxomas in different locations. It has the
capacity to section the mass and view it from different
angles, demonstrating maximum dimension using
multiplane probes or stacked 2D planes (Movie clips
70.11A to F), giving the examiner a more comprehensive
assessment of the mass and possibly additional
information about echolucency (Figs 70.16A to C; Movie
clips 70.16A and B–D).5,7,9 The attachment of the tumor
Fig. 70.15: Two-dimensional transthoracic echocardiography.
is much better delineated by the 3D technique (see Fig.
Fat in the tricuspid valve annulus. Arrow points to a prominent
echodensity in the anterior tricuspid valve annulus indicative of 70.6B and C). Contrast echo is another useful technique.
fibrofatty tissue. This should not be mistaken for a mass lesion. With contrast echo, the appearance of hyperenhancement
(RA: Right atrium; RV: Right ventricle) (Movie clip 70.15). of a suspected myxoma is supportive evidence.
After diagnosis, surgery should be performed urgently,
in order to prevent complications such as embolic events
mass that lie within the body of the RV during diastole or obstruction of the mitral orifice. Follow-up examination,
and extend toward the right ventricular outflow tract or including echocardiography, should be performed
prolapse through the pulmonic valve during systole.48 regularly.50 Recurrence rates reported for cardiac myxomas
The differential diagnosis may include: thrombus, tumors are 4–7% for sporadic cases and 10–21% for familial cases.
A B
Figs 70.16A to C: Three-dimensional (3D) echocardiographic

images cropped to show a left atrial myxoma attached to the
interatrial septum (A to C). 3D images demonstrate the finely
speckled appearance typical of myxomas.
Source: Dr Fredrich Albrecht, Suburban Cardiology, Buffalo, NY
C (Movie clips 70.16A and B).
Although recurrence rates are high, second recurrences suggesting a post-traumatic tumor or as a degenerative
are rare.51 process.55,57 Other etiologic hypothesis include relation to
Follow-up echocardiography is recommended after organized thrombi,58 or a latent development postradiation
surgical tumor resection. Particularly in large size myxoma, exposure.59 Gross pathological specimen resembles a sea
where there is a significant recurrence rate of 5%. anemone.
Because of the papillary configuration and soft, fragile
Papillary Fibroelastoma nature, PFEs are a potential cause of ostial coronary obs-
truction, leading to myocardial ischemia or infarction.60,61
Papillary fibroelastomas (PFEs) are the second most
common primary cardiac neoplasms, accounting for 5% of The multiple fonds with recesses may also act as a substrate
all tumors of the heart52 and 7.9% of benign primary cardiac for fibrin and platelet aggregation, with subsequent
tumors. However, they are the most common valvular peripheral or central embolization, depending on their
tumors.53,54 The true incidence may be underestimated55 cardiac location.62 The risk of embolization is higher in
because PFEs are often asymptomatic. It is frequently PFE compared to myxomas (34% compared to 24%)54
observed between the fourth and the eighth decade of because PFE commonly arise in the high flow and high
life.56 The pathophysiology of this tumor origin is variable. pressure systemic outflow tract of the heart (e.g. aortic valve
It can be seen in patients with long-standing heart disease or LV).63
A B1
Figs 70.17A and B: (A) Two-dimensional transthoracic echocar-

diography (parasternal long-axis view) of a patient with a papillary
fibroelastoma presenting as an 0.8 × 0.8 cm mobile echogenic
mass at the level of the sinotubular junction attached to the aor-
tic surface of one of the aortic valve leaflets. Source: Dr Hashmat
Ashraf, Chief of Cardiac Surgery, Buffalo General Medical Center,
Buffalo, NY; (B) Two-dimensional transesophageal echocardiogra-
phy showing a papillary fibroelastoma attached to the aortic valve
causing aortic insufficiency (AI) as shown by color Doppler (right
panel). (Ao: Aorta; LA: Left atrium; LV: Left ventricle).
Source: Dr. Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY (Movie clip 70.17). Transesopha-
geal echo movie clip showing a papillary fibroelastoma attached to the
aortic valve.
Source: Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
B2 General Medical Center, Buffalo, NY.
Aortic (Figs 70.17 and 70.18; Movie clips 70.17 and pedicled65 with a short stalk attached to the valve. Finger-
70.18A and B) and mitral leaflets are the most frequent like projection on the fibroelastoma produce a prominent
sites of origin, representing 60–90% of the diagnosed fluttering appearance of the tumor surface, which is a
cases, followed by tricuspid (Figs 70.19 and 70.20)64 and distinguishing feature on real time 2D TTE.
pulmonary valves [Figs 70.21A to F; Movie clips 70.21B, These tumors rarely exceed 1 cm in diameter and are
C(Parts 1and 2) and E, B–E].10,53,56,65 Approximately 77% of usually attached by a small pedicle to one of the cardiac
these tumors originate on the valves, and the other 23% in valve leaflet edges. Other less common anatomical sites
the endocardial nonvalvular surface.53 for PFE origin include: right atrium, RV, left atrium, LV,
Echocardiography remains the modality of choice to left ventricular outflow tract (LVOT), atrial or ventricular
identify and characterize PFEs.66 PFEs normally appear as septum, and coronary ostia.69 Doppler echocardiography
mobile, small, pedunculated, and echodense formations.67 usually demonstrates either minimal or mild regurgitation
The rounded, centrally radiolucent tumor is outlined but rarely significant regurgitation or stenosis.70,71
with a refractile linear echo.68 They are well demarcated TTE has a sensitivity and specificity of 88.9% and 87.8%,
and homogeneously textured in appearance, having a respectively, with an overall accuracy of 88.4% for the detection
speckled interior with stippling near the edges, which of PFE ≥ 20 mm. An overall TTE sensitivity of 61.9% is reported
correlates with the papillary projections on the surface of in case of tumor dimension ≤ 20 mm.65 A higher sensitivity is
the tumor (Figs 70.22A and B; Movie clip 70.22; Figs 70.23A reached with TEE, particularly for smaller PFEs.65 3D TTE has
and B; Movie clips 70.23A and B).65 Half of PFEs appear also been successfully employed in PFE diagnosis.10,72,73
A B
Figs 70.18A to C: (A) Enlarged two-dimensional (2D) echocar-

diogram apical four-chamber view; (B) two-dimensional transeso-
phageal echocardiogram (2D TEE), and (C) three-dimensional
transesophageal echocardiogram (3D TEE) showing a papillary
fibroelastoma attached to the chordal apparatus of the mitral valve.
(LA: Left atrium; LV: Left ventricle; RV: Right ventricle). Source:
Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo General
Medical Center, Buffalo, NY (Movie clip 70.18A). 2D echo movie
clip showing a papillary fibroelastoma attached to a chordal
apparatus of the mitral valve.
Source: Dr. Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY (Movie clips 70.18A and
70.18B). 2D TEE movie clip showing a papillary fibroelastoma
attached to a chordal apparatus of the mitral valve. Source: Dr
Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo General Medical
C Center, Buffalo, NY.
Fig. 70.19: Transesophageal echocardiogram of a patient with Fig. 70.20: Live/real time three-dimensional transthoracic echocardio-
a papillary fibroelastoma on the atrial surface of the anterior leaf- graphic evaluation of tricuspid valve fibroelastoma. The arrow shows a
let of the tricuspid valve. The mass presents as a large (1.56 × fibroelastoma attached to the septal leaflet (S) of the tricuspid valve. (AO:
1.2 cm), sessile, heterogeneous, mobile density with shimmering at Aorta; LA: Left atrium; RV: Right ventricle) (Movie clip 70.20).
the blood–tissue interface. (RA: Right atrium; RV: Right ventricle). Source: Reproduced with permission from Pothineni KR,
Source: Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo Duncan K, Yelamanchili P, et al. Live/real time three-dimensional
General Medical Center, Buffalo, NY. transthoracic echocardiographic assessment of tricuspid valve
pathology: incremental value over the two-dimensional technique.
Echocardiography. 2007;24(5):541–52.
A B
C D
E F
Figs 70.21A to F: Pulmonary valve fibroelastoma. (A) Two-dimensional transthoracic echocardiography. Arrowhead points to finger-
like projections on the fibroelastoma; (B to E) Live/real time three-dimensional transthoracic echocardiography; (B) Arrowhead points to
fibroelastoma and the arrow to the echogenic stalk attaching it to the pulmonary valve (PV); (C) Cropping and sectioning the tumor and
viewing it en face shows no evidence of echolucency; (D) Arrowhead points to finger-like projections (fronds) on the fibroelastoma; (E)
Ex vivo imaging of the resected fibroelastoma shows a dense central core and multiple finger-like projections (arrowheads); (F) Gross
pathological specimen of fibroelastoma showing multiple frond-like structures resembling a sea anemone (left) and histopathology of the
surgical specimen demonstrating a central core of collagen and elastin covered by a single layer of endothelial cells (right). (AO: Aorta;
AV: Aortic valve; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle; RVO: Right ventricle outflow tract) (Movie clips 70.21B,
70.21C Parts 1 and 2, and 70.21E). (Source: Reproduced with permission from Singh A, Miller AP, Nanda NC, Rajdev S, Mehmood F,
Duncan K. Papillary fibroelastoma of the pulmonary valve: assessment by live/real time three-dimensional transthoracic echocardiogra-
phy. Echocardiography. 2006;23(10):880–3.
A B
Figs 70.22A and B: Aortic valve fibroelastoma. Two-dimensional transesophageal echocardiography. Parasternal long-axis (A) and
short-axis (B) views. Arrowhead in A points to a mobile rounded mass attached to the right coronary cusp (arrow in B). The mass has
prominent, short, discrete projections on the surface, resembling a sea urchin or fronds of a curtain, and an echogenic central area from
collagen deposition. These findings are typical of a fibroelastoma. (AO: Aorta; LA: Left atrium; LAA: Left atrial appendage; RA: Right
A B
Figs 70.23A and B: Tricuspid valve fibroelastoma. Two-dimensional transthoracic echocardiography. Right ventricular inflow (A) and
parasternal short-axis; (B) views show an echodensity with small, multiple spicule-like structures typical of a fibroelastoma (arrowhead).
(AV: Aortic valve; RA: Right atrium; RV: Right ventricle) (Movie clips 70.23A and B).
PFEs are easily distinguished from myxomas, by size be difficult to distinguish from valvular myxoma, giant
and location. Myxomas commonly arise in the left atrium Lambl’s excrescences (Figs 70.24A and B; Movie clip
with a pedicle attached to the region of the fossa ovalis.22 70.24), and valvular vegetations. PFEs usually attach on
Myxomas are seen less frequently in the right atrium or the downstream side of the valve, whereas vegetations
ventricles and rarely involve the mitral valve. PFEs may be occur on the upstream side and are usually associated
differentiated from fibromas, which are nonencapsulated with clinical signs of sepsis or symptoms of endocarditis.
and highly refractile, ovoid, and usually solitary masses Primary surgical excision is the recommended therapy
that occur primarily in children and commonly involve for symptomatic PFEs, with left heart side location and
the LV, interventricular septum, and RV.74,75 PFE may with a specific clinical picture suggestive for possible
A B
Figs 70.24A and B: Two-dimensional transesophageal echocardiography. Lambl’s excrescence. Arrowhead points to a Lambl’s
excrescence on an aortic (AO) valve cusp. (LA: Left atrium; LV: Left ventricle; RV: Right ventricle) (Movie clip 70.24).
embolization.53,65 Asymptomatic patients with large mobile usually associated with the interventricular septum. On
masses (≥ 1 cm) have an increased risk of cardiovascular occasions, they can occur on the lateral wall or in the apex
complications from embolization and sudden cardiac of the RV.
death and should undergo a curative surgical excision.65 Echocardiography helps in confirming the presence of
Asymptomatic patients with small nonmobile lesions (< 1 a fibroma that is found primary within the interventricular
cm) should be closely followed up with echocardiography septum or anterior free wall of the RV.82 Central calcification
until symptoms develop or tumors enlarge and become is frequent. It can obstruct the mitral orifice and produce
mobile.76–78 TEE performed before and during surgery can significant obstruction to inflow. It can also present as
demonstrate the exact location of the tumor, influence massive tumor causing right ventricular outflow obstruction
surgical approach, and guide complete tumor excision.61 or displace the septum against the free wall of the LV, or shift
the tricuspid valve backward toward the atrium.
Cardiac Fibroma Fibromas are difficult to differentiate from rhabdo-
myomas, as both can present as a solitary, large mass,
Benign cardiac fibromas, when diagnosed, are seen particularly if intracavitary. However, their characteristic
almost exclusively in the pediatric population.79 Adult location within the interventricular septum83 or within
cases have been reported but are exceedingly rare.80,81 It is the ventricular free wall84 often aids in the appropriate
the second most common primary ventricular neoplasm diagnosis.
after rhabdomyomas. Grossly, these tumors are firm and Cardiac magnetic resonance (CMR) is very helpful in
nonencapsulated, intramural lesions ranging in size from showing anatomical details, and differentiating primary
3 cm to >10 cm in diameter.22 cardiac tumors from thrombus.85 The heterogeneity of
Clinically, cardiac fibromas may present as shortness the tissue component presents as hypointense T1 signal
of breath, dizziness, syncope, and heart failure. Symptoms with absent early vascular enhancement on perfusion
occur when the tumor increases in size causing obstructive imaging, and avid delayed enhancement suggesting a
disease. Cardiac fibromas may also be responsible for benign etiology and fibrous tissue.86 These tumors require
asymptomatic murmurs since childhood, or be an origin surveillance imaging due to the potential for recurrence.
for arrhythmias. Fifty percent are asymptomatic. Cardiac
fibromas can be found incidentally as calcification in
lower mediastinum on thoracic spine radiograph, or on
Cardiac Rhabdomyoma
CT of the chest. They most commonly occur in the RV Cardiac rhabdomyoma is the most common primary
(Figs 70.25A to C; Movie clips 70.25AB, CB–C)7 and are cardiac tumor in pediatric age group.87,88 This tumor is
A B
Figs 70.25A to C: Right ventricular fibroma. Two-dimensional

(A) and live/real time three-dimensional (B and C) transthoracic
echocardiography. (A) Arrowhead points to a single mass in the
right ventricle (RV); (B and C) The arrowhead (B) points to a highly
echogenic mass occupying right ventricle (RV) cavity and outflow
tract. Sectioning the mass and viewing it en face demonstrates two
separate tumors (arrowheads in C). (AO: Aorta; LA: Left atrium;
LV: Left ventricle; RA: Right atrium) (Movie clips 70.25B–C Parts
1 to 3).
Source: Reproduced with permission from Reddy VK, Faulkner M,
Bandarupalli N, et al. Incremental value of live/real time three-dimen-
sional transthoracic echocardiography in the assessment of right
C ventricular masses. Echocardiography. 2009;26(5):598–609.
multicentric and can occur in the ventricular free wall and When outflow obstruction is present, pulsed and
interventricular septum.88–90 It occurs in equal frequency continuous wave Doppler can determine the degree
in the right and left ventricles. of outflow gradient and help guide management. The
Diagnosis can occur in the prenatal period; however, presence of multiple nodular masses in several chambers
the majority are diagnosed in infancy. The incidence of of the heart helps differentiate rhabdomyoma from other
tuberous sclerosis in patients with cardiac rhabdomyoma primary cardiac tumors (e.g. myxoma, fibroma, or other
has been reported to be between 30 and 50%.91 Clinically, benign tumors).
rhabdomyoma can lead to life-threatening ventricular 2D echocardiogram is also a useful method of screening
arrhythmia or obstruction of ventricular filling or outflow asymptomatic infants and children with tuberous sclerosis
with intracavitary extension seen in 50% of patients. for the presence of cardiac tumors.91 Surgical intervention
Echocardiography is an ideal tool to diagnose may be required for children if a rhabdomyoma causes
rhabdomyomas. These tumors tend to appear as multiple severe clinical symptoms either due to life-threatening
homogenous, well-circumscribed, echogenic ventricular dysrhythmia or outflow obstruction refractory to medical
treatment.93 Spontaneous regression has been reported.94
masses. They may be intracavitary or intramural masses in
RV and LV. They have been described as having a speckled
appearance. A pedunculated rhabdomyoma occupying or Cardiac Lipomas
protruding into the ventricular cavity and aortic valve with Lipomas of the heart are most frequently located in the
significant LVOT obstruction has been reported.92 LV (Figs 70.26A to C; Movie clips 70.26AB–CA–C), RV
A B
Figs 70.26A to C: Left ventricular lipoma. Live/real time three-

dimensional transthoracic echocardiography. The arrowheads
in A and B point to a highly echogenic mass in the left ventricle
(LV). Further cropping of the three-dimensional data set shows
the presence of two lipomas (arrowheads in C) in the left ventricle.
Arrow in the movie clips 70.26A and B points to the lipoma that
is highly echogenic and shows no echolucencies on cropping.
Movie clip 70.26C shows another patient with a left ventricular
lipoma (arrow) together with the surgically resected specimen.
A few trabeculations are also present adjoining the lipoma in
the left ventricular apex. (RV: Right ventricle). (Movie clips
C 70.26A–B, C).
(Figs 70.27A and B; Movie clips 70.27 (Parts 1 to 3),7 Valvular lipomas are not easy to differentiate from
and right atrium (Figs 70.28A to C; Movie clip 70.28).22 other valvular tumors such as myxomas, PFEs or fibromas,
Clinically, these tumors are silent, although conduction or valvular vegetations. MRI may aid confirming the fatty
disturbances have been reported.95 nature of the tumor.98
Subepicardial tumors are large, often pedunculated,
whereas subendocardial lipomas are typically sessile. Cardiac Hemangiomas
About 25% are completely intramyocardial.22 Valvular
lipomas are rare, and have been described on the mitral Hemangiomas are the most common benign vascular
and tricuspid valves.96 tumors of the heart (Figs 70.30A to D; Movie clip 70.30 B).8
Lipomas are differentiated from left ventricular These tumors are usually solitary but may be multiple.99
myxomas in that lipomas are less mobile and generally They may occur anywhere in the heart including the
more echodense. These tumors need to be differentiated epicardium, often in association with pericardial effusion.99
from lipomatous hypertrophy of the interatrial septum They may be either intracavitary or intramural. Intramural
(Figs 70.29A and B), which is characterized by accumulation hemangiomas occur most commonly on the right side of
of adipose tissue in the atrial septum.97 In the subcostal views, the heart (Figs 70.31A to D; Movie clips 70.31A, B, and D
globular thickening of the interatrial septum with central Parts 1–2),34 particularly in the interventricular septum.99
sparing gives what has been described as a “dumbbell Clinical signs depend on anatomical location.
shape” to the atrial septum with lipomatous hypertrophy. Congestive heart failure occurs with large intracavitary
A B
Figs 70.27A and B: Right ventricular lipoma. Live/real time three-dimensional transthoracic echocardiography. (A) The arrow points to
a large markedly echogenic lipoma interdigitating and infiltrating the right ventricle (RV) free wall. (B) Surgical specimen. (LA: Left atrium;
LV: Left ventricle) (Movie clips 70.27 Parts 1 to 3).
A B
Figs 70.28A to C: Two-dimensional echocardiography showing

a cardiac lipoma attached to the apicoseptal left ventricular wall
(A). CT with (B) and without (C) IV contrast showing a hypodense
LV lipoma. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
Source: Dr Steven Horn, Medical Director Noninvasive Cardiology
C Lab, Kalieda Health, Buffalo, NY (Movie clip 70.28).
A B
Figs 70.29A and B: Two dimensional transesophageal echocardiography. Lipomatous hypertrophy of the atrial septum. (A) Arrow
points to thickening of atrial septum that spares foramen ovale. This is caused by fatty infiltration of the septum and usually has no
clinical sequelae. (B) Massive lipomatous hypertrophy (arrowheads) affects the entire atrial septum and occupies most of the right
atrium (RA) in another patient. (LA: Left atrium; RA: Right atrium; RPA: Right pulmonary artery; SVC: Superior vena cava). (Courtesy
of Dr Allan Schwadron, Dothan, AL).
tumors restricting filling of right heart chambers.100 The mesotheliomas (16%), fibrosarcomas (11%), primary
tumor may simulate infundibular pulmonic stenosis when cardiac lymphomas (PCLs; 6%), osteosarcomas (4%),
they involve the upper portion of the interventricular neurogenic sarcomas (2%), leiomyosarcomas (<1%; Figs
septum.97 Recurrent pericardial effusions are frequent 70.34A to C; Movie clip 70.34)9, liposarcomas (<1%), and
presentation.101,102 synovial sarcomas (<1%).104 In addition to identifying the
2D echocardiographic imaging shows echo-free presence of a cardiac tumor, echocardiography provides
areas within the mass, representing vascular channels valuable information regarding the anatomical location
(Figs 70.32A and B; Movie clip 70.32)5 and cavernous (right vs left heart chambers, intracavitary vs intramural),
lakes. These tumors are generally nonpedunculated, tumor extension (Figs 70.35A to H; Movie clip 70.35;
nonhomogenous, solitary masses. When seen in the valves, arteries, veins, and pericardium), physiological
right heart and associated with a pericardial effusion, consequences such as valvular stenosis and regurgitation,
they are more suggestive of hemangioma than myxoma, ventricular inflow or outflow obstruction, chamber
rhabdomyoma, or other primary intracardiac tumors. obliteration and ventricular function, and associated
Spontaneous regression of cavernous hemangiomas has findings such as recurrent pericardial effusion. All of these
been documented.103 features taken together help to establish a differential
diagnosis of the type of tumor in question and assess
MALIGNANT PRIMARY CARDIAC its hemodynamic effects on cardiac function.105–107 3D
TTE has more recently become a helpful adjunct in the
TUMORS detection and characterization of intracardiac masses,
secondary to its improved imaging over 2DE.108 In addition
Sarcomas
to 2D, 3D, and M mode echocardiography, contrast
Malignant primary tumors of the heart account for one- echocardiographic perfusion imaging has become an
quarter of all primary cardiac tumors, the vast majority important supplement to help classify cardiac masses.
of which are sarcomas (95%; Fig. 70.33; Movie clips Compared with the adjacent myocardium, malignant and
70.33A and B).7 The most common primary malignant vascular tumors, such as sarcomas, hyperenhance with
cardiac neoplasms in the adult are angiosarcomas (33%) the use of contrast imaging, whereas stromal tumors and
and rhabdomyosarcomas (21%), followed by pericardial thrombi hypoenhance.20,109,110
A B
C D
Figs 70.30A to D: Hemangioma involving the mitral valve. (A) Two-dimensional transthoracic echocardiography. Parasternal long-axis
view. The arrow points to the mass attached to the anterior mitral leaflet (AML), with a few echolucencies within it; (B) Live/real time
three-dimensional transthoracic echocardiography. Apical four-chamber view. The arrow points to a mass attached to the mitral valve.
The presence of multiple echolucencies (arrowheads, inset A) within the mass is consistent with a hemangioma; (C) Two-dimensional
transesophageal echocardiography. Low esophageal modified five-chamber view. The arrow points to the mass on the AML, with a few
scattered echolucencies within it; (D) Histopathology. H&E stain 10x. In addition to the sheet-like areas of “epithelioid” endothelial cells
with small vascular spaces typical of epithelioid hemangioma (arrows), this lesion also contains larger, more cavernous vascular spaces
(arrowheads). (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle). (Movie clip 70.30B).
Source: Reproduced with permission from Dod HS, Burri MV, Hooda D, et al. Two- and three-dimensional transthoracic and transesoph-
ageal echocardiographic findings in epithelioid hemangioma involving the mitral valve. Echocardiography. 2008;25(4):443–5.
Angiosarcoma is the most frequent type of sarcoma pathological examination, cardiac angiosarcoma looks
in the adult population and the most common primary like a lobulated mass with a necrotic and hemorrhagic
malignant cardiac tumor. It occurs more frequently in appearance and is composed of malignant cells forming
middle-aged males,104 is predominately right-sided,111 vascular channels.114 On echocardiogram it often appears
and carries a dismal prognosis.107 Typically, the tumor as a bulky, mobile, heterogeneous broad-based RA
completely replaces the RA wall, fills the entire cardiac mass near the IVC, extending intracavitary and into the
chamber, and invades surrounding structures including pericardium, occasionally invading the caval veins or
the tricuspid valve, free wall of the LV, interventricular tricuspid valve.115 Because of its tendency to invade valves,
septum, right coronary artery, and great veins.112,113 On sarcomas of the tricuspid and mitral valves may mimic
A B
C D
Figs 70.31A to D: Right ventricular hemangioma. (A) Two-dimensional transesophageal echocardiography. The tumor (T) is located
in right ventricle (RV); (B to D) Live/real-time three-dimensional transesophageal echocardiography; (B) The tumor (arrow) is located
in RV; (C) Arrow points to multiple small echolucencies involving the whole tumor including the periphery, typical of hemangioma;
(D) The three-dimensional data set was cropped from bottom and rotated to view the echogenic area of tumor attachment (dotted lines).
It measured 2.76 × 1.40 cm, area 2.01 cm2. (AV: Aortic valve; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium; TV: Tricuspid
valve) (Movie clips 70.31A, B, D Parts 1 and 2).
Source: Reproduced with permission from Khairnar P, Hsiung MC, Mishra S, et al. The ability of live three-dimensional transesophageal
echocardiography to evaluate the attachment site of intracardiac tumors. Echocardiography. 2011;28(9):1041–5.
cardiac angiosarcomas. Angiosarcoma that replaces of suspicion.118 There may also be a possible etiological
the atrial wall can also lead to RA rupture, as diagnosed correlation between the HIV infection or Kaposi sarcoma
by color Doppler echocardiography and contrast echo- and cardiac angiosarcomas.119 Angiosarcoma is usually
cardiography.116 On CT imaging, angiosarcoma can be associated with complications including pulmonary
identified as a low-attenuation RA mass, which may be embolism, right heart failure, cardiac tamponade, SVC
irregular or nodular. On MRI, it shows moderate intensity syndrome, cardiac chamber and coronary artery fistulas,
on T1- and T2-weighted images with local nodular areas of or cardiac perforation.
increased signal intensity interspersed to give a cauliflower- Rhabdomyosarcoma is the second most common
like appearance.117 Clinical presentation is usually primary cardiac sarcoma, but the most common cardiac
nonspecific; however, recurrent pericardial effusions are tumor in children and adolescents. This tumor arises
a common manifestation and should raise a high index from embryonic cells in the septum, which may explain
Figs 70.32A and B: Left atrial hemangioma. Live/real time three-

dimensional transthoracic echocardiography. (A) Arrowheads
(arrow in Movie clip 70.32) point to two of the large number of
closely packed echolucencies in the tumor mass with sparse solid
tissue; (B) Resected specimen showing multiple vascular areas.
(LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA: Right
Source: Reproduced with permission from Mehmood F, Nanda
NC, Vengala S, et al. Live three-dimensional transthoracic echo-
cardiographic assessment of left atrial tumors. Echocardiography.
2005;22(2):137–43. B
Fig. 70.33: Right ventricular sarcoma. Live/real time three-

dimensional transthoracic echocardiography. Arrow points to a
mass in the right ventricle (RV) showing large echolucencies
surrounded by echogenic band-like tissue giving a “doughnut”
appearance. Movie clip 70.33B shows, in a different patient, renal cell
carcinoma invading the inferior vena cava (IVC) and the proximal right
atrium (RA). Cut section demonstrates a solid tumor with no evidence
of necrosis. Surgically resected specimen is also shown. (LA: Left
atrium; LV: Left ventricle) (Movie clips 70.33A and B).
Source: Reproduced with permission from Reddy VK, Faulkner M,
Bandarupalli N, et al. Incremental value of live/real time three-dimen-
sional transthoracic echocardiography in the assessment of right ven-
tricular masses. Echocardiography. 2009;26(5):598–609.
A B
Figs 70.34A to C: Left atrial leiomyosarcoma. (A) Live/real time

three-dimensional transthoracic echocardiography. The arrows
point to a “doughnut”-like appearance of the tumor mass located
in the left atrium; (B) Intermediate and high-power microscopic
appearances of the leiomyosarcoma. The tumor is composed
of pleomorphic spindle cells arranged in a fascicular pattern.
Abnormal mitotic activity (inset) was easily identified; (C) Low-
power microscopic appearance of necrotic tumor. The periphery
of a necrotic area within the tumor showed acellular stroma
and dilated/ectatic blood vessels (arrows) consistent with the
echocardiographic findings. (AO: Aorta; LA: Left atrium; LV: Left
ventricle) (Movie clip 70.34).
Source: Reproduced with permission from Suwanjutah T, Singh H,
Plaisance BR, Hameed O, Nanda NC. Live/real time three-dimen-
sional transthoracic echocardiographic findings in primary left atrial
C leiomyosarcoma. Echocardiography. 2008;25(3):337–9.
its high prevalence in children younger than 1 year. enhancement after contrast, although there may be areas
Rhabdomyosarcomas most commonly arise from either of low signal intensity due to central necrosis within the
the left or right ventricular wall and frequently invade tumor.123,124 Systemic metastases typically develop in the
cardiac valves or interfere with valve function secondary lung, bone, and brain. In most patients, survival time is
to their mass effect.120 On echocardiogram, it appears as an limited to <12 months.
echodense, multilobular pedunculated mass with irregular Fibrosarcomas are primarily fibroblastic in origin
borders. While it is most commonly seen originating from
and appear as firm, gray–white nodules with areas of
the ventricles, it has also been described on 2D echo as a
hemorrhage and necrosis. They can occur on the right and
lobulated mobile mass inside the left atrium projecting
left sides of the heart and invade the cardiac chambers and
into the left ventricle during diastole and provoking
turbulence.121 Additional case reports have also described the pericardium. Echocardiographically, they are most
an echodense mass that originates from the pulmonary often seen as a mobile, heterogeneous left atrial mass.
valve and obliterates the right ventricular outflow tract Because most occur on the left side of the heart, associated
with associated high peak transpulmonary gradients.122 signs and symptoms are related to pulmonary congestion,
On contrast CT, it shows as a hypodense mass, while on mitral stenosis, and pulmonary vein obstruction. Similar
MRI it appears isointense to myocardium on T1-weighted to angiosarcomas and rhabdomyosarcomas, survival is
images and demonstrates more or less homogeneous poor.105
A B
C D
E F
Figs 70.35A to F
G H
Figs 70.35A to H: Aortic leiomyosarcoma in a 43-year-old male. (A to F) A mass (arrowheads) in the descending thoracic aorta (DA).
Color Doppler examination shows prominent flow signals in the unobstructed portion of the aorta (AO). This makes thrombus unlikely,
because associated spontaneous contrast echoes caused by a low-flow state usually are present. Also, no dissection flap is identified.
The arrows in A to C and E to H show a large echogenic mass outside the AO, consistent with hematoma; (G and H) A hematoma
(arrow, arrowheads) is seen extending on both sides of the descending aorta (DA, AO), even where the tumor mass is not present. At
surgery, the mass was found to be a leiomyosarcoma that involved the aortic wall, resulting in perforation that caused the hematoma
(Movie clip 70.35).
Source: Reproduced with permission from Suwanjutah T, Singh H, Plaisance BR, Hameed O, Nanda NC. Live/real time three-dimen-
sional transthoracic echocardiographic findings in primary left atrial leiomyosarcoma. Echocardiography. 2008;25(3):337–9.
Primary Cardiac Lymphoma can appear as a frank multilobulated mass mostly located
in the right atrium,136 occasionally in the RV, and rarely in
Primary cardiac lymphoma (PCL) is defined as a non- the left cavities.137 Other echocardiographic findings seen
Hodgkin lymphoma (NHL) exclusively located in the heart
in clinical cases were limited thickening of the pericardium
or the pericardium.125–127 The accepted definition of PCL
or the cardiac wall, decrease of the cardiac kinetics with or
also includes a lymphoma presenting as cardiac disease,
without pericardial effusion, and cardiac tamponade.138
especially if the bulk of the tumor is intrapericardial.128
ECG-gated MRI helps determine tissue characteristics,
Cardiac 99NHL is commonly associated with HIV
infection.129–131 location, mobility, and its relationship with surrounding
The most typical locations for cardiac lymphomas are tissues.139
the right atrium and RV, and less often the left atrium and
LV.132,133 PCL is rare, comprising only 0.5% of all lymphomas Cardiac Plasmacytoma
and 1.3–2% of all heart tumors.127,128 More than 80% of PCLs
Primary plasmacytoma of the heart is extremely rare.
are diffuse B-cell NHLs, the majority of which are a large
These tumors, when present in the heart, are usually
cell type and less often T-cell lymphomas.
secondary to metastatic disease or direct extension from
The two most common manifestations are pericardial
effusion and heart failure.134 SVC syndrome, stroke, the mediastinum. The right atrium is the most common
pulmonary hypertension, aortic valvular obstruction,127 site of cardiac plasmacytoma.140 They also can originate in
and myocardial infarction have also been reported.128 the atrioventricular groove with a short stalk perforating
Atrioventricular and bundle branch blocks, low into the right atrium near the tricuspid valve annulus.
voltages, and negative T-waves have been reported on Heart failure is the most common clinical presentation.
electrocardiography (ECG).135 Plasma immunoelectrophoresis detection of monoclonal
Echocardiography is a good noninvasive diagnostic immunoglobulin G paraproteinemia and k light chains
tool to detect pericardial effusion and the presence of a increases the probability for this disease entity. Bence–
lymphoma mass (Figs 70.36A and B). Cardiac lymphoma Jones proteinuria may or may not be present. On gross
A B
Figs 70.36A and B: Two-dimensional transesophageal echocardiography. Hodgkin lymphoma involving the atrial septum. (A) Four-
chamber view demonstrating thickening of the atrial septum (arrowhead) produced by the tumor. Note sparing of the fossa ovalis;
(B) Arrowhead shows a large circumscribed echolucent area in the atrial septum containing multiple, bright echo densities (“coin lesion”).
(LA: Left atrium; MV: Mitral valve; RA: Right atrium; SVC: Superior vena cava; TV: Tricuspid valve).
Source: Reproduced with permission from Miller A, Mukhtar O, et al. Two- and three-dimensional TEE differentiation of lymphoma
involving the atrial septum from lipomatous hypertrophy. Echocardiography. 2001;18(3):205–9.
examination, these tumors usually present as a large, asbestos exposure, other suspected risk factors include
friable mass. Definitive diagnostic confirmation is made radiation exposure, infection (including SV40 and TB),
through postresection histological and histochemical dietary factors, and recurrent serosal inflammation.144
examination demonstrating the classic features of a Clinical manifestations include constrictive pericarditis,
plasmacytoma. A standard 2D echocardiogram reveals the pericardial effusion, cardiac tamponade, and heart
presence of a large mass in the right atrium (Figs 70.37A failure. Diagnosis can be challenging and often requires
to C; Movie clips 70.37A to C), often producing a subtotal a multimodal imaging approach including echocardio-
obstruction of the diastolic inflow through the tricuspid graphy, MRI, CT, and FDG-PET scans. TTE can show
valve.141 Treatment is usually palliative and consists of
a pericardial effusion comprising a partially organized
resection followed by chemotherapy.
fibrinous structure and a thickened pericardium. 2DE can
be misleading; however, as it can suggest the presence of
Hydatid Cyst an abnormal amount of pericardial fluid with a surgical
Cardiac hydatid cyst is a rare parasitic disease caused by plane for resection. As later pathological findings will
larvae of Echinococcus granulosus, which is still endemic reveal, the low-density echogenic region surrounding
in many sheep-raising countries. Although the LV is mostly the heart will be tumor rather than fluid.145 FDG-PET/CT
involved in this condition, all cardiac chambers and rarely scan demonstrates an intrapericardial accumulation of
the pericardium can be affected (Figs 70.38A to Z; Movie the tracer, seen with local infection or tumor.146 MRI is
clips 70.38A and B; Figs 70.39A to F). Patients can be emerging as the best modality for demonstrating the extent
asymptomatic, have nonspecific symptoms such as chest and nature of the constrictive process, and will show a soft-
pain, shortness of breath, or palpitations or present as an tissue mass within the pericardial space that is enhanced
acute complication of cyst rupture.142 Echocardiogram
with gadolinium DTPA.147 Pericardial fluid cytology
usually shows a unique finding of multiple cysts inside the
is often negative for malignant cells and a diagnosis
cardiac chambers or the pericardium.
usually requires tissue for histological evaluation.143,144
The prognosis of primary pericardial mesothelioma is
Pericardial Mesotheliomas extremely poor due to its late presentation, inability for
Primary pericardial mesothelioma is a very rare tumor, complete tumor eradication by surgery, and poor response
with a reported prevalence of <0.002%.143 In addition to to radiotherapy and chemotherapy.
A B
Figs 70.37A to C: Two-dimensional (2D) transesophageal

echocardiogram showing a periaortic right atrial mass representa-
tive of plasmacytoma (A to C). (A: Aorta; AV: Aortic valve; LAL Left
(Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
Buffalo, NY (Movie clip 70.37A). 2D echocardiogram showing a
periaortic right atrial plasmacytoma. Source: Dr Edward Span-
genthal, Roswell Park Cancer Institute, Buffalo, NY. Movie clip
70.37B.Transesophageal echocardiogram showing a periaortic
right atrial plasmacytoma seen anterior to the aortic valve. Source:
Dr Edward Spangenthal, Roswell Park Cancer Institute, Buffalo,
NY (Movie clip 70.37C). Transesophageal echocardiogram show-
ing a pariaortic right atrial plasmacytoma seen on the right atrial
side of the interatrial septum.
Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
C Buffalo, NY.
Secondary Cardiac Tumors primary tumors of the heart, metastases may imitate
valvular heart disease or cause cardiac failure, ventricular
While primary cardiac tumors are very rare, secondary or or supraventricular heart rhythm disturbances, con
metastatic heart tumors are the most common tumors of duction defects, syncope, embolism, or, quite often,
the heart. In clinical practice, secondary cardiac tumors pericardial effusion (Fig. 70.40). Not infrequently, cardiac
are rarely seen. The frequency of cardiac metastases tumor invasion contributes to the mechanism of death in
is generally underestimated—varying from series to affected patients.148–150
series, cardiac metastases were found in up to 25% of In principle, every malignant tumor can metastasize
postmortem patients who had died of malignancies.148–152 to the heart. The most common tumors with cardiac
Because of absent or nonspecific signs and symptoms of metastatic potential are carcinomas of the breast, the
cardiac involvement, most metastases are diagnosed post lung (see Fig. 70.40), the esophagus, leukemia, malignant
mortem. Cardiac metastases mostly appear in patients melanoma (Figs 70.41 and 70.42), and lymphoma (Fig.
with disseminated tumor disease; solitary metastases to 70.43).153–155 Of tumors that metastasize to the heart,
the heart are very rare. Despite their frequency, metastatic melanoma has the highest rate of cardiac metastasis,
heart tumors only rarely gain clinical attention. Signs although lung and breast carcinomas are more commonly
of cardiac involvement are often overlooked, since the encountered because of the higher prevalence of these
symptoms of disseminated tumor disease prevail. Like cancers.153–155
A B
C D
E F
Figs 70.38A to F
G H
I J
K L
Figs 70.38G to L
M N
O P
Q R
Figs 70.38M to R
S T
U V
W X
Figs 70.38S to X
Y Z
Figs 70.38A to Z: Live/real time three-dimensional transthoracic echocardiography. Hydatid cyst. Arrow in the apical four-chamber (A)
and short-axis (B) views in a young Asian Indian policeman shows a large mass in the left ventricle (LV), which represents a hydatid cyst
that had become smaller since the previous study done a few years ago. This collapse of the hydatid cyst could have been spontane-
ous or may be related to antihelminthic albendazole therapy, which had been given to this patient. (LA: Left atrium; RV: Right ventricle)
(Movie clips 70.38A and B). (C to Z) Live three-dimensional transthoracic echocardiographic assessment of hydatid cyst in the left ven-
tricle in another patient— a 37-year-old Asian Indian male. (C) The arrow points to the large hydatid cyst in the left ventricle (LV) seen on
the two-dimensional transthoracic echocardiogram. (B to I) Sequential transverse plane (TP) sections taken at various levels (# 1–5) of
the three-dimensional data set. The cyst is not visualized at the level of the body of the mitral valve(C, *), but its basal tip (arrow) comes
into view when the section is taken further down at the mitral valve leaflet tips (D). In H and I, the large arrowhead points to a tertiary or
grand-daughter cyst located within the secondary or daughter cyst. The small arrowhead in H shows a small great–grand-daughter cyst
budding from the tertiary cyst. The arrow points to the parent hydatid cyst. (J to M) Longitudinal plane (LP) sections (# 1 and 2) through
the hydatid cyst (arrow). (N) Combined TP and LP sections through the hydatid cyst. (O to T) Only FP (O), combined FP and TP (P and
Q), combined FP and LP (R), and combined FP, TP, and LP (S and T) sections through the hydatid cyst. (U to Y) Oblique plane (OP)
sections through the hydatid cyst. In X, the OP section is rotated to view the cyst en face. A tertiary or grand-daughter cyst is shown
attached to the secondary or daughter cyst by a stalk (small double arrowheads). (Z) Schematic of hydatid cyst. (LA: Left atrium; RA:
Right atrium; RV: Right ventricle; S: Stalk). (Movie clips 70.38C–Z).
(Source: Reproduced with permission from Sinha A, Nanda NC, Panwar R, et al. Live three-dimensional transthoracic echocardio-
graphic assessment of left ventricular hydatid cyst. Echocardiography. 2004;21(8):699–705.
A B
Figs 70.39A and B
C D
E F
Figs 70.39A to F: (A) Transthoracic echocardiography shows a large pericardial hydatid cyst (MS) compressing the left ventricular (LV)
lateral wall. It measures approximately 8 cm in diameter and appears to contain layered solid tissue. The patient was an 18-year-old
Asian Indian male who had emigrated to South Alabama at the age of 5 from India. He presented with a recent syncopal episode and the
electrocardiography (ECG) showed deeply inverted T-waves in anterolateral leads consistent with myocardial ischemia. Initially, the two-
dimensional echocardiogram appeared to show poor LV function but it soon became apparent that this was artifactual and resulted from
the ultrasonic beam passing directly from the aortic root into the cyst; (B and C) The arrows point to multiple intramyocardial coronary
vessels imaged within the compressed LV free wall. These were visualized using a high-resolution color Doppler system. Note that the
Nyquist limit is set at a very low velocity of 0.16 m/s; (D) The arrows demonstrate a high velocity of 1.2 m/s obtained by color Doppler-
guided pulsed Doppler interrogation of an intramyocardial coronary vessel (normal velocity < 0.6 m/s). Thus, there was compression
of the LV lateral wall by the cyst that served to explain the ECG changes. A coronary arteriogram also showed systolic emptying of the
first marginal branch of the circumflex artery. The cyst was surgically resected and the patient is doing well; (E) Left: Three-dimensional
reconstruction demonstrates membrane-like solid tissue in the cyst. Right: Three-dimensional reconstruction of intramyocardial coro-
nary vessels (arrows) in the compressed LV wall; (F) Left upper panel: Hydatid cyst wall and surrounding soft tissue. Left lower panel:
Close-up of hydatid scolex with double rows of hooklets (100x). Right panel: Laminated membrane. (C: Pericardial cyst; LA: Left atrium;
RA: Right atrium; RV: Right ventricle) (Movie clip 70.39).
Source: Reproduced with kind permission from Kluwer Academic Publishers, Advances in Echo Imaging using Contrast Enhance-
ment, 2nd edition, 1997, Ch. 41 “Echocardiographic detection of intramyocardial coronary obstruction produced by pericardial hydatid
CPT,” Fig. 2B. Jamil F, Nanda NC, et al. Echocardiographic Detection of Intramyocardial Coronary Obstruction Produced by Pericardial
Hydatid Cyst. Echocardiography. 1997;14(5):459–60.
Fig. 70.40: Apical four-chamber view showing a left ventricular Fig. 70.41: Apical four-chamber view showing metastatic mela-
intracardiac metastasis of a ureteral sarcomatoid tumor (blue). noma with metastasis to the papillary muscle. (LA: Left atrium; LV:
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right Left ventricle; RA: Right atrium; RV: Right ventricle).
ventricle).
Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
Buffalo, NY.
A B
Figs 70.42A and B: Two-dimensional transesophageal echocardiography. Metastatic melanoma involving the right ventricle. (A and
B) A huge tumor mass (M, T) is noted in the right ventricle (RV) reaching up to the pulmonary valve. (AO: Aorta; LV: Left ventricle; PA:
Pulmonary artery).
Source: Reproduced with permission from Nanda NC, Domanski MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2007:519.
2DE plays an essential role in the detection of cardiac lesions.155,156 In cases of peri- or paracardial lesions, the
metastases and their complications (Figs 70.44 and 70.40). transesophageal approach is superior to the transthoracic
It can generally be used to determine the location, size, approach (see Figs 70.42A and B).157,158
shape, attachment, and mobility of the cardiac tumor. Tumors can spread to the heart through four
It has a good sensitivity to detect intracardiac tumors, alternative pathways: (a) by direct extension, (b) through
but a lower detection rate for pericardial or paracardial hematogenous spread, (c) through the lymphatic system,
Fig. 70.43: Transthoracic two-dimensional (2D) echocardiogram Fig. 70.44: Subcostal view showing pericardial metastasis (P)
(parasternal long-axis view) of a patient with lymphoma and and circumferential pericardial effusion (PE) in a patient with met-
evidence of a very large and uniform echodense mass anterior astatic lung cancer. (LA: Left atrium; LV: Left ventricle; RA: Right
and spanning the entire heart with possible extension into the atrium; RV: Right ventricle).
pericardial space. (AV: Aortic valve; LA: Left atrium; LV: Left Source: Dr Edward Spangenthal, Roswell Park Cancer Institute,
ventricle; L: Lymphoma). Buffalo, NY.
and (d) by intraluminal venous extension. About two- Some tumors diffuse along the IVC reaching the right
thirds of all cardiac metastases involve the pericardium, atrium and producing an intracavitary lesion, leading
one-third the epicardium or the myocardium, and only 5% occasionally to obstruction. These include carcinoma of
the endocardium.148 the kidney, hepatocellular carcinoma, leiomyoma of the
Tumors such as the bronchial, breast, and esophageal uterus, and carcinoma of the adrenal cortex. This type of
carcinoma, which develop near the heart, may expand by lesion can become so obstructive that it fills the chamber
direct extension into the heart, but predominantly by the completely and blocks tricuspid movement, resulting
lymphatics. All preferentially affect the pericardium.148–153 in a clinical pattern similar to pericardial constriction
In the case of pericardial involvement, echocardiography or myocardial restrictive disease. Invasion of the heart
can show dense pericardial bands reflecting thickening through the SVC into the atrium can occur in the case of
carcinoma of the lung and thyroid gland.148
by inflammation or tumor infiltration. Pericardial effusion
Carcinoid heart disease is also another entity, which
can be proven quickly, with high sensitivity. When the
can be diagnosed by echocardiography. Metastatic
pericardium is involved, echocardiography is usually
carcinoid tissue in the liver produces biologically active
used both as a guidance for pericardiocentesis and for the
substances, including serotonin, which cause abnormalities
follow-up of effusions. Tumor cells within the pericardial
of the right-sided cardiac valves and endocardium. Typical
fluid may verify diagnosis of metastatic pericardial
changes include thickening, retraction, and increased
involvement (see Figs 70.43 and 70.44). rigidity of the tricuspid and pulmonic valve leaflets,
Malignant melanoma (see Figs 70.41 and 70.42), resulting in valvular regurgitation or, less often, valvular
lymphoma (see Fig. 70.43), leukemia, soft tissue, and bone stenosis. Left-sided valvular involvement is rarely seen.160
sarcoma usually spread hematogenously. Myocardial Cardiac involvement is present in one-half of the patients
metastases can involve any one of the heart chambers with carcinoid tumors. Heart failure resulting from severe
(Figs 70.45A to E, Movie clips 70.45B, C and DE; Figs tricuspid regurgitation is a common cause of mortality in
70.46A to E; Movie clips 70.46A and B).13,159 Intracavitary these patients. Although metastatic carcinoid disease is
cardiac metastases are the least common variety. Echo- rare, the echocardiographic findings are pathognomonic
cardiography cannot distinguish intracavitary metastatic and may lead to the diagnosis in a patient in whom it was
tumor from primary cardiac tumors. not considered previously.160
A B
C D
Figs 70.45A to E: (A) Microscopic examination of a biopsy specimen
showing malignant tumor cells with marked nuclear pleomorphism,
prominent nucleoli, and high mitotic rate. No glandular or squamous
differentiation was noted; (B to E) Two-dimensional transthoracic
contrast echocardiography; (B) Precontrast study showing two
masses (arrow heads) in the left ventricle (LV) attached to the
ventricular septum; (C to E) Postcontrast study. Initial frames
showed contrast filling LV cavity and outlining the two masses,
but there was no significant tumor enhancement (C). Subsequent
beats (D and E) demonstrate prominent enhancement of both
metastatic tumor masses consistent with high vascularity seen in
a poorly differentiated malignant tumor. RV, right ventricle. (Movie
clips 70.45B, C and DE).
Source: Reproduced with permission from Yelamanchili P,
Wanat FE, Knezevic D, Nanda NC, Patel V. Two-dimensional
transthoracic contrast echocardiographic assessment of metastatic
E left ventricular tumors. Echocardiography. 2006;23(3):248–50.
A B
C D
Figs 70.46A to E: Metastatic chordoma. (A) Real-time two-
dimensional transthoracic echocardiography. The arrow points to
the chordoma in the right ventricle (RV); (B and C) Live/real time
three-dimensional transthoracic echocardiography. Arrows point
to echolucencies consistent with hemorrhages and cystic areas,
and the arrow heads denote echodense areas produced by fi-
brotic bands. The less intense areas represent the myxoid stroma;
B and C represent in-vivo and ex-vivo studies, respectively;
(D and E) Pathological specimen showing the lobulated resected
tumor (D); The cut surface (E) shows hemorrhagic and cystic areas
(arrows) and dense fibrotic bands (arrow heads). (LA: Left atrium;
RA: Right atrium; TV: Tricuspid valve) (Movie clips 70.46A and
70.46B).
E Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Bell WC, Post JD. Live/real time three-dimensional
transthoracic echocardiographic description of chordoma meta-
static to the heart. Echocardiography. 2008;25(4):440–2.
Normal Variants and Other Masses membrane of the right atrium. It can be mistaken for a
RA mass on echocardiogram, and varies anatomically
There are several normal variant cardiac structures that more than the Eustachian valve. On echocardiogram, it
may mimic a cardiac mass. In conjunction with clinical has been described as an echodense, crescentic fold that
presentation, evaluation of their size, shape, location, resembles bars and bands or threads and networks. It can
mobility, and site of attachment by echocardiography
have fenestrations or present as multiple fine strands at the
helps to differentiate them from malignant pathology.
coronary sinus.161 Clinical practice suggests that the valve
may present difficulties for cannulation of the coronary
Chiari Network sinus. In autopsy cases, thebesian valves are present in
The Chiari network is an embryological remnant that results over 70% of hearts.162,163 Over 15% of hearts have a valve
from incomplete reabsorption of the right valve of the sinus that covers more than 75% of the ostium and can be devoid
venosus. It appears as a web-like structure with thread-like of any fenestrations.162
components and is present in 2% of the population. Its widely
mobile and serpiginous appearance within the right atrium Crista Terminalis
can be confused with other highly mobile pathological
The crista terminalis is a well-defined fibromuscular
structures including vegetations, flail tricuspid leaflet,
structure formed by the regression of the septum
ruptured chordae tendinae, thrombus, or right heart tumor.46
spurium as the sinus venosus is incorporated into the
The key differential points by echocardiogram include (a)
RA wall. Depending on the amount of regression, the
identification of two, and ideally three, normal appearing
crista terminalis can also be very prominent and mimic
tricuspid valve leaflets; (b) presence of a bright, rotatory,
a RA mass. On TTE, the crista terminalis is seen as an
highly mobile echocardiographic target that does not move
echodense linear ridge in the posterior RA wall, extending
into the right ventricular inflow tract or RV in diastole as
laterally from the atrial septum. On 3DE, it can be more
would be typical of a tricuspid leaflet vegetation; and (c)
in the four-chamber apical or subcostal view, the typical clearly defined as a thick and tapering linear structure in
posterolateral orientation and anteroinferior and medial the posterior wall of the right atrium.16
course of this structure across the right atrium. The use of
intravenous bolus contrast material to outline the course of Moderator Band
the IVC, right atrium, tricuspid valve, and RV can also be of The moderator band, or septomarginal trabeculation,
additional benefit in excluding the presence of RA mass and is a muscular trabeculation extending from the lower
tricuspid leaflet disruption.46 interventricular septum to the anterior RV wall. It is easily
identified because of its location in the RV, carrying the
Eustachian Valve right bundle branch from the bundle of His. It is best seen
The Eustachian valve, an embryological remnant of the in the apical four-chamber view and subcostal views, and
valve of the IVC, commonly appears as a thin flap arising in the apical view, can be seen as it transverses the RV
from the anterior rim of the IVC orifice. However, it can also cavity at midventricular level, connecting the free wall to
persist as a mobile, elongated structure that projects several the interventricular septum. In the parasternal long-axis
centimeters into the RA cavity and has been misinterpreted view of the RV, it can be seen as a large muscular band that
as an intra-atrial thrombus. Eustachian valves move in a crosses the apex of the RV obliquely.
more restricted manner than Chiari networks, and should be
imaged from the apical four-chamber view, right inflow tract, Coumadin Ridge
and subcostal views to distinguish them from vegetation,
The “Q-tip” sign, also known as the “Coumadin ridge”, is
tumor, or thrombus in the right atrium. On echocardiogram,
a prominent muscular structure formed between the left
it appears as a linear, echodense object.
atrial appendage (LAA) and the atrial insertion of the left
upper pulmonary vein. It was often misdiagnosed as a
Thebesian Valve thrombus until it became well described in the literature.
The thebesian valve, also known as the valve of the It can be differentiated from a thrombus by its lack of
coronary sinus, is a semicircular fold of the lining mobility and characteristic location. This tissue may
accumulate fat, creating a mass-like appearance, usually setting, TEE is the gold-standard technique, with a great
with a thin, proximal part and a thicker, more bulbous, sensitivity and specificity to detect LAA thrombi.164 These
distal part seen on echocardiogram. thrombi are seen as echo-reflecting masses in the atrial
body or in the apex of the LAA, distinct from the underlying
Thrombus endocardium, observed in more than one imaging plane,
and not related to pectinate muscles. This should be
Cardiac thrombi can be located within the atrial (Figs differentiated from the mass-like effect, which is a normal
70.47A and B, Movie clips 70.47, Figs 70.48A to E)7 and variant rarely seen in the LAA on TEE (Figs 70.53A to D;
ventricular chambers (Figs 70.49 and 70.50),7 and it can Movie clip 70.53). Other signs that are usually associated
extend to the heart from the vena cava (Figs 70.51A to
with thrombosis in the atrium are low LAA emptying
C) or from the heart to the pulmonary arteries (Figs 70.51A
velocities, dilated (area > 6 cm2) and multilobulated LAA,
to C and 70.52). They may develop as a consequence of
and spontaneous echo contrast.
multiple underlying cardiac disorders affecting the valves
and myocardium.
Echocardiography serves as a cornerstone in the Left Ventricular Thrombus
evaluation and diagnosis of these patients. It is considered In the ventricles, thrombi are usually located in the
to be the first-line imaging modality in such patients. apex. They usually occur in the setting of significant left
ventricular dysfunction and after acute anterior and/or
Atrial Thrombus apical myocardial infarction. It is extremely rare to have a
Within the atria, thrombi can be found in the atrial appen- LV thrombus on the top of a normally contracting LV wall; it
dages, within the body of the atrium, or in a combination is usually adjacent to a hypokinetic and akinetic area of the
of these areas. They are usually the consequence of poor LV wall. These thrombi have to be seen in at least two views
atrial emptying and blood stasis in conditions such as atrial (usually apical and short axis). LV thrombus is frequently
fibrillation, atrial flutter, and mitral stenosis. They may also suspected. Beside ultrasound artifacts, normal structures
be associated with indwelling catheters (see Figs 70.51A to and normal variants can be mistaken as thrombus, such
C). The most common location for thrombi in the atrium as normal or aberrant trabeculae or false tendon, muscle
is the LAA, which cannot be regularly seen by TTE. In this tendon such as moderator band, and papillary muscles.
A B
Figs 70.47A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of right atrial thrombus. (A)
Arrowhead points to a large mobile serpiginous thrombus in the right atrium (RA) prolapsing into the right ventricle (RV); (B) Cropped
segments of the thrombus (arrowheads) demonstrate a homogenous appearance with no echolucencies, indicating absence of clot lysis
(Movie clip 70.47).
Source: Reproduced with permission from Nanda NC, Hsiung MC, Miller AP, Hage FG: Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.
A B
C D
E
Figs 70.48A to E: Live/real-time three-dimensional transthoracic echocardiography. (A and B) Apical (A) and parasternal short-axis
(B) views show a large mass (arrow) consistent with thrombus in the right atrium (RA) with possible attachment to the RA free wall;
(C and D) Right parasternal views showing the attachment of the thrombus to the RA free wall in the vicinity of the inferior vena cava
(IVC) and tricuspid valve (TV) directly opposite the superior vena cava (SVC) entrance into the RA. (LA: Left atrium; LV: Left ventricle;
RV: Right ventricle); (E) Schematics of (a) clot, (b) myxoma, (c) sarcoma/chordoma, and (d) hemangioma. The horizontal arrowhead
in (a) points to central lysis in a clot, in (b) it points to an area of hemorrhage/necrosis in a myxoma, and in (c) to an area of necrosis
surrounded by thick, band-like tissue containing collagen, giving a doughnut-like appearance seen with chordoma and sarcoma. The
vertical arrowhead in (b) points to dense calcification in the myxoma. (d) Demonstrates a hemangioma that is completely vascular and
the echolucencies involve the whole tumor, including periphery (Movie clips 70.48A–D).
A B
C D
Figs 70.49A to D: Two-dimensional (A) and live/real-time three-dimensional (B to D) transthoracic echocardiography in right ventricular
thrombus. (A) The arrowhead points to one bifid or possibly two clots in the right ventricle (RV). (B and C) Cropping the three-dimension-
al (3D) data set demonstrates three separate clots in the RV (arrowheads). (D) Another patient with clots in RV showing central lysis in
a cropped 3D image. (LA: Left atrium; LV: Left ventricle; RA: Right atrium) (Movie clips 70.49A, B–C parts 1 to 4, D).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time
three-dimensional transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598–
609.
LV thrombus may be protruding within the LV clearly defined and the presence or absence of a thrombus
cavity or flat (mural) lying along the LV wall. It may be may be very difficult to establish. In such cases, the use
homogeneously echogenic, or have a heterogeneous of contrast ultrasound agent injected intravenously can
texture often with central lucency. Thrombi may be fixed clearly identify the presence of a thrombus and decrease
along LV wall or present an independent motion to a both intraobserver and interobserver variability.165
variable extent. Motion commonly involves a portion of
the thrombus but might involve the entire thrombus. What
Vegetation
differentiates LV thrombus from an artifact is that the
motion of the thrombus is independent of the underlying Cardiac vegetation presents as an oscillating or nonosci-
myocardium. Color Doppler tissue imaging may further llating intracardiac mass attached to the valves, other
facilitate this differential diagnosis. endocardial structures, or implanted on intracardiac
Although TTE has high sensitivity and specificity in material. It is considered to be the pathological hallmark
diagnosing LV thrombus, very often the LV apex cannot be of endocarditis. Endocarditis can involve any of the heart
A B
C D
Figs 70.50A to D: Live/real-time three-dimensional transthoracic echocardiogram. (A) Four-chamber view showing no thrombus or
aneurysm in the left ventricle (LV); (B) Anterior–posterior cropping displays the large aneurysm containing thrombus (arrow); (C and D)
Sectioning of the thrombus (C) and viewing it en face (D) shows no evidence of lysis or liquefaction. (RV: Right ventricle) (Movie clip
70.50).
Source: Reproduced with permission from Duncan K, Nanda NC, Foster WA, Mehmood F, Patel V, Singh A. Incremental value of live/
real time three-dimensional echocardiography in the assessment of left ventricular thrombi. Echocardiography 2006;23(1):68–72.
valves, with predilection to prosthetic valves. Diseased vegetation; it provides clinically important information on
valves are more prone to be affected during infectious the presence and degree of valvular destruction and their
states. Vegetations are typically attached to the low- hemodynamic consequences, as well as on the existence
pressure side of the valve structure, but may be located of perivalvular infection.
anywhere on the components of the valvular and the Overall, the detection rate for vegetations by TTE
subvalvular apparatus, as well as the mural endocardium in patients with a clinical suspicion of endocarditis
of the cardiac chambers or the ascending aorta. When averages around 50 to 75%.166,167 The diagnostic yield of
large and mobile, vegetations are prone to embolism and the technique in the detection of vegetations is influenced
less frequently to valve or prosthetic obstruction. by several factors—image quality; echogenicity and
Two-dimensional and transoesophageal echocardio- vegetation size; vegetation location; presence of previous
graphy serves as the cornerstone in noninvasive detection valvular disease or valvular prosthesis; experience
of vegetation. In the setting of endocarditis, the role and skill of the examiner; and pre-test probability of
of echocardiography is not just the identification of endocarditis.
A B
Figs 70.51A to C: Two-dimensional transesophageal echo-

cardiography. Thrombus in the pulmonary artery and the superior
vena cava. (A to C) Large thrombi (M,C) are noted in the supe-
rior vena cava (SVC) and the right pulmonary artery (RPA) in this
patient with an infusion catheter, which acted as the nidus for
thrombus. (AO: Aorta; ASC AO: Ascending aorta; LA: Left atrium).
Source: Reproduced with permission from Nanda NC, Domanski
MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
C Philadelphia: Lippincott Williams & Wilkins; 2007:521.
TEE enhances the sensitivity to 85–90% for the diagnosis series of prosthetic endocarditis, TEE has shown an 86
of vegetations, while more than 90% specificity has been to 94% sensitivity and 88–100% specificity for vegetation
reported for both TTE and TEE.167–169 Both TTE and TEE diagnosis, while TTE sensitivity was only 36–69%.169
do not permit differentiation between septic and other In the setting of tricuspid vegetations, TTE allows
aseptic vegetations present in conditions such as Libman– an easy and correct diagnosis, probably because the
Sacks endocarditis in systemic lupus erythematosus, majority of patients with tricuspid endocarditis are young
antiphospholipid syndrome, and marantic endocarditis. intravenous drug abusers with large vegetations. The
Vegetations on prosthetic valves are more difficult vegetations are located on the atrial side of the tricuspid
to detect by TTE than those involving native valves. valve, in the way of the regurgitant jet. TEE did not increase
Therefore, TEE should always be used if the diagnosis the accuracy of TTE in the detection of vegetations in
of prosthetic endocarditis is suspected. The sewing ring tricuspid endocarditis in one study.168,170
and support structures of mechanical and bioprosthetic Infection or endocarditis on a pacemaker lead is
valves are strongly echogenic and may prevent vegetation difficult to diagnose by TTE, since pacemaker leads
detection within the valve apparatus or its shadow. produce reverberations and artifacts that may mask or
Thrombus, pannus, and strands can be mistaken as make difficult the recognition of vegetations close to these
vegetative material given their similar appearance structures. In addition, when vegetations were visualized,
(Figs 70.54A to C; Movie clips 70.54A to C). In large it was difficult to determine whether tricuspid valve
A B
C D
Figs 70.52A to E: Two-dimensional transesophageal echocardio-
graphy. A 78-year-old black female. (A and B) Thrombic obstruction
of distal left and right pulmonary arteries. Arrowhead points to a
large thrombus in the distal left pulmonary artery (LPA, A) and in
the distal right pulmonary artery (RPA, B); (C to E) Thrombotic
occlusion of LPA descending lobar branches; (C) Large arrowhead
points to the thrombus (TH) and small arrowhead points to an
echolucent area of clot lysis. Arrow shows extension of the TH into
a descending lobar branch of the LPA; (D) Turbulent flow signals
with prominent flow acceleration are noted in a descending lobar
branch (labeled 2). Arrow points to the left pulmonary vein (LPV),
and arrowhead shows the TH; (E) Color Doppler-guided continuous
wave Doppler interrogation (arrow) shows a high systolic velocity
of 2.2 m/s and a high diastolic velocity of 1.0 m/s indicative of
significant obstruction. (AO: Aorta; T: Transverse plane) (Movie
clips 70.52A to E).
Source: Reproduced with permission from Kang SW, Nekkanti R,
Nanda NC, et al. Transesophageal echocardiographic identifica-
tion of thrombus producing obstruction of left pulmonary artery de-
scending lobar branches and bronchial artery dilatation. Echocar-
E diography. 2002;19:83–8.
A B
C D
Figs 70.53A to D: Transesophageal echocardiographic finding of LAA lobe mimicking a mass lesion. (A) The arrowhead points to what
appears to be a mass in or adjacent to LAA; (B) Schematic; (C) Keeping the mass-like lesion in the middle of the monitor screen and
rotating the transducer from 0° to 180° shows that the mass-like effect is produced by a lobe (#3) of the LAA; (D) Schematic. Numbers
1 and 2 denote the other two lobes of the LAA. (AO: Aorta; LA: Left atrium; LAA: Left atrial appendage; LV: Left ventricle; M: Mass; MV:
Mitral valve; PA: Pulmonary artery; PE: Fluid in the transverse sinus of pericardium) (Movie clip 70.53).
Source: Reproduced with permission from Giove GC, Singla I, Mishra J, Nanda NC. Transesophageal echocardiographic finding of left
atrial appendage lobe mimicking a mass lesion. Echocardiography. 2011;28:684–5.
endocarditis, lead infection, or both were present. TEE was valves than mitral valves. This carries a poor prognosis in
clearly superior to TTE in this clinical setting (sensitivity most cases. TTE appears more useful in detecting mitral
23% vs 94%).171 than aortic perforations. Color flow Doppler imaging
Regurgitation of the infected valve is present in allows location of abnormal flows at the areas of anatomical
most cases resulting from a variety of mechanisms. They interruption and, therefore, it helps to differentiate mitral
include prevention of proper leaflet or cusp coaptation by cusp perforation from true mitral regurgitation. TEE is
the vegetation and valvular destruction that can result in recommended if a valve perforation is suspected and TTE
a small perforation in a cusp, or lead to a complete flail is negative or equivocal.
leaflet. Valvular perforation is a frequent complication that In the case of mechanical mitral prosthesis, TEE with
may cause severe insufficiency with an acute onset and color flow mapping is of importance in the diagnosis
precipitate heart failure. This is more common in aortic of paravalvar regurgitation. The presence of a new or
A B
Figs 70.54A to C: Two-dimensional (A and B) and three-dimensional

(C) transthoracic echocardiography. Tumor mimic following mitral
valve replacement. Arrowhead points to subvalvular apparatus
(chordae and papillary muscle) remaining in the left ventricle
following resection of the native mitral valve and replacement
with a prosthetic valve. This could be misdiagnosed as tumor or
vegetations. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA:
C Right atrium; RV: Right ventricle) (Movie clips 70.54A to C).
increasing paravalvar regurgitation or valve dehiscence is abscess is considered to be present when a definite region
a major criterion for the diagnosis of endocarditis. of reduced echodensity is found on the echocardiogram,
Extension of the infection to the perivalvar tissues is or when echolucent cavities within the valvular annulus
a sign of poor prognosis in the evolution of the disease. or adjacent myocardial structures are found in the setting
Extravalvar extension may lead to endothelial erosion, of valvular infection. Sensitivity and specificity of TTE
perivalvar abscess, mycotic aneurysm, and intracardiac for abscess detection were 28% and 99%, respectively,
fistulae. compared with 87% and 95%, respectively, with TEE. TEE is
especially useful in prosthetic endocarditis. The diagnosis
Perivalvular Abscess of aortic abscesses were easier than mitral abscesses, both
with TTE (42% vs 9%) and TEE (86% vs 57%).8
Perivalvar cavities are formed when annular infections
break through and spread into contiguous tissue. In native
aortic valve endocarditis, they generally occur through
MICE
the weakest portion of the annulus, which is near the Mesothelial/macrophage incidental cardiac excrescences,
membranous septum. Perivalvar abscesses are particularly or MICE, are made up of mesothelial cells, macrophages,
common in prosthetic valve endocarditis, since the scattered inflammatory cells and fibrin, and lack a vascular
annulus is the usual primary site of infection. A perivalvar network or supporting stroma.172 Lack of vascularity is an
A B
Figs 70.55A and B: Real time two-dimensional transthoracic echocardiography. Arrowhead points to a large echogenic mass involving
the posterior mitral annulus, consistent with calcification. An echolucent area consistent with liquefaction is seen in B. (AO: Aorta; LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clips 55A and B).
(Source: Reproduced with permission from Assudani J, Singh B, Samar A, et al. Echocardiography. 2010;27:1147–50).
important feature distinguishing them from localized

mesothelial hyperplasia, which can also form incidentally
detected excrescences and contain sheets of mesothelial
cells, macrophages, and fibrin. They are more commonly
found in the left heart chambers and on valve surfaces
especially during aortic and mitral valve surgery or in
endomyocardial biopsy specimens. On echocardiography,
it can appear as a free-floating mass or loosely adherent.
It has been described as isointense on MRI compared to
myocardium on T1-weighted images and hyperintense
on T2-weighted images, without enhancement with
contrast.173 While they are benign, they may be potentially
mistaken for a primary or metastatic malignancy.
Mitral Annular Calcification Fig. 70.56: Live/real time three-dimensional transthoracic echo-
cardiography. Cropped three-dimensional data set. The lower
Mitral annular calcification (MAC) is a common echo- arrowhead points to a highly echogenic calcification while the upper
cardiographic finding seen mainly in older patients and arrowhead shows a less echogenic area of uniform appearance.
those with chronic renal failure. Caseous calcification (MV: Mitral valve; TV: Tricuspid valve) (Movie clip 70.56).
Source: Reproduced with permission from Assudani J, Singh B,
is a rare variant seen as a large, round, echodense
Samar A, et al. Echocardiography. 2010;27:1147–50.
mass with smooth borders situated in the periannular
region, with no acoustic shadowing artifacts and definitive diagnosis and prevent more invasive testing
containing central areas of echolucencies resembling and even exploratory cardiotomy.
liquefaction.174 The diagnosis is made by TTE in most
cases but 2D or 3D transesophageal echocardiogram
(Figs 70.55 to 70.59; Movie clips 70.55A and B to 70.58A
Cardiac Calcified Amorphous Tumor
Parts 1 and 2, B, CD Parts1 and 2-D) are helpful in some Cardiac calcified amorphous tumor (CAT) is another
cases where the diagnosis is in question to make more rare benign cardiac mass that can mimic malignancy and
Fig. 70.57: Real time two-dimensional transesophageal echo-

cardiography. The arrowhead denotes posterior mitral annular
calcification. Note shadowing and reverberations beneath the
calcification. (LA: Left atrium; LV: Left ventricle) (Movie clip 70.57).
Source: Reproduced with permission from Assudani J, Singh B,
Samar A, et al. Echocardiography. 2010;27:1147–50.
A B
C D
Figs 70.58A to D: Live/real time three-dimensional transesophageal echocardiography in caseous mitral annular calcification.
(A and B) Four-chamber views. The arrowheads in A and B point to a large echogenic mass involving the posterior mitral annulus,
consistent with calcification. In B, the mass shows multiple, discrete, band-like, and punctuate echodensities surrounded by a highly
echogenic border. (C and D) Cropping of the four-chamber data set shows both a highly echogenic (lower arrowhead) and a relatively
less echogenic (upper arrowhead) components of the mass. Transverse cropping (D) also reveals a pattern of highly echogenic
component as well as a relatively less echogenic area with multiple, small, discrete band-like, and punctate echodensities. (AML:
Anterior mitral leaflet; AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). [Movie clips 70.58A (Parts 1 and 2), B to D
(Parts 1 and 2)].
Source: Reproduced with permission from Assudani J, Singh B, Samar A, et al. Echocardiography. 2010;27:1147–50.
A B
Figs 70.59A and B: Two-dimensional transesophageal echocardiography. Extracardiac tumor. (A and B) Tumor (T, arrow) is seen pos-
terior to the aortic root bulging into the left atrium (LA). (AO: Aorta; LV: Left atrium; RV: Right ventricle).
Source: Reproduced with permission from Nanda NC, Domanski MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2007:524.
cause symptoms due to obstruction or embolization of Hiatal hernias can also be seen on TTE and TEE
calcific fragments.175,176 Typical findings for CAT include as an extracardiac mass compressing the left atrium
calcium nodules within amorphous fibrinous materials with associated mild left ventricular hypertrophy with
often displaying characteristic histological findings that normal function and no thrombus in the LA. If a hiatal
include nodular calcium deposits, chronic inflammatory hernia is suspected, use of oral contrast or soda during
cell infiltration, hyalinization, and degenerating blood TTE may be diagnostic, but during TEE it carries the
elements.175 Intracardiac echocardiogram will demonstrate risk of aspiration.178 Juxtacardiac pulmonary atelectasis
curvy linear densities representative of endomyocardial or lobar collapse can also simulate pericardial tumor
calcifications.177 implants on echocardiographic examination (“pericardial
pseudotumor”) from surrounding compressive effusive
Extracardiac Masses fluid.179 Echocardiographic delineation of pericardial and
pleural anatomy can be used to delineate the atelectatic
In addition to normal cardiac variants, certain extracardiac nature of these masses, combined with ancillary
masses can also mimic cardiac tumors by compressing radiographic and CT studies. In addition, drainage of
the cardiac chambers from the outside of the heart and pleural fluid will lead to the disappearance of the masses
creating a mass effect. Examples include tumors or diseases on echocardiographic examination.
of the mediastinum (Figs 70.59 to 70.63), hematomas
(Fig. 70.64), coronary aneurysms, and pseudoaneurysms.
Although not uncommon, pericardial fat deposition
Intracardiac Hardware
when prominent can masquerade as extracardiac tumor In addition to intrathoracic masses mimicking cardiac
(Figs 70.65A and B; Movie clips 70.65A and B). tumors, intracardiac hardware such as Impella per-
Sclerosing mediastinitis is a rare disorder characterized cutaneous left ventricular assist device catheters, pacing
by the invasive proliferation of fibrous tissue within the leads, Swan–Ganz catheters, and central line catheters
mediastinum and frequently results in the compression are also commonly seen left atrial foreign bodies on
of vital mediastinal structures including the heart. It can echocardiography. Catheters and pacemaker leads are
cause compression of the atrial or ventricular cavities usually seen on echocardiography as two parallel thin
and can cause obstruction of the SVC and the pulmonary linear echodensities separated by an echolucent slit that
arteries (Figs 70.60 to 70.63). produce typical reverberations and side lobe artifacts
A B
Figs 70.60A to C: Two-dimensional transesophageal echocardi-

ography. Sclerosing mediastinitis. This 43-year-old man presented
with respiratory failure. (A) A mass (M) surrounds the left atrium
(LA), right pulmonary artery (RPA), and superior vena cava (SVC);
(B and C) The mass appears to infiltrate and invaginate into the LA
and extends up to the base of the left atrial appendage (LAA). This
resembles an intracardiac tumor. (AO: Aorta; AV: Aortic valve; PA:
Pulmonary artery; RA: Right atrium; RVO: Right ventricular out-
flow).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in scle-
C rosing mediastinitis. Echocardiography. 1996;13:103–8.
A B
Figs 70.61A and B
Figs 70.61A to C: Two-dimensional transesophageal echo-

cardiography. Sclerosing mediastinitis. Same patient as in Figures
70.60 A and B. Both the right lower pulmonary vein (RLPV) and
right upper pulmonary vein (RUPV) demonstrate obstruction near
their entrance in the left atrium (LA). The exact sites of obstruction
in the lower and upper pulmonary veins, shown by the arrow and
the arrowhead, respectively, mark the transition from laminar (red)
to disturbed (mosaic) flow; (C) Pulsed Doppler interrogation of
the mosaic flow reveals a high velocity of 2.58 m/s, indicative of
obstruction. (LV: Left ventricle; M: Mass; RVO: Right ventricular
outflow; SVC: Superior vena cava).
Kim KS, et al. Transesophageal echocardiographic findings in
C sclerosing mediastinitis. Echocardiography. 1996;13:103–8.
A B
Figs 70.62A to C: Two-dimensional transesophageal echocardio-

graphy. Sclerosing mediastinitis. Same patient as in Figures 70.60
and 70.61. (A and B). The arrow points to the site of obstruction in
the superior vena cava (SVC) near its junction with the right atri-
um (RA). Color Doppler examination shows a thin mosaic flow jet
in (B), indicative of obstruction; (C) Pulsed Doppler interrogation
reveals a high velocity of at least 1.61 m/s. (LA: Left atrium; M:
Mass; RPA: Right pulmonary artery).
Kim KS, et al. Transesophageal echocardiographic findings in
C sclerosing mediastinitis. Echocardiography. 1996;13:103–8.
A B
Figs 70.63A and B: Sclerosing mediastinitis. Histology of mediastinal biopsy tissue from the same patient shown in Figures 70.60 to
70.62. (A) Photomicrograph of sclerosing process impinging on mediastinal adipose tissue (congo red, original magnification ×125);
(B) Photomicrograph of collagenization of blood vessel wall with narrowing of the lumen (center), a region of cellular fibrosis (above),
and a region of acellular fibrosis (below; hematoxylin and eosin, original magnification ×125).
Source: Reproduced with permission from Kovach TA, Nanda NC, Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:103–108.
Fig. 70.64: Two-dimensional transesophageal echocardiography.

Hematoma following cardiac surgery. Arrowheads show hema-
toma that developed around the aortic root following aortic valve
replacement. It had a benign course. (AO: Aorta; LA: Left atrium;
LAA: Left atrial appendage).
Source: Reproduced with permission from Nanda NC and
Domanski MJ, editors. Atlas of Transesophageal Echocardiography.
2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:535.
shadowing other structures. Clinical correlation is important of a cardiac mass or tumor. Serial studies can provide
in assessing the echocardiographic presence of such objects. useful information regarding progression, regression, and
In summary, the integration of the echocardiographic results of treatment of the mass or tumor. In some cases,
findings with the patient’s medical history and clinical additional imaging modalities such as cardiac CT and MRI
scenario is critical to establishing a differential diagnosis can provide complementary information that enhances
and to determining the hemodynamic consequences the echocardiographic findings.
A B
Figs 70.65A and B: Two-dimensional transthoracic echocardiography. Epicardial fat pad. Arrows point to prominent epicardial fat depo-
sition both anteriorly and posteriorly visualized in parasternal long-axis view and around the cardiac apex in the apical four-chamber
view (Movie clips 70.65A and B). (LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
10. Singh A, Miller AP, Nanda NC, et al. Papillary fibroelastoma

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SECTION 6
Congenital Heart Disease
Chapters
Chapter 71 Fetal Cardiac Imaging Chapter 74 Three-Dimensional Echocardiography in
Chapter 72 M-Mode and Two-Dimensional Echocardiography Congenital Heart Disease
in Congenital Heart Disease Chapter 75 Echocardiography in the Evaluation of
Chapter 73 Real time 3D Echocardiography for Adults with Congenital Heart Disease
Quantification of Ventricular Volumes, Mass and Chapter 76 Echocardiographic Evaluation for Acquired Heart
Function in Children with Congenital and Acquired Diseases in Childhood
Heart Diseases
1527
CHAPTER 71
Fetal Cardiac Imaging
Aarti H Bhat
Snapshot

Scope of Fetal Cardiology 
Extracardiac Reasons and AssociaƟons for Fetal Heart

IndicaƟons for Fetal Cardiac EvaluaƟon Disease

Fetal Physiology 
Fundamentals of Fetal Cardiac Imaging

IndicaƟons for Fetal Echocardiography 
Case Studies
INTRODUCTION outflow views yields a sensitivity of 52 to 92% in detecting

congenital heart defects. The range is similar in high-
The field of fetal cardiology has made significant advances risk populations. A fetal echocardiogram (traditionally
along with all aspects of prenatal evaluation over the last acquired if the screening ultrasound is positive and/or in
three decades. Almost all structural fetal heart disease is high-risk pregnancies) has a sensitivity of 42 to 100% for
amenable to a detailed in utero diagnosis that can then
detection of congenital heart disease.4–11 Over the years,
be used to develop plans for fetal as well as postnatal
many studies have proven aspects of improved survival
management of a pregnancy. Fetal ultrasound and two-
or decreased morbidity in groups of newborns that have
dimensional imaging are central to this diagnostic process.
received a prenatal cardiac diagnosis that translates into
Improvements in ultrasound techniques as well as our
a specific birth plan and postnatal management including
enhanced understanding of fetal cardiac anatomy and
emergent neonatal intervention.12–17 Identification of
physiology have facilitated and broadened the scope of
critical heart disease can allow in utero surveillance
this specialty. The purpose of this chapter is to familiarize
culminating in therapy in the form of maternal medications,
the reader with the basic framework of fetal cardiology
fetal cardiac intervention, and a heightened awareness
using a basic description of echocardiographic anatomy.
of immediate requirements in the delivery room or soon
Liberal use of illustrative labeled screen images as well as
after.18,19 Despite the presence of the need for prenatal
movie clips (on-line version) are intended to orient the
diagnosis and the presence of robust tools to achieve this,
reader as well as provide an example of the anatomical
the overall rate of prenatal detection by routine obstetric
defect or finding being discussed.
scan is unfortunately low.20–22
Heart defects in a fetus may be “isolated” if there is no
SCOPE OF FETAL CARDIOLOGY other organ or genetic abnormalities or “associated” with
Cardiac malformations occur in 4 to 8/1,000 live births other organ anomalies or genetic problems. Determining
and account for one-third of perinatal mortality due to a possible abnormality in fetal life allows for a more
congenital anomalies.1–3 In the low-risk general popu- detailed evaluation of the other organ systems, fetal
lation, an obstetric scan using the four-chamber and growth and well-being, and chromosomal disorders. Such
1528 Section 6: Congenital Heart Disease
a process may lead to an overall complete fetal diagnosis, referral if there are concerning or unusual findings, and
allowing the involved teams to plan a process of pregnancy improved access to fetal cardiologists are each a key step
management. Practice care models are unique to each in overall improvement in the rates of prenatal diagnosis
institution and vary greatly depending on the setting of fetal heart defects. Critical congenital heart disease such
(primary vs. tertiary), specialty (obstetric, pediatric, or as atresias of the atrioventricular valve with or without
combined) or skill and resource level (screening center accompanying hypoplastic ventricles, cardiac dysfunction,
or referral center). There are potentially many good and and pericardial effusion are amenable to detection from a
appropriate ways to achieve the endpoints of prenatal four-chamber view. However, other critical diseases such
cardiac care, which are (a) as accurate and complete a as transposition of the great arteries and semilunar valve
diagnosis as possible; (b) a complete, fact-driven, and hypoplasia/atresia can only be detected if biventricular
compassionate counseling; and (c) securing a safe birth outflow view can be obtained. The parallel orientation of
and transition plan for the maternal–fetal dyad and the the great arteries can be easily determined in the outflow
newborn. This chapter is aimed at providing the reader with view even if the four-chamber view indicated no obvious
a good understanding of the first necessary undertaking of abnormality [for example, in a dextro Transposition of great
anatomical diagnosis. arteries (dTGA) without ventricular septal defect (VSD)].
Prenatal counseling is very stressful on the parents Nuchal translucency (NT) detects the echo-free space at
for whom the hope and prospect of a normal baby is the back of the neck, typically measures at 11 to 14 weeks
now changed. A genuine compassionate and honest of gestation. Increased NT (typically > 4 mm) is strongly
approach to the interview session needs to secure their associated with abnormal chromosomes, with or without
understanding and trust, as the whole team establishes congenital heart disease.24,25 Often times, at this stage,
common goals. Parental grief, stress, and depression need with or without a confirmed diagnosis of fetal aneuploidy
to be acknowledged as these are known to continue from depending on when and if the parents and providers are
the prenatal to the postnatal periods.23 proceeding with an amniocentesis or chorionic villus
biopsy (CVS), the pregnancy is referred for a fetal cardiac
INDICATIONS FOR FETAL CARDIAC evaluation. Even in the absence of confirmed aneuploidy,
EVALUATION elevated fetal NT in the first trimester and other “soft
markers” such as intracardiac echogenic focus (IEF) and
A screening obstetric ultrasound at about 18 to 20 weeks intrauterine growth retardation (IUGR) or extracardiac
is usually performed in most obstetric programs. The pathology may be indications for fetal echocardiography.
primary aim of this scan is to acquire all pertinent fetal and
uteroplacental information in a particular pregnancy. An
abnormal fetal cardiac or extracardiac scan at this gestation
FETAL PHYSIOLOGY
as well as a suspected or proven chromosomal anomaly Fetal circulation appears uniquely adapted to maximize
prompts a fetal cardiac evaluation and is the single most efficiency and oxygen delivery. Some of the most
robust source for fetal cardiac pathology. The vast majority exhaustive information on fetal physiology was originally
of these patients do not have any of the risk factors typically derived from fetal sheep.26 Similar data from the human
considered as “high risk.” Pregnancies considered at “high fetus is incomplete. Oxygenated blood is delivered to
risk” for fetal cardiac and noncardiac pathologies are the fetus through the umbilical vein. This vein enters the
the dominant cause for referral to the fetal cardiologist, porta hepatis and gives several branches to the left lobe
but, the relative yield of abnormal fetal cardiac exams is of the liver, distal to these is the ductus venosus. The
relatively lower from this indication group. This brings umbilical veins then arches toward the right lobe of the
up an interesting aspect while planning a fetal cardiac liver, is joined there by the portal vein, and gives branches
program aiming to increase prenatal diagnosis rates in to the right lobe of the liver. The ductus venosus then
that most of the abnormal fetal heart diagnoses actually continues on and connects to the inferior vena cava, as
come from a “low-risk” group. This fact implies that any do the left hepatic veins. Flow from the ductus venosus is
increase in fetal cardiac diagnosis over a population preferentially directed by the Eustachian valve, across the
can only come from an improvement in the obstetric foramen ovale to the left atrium. Nearly the entire return
“screening” scan. Improving the awareness of obstetric from the superior vena cava enters the right ventricle
sonographers, close attention to and low threshold for through the tricuspid valve.27–31 Right ventricular output
Chapter 71: Fetal Cardiac Imaging 1529
[55% of the combined biventricular output (CVVO)] is for fetal echocardiography. Maternal metabolic disorders
directed across the pulmonary valve, most of this enters such as diabetes and phenylketonuria are known to
the ductus arteriosus, and joins the aortic arch at the increase the risk for congenital cardiac defects. Maternal
isthmus. Just under 20% of the combined cardiac output diabetes in particular has been the cause of much
enters the pulmonary bed through the pulmonary arteries controversy in terms of screening recommendations.36–40
and returns to the left atrium. Left ventricular output The incidence of fetal cardiac defects is noted to be
(45% of the CVVO) is directed across the aortic valve to the as much as five time higher than euglycemic pregnancies
ascending aorta. Most of this amount flows to the coronary and worsens with HbA1c levels > 6.3% in the first trimester.
arteries, and the head and neck vessels, so that only 10% of Existing strategies are based on resource utilization
the CVVO actually crosses the isthmus, the region of the models (comprehensive fetal obstetric ultrasound in
distal aortic arch between the origins of the left subclavian the pregestational diabetic population, followed by fetal
and the insertion of the ductus arteriosus. The aortic and cardiac referral in the presence of concerning findings)
ductal arches merge, and the descending aorta carries or risk-based models (risk increases with elevated HbA1c
supply to the abdominal organs and then to the umbilical levels).41 Maternal autoimmune disease such as Sjogren
artery, headed to the placenta. In this manner, the entire syndrome or systemic lupus erythematosus has an
umbilical venous flow is returned to the umbilical arterial increased incidence of fetal congenital complete heart
side. block (CCHB) that can be detected as well as monitored
The CVVO in the fetus is about 450 mL/min/kg of by fetal echo and for which therapeutic options exist.42
estimated fetal weight and the right:left ventricular output Maternal teratogen exposure, for example, alcohol, lithium,
ratio is about 1.2 to 1.3.32,33 CVVO is determined by heart retinoic acid anticonvulsants, and SSRI antidepressant
rate and stroke volume, and the latter is determined by have noted increase in fetal cardiac and extracardiac
preload and afterload, very similar to the physiological malformations. Maternal and intrauterine infections such
inter-relationships seen in children and adults. However, as congenital rubella and coxsackie virus can be seen with
fetal myocardium generates less tension at similar muscle cardiac defects. The use of in vitro fertilization or other
lengths as compared to adult myocardium and there may reproductive assist techniques are being increasingly
be differences in the sarcoplasmic reticulum as well as recognized with elevated rate of congenital cardiac
calcium uptake function.34,35 malformations.
In the fetus, the two natural right–left communications Fetal indications such as a fetal heart abnormality
at the level of the foramen ovale and the ductus arteriosus suspected on a screening obstetric ultrasound, extra-
allow equalization of the right and left pressures at the cardiac structural abnormalities, single umbilical artery,
atrial and great arterial levels. The left ventricular–aortic chromosomal abnormalities—confirmed or suspected—
output upto and including the aortic isthmus is likely a fetal arrhythmia, multiple gestations, and fetal non-
higher afterload as compared to the right ventricular– immune hydrops.
pulmonary trunk output that has a higher compliance
placental bed distally.
EXTRACARDIAC REASONS AND
INDICATIONS FOR FETAL ASSOCIATIONS FOR FETAL
ECHOCARDIOGRAPHY HEART DISEASE
Twin gestations can have unique cardiovascular findings,
Maternal Indications more common in monochorionic gestations.
Twin–twin transfusion syndrome (TTTS) can occur due
Family History of Congenital Heart Disease to abnormal placental vascular connections in about 15%
Family history of transmissible genetic malformations of monochorionic twin pregnancies, increasingly seen due
that have known cardiac abnormality such as 22q11 to more widespread use of reproductive technologies.43
deletion syndromes, Marfan or Noonan syndrome as well There is discordance in fetal size. The recipient twin is
as multifactorial-etiology type defects such as ventricular larger, has polyhydramnios, cardiomegaly, biventricular
septal defect or bicuspid aortic valve-hypoplastic left heart hypertrophy and dysfunction, and atrioventricular valve
syndrome spectrum in first order relatives is an indication incompetence. In approximately 10%, there is acquired
right ventricular outflow tract obstruction, pulmonary dimensions is also noted, possibly due to decreased filling,
stenosis, and even reversal of ductal flow.44,45 Eventually, but may also eventuate in obstructive left-sided lesions.
recipient cardiomyopathy from systolic as well as diastolic Sacrococcygeal teratoma is a pluripotential tumor
dysfunction can lead to hydrops and fetal demise. The arising from the sacrococcygeal region. This is the
donor twin, on the other hand, is likely to have a structurally commonest tumor seen in the fetus, albeit rare and
and functionally normal heart, evidence of elevated its major cardiac impact is to cause high-output heart
placental vascular resistance, relative hypovolemia, and failure and hydrops in the fetus. Serial evaluation of the
oligohydramnios. Scoring systems are used to gauge CVVO is indicated to decide on need and timing for fetal
disease severity.46,47 Laser photocoagulation of the intervention in the form of a cyst aspiration, laparoscopic
placental vascular connections is currently the treatment laser ablation, surgical debulking, or early delivery.
of choice for TTTS.48 The abnormal cardiovascular findings
can improve after this therapy, although complete norma- FUNDAMENTALS OF FETAL
lization is unlikely in the subgroup that has developed
anatomical pulmonary atresia.49
CARDIAC IMAGING
Twin reverse arterial perfusion (TRAP) occurs in 1% of Two-dimensional imaging is the principal imaging
monochorionic pregnancies, in which one twin is acardiac modality for fetal heart disease detection. Basic ultrasound
with a primitive or nonfunctional heart. The normal twin principles apply to fetal imaging, although with many
heart pumps blood through superficial arterial–arterial additional levels of technical challenges as well as
placental anastomosis to this acardiac twin. As a result, the opportunities. In general, a combination of obstetric and
direction of flow in the umbilical artery of the acardiac– pediatric cardiology skills is required. Flexibility, familiarity
acephalic twin is from placenta to fetus (reversed). Serially as well as perseverance are essential to get as complete
evaluating the combined cardiac output of the normal twin and accurate a fetal cardiac examination as possible. The
can monitor for the increased cardiovascular workload to small size of fetal cardiac structures as well as the relatively
this heart, which can culminate in heart failure, hydrops, faster fetal heart rate can challenge the spatial as well as
and demise.50 temporal resolution capabilities of the ultrasound system.
Congenital cystic adenomatoid malformation (CCAM) Accordingly, a higher frequency transducer, minimal
is a rare developmental abnormality of the lung due distance between the fetal heart and the transducer on
to overgrowth of the terminal bronchioles, causing a the maternal abdomen, appropriate depth and width
dysplastic lung mass. This mass can cause compression of the region of interest, and dynamic focusing are all
of the developing fetal heart, alter its position within the important considerations and need to be optimized. Since
thorax, alter the angulation of the inferior vena cava as it axial resolution typically exceeds radial resolution, the
enters the displaced heart, decrease cardiac filling, and transducer can be moved on the maternal abdomen so as
potentially lead to fetal hydrops. Due to the increased to insonate the region of interest at minimal angulation.
intrathoracic pressure from the lung mass, hydrops is not Similar to the pediatric cardiac exam, each region of
typically accompanied by pleural or pericardial effusions.51 interest needs to be seen in multiple views to get an accurate
Congenital diaphragmatic hernia (CDH) is a defect impression. Again, similar to a pediatric cardiac exam,
in the muscular diaphragm between the chest and a segmental completeness is necessary. Fetal position,
abdominal cavities. Abdominal contents can herniate movement, and variable transducer positions make this a
through this defect and occupy space in the chest, literal moving target and while a protocolized progression
causing varying degrees of displacement and extrinsic of sweeps and views cannot be realistically applied,
compression to the heart and lungs. Since this process every attempt should be made to demonstrate as many
happens in early gestation, the presence of herniated anatomical and functional components of each segment.
contents can adversely and proportionately impact the A lower frequency transducer, even a nonobstetric one
development of lung parenchyma as well as vasculature may be occasionally helpful in a later trimester gestation,
causing pulmonary hypoplasia. CDH can occur along with where more penetration is required. This strategy may also
structural heart disease as part of a syndrome (e.g. Wolf– be required in the rare event of an aliasing Doppler signal
Hirschhorn syndrome, trisomy 18, vitamin A exposure), with the usual obstetric transducer. Harmonic imaging
the commonest being ventricular septal defect. Curiously, may enhance the blood–tissue interface and permit
a decrease in left ventricular wall as well as cavity better assessment in some challenging cases. In early
gestation and while the pregnancy is still pelvic, the use

of endovaginal transducers can permit the best resolution
studies in the first trimester and may add information to
transabdominal scans.52,53 Guidelines have been published
for the “basic” and “extended basic” fetal cardiac scan and
the reader intending to formalize a fetal echo protocol is
encouraged to read them.54,55
By convention, the transducer notch is always pointing
toward the maternal left side. Even as multiple sweeps
will require clockwise and anticlockwise rotation, it is
important to keep in mind that the notch must be kept in
the left two quadrants to avoid confusion. The left/right
invert button may allow better pattern recognition in some
situations and readers, but, overall, inadvertent switching
can cause tremendous errors in situs assessments and Fig. 71.1: Cardiac situs, cardiothoracic ratio, and cardiac axis
determination. Fetal cardiology consult was requested due to
should be avoided or vigilantly supervised. A fetal cardiac
older sibling with tricuspid atresia. Gestational age is 28 3/7 weeks.
exam must begin with knowledge of the fetal indication, In the Cordes technique demonstrated, the fetal position within the
obstetric findings, and other concerning extracardiac uterus is determined and a short axis of the fetal chest is obtained
anatomical findings in the fetus. The key elements are with the fetal head on the right of the screen. Fetal heart and
quite comprehensive and include assessment of the cardiac apex to the right of the fetal spine in this technique indi-
cate levocardia. Situs can also be cross-checked with the second
fetal number and position in the uterus. Determining technique demonstrated in Figure 71.2. The cardiothoracic ratio
the abdominal and cardiac situs needs to be established is determined as a ratio of the fetal heart compared to the tho-
at the very beginning of the study. This can be done by racic area. In this example, it is 8.27/35.31 cm2 = 23% and normal.
establishing the fetal presentation followed by the fetal left The cardiac axis is the imaginary axis between the line joining the
fetal spine with the middle of the fetal frontal chest and the line
and right side—the use of a doll to illustrate the relative
drawn along the plane of the interventricular septum. It is 30°
location of a fetus in the uterus is often helpful to interpret in this example and normal. This Figure correlates with Movie
a uniplanar display. Another method that has been used clip 71.1.
requires the fetal scan to demonstrate the fetal head to the
right of the screen. From this position, a 90° rotation of the and need to be pursued in a fractionated manner
transducer will bring the fetal heart into a cross-sectional (Figs 71.1 to 71.37, Movie clips 71.1 to 71.26). It is helpful to
view. Levocardia is interpreted from a fetal heart and apex keep a mental checklist that allows a complete anatomical
that are located and directed to the right of the fetal spine and functional three-dimensional (3D) picture to form
in this position.56 Ideally, the fetal cardiologist should be while the study is being conducted or reviewed.
able to interpret both methods and even use both in each
case as a mechanism for cross checking. As part of this situs
Four-Chamber View
sweep, the location of the heart and stomach on the left,
and inferior vena cava on the right establishes abdominal This view is part of the obstetric standard for anatomy
as well as cardiac situs. The position of the heart within the scan. Some very important and critical cardiac lesions are
fetal chest may itself indicate potential abnormality if it is readily picked up in this view such as hypoplastic left or
located in the right chest, apex is pointed to the right side of right heart, mitral or tricuspid atresia, atrioventricular (AV)
the fetus, or if the cardiac axis is abnormal. The fetal cardiac canal defects balanced or unbalanced, double inlet left
axis is the angle between the plane of the interventricular ventricle, single ventricle type situations, and large septal
septum and the presumed midline and has been noted to defects. While such a diagnosis may be striking at the very
be 43 ± 7.57 The cardiothoracic area should be under 40%— onset of the examination, this view alone is inadequate
a relatively larger heart occurs in cardiac lesions such as for a complete assessment. In more subtle situations of
Ebstein’s anomaly and a relatively larger lung component ventricular or atrioventricular valve discrepancy, each of
is noted in severe pulmonary cystic lesions. The following these structures needs to be measured in the cardiac cycle
views comprise a complete exam, although of course they that reaches its maximal dimension and compared to
may not be acquired in this order and may be incomplete gestational age-based norms.58–60 This view demonstrates
Fig. 71.2: Situs determination and ventricular morphology. The Fig. 71.3: Inferior vena cava (IVC), hepatic veins (Hep V), and
fetus is in vertex position. With the transducer notch toward the left ductus venosus (DV). The inferior vena cava is right-sided in this
of the maternal abdomen, this image indicates the heart is within the fetus with levocardia. It is joined by the right and left hepatic veins
left chest. Situs can also be cross-checked with the second tech- as well as the ductus venosus, which can be identified as a turbu-
nique demonstrated in Figure 71.1. The descending aorta (DAo) is lent region on color Doppler along the course of the umbilical vein
seen in cross section to the left of the fetal spine. The left atrium is to the IVC. These venous structures connect to the right atrium
the left-sided upper chamber, identified by the presence of a right (RA). This Figure correlates with Movie clip 71.3.
and a left-sided pulmonary vein entering it posteriorly (marked *).
The redundant foraminal membrane is seen to separate the right
and left atria and is usually bulging into the left atrium secondary to
right-to-left shunting at this level across the patent foramen ovale
(PFO). The primum component of the interatrial membrane is noted
to be intact. The morphologically right ventricle (RV) is noted with a
trabeculated apex. The left ventricle (LV) has a more rounded apex
with less trabeculations. The tricuspid valve (TV) and mitral valve
(MV) are seen on the right and left sides, respectively. This Figure
correlates with Movie clip 71.2.
Fig. 71.4: Two-dimensional (2D) bicaval view. In this transverse Fig. 71.5: Color Doppler across interatrial membrane. The intera-
view, the fetal head is to the left of the screen and the fetal abdo- trial membrane region is marked * and indicates right-to-left shunt-
men is toward the right. The inferior vena cava (IVC) receives the ing from the right atrium (RA) to the left atrium (LA) in this normal
hepatic veins (**) before joining the right atrium (RA). The right fetus. (Movie clip 71.5).
superior vena cava (RSVC) is seen entering the RA from the cra-
nial aspect of the fetus. The diaphragm separates the liver and
abdominal contents from the lung and the heart in the thorax. The
azygous vein (*) is seen joining the RSVC. (Movie clip 71.4).
Fig. 71.6: There is laminar color Doppler flow across the tricuspid Fig. 71.7: Pulsed wave Doppler across the tricuspid valve showing
(TV) and mitral (MV) valves from the right (RA) and left atria (LA) “a” wave dominance in this 28-week fetus. (Movie clip 71.7).
to their respective ventricles (RV, LV). There is no obvious shunt-
ing at the interventricular septal level. (Movie clip 71.6).
Fig. 71.8: Pulsed wave Doppler across the mitral valve showing in Fig. 71.9: Color Doppler of pulmonary veins. The pulmonary
this 28-week fetus. (Movie clip 71.8). venous connections are elicited by applying color Doppler in views
demonstrating the back of the left atrium and lowering scales until
they can be easily visualized and interrogated with pulse Doppler.
This Figure correlates with Movie clip 71.7. (DAo: Descending
aorta; LLPV: Left lower pulmonary vein; LUPV: Left upper pulmo-
nary vein; PFO: Patent foramen ovale; RUPV: Right upper pulmo-
nary vein). (Movie clip 71.9).
Fig. 71.10: Pulse Doppler of pulmonary vein. In this image, the right
upper pulmonary vein is being interrogated after it was identified on
color Doppler. Note that there is some contamination of the signal
with the right pulmonary artery signal—seen as the sharp systolic
wave below the baseline. This coincidentally demonstrated the “s”-
wave of the pulmonary venous Doppler, followed by the “d”-wave.
(Movie clip 71.10).
Fig. 71.11: Short-axis mitral valve, interventricular septum, mod- Fig. 71.12: Short axis of interventricular septum (IVS). Multiple
erator band attachment. The left ventricle (LV) is identified by its sweeps up and down of the interventricular septum need to be
circular contour, presence of two papillary muscles and mitral valve performed to determine the ventricular morphology as well as look
(MV), and smooth interventricular septum. The right ventricle is for any obvious drop-outs suggestive of ventricular septal defects.
the anterior chamber and its outflow is seen wrapping around the This Figure correlates with Movie clip 71.12.
left ventricle in a relatively oblong manner. The septal attachment
of the moderator band is identified. (Movie clip 71.11).
Fig. 71.13: Color Doppler ventricular septum short axis. Color Fig. 71.14: Two-dimensional (2D) biventricular outflow views show
Doppler is applied to images in Figure 71.12. The interventricular crisscrossing. From the four-chamber—equivalent view of the
septum is intact in this view. This Fig. correlates with Movie clip fetal heart—sweeping anteriorly in the fetal chest brings out the left
71.13. ventricular outflow tract (marked in cross hatched line) and then
the right ventricular outflow tract (marked by continuous line) as
they arise from their respective ventricles. The outflow tracts are
noted to crisscross and this aspect is important to identify normal
outflow anatomy. This Figure correlates with Movie clip 71.14.
atrial as well as ventricular morphology and atrioventricular and bilateral AV valve morphology and function. The left
concordance. A posterior sweep to the coronary sinus and atrium is the most posterior structure and at least one right-
anterior sweep to the pulmonary valve will show critical and one left-sided pulmonary vein can be seen entering it
intervening cardiac structures such as septum primum, in this view, confirmed on color and spectral Doppler. The
inlet, membranous and muscular interventricular septum, coronary sinus can be seen as a small structure parallel
Fig. 71.15: Two-dimensional (2D) right ventricular outflow, pulmo- Fig. 71.16: Two-dimensional (2D) left ventricular outflow tract. The
nary valve (PV). The right ventricle (RV) is the anterior chamber left ventricle is identified. Sweeping slightly anteriorly in the fetal
and its outflow is seen wrapping around the left ventricle in a rela- chest and with some angulation to bring out the left ventricular
tively oblong manner. The PV and main pulmonary artery (MPA) outflow tract, which is seen to be unobstructed and open in this
are seen in this image. The aortic valve is seen at the crux of the view. The aortic valve (AoV) and ascending aorta (Ao) are noted.
heart at the same level as the PV and is marked as X in this image. The right pulmonary artery is seen to traverse behind the ascend-
This image correlates with Movie clip 71.15. ing aorta. The membranous region of the interventricular septum
between the aortic valve and the tricuspid valve is additionally
seen in this particular image (*). This image correlates with Movie
clip 71.16.
Fig. 71.17: Color Doppler laminar flow across pulmonary Fig. 71.18: Color Doppler laminar flow across aortic valve ( )
valve (X). (Movie clip 71.17). and aortic arch (AoAr). (Movie clip 71.18).
to the posterior left atrioventricular groove, entering into and the bulging of thin foraminal rims into the left atrium
the inferior aspect of the right atrium—obvious dilatation indicates the direction of shunting at this level from right
can be seen in conditions where it receives a left-sided atrium to left atrium—this can be confirmed on color
superior vena cava (LSVC). An intact septum primum Doppler in orthogonal views of the interatrial membrane.
can be critical to the diagnosis of an endocardial cushion The tricuspid valve and the right ventricle are mildly
defect or atrioventricular canal defect (AVCD). A large dominant toward latter gestation, although both sides
drop out in the interatrial membrane is readily obvious appear relatively symmetric in earlier gestation. Defects
Fig. 71.19: Pulse Doppler across pulmonary valve. (Movie clip Fig. 71.20: Pulse Doppler across proximal transverse aortic arch.
71.19). (Movie clip 71.20).
Fig. 71.21: Short axis of aortic valve (marked as X). The symmet- Fig. 71.22: Two-dimensional (2D) short axis both semilunar
ric and trileaflet anatomy of the normal aortic valve is seen in this valves and right pulmonary artery (RPA). The pulmonary valve (P)
image. This view is particularly useful to illustrate the morphology is more anterior and slightly larger. The main pulmonary artery and
of an abnormal aortic valve as annular hypoplasia, valve thicken- its bifurcation into the right and left pulmonary arteries are noted
ing, and asymmetry. The tricuspid valve (TV) leading to the right by cranial angulation from this view or by opening these vessels
ventricle and right ventricular outflow tract (RVOT) is noted. The out from the three-vessel view. The right pulmonary artery trav-
patent foramen ovale (PFO) is well profiled in this view. (Movie erses behind the ascending aorta. (Movie clip 71.22).
clip 71.21).
in the inlet, membranous, muscular, and apical regions of side. This comparison has been found to be very useful
the interventricular septum may be evident in this view, in situations of ventricular hypoplasia, unbalanced AV
but all components of the interventricular septum should canal defects, severe semilunar valve obstructions, fetal
be assessed in multiple views and specifically in views coarctation and heart failure, or high-output situations.
perpendicular to the plane of the defect so as to minimize Fractional area of shortening may be used to assess systolic
drop out artifact due to parallel beam orientation. function. Color Doppler in this view demonstrates lack of
Deviation ≥ 2 standard deviations or z score ≥ 2 compared antegrade flow in AV valve atresia, valve incompetence,
to gestation-based norms as well as serial evaluation of VSD flow, and outflow directions. Spectral Doppler
ventricular major and minor dimensions are very helpful should be used to ascertain the inflow pattern of the right
in determining if one side is larger or smaller by itself or as well as left side as well as incompetence jets. Tissue
merely appears to be so due to a smaller or larger other Doppler has been used in an attempt to analyze systolic
Fig. 71.23: Two-dimensional (2D) of ductal and aortic arches, isth- Fig. 71.24: Transverse aortic arch, and head and neck vessels.
mus (*). The ductal arch (DuAr) is anterior and describes a gradual The fetal head is to the left of this screen image. The transverse
curve slightly rightward and posteriorly where it is met at the isth- aorta (TrAo) has been opened out to demonstrate each of the
mus (*) by the more posterior and acutely angulated aortic arch head and neck vessels, the last one being the left subclavian
(AoAr). (Movie clip 71.23). artery (LSCA). This image set is important to demonstrate head
and neck vessels as well as indicators or aortic arch anomalies.
The aortic isthmus is distal to the LSCA and needs to be clearly
visualized to rule out aortic coarctation. (Movie clip 71.24).
Fig. 71.25: Two-dimensional (2D) of three-vessel view and pul- Fig. 71.26: Two-dimensional (2D) features of the ductal arch.
monary artery (PA) bifurcation. The orientation of the fetal chest The ductal arch is the continuation of the pulmonary artery after
is marked. The fetal spine (Sp) marks the posterior aspect. The it arises from the right ventricle (RV) and pulmonary valve (PV),
descending aorta (DAo) is seen just to the left and anterior of the and after this vessel has given rise to the right pulmonary artery
spine. The three vessels noted from left to right as well as anterior (*) and left pulmonary artery (**). The ductal arch is the larger
to posterior are the PA, aorta (Ao), and right-sided superior vena of the two arches and dives posteriorly to join the aortic arch at
cava (RSVC) in that order of location as well as decreasing size. the aortic isthmus. In this Figure, the isthmus is not well profiled,
Identification of the location as well as relative sizes of the great and that view will need to be developed along with the aortic arch
vessels in this view is critical in detecting and confirming presence to establish anatomy as well as flow in the isthmic region. This
of obstruction, size discrepancy as well as arch anomalies. The is important to predict fetal aortic coarctation. (Movie clip
right pulmonary artery (RPA) is seen to arise from the main PA and 71.26).
traverses posterior to the aorta as well as RSVC as it heads to the
right lung hilum. (Movie clip 71.25).
Fig. 71.27: Two-dimensional (2D) features of the aortic arch. Fig. 71.28: Two-dimensional (2D) three-vessel view. In this ad-
The ascending aorta (AscAo) gives rise to the right innominate ditional example of a normal three-vessel view, the orientation of
(*), left common carotid artery (**), and the left subclavian artery the fetal chest is marked. The fetal spine (Sp) marks the posterior
(***) before it joins the ductal arch at the aortic isthmus. (Moive aspect. The descending aorta (DAo) is seen just to the left and
clip 71.27). anterior of the spine. The three vessels noted from left to right as
well as anterior to posterior are the pulmonary artery (PA), aorta
(Ao), and right-sided superior vena cava (RSVC) in that order of
location as well as decreasing size. Identification of the location
as well as relative sizes of the great vessels in this view is critical
in detecting and confirming presence of obstruction, size discrep-
ancy as well as arch anomalies. The right pulmonary artery (RPA)
is seen to arise from the main PA and traverses posterior to the
aorta as well as RSVC as it heads to the right lung hilum. (Movie
clip 71.28).
Fig. 71.29: Two-dimensional (2D) evaluation of the aortic isthmus. Fig. 71.30: Pulse Doppler across ductus venosus.
The aortic outflow in the crux of the heart is marked (**) and gives
rise to the aortic arch. The head and neck vessels are seen aris-
ing from the top of this arch. The isthmus is seen as the region
after the left subclavian opposite the insertion of the ductus
arteriosus (*).
Fig. 71.31: Pulse Doppler across inferior vena cava (IVC). Fig. 71.32: Pulsed wave Doppler of hepatic vein.
Fig. 71.33: Pulse Doppler across umbilical artery. Fig. 71.34: Pulse Doppler across umbilical vein.
and diastolic function of both ventricles,61,62 although

this tool has not been fully explored secondary to the
numerous unknown factors that can impact it and also
lack of robust interobserver variability. From this nodal
four-chamber view, a more anterior tilt will bring out the
left ventricular outflow. The aortic valve can be well seen
and measurements made in this view. Outflow tract as
well as aortic valve issues can be seen in this view and
further interrogated with color as well as spectral Doppler.
The left ventricular cardiac output can be calculated by
determining the systolic dimension of the aortic annulus
and the velocity time integral of the transaortic pulsed
wave Doppler. On further tilting toward the anterior fetal
chest, the right ventricular infundibulum is seen to lead
Fig. 71.35: Umbilical cord vessels. The umbilical vein (*) is rel- to the pulmonary valve. Crisscrossing of the great arteries
atively thin walled and of larger caliber. There are two umbilical with the pulmonary artery being the more anterior and
arteries (-) that are thicker walled and of smaller lumen. leftward is reassuring for normal great artery relationship.
Fig. 71.36: Situs sweep in this 24-year-old Gravida 2 referred due Fig. 71.37: Two-dimensional (2D) ductal and aortic arches. The
to maternal history of aortic stenosis. This echo was performed at ductal arch (DuAr) is anterior and describes a gradual curve
31 1/7 week gestation. The reader is basically oriented with the slightly rightward and posteriorly where it is met at the isthmus ( )
use of labels as the “Cordes technique” is used to determine situs. by the more posterior and acutely angulated aortic arch (AoAr).
The horizontal bar (/) indicates that the more apically displaced (Movie clip 71.37).
tricuspid valve is located on the right side—this is the normal tri-
cuspid valve leading to the morphological right ventricle. (Movie
clip 71.36).
Loss of this relationship will be obvious in other views the most rightward and posterior location (RSVC). This
and allows diagnosis of malposition syndromes. However, view can provide insight into flow discrepancies in the
semilunar valve function may not be easily separated in subpulmonic and subaortic ventricle, in the respective
this view. semilunar valves, or in the great arteries.69 For example,
Short-axis views are possible in multiple planes the three-vessel (3V) view in TOF will demonstrate a
perpendicular to the long-axis view. A central reference smaller and mildly posteriorly (and cranially) displaced
image is of the right ventricular outflow tract as it wraps pulmonary artery. A smaller aortic dimension is seen with
around the left ventricle. Ventricular septal defects with HLHS and its variants. Bilateral SVC is suspected if there is
outlet or conal extension can be seen in this scan-plane another circular signal to the left of the pulmonary artery
and deviation of the conal septum can be appreciated in and at the same anteroposterior level as the RSVC. Various
this view. Presence of conal deviation, even in the absence aortic arch anomalies including coarctation, interruption,
of significant outflow obstruction, merits serial follow-up double aortic arch, and vascular slings can also be
to monitor for in utero progression to a potential ductal- appreciated in this view. If the PA and aorta are opened out
dependent situation. Such progression of intracardiac in this view, these vessels will be seen to dive posteriorly as
findings has been shown in tetralogy of Fallot (TOF) as they transit into the ductal and aortic arches, respectively.
well as hypoplastic left heart syndrome (HLHS).63–68 Once again, this view is a visual demonstration of expected
A common underpinning of such in utero progression size proportion. The ductal and aortic arches meet in
is a commonly believed concept of antegrade flow being a the aortic isthmus and measurements as well as color
stimulus for growth of valves and cavities. Slight leftward and Doppler here can raise concern for coarctation if
angulation from this view will bring the pulmonary valve, the isthmus is narrowed or if there is flow turbulence or
main pulmonary artery, right and left branch pulmonary increased velocity across this region. Also, if there is an
arteries as well as ductal arch into view. A more cephalad abnormal flow pattern in either arch, it will be readily
sweep with some rightward angulation will develop the noted on color Doppler, although the size discrepancy
“three-vessel view” in the superior mediastinum of the in severe stenosis situations can make it difficult to open
fetus. The main pulmonary artery, aorta, and right-sided these two arches in single view consistently. Focusing on
superior vena cava are lined as in decreasing order of the aortic arch, the sidedness can be determined based on
size from the leftward and anterior most location (PA) to the direction of a left-sided arch diving from a rightward
anterior location to its leftward posterior location to the vessels from the top of the curve. The ductal arch describes
left of the trachea. In most of these cephalad planes, the a tighter curve and is described as a “hockey stick” as it
thymus can be seen in the anterior mediastinum—its joins the aortic arch. Sweeping back and forth amidst
hypoplasia along with a conotruncal lesion may indicate these two arches allows separation of these arches. A
22q11 deletion spectrum. proportionate size, as well as laminar antegrade flow on
Ventricular long-axis view is obtained by aligning the color Doppler are reassuring for preserved fetal physiology
transducer along the left ventricular outflow. Ventricular and right-to-left shunting at the ductal level. Resistance
septal defects, aortic override (pathognomonic of TOF), and pulsatility indices can be calculated and are valuable
aortomitral continuity, goose-neck deformity (pathog- indicators to differentiate increased flow across the
nomonic of AVCD), mitral and aortic valve sizes as well as ductus (increased right ventricular output) from ductal
ventricular morphology can be well seen in this view. Fetal constriction (due to maternal nonsteroidal ingestion).70,71
noncompaction of the myocardium can be a controversial A size discrepancy needs further assessment. In ductal-
diagnosis in early gestation but may be remarkable enough dependent systemic circulations, there is antegrade flow in
to appear convincing in later gestation. Left ventricular the ductal arch, possible abnormal flow in the isthmus, and
basal wall function as well as overall systolic function potentially retrograde flow in the transverse aortic arch. In
can be assessed. Unusual morphologies such as left ductal-dependent pulmonary circulation, the ductus is
ventricular diverticulum and fetal rhabdomyomas may be abnormally angulated and arises more “vertically” from
the undersurface of the aortic arch, and has retrograde
well delineated in this view. Spectral Doppler in this or the
flow indicating that even in the fetal stage, pulmonary
four-chamber view with the pulsed wave Doppler gates
circulation is dependent on a retrograde ductus. Identifying
across the mitral as well as aortic valves can be optimized
both these entities is critical as it has ramifications on fetal
to show the “inflow–outflow Doppler” to calculate. This
counseling, preparing a more detailed birth plan including
is an accepted method to determine the mechanical
initiation of prostaglandins soon after birth.
PR interval in maternal systemic lupus erythematosus
or Sjogren’s syndrome, where there is risk for fetal heart
block, to calculate isovolumic relaxation time and the fetal Fetal Cardiac Function
Tei index.61 Left ventricular shortening fraction can be calculated
Caval long-axis view lines up the inferior vena cava using either two-dimensional images or M-mode
(IVC) as well as SVC entering the right atrium. From interrogation at the level of the papillary muscles on the
this view, the right pulmonary artery passing behind the left side and at midlevel in the right side. Normal left and
RSVC and sometimes a prominent azygous vein entering right ventricular shortening fraction is 34% ± 3% after 17
the RSVC can be appreciated. A dilated RSVC may be an weeks gestation.72 Poor reproducibility of modified biplane
indicator of an arteriovenous malformation in the head Simpson technique-based ventricular ejection fractions
and neck region, commonly in the cerebral territory. contrasts with the better reproducibility and variability
Such a finding may also raise suspicion for anomalous in three- and four-dimensional (4D) measurements of
pulmonary venous drainage and should be followed by ventricular volumes and mass.73–75 A cardiovascular profile
attempts to delineate at least three of the pulmonary veins. score has been widely used to reflect fetal cardiac status.
At this level, the IVC as well as the hepatic vein should be This composite score gives 2 points each for presence or
interrogated by spectral Doppler and as the IVC is traced absence of hydrops, Doppler pattern in the umbilical vein
further to ensure that it is uninterrupted. An interrupted and ductus venosus, heart size based on cardiothoracic
IVC is a marker for heterotaxy syndrome, usually of the ratio, cardiac function based on inflow pattern, AV valve
left atrial isomerism type. From this view, color Doppler incompetence and shortening fraction, and finally
over the liver region can identify the ductus venosus as a Doppler pattern in the umbilical artery.76
narrowing in the course of the umbilical vein to the IVC. Across both mitral and tricuspid valve, the E-wave is
Once identified on color Doppler, spectral Doppler is used smaller and the A-wave is dominant and the E/A ratio is
to show the flow pattern in this structure. usually <1, presumably due to the reduced compliance
Ductal and aortic arch views are best developed of the ventricular myocardium toward the end of the first
perpendicular to the three-vessel view. The aortic arch is trimester. As pregnancy progresses, improved ventricular
higher and describes a wider arch, likened to the “candy compliance is manifested as increased E-wave and
cane”. It is identified by the origin of the head and neck increasing E/A ratio across both valves.77–81 Similar to
the postnatal and adult experience, a fetal myocardial 71.38 to 71.93 and the corresponding movie clips, and also
performance index can be derived based on inflow and Movie clip 71.94).
outflow Dopplers. A higher myocardial performance
index indicates a greater amount of dysfunction, although Fetal Cardiac Rhythm Assessment
it may not separate out systolic from diastolic dysfunction.
Rhythm assessment is integral to a screening echocar-
This Tei index can be compared with recently published
diogram. Although the primitive heart tube demonstrates
norms and is shown to be relatively stable across gestation
organized contractions by 12 days postconception, the
(mean MPI = 0.36, range 0.28–0.44).61,82–84 The fetal
conduction system is developed by 16 weeks of gestation,
isovolumic relaxation time is the interval between closure later than the fetal cardiac structures at 12 weeks.87 Fetal
of the semilunar valves and opening of atrioventricular heart rate varies through gestation, ranges about 110 to
valves with a mean value of 34 ms (range is 26–41 ms).82,85 180 beats/minute (bpm) in the first trimester, maximum
The greatest utility of these parameters may be in the rate achieved at 9 weeks gestation, and averaging about
longitudinal follow-up in a particular fetus with cardiac or 135 bpm (110–150 bpm) by later trimesters.88,89
extracardiac pathology affecting this parameter. Pulse Doppler or M-mode techniques can be used
to detect the rate of cardiac events. To determine the
Core and Cord Dopplers atrioventricular synchrony, simultaneous inflow–outflow
Dopplers such as SVC and aorta, mitral valve and aorta,
This term describes the spectral Doppler pattern in pulmonary artery and pulmonary vein, and M mode
the umbilical artery, umbilical vein, ductus venosus, through atrial as well as ventricular structures can to be
IVC, hepatic vein, and middle cerebral artery. Venous analyzed.90,91 Unfortunately, all these techniques require
structures closest to the heart are the first to manifest persistence and skill due to challenges from fetal position,
changes secondary to increased filling pressures. Both the fetal movement, and image resolution. Fetal arrhythmias
hepatic vein and the IVC show a triphasic pattern similar and premature beats can be quite striking at the time of an
to the “s,” “d,” and “a” wave pattern seen in mature venous obstetric scan, but the vast majority of referrals for reasons
systems. Prominent “a”-wave reversal indicates elevated of fetal arrhythmia reveal premature atrial beats or no
filling pressures, low velocity “s”-waves indicate presence rhythm issues.92 Premature atrial contractions are 10 times
of important tricuspid incompetence. It is recommended more common than premature ventricular contractions;
to acquire umbilical artery (UA) and umbilical vein (UV) both have a combined occurrence of about 1.7% in third
signals in a free loop of umbilical cord midway between the trimester studies.93 The relationship between atrial and
placenta and the fetus. The umbilical venous flow carries ventricular contractions and their respective rates usually
blood from the placenta to the fetus and is a low velocity, allows identification of supraventricular tachycardia
continuous, nonphasic flow. Appearance of “notching” (SVT), atrial flutter, junctional ectopic tachycardia, and
with decrease in centripetal flow during the cardiac cycle is ventricular tachycardia. Fetal SVT with 1:1 atrial (A):
abnormal. The umbilical artery carries fetal deoxygenated ventricular (V) contraction is the commonest at 66–90% of
blood from the fetus to the placenta. The low placental all fetal tachyarrhythmia and is the cause of nonimmune
vascular resistance allows systolic as well as diastolic flow, hydrops in many. These can be further divided into short
which can be quantified using the pulsatility index. This and long V-A types.91 The ventricular rate is typically 250
index is elevated in intrauterine growth retardation and in bpm, and there is 1:1 A:V relationship. The latter feature
the donor of TTTS. Middle cerebral artery is interrogated separates fetal SVT from the second commonest fetal
midway after its origin from the circle of Willis and the tachyarrhythmia, which is atrial flutter.
lateral cranium. Unlike the UA, there is minimal diastolic In atrial flutter, atrial rates may range from 350–
flow in this vessel because of elevated cerebrovascular 500 bpm, eventual ventricular rate depending on AV
resistance—in situations where the fetus is adapting to conduction. Rapid ventricular rates without V-A synchrony
a poor circulatory situation by decreasing its vascular suggest ventricular and junctional ectopic tachycardia.
resistance; there is more diastolic flow in this artery.86 Besides meticulous rhythm studies in affected fetuses,
Accompanying case studies illustrate all the above the structure of the fetal heart must also be assessed in
principles, multiple imaging views, and diagnostic infor- detail since structural defects can coexist in an important
mation in a variety of anatomical situations. (See Figs number. Tachycardias are described as “sustained” if
Case VSD 1. Fig. 71.38: Ventricular septal defect in four-chamber Case VSD 1. Fig. 71.39: Two-dimensional (2D) ventricular long
view. The right and left atria (RA, LA) and ventricles (RV, LV) as axis with ventricular septal dropout in high muscular region.
well as respective atrioventricular valves are labeled (TV, MV). (Movie clip 71.39).
A drop-out (*) is noted at the crest of the interventricular septum in
this image and is suspicious for a ventricular septal defect. (Movie
clip 71.38).
Case VSD 1. Fig. 71.40: Color Doppler ventricular long axis with Case VSD 1. Fig. 71.41: Three-vessel view (3VV) with Bilateral
ventricular septal dropout in high muscular region confirms the superior vena cava (BLSVC). The ductal arch (DuAr) and aortic
presence of a septal defect in this region. (Movie clip 71.40). arch (AoAr) are appropriate in their relative orientation as well as
size. However, there are two good-sized bilateral superior vena
cava [right-sided superior vena cava (RSVC) and left-sided supe-
rior vena cava (LSVC)] noted in this view. (Movie clip 71.41).
Case VSD 1. Fig. 71.42: Postnatal confirmation of high muscular

ventricular septal defect (VSD). (Movie clip 71.42).
Case VSD2. Fig. 71.43: Midmuscular ventricular septal defect Case VSD 2. Fig. 71.44: Orthogonal view of midmuscular ventri-
(VSD; *). This defect is suspected in the muscular septum just cular septal defect (VSD). This is the same patient and imaging
proximal to the moderator band in the right ventricle. Note that in session as in Figure 71.1. In this image, the previously suspected
this instance, the angle of insonation is parallel to the drop-out and ventricular septal defect is confirmed on color Doppler as well as a
suspected defect. nearly perpendicular angle of insonation.
Case AVCD. Fig. 71.45: Four-chamber identification of atrioven- Case AVCD. Fig. 71.46: Atrial morphology and atrioventricular
tricular canal components. A fetal echocardiogram was performed canal defect (AVCD). The right atrial appendage is broad-based
for this 38-year-old G6P4 with Marfan syndrome and a fetus at 28 triangular and with dense pectinate muscles—this identifies
5/7 weeks gestation with trisomy 21 confirmed on amniocentesis. the right atrium and is seen in this image as “a.” The left atrial
The left ventricle is left-sided and on the left of the screen. The appendage is smaller, thin, and finger-like and has fewer pectinate
right ventricle is identified with its apical trabeculations. A large muscles—seen in this image on the left side and labeled “l.” The
atrial septal defect including the septum primum (**) is seen along pulmonary veins are seen to enter into the back of the left atrium.
with a large ventricular septal defect (*). The common atrioven- The large common atrium communicates via a common atrioven-
tricular valve is seen to sit astride this large defect in the crux of tricular valve with the ventricles distally. The ventricular septal
the heart. defect (VSD) is noted (*).
they occur for >50% of the study period, otherwise they to decide on when to start maternal medications and also
are considered “nonsustained.” Functional parameters to follow up the effect of the rhythm disturbance as well
including ventricular dimensions, cardiothoracic ratios, as medication. Most premature beats and nonsustained
presence and severity of AV valve incompetence, output, tachycardias can be monitored without intervention while
cord and core Dopplers, and presence of hydrops need to fetal well-being is monitored. Oral maternal medications
be serially collected and interpreted—these are invaluable in th e form of digoxin, flecainide, sotalol, terbutaline, and
Case AVCD. Fig. 71.47: Short-axis view of common atrioventricu- Case AVCD. Fig. 71.48: Left-sided superior vena cava (LSVC)
lar (AV) valve demonstrates the single valve, and its anterior and to CS, smaller aorta. The LSVC is noted to drain into the right
posterior leaflets as well as right and left components. The anterior atrium and the roof of the coronary sinus appears to be intact on
leaflet has connections to the crest of the interventricular septum this two-dimensional (2D) image. This was confirmed on color
in this example, indicating that it is likely to be Rastelli type 1. The Doppler. The aorta (X) is seen in the crux of this view and appears
pulmonary valve (PV) is noted anteriorly and is of a good size. to be smaller than the anterior pulmonary valve.
amiodarone are important decisions that allow rate as well

as rhythm control but have important implications for the
mother as well as fetus.94–98
Fetal bradycardia is diagnosed when the ventricular
rates are < 100 to 110 bpm. These low heart rates may
occur transiently and with no adverse outcome secondary
to vagal stimulus. More persistent bradycardia is an
indicator of a compromised fetus. CCHB with overall
ventricular rates of 30 to 120 bpm can arise due to
maternal autoimmune illnesses such as systemic lupus
erythematosus (SLE) and Sjögren’s syndrome and also
from fetal structural heart disease such as heterotaxy
syndromes (left atrial isomerism), l-looped ventricles,
rarely idiopathic. CCHB due to maternal autoantibodies
Case AVCD. Fig. 71.49: Three-vessel view—discrepancy and anti-Ro (anti-SSA), and anti-La (anti-SSB) is presumably
left-sided superior vena cava (LSVC). The reader is encouraged due to affinity of these transplacentally transmitted
to orient the structures and note that the three-vessel view is ab- antibodies to the fetal conduction system and rarely to
normal. Firstly, the aorta (Ao) is much smaller than the pulmonary the myocardium. First and second degree heart block
artery (P). Secondly, there is a left-sided structure in the same are amenable to diagnosis and treatment with maternal
plane as the right-sided superior vena cava and of equivalent size steroids; third degree heart block is generally thought to
on the left of the pulmonary artery—this indicates the presence of
be irreversible. Stabilization and even reversal of first and
the left SVC as also demonstrated in Figure 71.4. The relative dis-
proportion is concerning for left ventricular outflow tract or aortic second degree AV block with maternal fluorinated steroids
abnormalities or for smaller aortic arch. The ascending as well as has resulted in this being the treatment of choice. Others
transverse arch measured small, and the last head and neck ves- have reported additional improvement in outcome with
sel seemed to be directed toward the fetal neck with no continuity maternal use of intravenous immunoglobulins, especially
with the remaining ductal arch. This anatomy was suspicious for if the myocardium is also affected.99–103 Fetal bradycardia
interrupted aortic arch.
can also be seen in long QT syndrome.104
Case Dextrocardia. Fig. 71.50: In this situs sweep, the cardiac Case Dextrocardia. Fig. 71.51: Situs sweep with dextrocardia and
apex is directed toward the right of the fetal chest. This fetus was right-sided stomach. This indicates that the heart as well as the
referred due to abnormal obstetric scan and this study was per- stomach are located in the right side of the body, that is, cardiac as
formed at 26 3/7 weeks gestation. The fetus is in breech position. well as abdominal situs inversus. (Movie clip 71.51).
Case Dextrocardia. Fig. 71.52: Asymmetric four-chamber view, Case Dextrocardia. Fig. 71.53: Pulmonary veins to right-sided left
atrioventricular canal defect (AVCD). A common atrioventricu- atrium. The right and left pulmonary veins are seen to join the back
lar valve is suspected accompanied by an atrial and ventricular of the right-sided left atrium in this color Doppler image. (Movie
septal defect (*). Left-sided atrium appears to have the right atrial clip 71.53).
appendage (RAAp) and hence the right atrium. The left atrium is
right-sided and receives the pulmonary veins. The right-sided left
ventricle appears to be smaller than the other ventricle. (Movie
clip 71.52).
Safety of Fetal Ultrasound ultrasound energy can be theoretically secondary to its

thermal effect relating to increase in temperature in the
Prolonged and/or repeated fetal ultrasound is sometimes region of insonation, or mechanical, relating primarily to
required in fetal diagnosis. Despite theoretical concerns, cavitation. Most ultrasound systems allow for display of
no confirmed harmful effects have been detected over potential increase in temperature in the field by displaying
the years. The use of each imaging modality is associated the thermal index for either soft-tissue (TIS), or bone
with ultrasound energy expenditure, most with the use of (TIB). The TI represents an estimate of the temperature
color Doppler in a small region of interest. Bioeffects of rise in the field and is approximately proportional to
Case Dextrocardia. Fig. 71.54: Double outlet right ventricle. In this Case Dextrocardia. Fig. 71.55: Smaller pulmonary artery and bi-
image, the left-sided right ventricle gives rise to both the great furcation. The right pulmonary artery (RPA) is red on color Doppler
arteries. There is some deviation of the conal septum, so that as it moves toward the transducer; the left pulmonary artery (LPA)
this protrudes below the pulmonary valve. The pulmonary valve is blue as it moves away. The main pulmonary artery (MPA) is of
appears to be smaller than the aortic valve (identified as the non- smaller caliber than the aorta (Ao) that is directed leftward from its
dividing vessel). The pulmonary artery (PA) is identified as the anterior position. (Movie clip 71.55).
vessel that bifurcates distally. The aorta is anterior and larger than
the pulmonary artery. This great artery relationship should be
confirmed in other views. (Movie clip 71.54).
Case Dextrocardia. Fig. 71.56: En face view of unbalanced CA Case Dextrocardia. Fig. 71.57: Abnormal three-vessel view with
valve. The left-sided right ventricle (RV) is more dominant. The aorta anterior and leftward. The aorta is most anterior (**); a
smaller right-sided left ventricle (LV) is seen posterior and right of smaller pulmonary artery is seen to its right and more posteriorly.
the RV. The common atrioventricular valve is seen on short axis in Bilateral superior vena cava (SVC) are once again suspected in
this view. (Movie clip 71.56). the same plane (*).
the temperature increase in degree Celsius. The risk of as Doppler applications assessing tissue motion and real-
mechanically induced ultrasound damage is displayed by time 3D imaging continue to develop, bioeffects on the
the mechanical index (MI), which is defined as the ratio fetus will need to continue to be monitored. As there are
of maximal peak rarefactional pressure to the square root no strictly defined limits established, use of ultrasound
of the ultrasound frequency. The risk of mechanical injury energy in fetal echocardiography is best expressed by the
rises with increasing MI.105–107 As newer modalities such “ALARA” principle—as low as reasonably achievable.108
Case TA. Fig. 71.58: Four-chamber asymmetry. This four-cham- Case TA. Fig. 71.59: d-malposition of the aorta. The aorta (Ao)
ber equivalent view demonstrates asymmetry in ventricular cham- is identified due to its relationship with head and neck vessels.
ber size, the right-sided right ventricle (RV) is smaller, and its apex This vessel arises from the anterior and rightward bulboventricu-
does not reach up to the cardiac apex. The tricuspid valve above lar chamber (BVC). Blood enters the BVC through a currently
it seems to have plate-like atresia between the right atrium (RA) unrestrictive ventricular septal defect (VSD) from the left ventricle
and RV. A large ventricular septal defect (*) is seen separating (LV). There is no aortomitral continuity and the aorta is of good
the smaller right ventricle from the larger and apex-forming left size. (Movie clip 71.59).
ventricle (LV). The mitral valve (MV) is seen in its open diastolic
position. The interatrial foraminal membrane is seen bulging into
the left atrium (LA), suggesting that there is right-to-left flow at this
level. (Movie clip 71.58).
Case TA. Fig. 71.60: d-malposition of the great arteries. The Case TA. Fig. 71.61: Origin of malposed great arteries, subpul-
pulmonary valve (PV) leads to a good-sized pulmonary artery monic ventricular septal defect (VSD). The aorta (Ao) is anterior,
that is seen to bifurcate distally. This great vessel is seen to arise smaller, and arises from the anterior diminutive right ventricle
from the left ventricle (LV). The ventricular septal defect (VSD; *) (RV). The pulmonary artery (P) arises posteriorly from the larger
connecting this left ventricle to the bulboventricular chamber is not and dominant left ventricle (LV). This indicates ventriculoarterial
well profiled in this view. (Movie clip 71.60). discordance. A subpulmonic VSD (*) and its relationship to the
great arteries is seen clearly. Note that the subpulmonic conus
is minimally thickened and deviated into the subpulmonic region.
(Movie clip 71.61).
Case TA. Fig. 71.62: Abnormal 3VV in DTGA. The fetal spine (Sp) Case TA. Fig. 71.63: DTGA, anterior aorta bulboventricular cham-
and anterior aspects are marked. The aorta is rightward and an- ber. The reader is encouraged to use the previous images to de-
terior to the pulmonary artery (d-malposed). The right pulmonary termine the anatomy in this labeled image. (Movie clip 71.63).
artery (RPA) origin from the pulmonary artery (PA) is seen before
this vessel continues as the ductal arch (DuAr) to the descending
aorta (DAo). (Movie clip 71.62).
Case HLHS. Fig. 71.64: Asymmetry of the ventricles. A significant Case HLHS. Fig. 71.65: Bicaval view showing foraminal flap. The
fetal structural heart defect is suspected in the early part of this superior (SVC) and inferior (IVC) vena cavae are seen to enter
scan due to the obvious asymmetry of the ventricles. The right the right atrium in this bicaval view. The foraminal membrane is
atrium (RA) and right ventricle (RV) are identified to be of ade- seen with the crest of the flap directed toward the right atrium (*),
quate size. The left ventricle (LV) cannot be easily opened out and indicating left-to-right flow at this level. Normal atrial level shunting
the atrioventricular valve above it appears plate-like and does not in the fetus is always from the right to the left and presence of an
open well. A hypoplastic left heart variant is suspected in this im- opposite direction of flow indicated impaired left atrial drainage
age. (Movie clip 71.64). from anatomical or functional causes. (Movie clip 71.65).
Three-Dimensional Imaging two planes, a 3D data point is called a voxel. This comprises
information in all three dimensions. An immense
3D and 4D (3D + fourth dimension of time) fetal echo- advantage of 3D fetal scanning is that postprocessing
cardiography requires acquisition of a volume of imaging can allow navigation within this data set to yield virtual
data. Just as two-dimensional data is defined as a pixel in and customizable scan planes that can better depict
Case HLHS. Fig. 71.66: Color Doppler showing left-to-right (LA Case HLHS. Fig. 71.67: Asymmetric three-vessel view. The
to RA) shunting at the atrial level (*). This image correlates with pulmonary artery is dominant and anterior. A smaller aorta is seen
Movie clip 71.66. to its right, followed by a right-sided superior vena cava. The
pulmonary artery goes on to bifurcate and the right pulmonary
artery (RPA) continues behind the aorta. The transverse aorta is
suspected to be the diminutive vessel that is headed from the
ascending aorta (Ao) toward the leftward aspect of the fetus.
(Movie clip 71.67).
Case HLHS. Fig. 71.68: Color Doppler of both arches. Further Case HLHS. Fig. 71.69: Color Doppler showing retrograde trans-
cranial sweep from the location in Figure 71.4 reveals the aortic verse aortic arch flow. This image is derived by rotation of the
and ductal arches. On applying color Doppler, there is antegrade transducer from the position that demonstrated Figure 71.5. Here
flow through the ductal arch. The point (*) is the isthmus and there the aortic arch is identified by the head and neck vessels, and the
is retrograde filling of the aortic arch from this region (red flow transverse aorta (AoAr) is seen to fill retrograde from the isthmus
toward the transducer). Both arches should be antegrade and and the antegrade ductal arch (DuAr). (Movie clip 71.69).
symmetric. Presence of asymmetry is a subtle clue to possible hy-
poplasia, but retrograde flow indicates severe obstruction of one
of these arches and is a ductal-dependent pulmonary or systemic
circulation at birth. (Movie cilip 71.68).
anatomy as well as relationships between intracardiac terminology) it is considered 4D ultrasound. This modality
and extracardiac structures. When acquired over time acquires a series of B-mode images and stacks them
and multiple events in one or more cardiac cycles (STIC up to result in a data volume across a cardiac cycle.
= spatiotemporal image correlation and other 4D imaging Such a volume may potentially contain all the imaging
Case HLHS. Fig. 71.70: Spectral Doppler of retrograde aortic arch Case HLHS. Fig. 71.71: Retrograde arch flow on power color
flow shows that the flow is coming anteriorly and toward the aortic Doppler. Power color Doppler can be used to bring out color flow
root rather than the normal flow posteriorly and away from the phenomenon to advantage. (Movie clip 71.71).
aortic root.
Case HLHS 2. Fig. 71.72: Four-chamber view—smaller left ven- Case HLHS 2. Fig. 71.73: Four-chamber view with no color flow
tricle (LV) and mitral atresia. In this case, a large and hypertrabec- across mitral valve. In this image, while there is antegrade flow
ulated right ventricle (RV) contrasts with the smaller, non-apex- across the tricuspid valve into the right ventricle, there is no flow
forming LV. The smaller left atrium (LA) is separated from the across the mitral valve into the left ventricle in the presence of
larger right atrium (RA) by a slightly thickened interatrial septum plate-like mitral atresia. (Movie clip 71.73).
with a central defect; its edges are bowing into the right atrium.
(Movie clip 71.72).
information that is required for fetal cardiac screening.109,110 technologies with color Doppler may be a promising tool
Volume reconstruction and rendering are postprocessing for multiplanar display of flow phenomenon.111,112
applications that allow manipulation of the data set to extract With the recent availability of live 3D scanning, this
relevant information and display. For example, volume technology has been utilized in assessing the fetal heart.113
rendering of a data set with a fetal ventricular septal defect B-Flow is a non–Doppler-based imaging technology
will demonstrate the location, rims, and extent of the defect. by digital encoding of emitted ultrasound beams into
Color flow Doppler has been used as a way of mapping two sub-beams. One of these beams is a high frame rate
flow in the fetal heart and a combination of 3D and 4D B-mode display of color flow that is enhanced to increase
Case HLHS 2. Fig. 71.74: Color flow across large apical ventricu- Case HLHS 2. Fig. 71.75: Color Doppler, mitral atresia, and
lar septal defect (VSD). This is the only source of blood flow into left-to-right flow at patent foramen ovale (PFO). (Movie clip
the left ventricle from the right ventricle. (Movie clip 71.74). 71.75).
Case HLHS 2. Fig. 71.76: Color contrast in pulmonary veins and Case HLHS 2. Fig. 71.77: Asymmetric 3VV in mitral atresia—clue
patent foramen ovale (PFO). This image illustrates the complex- for arch hypoplasia. The large ductal arch is a stark contrast to the
ity as well as variable directionality of multiple flows in a small much smaller and tortuous aortic arch. (Movie clip 71.77).
region of interest. Careful attention will allow interpretation and the
information thus gathered should appear to be internally consist-
ent and unifying toward the anatomy and physiology. (Movie clip
71.76).
signal return from normal flow phenomenon. As a result, They have been found to be particularly useful in first and
the lumen dimensions as well as overall cardiac flow early second trimester scans.118
phenomenon can be easily visualized. Some investigators Fetal cardiac interventions are intended to “normalize”
have found it to be useful to measure vessel dimensions, the physiological disturbance that has come about in the
others have found it to be highly sensitive to vessel developing fetus because of a structural problem. This
anomalies such as transposition of the great arteries.114–116 stems from the observation that improved flow across
Power Doppler and high definition power Doppler valves and structures improves their size and function
are used in conjunction with 3D static imaging or STIC as also the observation that these techniques are both
imaging, respectively. They operate at lower velocities and feasible and bear good results. Theoretically at least, it is
enhance visualization of flow phenomenon with color.117 possible to “improve” the anatomy of the developing heart
Case HLHS 2. Fig. 71.78: Aortic arch deceptively normal in long Case HLHS 2. Fig. 71.79: Pulmonary venous Doppler showing
axis. On this static image, it almost appears as though the aortic consistent but low velocity “a”-wave reversal. This feature will need
arch is being demonstrated and appears to be of good size. In to be serially followed in this fetus as pregnancy advances, since
fact, the left subclavian artery is arising from the top of the ductal the natural history of such restrictive atrial septums is to further
arch where it meets the diminutive aortic arch. The aortic arch is decrease with time. Presence of atrial level restriction to left-
antegrade due to the flow coming into it from the apical ventricular to-right flow adds significant morbidity as well as mortality and
septal defect (VSD). However, often, a diminutive aortic arch is dif- may be an indication for fetal intervention or emergent postnatal
ficult to trace out along its entire distance and multiple views and intervention.
clues need to be used. (Movie clip 71.78).
Case PA. Fig. 71.80: Asymmetric four chamber showing tricus- Case PA. Fig. 71.81: Longitudinal view showing aortic outflow and
pid and right heart hypoplasia. The tricuspid annulus is small and ventricular septal defect (VSD; *). The left atrium (LA) and left ven-
appears to be dysplastic. The right ventricle is smaller and non– tricle (LV) are noted here, with presence of aortomitral continuity.
apex-forming. (Movie clip 71.80). The aortic outflow (Ao) is astride a ventricular septal defect (*).
(Movie clip 71.81).
or even temporize it enough to allow for survival, and septum or in d-transposition of the great arteries, and
diminish postnatal mortality and morbidity. Thus, balloon intact interventricular septum all bear potential interest.
dilatation of the aortic valve in critical aortic stenosis The greatest constraint facing this field is the substantial
and HLHS, pulmonary valve balloon dilatation in critical ethical issues as well as logistics around it. Encouraging
pulmonary stenosis or pulmonary atresia with an intact results have been noted in the hands of experienced
interventricular septum, balloon dilatation of the interatrial centers performing interventions at the extreme end of the
septum in cases with HLHS with restrictive interatrial spectrum of congenital heart disease.119–123
Case PA. Fig. 71.82: Retrograde ductal arch (DuAr) in fetus with
pulmonary atresia. (Moive clip 71.82).
Case PR. Fig. 71.83: Four chamber with biventricular apical Case PR. Fig. 71.84: Dysplastic pulmonary valve. The pulmonary
hypertrophy (*), hyperechogenic ventricular walls (~), and right annulus itself is of a normal size (--), seen here distal to a normal
ventricular chamber dilatation. Accompanying biventricular appearing right ventricular outflow tract (RVOT). However, the pul-
systolic dysfunction is better seen in the accompanying movie monary valve leaflets are not discernible—rolled up and thickened
(Movie clip 71.83). leaflet tissue is seen in this location. The main pulmonary artery
(MPA) is dilated. (Moive clip 71.84).
Case PR. Fig. 71.85: Color Doppler right ventricular outflow show-
ing free pulmonary incompetence and ductal reversal. A wide
regurgitant jet is seen to enter the right ventricle (RV) from across
the plane of the previously noted pulmonary annulus. In fact, this
reversal is seen to extend into and include the main pulmonary ar-
tery (MPA) and the ductal arch (DuAr). (Movie clip 71.85).
Case PR. Fig. 71.86: 3VV dilated pulmonary valve and branch Case PR. Fig. 71.87: Color Doppler both arches flow reversal in
pulmonary artery (PA). (Movie clip 71.86). ductal arch.
Case PR. Fig. 71.88: Pulse Doppler in ductal arch showing dias- Case PR. Fig. 71.89: Pulse Doppler across ductus venosus show-
tolic reversal or to–fro flow. ing return to baseline.
Case PR. Fig. 71.90: Pulse Doppler of umbilical artery showing Case PR. Fig. 71.91: Pulse Doppler of umbilical vein with no obvi-
loss of antegrade diastolic flow. ous or significant venous notching.
Case PR follow-up. Fig. 71.92: Two-dimensional (2D) dilated right Case PR follow-up. Fig. 71.93: Color Doppler with moderate tri-
ventricular outflow tract (RVOT), main pulmonary artery (MPA), cuspid regurgitation (TR). The right ventricle (RV) is dilated and
and minimal pulmonary valve tissue. There has been marked en- dysfunctional; the right atrium is dilated. (Movie clip 71. 93).
largement of the pulmonary annulus (----) even accounting for the
advancing gestation. (Movie clip 71.92).
CASE STUDIES 7. American College of Radiology. ACR practice guideline for

the performance of antepartum obstetrical ultrasound—
1. Case VSD 1 (ventricular septal defect) Revised 2003 (Res. 19). Amended 2006 (Res. 35). ACR
2. Case VSD 2 (ventricular septal defect) Practice Guidelines and Technical Standards, 2006 ed. Am
3. Case AVCD (atrioventricular canal defect)
Coll Radiol. 2006;895–901.
4. Case Dextrocardia
8. ACOG Committee on Practice Bulletins. ACOG Practice
5. Case TA (tricuspid atresia)
6. Case HLHS 1 (hypoplastic left heart syndrome) Bulletin No. 58. Ultrasonography in pregnancy. Obstet
7. Case HLHS 2 (hypoplastic left heart syndrome) Gynecol. 2004;104:1449–58.
8. Case PA (pulmonay atresia) 9. Rychik J, Ayres N, Cuneo B, et al. American Society of
9. Case IEF Echocardiography guidelines and standards for perfor-
10. Case PR (pulmonary regurgitation). mance of the fetal echocardiogram. J Am Soc Echocardiogr.
2004;17(7):803–10.
10. Small M, Copel JA. Indications for fetal echocardiography.
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1561
CHAPTER 72
M-Mode and Two-Dimensional
Echocardiography in Congenital
Heart Disease
Neeraj Awasthy, Savitri Shrivastava
Snapshot

PaƟent PreparaƟon 
Double Outlet Right Ventricle

Imaging 
Truncus Arteriosus

Dextrocardia 
TransposiƟon of Great Vessels (TGA)

Principles of SequenƟal Chamber Analysis 
Atrioventricular and Ventricoarterial Discordance

General Features: Shunt Lesions 
Normal Flow PaƩern of Pulmonary Veins

Atrial Septal Defects 
Anomalies of Pulmonary Veins

Ventricular Septal Defect 
Total Anomalous Pulmonary Venous ConnecƟon

Patent Ductus Arteriosus 
Anomalies of Systemic Veins

Aortopulmonary Window 
Coronary Artery Anomalies

Gerbode Defect 
Coronary Arteriovenous Fistula

Atrioventricular Septal Defects 
Coronary Aneurysms

Congenital Anomalies of Mitral Valve 
Abnormal FormaƟon of Arch

Congenital AbnormaliƟes of Tricuspid Valve 
CoarctaƟon of Aorta (CoA)

Valvular AorƟc Stenosis 
InterrupƟon of AorƟc Arch

Subvalvular AorƟc Stenosis 
AorƟc Aneurysm

Supravalvular AorƟc Stenosis 
Univentricular Atrioventricular ConnecƟons

AorƟc RegurgitaƟon 
Tricuspid Atresia

Sinus of Valsalva Aneurysm 
Mitral Atresia and HypoplasƟc LeŌ Heart Syndrome

Aortocameral CommunicaƟons 
Heterotaxy Syndrome

AorƟc Override
PART 1: BASICS OF IMAGING AND SEQUENTIAL SEGMENTAL ANALYSIS
Echocardiography is the most useful modality of imaging toys and music. A cheerful environment, interaction
in the diagnosis of congenital heart disease (CHD). The with the patient, allowing the parents to be around,
following special issues should be remembered while and familiarizing the patient with equipment are a few
performing pediatric echocardiography: factors that help in achieving the child’s cooperation.
Sedation may be appropriate if the above methods fail.
Sedation should be given in the presence of a parent or a
PATIENT PREPARATION1–4
relative with whom the child is familiar. This helps in the
Prior to taking the patient for an echocardiographic acceptance of the drug and gives a better sedative effect.
study, all the available clinical data should be carefully Also, a familiar parent is able to detect changes in the
perused and the oxygen saturation checked. This helps in sensorium of the patient. Drugs commonly used are oral
total evaluation of lesions. Pediatric echocardiographic chloral hydrate and nasal midazolam. Chloral hydrate is
examination should be performed when the child is quiet given in a dose of 50 to 100 mg/kg weight (the total dose
and cooperative or else one may miss certain important should not exceed 1.5 g).1 Children may become restless
observations. In a crying baby alter intrathoracic pressures and agitated with chloral hydrate. This usually occurs
resulting in fallacious gradients and other vital findings. when the medicine is given when they have just woken
Most of the neonates and infants can be quietened from sleep or are hungry. Midazolam nasal drops can also
by a feed, handling by an experienced nurse, using a be used in infants and young children (up to 3 years), with
pacifier, and keeping them adequately warm. Wrapping a dose of 0.2 mg/kg bodyweight in the nostrils.2,5,6 If the
a small child is in itself not only a proper positioning patient does not go to sleep or becomes uncooperative,
method for small children and infants but a cuddled the same dose can be repeated. Many patients do not fall
up wrapped infant can be easily quietened for a proper asleep with midazolam but become passive, cooperative,
echocardiographic examination (Fig. 72.1). Certain and drowsy; thus, the study can be performed. The other
children can also remain quiet by familiarizing them with important aspect is patience. It is rewarding to patiently
the surroundings and a friendly environment with some wait till the child quietens down and then proceed with
Fig. 72.1: The steps for appropriately wrapping up an infant for proper cardiac evaluation. Cuddled up neonate is well suited and coop-
erative for any echocardiographic examination. Step1: Laying the sheet with single fold on a flat surface (bed); Step 2: Demonstrates
the end, which is picked up and wrapped on the baby’s opposite feet; Step 3: Demonstrates step 2 on the baby; Step 4: Wrapping the
left over upper end of the sheet on the upper limb; Step 5: Repeating the step of wrapping the upper arm for the other arm; Step 6: Child
with restrained both upper limbs; Step 7 and Step 8: The lower part of the sheet is wrapped around the lower limbs to cuddle up the child.
Chapter 72: M-Mode and Two-Dimensional Echocardiography in Congenital Heart Disease 1563
the study. In sedated babies, oxygen saturation should be pulsed and continuous wave Doppler, velocities across all
monitored during the study. There is a wide variation in valves are recorded. If any unusual turbulence on CFI is
the age of the patients with CHD, as such transducers of noted, it should be carefully interrogated using pulsed and
different sizes are needed. In cardiac imaging the sound continuous wave Doppler.
waves travel through the chest wall, pericardium, and Conventional views for echocardiography are detailed
cardiac structures. A portion of the energy is absorbed below.1,2,4-8
and the rest is transmitted. The absorption increases with Because of the limitations imposed by the air-filled
frequency; therefore, a high frequency ultrasound beam, lungs and bony thoracic structures, the echocardiographic
that is, 10 MHz will not penetrate as far as a low frequency planes for the heart are obtained from only four areas of
transducer like a 2.0 MHz frequency will. This is one of the the body (Table 72.1):
reasons why high frequency transducers are needed for • Subcostal.
the new born and infants while low frequency transducers • The cardiac apex—apical four-chamber (4C) views.
are used for grown up children and adults.3 • Parasternal region—adjacent to sternum—second,
For small and premature infants, a 12 MHz transducer third, and fourth intercostal spaces.
is used. It has excellent near-field axial and lateral • Suprasternal notch.
resolution. However, its ability to penetrate the far field is
limited. For optimal resolution of the image, the highest Subcostal Window7
frequency transducer should be tried first. If penetration is Evaluation of congenital heart lesions generally starts with
not adequate, progressively lower frequency transducers the subcostal view. This provides information regarding
can be used till an optimal image is obtained. Multiple the situs, which is a crucial step in the diagnosis of CHD.
transducers may be used in the same patient for optimal Imaging the inferior vena cava (IVC) and abdominal aorta
imaging. For subcostal imaging generally a transducer with in cross section gives this information (Fig. 72.2). In small
a lower frequency than used for transthoracic imaging gives infants if the transducer is pressed too deep, impairment
better results. The probe transducers which are commonly of respiration may occur. Intensive pressure may also
used for pediatric echocardiographic are generally as per compress on the IVC impairing the venous return.
the age group of the patient. While a 12 MHz probe At times, it may lead to nonvisualization of an otherwise
transducer is used for small children, a transducer with a
patent IVC. The pulsations, color flow mapping, and pulse
frequency range from 5 MHz to 8 MHz is used for grown-
Doppler interrogation identifies the IVC and the aorta.
up children and adolescents. In adults, a lower frequency
probe transducer (4–5 MHz) is generally used.
Table 72.1: Conventional Views of Echocardiography
Subcostal
IMAGING
Sagittal (B1 caval)
The anatomical reference for the echocardiographic
Coronal
planes is the major axis of the heart and not the major
axis of the body. Displaying images in an anatomically Paracoronal view
correct fashion allows better understanding of anatomy, Apical
particularly in cases of complex CHD. For an anatomically 4C view
correct display, the apex of the imaging sector is placed at 5C view with aorta
the bottom of the screen in the subcostal and the apical
5C view with pulmonary artery
views, and for the rest of the views it is placed at the top of
Parasternal
the screen. The aim of the study is to image all structures
of the heart and great vessels in all possible planes so that Parasternal long-axis view
one can get a three-dimensional image from the two- Psax ( parasternal short-axis view)
dimensional (2D) study. The study should be performed High parasternal short-axis view (ductal view)
in a systematic sequence in all patients so as to minimize Suprasternal windows
errors in diagnosis. In each plane, the transducer should
Long-axis view
be swept across the heart and great vessels in a specific
direction with and without color flow imaging (CFI). Using Short-axis view
The position of the heart in the thorax is best appreciated Following this, one proceeds to image other cardiac
in the subcostal views, which is helpful in planning the structures from the subcostal view (Figs 72.3A to F).
whole study. This view also shows the movements of both The interatrial septum is viewed in the sagittal plane
domes of the diaphragm, any pleural collections, and the (Bicaval view) with the transducer placed below the
position of the liver. xiphisternum. This approach helps to view the flap of
foramen ovale, superior vena cava (SVC), IVC, and both
right upper and lower pulmonary veins. Their connection
can be identified; color flow mapping and pulse Doppler
interrogation of these veins are done to rule out any
obstruction. In venous obstruction, the first to get altered
is the loss of phasic character of venous flow; later, the total
velocity also increases. The atrial septum should also be
imaged in coronal plane so as to profile the atrial septum
completely. Then sweep in the sagittal plane at the level of
the ventricular septum, atrioventricular (AV) valves, and
great vessels. The sweeps will profile the right ventricle
(RV), RV out flow and pulmonary arteries (with a straight
anterior tilt), interventricular septum, left ventricle (LV)
and left ventricular out flow tract from right to left, AV
valves and the great vessels, then the operator should
Fig. 72.2: Two-dimensional echocardiography. Subcostal short- proceed with the coronal section of the ventricle and the
axis at the level of abdominal vessels. Inferior vena cava and aorta out flow tract by tilting the plane of transducer posterior
are shown in cross section with aorta (red) to left of spine and to anterior. By giving a counterclockwise rotation to the
inferior vena cava (blue) to right of spine (*).
transducer, one can obtain a paracoronal view showing RV
A B C
D1 D2 E1 E2
Figs 72.3A to E
F1 F2
F3 F4
Figs 72.3A to F: Two-dimensional echocardiography subcostal window. (A) Subcostal coronal (four-chamber equivalent) view showing
interatrial septum, connection of right upper pulmonary vein (RUPV) to left atrium. Descending aorta ( ) in short axis is imaged behind
the left atrium; (B) Subcostal coronal view with posterior-to-anterior sweep showing left ventricular outflow tract and aorta with color flow
mapping (blue); (C) Far anterior tilting shows right ventricular outflow tract; (D1 and D2) Subcostal paracoronal view with right anterior
tilt showing right ventricle (RV) and right ventricular outflow tract. (E1 and E2) Subcostal paracoronal view with color compare showing
right ventricular outflow (*) tract, main pulmonary artery, and right pulmonary artery; (F1 to F4) Subcostal sagittal view of the muscular
septum with right-to-left sweep. This view demonstrates the short axis of the left ventricle (LV) at the level of the mitral valve with sweep
up to the apex of the heart. The color comparison at the scale of “35–36” is especially important to look for a small muscular ventricular
septal defect (VSD), especially in the setting of the increased pulmonary artery pressures (not shown in the illustration).
outflow and right pulmonary artery (PA). This view is also Subsequently, one should proceed with the
very useful to profile perimembranous and subpulmonary parasternal apical, long-axis, short-axis, and supra-
areas of interventricular septum. In coronal section, sternal views. These will be detailed later. The sequence
movement of both domes of the diaphragm can be may need to be altered in a few children till they are well
visualized during respiration. sedated.
Sagittal view is similar to the left anterior oblique Apical View (4C View; Figs 72.4A to D)
view and paracoronal view is similar to the right anterior
oblique view in the angiographic studies. Apical view is commonly called the four-chamber view
In the sagittal view, one should sweep the transducer or 4C view. The apical impulse corresponds to the site of
from right to left and reverse, and in the coronal view, optimal placement of the probe for 4C view. After obtaining
the sweep should be from the posterior to anterior and an adequate window from the apex, the transducer should
reverse, in order to obtain various sections of the heart. be swept from the posterior to anterior plane and reverse.
A B
C D
Figs 72.4A to D: Two-dimensional transthoracic echocardiography. (A) Apical four-chamber view in posterior plane showing coronary
sinus (CS) coursing from left to right in atrioventricular groove; (B) Apical four-chamber view showing all four cardiac chambers, the
interatrial septum, and interventricular septum. Offsetting of atrioventricular valves (attachment of tricuspid valve more toward apex than
mitral valve—arrow) is seen; (C) Apical four-chamber view in anterior plane (apical five-chamber view) showing left ventricular outflow
tract and aorta; (D) Same view in far anterior tilt visualizes right ventricular outflow tract and main pulmonary artery. (Ao: Aorta; CS:
Coronary sinus; LA: Left atrium; LV: Left ventricle; MPA: Main pulmonary artery; RA: Right atrium; RV: Right ventricle).
In the most posterior plane, the coronary sinus can be Parasternal Long-Axis View (Figs 72.5A to D)
imaged coursing at the base of the left atrium (LA) and
opening into right atrium (RA; Fig. 72.4A). The classical 4C This view is obtained with the marker of the probe
view shows all four chambers, AV valves, and the offsetting directed to the right shoulder in the long axis of the
of AV valves (Fig. 72.4B). With the anterior tilt, the aorta can heart. The parasternal long-axis (PLAX) view visualizes
be seen and on a further anterior tilt the PA can be profiled the RV, the interventricular septum, and LV; by
if the great arteries are normally related (Figs 72.4C and D). sweeping from left to right, one visualizes the aorta
The apical five-chamber and two-chamber views are and LA and the LV (Fig. 72.5A). The sweep toward the
commonly used in adult practice to image various left and superiorly allows visualization of the RV out
segments of the LV. As the transducer is swept anteriorly flow tract (Fig. 72.5B). The rightward and inferior tilt
from the apical view, image quality deteriorates because profiles the tricuspid valve and the adjacent RV inflow
of rib shadowing. (Figs 72.5C and D).
A B
C D
Figs 72.5A to D: Two-dimensional transthoracic echocardiography. (A) Parasternal long-axis view of left ventricle showing the left
ventricular inflow and outflow tract with anteriorly placed RV cavity; (B) Parasternal long-axis view with anterior tilt showing right
ventricular outflow tract. (C and D) Parasternal long axis with posterior tilt and color compare showing right ventricular inflow. (Ao: Aorta;
LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right atrium; RVOT: Right ventricular outflow tract).
Parasternal Short-Axis View perpendicular to the PLAX view. Serial cross sections of
the heart are obtained, demonstrating various structures
(Figs 72.6A to D) by sweeping superiorly and inferiorly. The superior sweeps
Parasternal short-axis (PSAX) view is obtained with show the great vessels and the inferior sweeps show serial
the transducer pointing to the left and probe marker cross sections of the ventricles from base to apex.
A B
C D
Figs 72.6A to D: Two-dimensional transthoracic echocardiography. (A) Parasternal short-axis view at the base of the heart showing
aorta (Ao), right ventricular outflow tract, pulmonary valve, main pulmonary artery, bifurcation of main pulmonary artery, and right and
left pulmonary arteries; (B) Parasternal short-axis view of left ventricle at the level of the mitral valve showing the anterior mitral leaflet
(arrow) and the posterior mitral leaflet (arrow); (C) Parasternal short-axis view of left ventricle at the level of papillary muscle showing
the location of papillary muscles, anterolateral at 4 o’clock and posteriomedial at 8 o’clock, trabecular part of interventricular septum,
and right ventricular cavity; (D) Parasternal short-axis view toward apical part of ventricular septum. (LPA: Left pulmonary artery; LV: Left
ventricle; MPA: Main pulmonary artery; RPA: Right pulmonary artery; RV: Right ventricle).
Parasternal Short-Axis View for arteries can usually be visualized. The coronary arteries
can also be visualized from a subcostal window in the
Coronary Arteries (Fig. 72.7)1,2,8 coronal view of left ventricular out flow.
The coronary arteries are better visualized with higher
frequency transducers in PSAX views at the level of aortic High Parasternal or
cusps. Using zoom and low color flow gain, one should
check the color flow in both coronary arteries. The right
Ductal View (Fig. 72.8)
coronary artery (RCA) is at a more superior plane, hence The classic short-axis view visualizing the aorta and PA is
the transducer needs to be rotated counterclockwise, and initially obtained from just below the clavicle, a level higher
for the left coronary artery (LCA) a clockwise rotation of than the usual parasternal views. From this location, the
the transducer is required. The origin of both the coronary transducer is rotated anticlockwise. During this process,
the right PA gradually goes away from view. The main PA
is then seen continuing as the left PA. The descending
thoracic aorta then is in view. The duct is located at the
junction of left PA with the descending aorta. This is called
ductal view.
Suprasternal Views (Figs 72.9 and 72.10)

This view is profiled by placing the probe in the suprasternal
notch. It is helpful to place a small pillow below the neck
to extend the neck.
Long-Axis View (Fig. 72.9)

The direction of the marker of the probe is toward the
left shoulder. This oblique view profiles the arch and
Fig. 72.7: Two-dimensional echocardiography. Parasternal short- descending aorta lengthwise. In case of right aortic arch,
axis view showing origin of both the right and left coronary arteries. the transducer needs to be rotated anticlockwise to profile
(LCA: Left coronary artery; RCA: Right coronary artery; Ao: Aorta).
the arch and descending aorta.
Fig. 72.8: Two-dimensional echocardiography. High parasternal Fig. 72.9: Two-dimensional echocardiography. Suprasternal
short-axis view with anticlockwise tilt to profile ductal area with long-axis view with color flow mapping showing arch of aorta and
color flow imaging shows Desc. aorta, main pulmonary artery, and descending aorta with arch branches. (A: Transverse arch; Desc.
left pulmonary artery in short axis. (AO: Aorta; LPA: Left pulmonary Aorta: Descending aorta).
artery; MPA: Main pulmonary artery; Desc. Ao: Desending aorta).
Fig. 72.10: Two-dimensional echocardiography with color flow compare. Suprasternal short-axis view showing aorta in short axis, right
pulmonary artery in long axis and innominate vein joining superior vena cava, all four pulmonary veins joining left atrium. (LA: Left
atrium; RPA: Right pulmonary artery; SVC: Superior vena cava; AO: aorta; Inn V: Innominate vein).
Short-Axis View (Fig. 72.10)

The direction of probe is parallel to the body plane. This
short-axis view profiles the aorta in its short axis and
opens up the right PA below the aorta in its entire length
till the first branch. Below the right PA, one can see the LA
receiving all four pulmonary veins, also called the crab
view. By tilting the transducer to left and anteriorly, left PA
can be profiled. By tilting the transducer superiorly and to
the right, one can profile the innominate vein and the SVC.
In the suprasternal notch view try to look for the branching
of the arch vessels. If the first branch courses to right and
divides it, this indicates a left aortic arch, and if the first
branch divides and courses to the left side, it is diagnostic
of the right aortic arch. Fig. 72.11: Two-dimensional echocardiography. Subcostal view
showing situs solitus with dextrocardia and L-looped ventricles.
DEXTROCARDIA (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle).
For patients with dextrocardia, attempts should be made
to perform the same views keeping the transducer on
the right side of the chest (Fig. 72.11). The marker on the
Color Flow Doppler
transducer should point in the same overall direction as Color is superimposed on 2D images to demonstrate
for patients without dextrocardia. This enables consistency the flow information. While using color flow mapping
in image orientation and display. the 2D image should be idealized and 2D gain reduced.
Table 72.2: Pitfalls in Imaging of Congenital Heart Disease

Chest wall deformities: These are common in patients with congenital heart disease. Thus, pectus excavatum or carinatum produces
poor parasternal images. Also, scoliosis causes the heart to shift to the ipsilateral side and produces difficulty in imaging
Diaphragmatic hernia: In this condition, the bowel loops can come in front of the chest wall. Since air is a poor conductor of
ultrasound, it can cause unexplained difficulties in imaging. The plain X-ray of the chest easily diagnoses the condition
The postoperative state: Chest wall edema, bandages, and chest wall drains in the immediate postoperative period and
deformity of the sternum in the late postoperative state frequently cause problems in imaging. This is especially true for midline
structures. Following the arterial switch and the LeCompte maneuver, the pulmonary artery frequently lies behind the sternum.
Similarly, midline structures like conduits may be poorly visualized.
Grown up congenital heart disease: It is frequently difficult to get good images in a grown up patient with congenital heart disease.
This is especially true for structures like the pulmonary artery, pulmonary and systemic veins, descending thoracic aorta, ductal
area, etc. even with use of adult transducer. Associated with the poor window is the inability to get good color flow images or
continuous wave Doppler envelopes
A B
Figs 72.12A and B: Nonoptimal scales can result in overestimation of tricuspid valve regurgitation. (A) Apical four-chamber view
showing tricuspid valve regurgitation with splaying of the colors (at color scale of 32); (B) Apical four-chamber view in the same patient
showing tricuspid valve regurgitation with no splaying of the colors at color scale of 72.
Red color indicates flow toward the transducer and blue • Size of color sector: If the color sector is very large,
indicates away from the transducer. Color Doppler allows the frame rate is reduced considerably. This results
rapid display of a great deal of information. One can easily in poorly defined color flow images with potential for
visualize small disturbed jets and study them in depth. both overestimation and underestimation of lesion
CFI is an extremely useful modality that can considerably severity. The size of color sector should be kept as
improve the speed and accuracy of an echocardiographic small as possible. The use of the “zoom” switch allows
examination. If used incorrectly, however, it can result for improved visualization with the smallest possible
in an erroneous interpretation of information. The size of the color sector.
echocardiographer needs to be aware of the following • Failure to optimize gain and scale settings (Figs 72.12A
pitfalls of CFI to make optimal use of color Doppler and B): Excessive gains can result in overestimation of
(Table 72.2). regurgitant lesions such as mitral, tricuspid and aortic
• Failure to optimize 2D images before using color regurgitation. The color Doppler gain should be ad-
Doppler results in poor quality color flow images: justed in a manner that no color is seen in areas where
The echocardiographer should endeavour to obtain blood doesnot flow.
maximum possible information from 2D images before • Failure to optimize color scales (Figs 72.13A and B):
using color. This allows CFI to be specifically targeted Low velocity flows (venous flows, flows in the atria,
to areas of interest. etc.) are best demonstrated by low color scale settings.
A B
Figs 72.13A and B: Optimal color gain settings are essential to prevent “graining” in the field of imaging. (A) Four-chamber (inverted
view) showing graining in the field of interest with gain settings of 90%; (B) The same patient with trace regurgitation and minimal grain-
ing after optimizing color gains.
The same settings will overestimate the severity of high The Doppler cursor should be placed in the direction of
velocity regurgitant jets such as aortic regurgitation the flow being interrogated as seen on color flow mapping.
and mitral regurgitation. Wherever high velocity flows There are limits to the maximum frequency shifts that
are anticipated, the color scale settings should be can be unambiguously displaced at any given point; the
appropriately increased. maximum detectable frequency shift in one direction is
• Multiple color jets: Multiple color flow jets require one half of the sampling rate. The maximum detectable
careful interpretation. Examples include multiple frequency is called the Nyquist limit. The pulsed Doppler
ventricular septal defects (VSDs) or a combination mode selectively provides the flow velocities at the area
of VSD with infundibular pulmonary stenosis. In being interrogated.
this situation, some lesions may be missed. Careful
attention to 2D images can often resolve the individual Continuous Wave Doppler
lesions. The continuous wave Doppler transducer has two crystals,
one continuously transmits an ultrasound signal whereas
Interpreting Color Flow the other continuously receives the back-scattered ultra-
Information in Isolation sound; therefore, Doppler signals from all blood flow tra-
versed by the ultrasound beam are received and displayed.
A number of criteria are available for quantification of
The main advantage of plural wave of continuous wave
regurgitation jets by CFI. However, using them in isolation
Doppler is that there is no limit to the maximum limits that
may result in incorrect interpretation of lesion severity.
can be measured. Therefore, very high velocity jets can be
The information obtained on CFI should be combined
correctly interrogated.
with the 2D information to improve accuracy. Examples
include incorporation of left atrial size, left ventricular
Cautions in Using Pulsed and Continuous
size and function while estimating severity of mitral
regurgitation, and left ventricular size and function for Wave Doppler Evaluation
estimation of severity of aortic regurgitation. Poor Echo Windows
If the images are suboptimal, Doppler signals are
Pulsed Doppler (Figs 72.14A and B) suboptimal as well. The nonimaging continuous wave
A single ultrasound crystal alternatively transmits and Doppler probe may be used in selected circumstances,
receives the ultrasound signal. The vessels or valve keeping in mind the potential pitfalls of using the
being interrogated is imaged by 2D echocardiography. nonimaging probe.
A B
Figs 72.14A and B: (A) Doppler signal with inappropriate gain setting resulting in falsely increased gradients. Note excessive splaying
with a gradient of 85 mm Hg; (B) The same patient with optimized gains with a gradient of 51 mm Hg (+).
Inappropriate Transducer Frequency repetition frequency (PRF). High velocity signals alias
despite maximum PRF. For measuring their velocity,
High quality Doppler signal with clear display of high
continuous wave Doppler will have to be used.
velocity signals requires lower transducer frequencies
than those required for optimal images. Serious
underestimation of stenotic lesions may result from use Inappropriate Signal Alignment
of high frequency transducers for obtaining Doppler (Figs 72.15A and B)
gradients. This is particularly true for Doppler interrogation
It is the most common cause of underestimation of
of deeper lesions such as coarctation. Whenever possible,
Doppler gradients. The correct use of CFI and interrogation
gradients obtained during imaging with high frequency
in multiple views considerably minimizes the likelihood
transducers should be confirmed by using lower frequency
of poor alignment. For example, in a patient with valvular
transducers.
aortic stenosis the gradient should be obtained in the
Inappropriate Doppler Gain and Filter apical, the right parasternal, and the suprasternal views.
Settings (Figs 72.12 and 72.13) The maximum value obtained represents the true Doppler
gradient. However, the gain settings should be optimized to
High velocity signals require high filter settings. For low
get a clear envelope. For a patient with a perimembranous
velocity signals such as venous signals, low filter settings
VSD, Doppler signals may have to be recorded in the
are appropriate. The Doppler gain should be adjusted to
subcostal, the apical, and the parasternal views to obtain
minimize signal-to-noise ratio. Increased gain settings
the best aligned signal.
result in noisy signals with poorly defined spectral margins.
This can result in overestimation of the gradients.
Pitfalls Relating to the Use of the Nonimaging
Inappropriate Doppler Scales Continuous Wave Doppler Probe
(Figs 72.14A and B) There is a likelihood of mistaking one signal for another
Aliasing of the signal results when the Doppler scales if the nonimaging probe alone is used for gradient
are inappropriately low. Once aliasing occurs, the signal estimation. For example, while interrogating from the
cannot be accurately quantified. Increasing the scales of apex, the systolic gradient of mitral regurgitation may be
the Doppler signals can minimize aliasing. For pulsed mistaken for aortic stenosis. Again, interrogation from
Doppler, this would require increasing in the pulse multiple views can help reduce the chances of errors.
A B
Figs 72.15A and B: (A) High frequency signal not getting aligned in view of the pulsed wave Doppler exceeding the Nyquist limit;
(B) The same patient by appropriately increasing the scale of the measurements, and changing from pulsed wave to continuous wave
Doppler.
Failure to Appreciate the Limitations of the and regurgitant lesions, stenotic lesion combined with a
shunt lesion [atrial septal defect (ASD) with pulmonary
Bernoulli Equation stenosis], and high output states such as anemia. In
Simply stated, the Bernoulli equation is as follows: patients with polycythemia, the viscous friction may be
Pressure difference between two points = convective significant and blood density (p) cannot be assumed to be
acceleration + flow acceleration + viscous friction. Of these the same as for normal, and in such a situation significant
three determinants of pressure difference, convective underestimation of Doppler gradients may result.
acceleration is considered the most significant. Convective
acceleration is defined by the formula 1/2p (V22 − V12); Pressure Gradients Across Trivial Lesions
“p” represents the fluid density of blood; V1 and V2 are
the velocity of blood at the two points across which the When the amount of blood flow is very small, such as in
pressure difference is being measured. After converting tiny muscular VSDs or in trivial tricuspid regurgitation,
blood density by using the conversion factor to mm Hg the pressure gradient may be underestimated by Doppler
and velocity to m/s, the coefficient calculates out as 3.98. despite optimal gain setting. This is possibly because the
This is rounded to 4. The assumptions that are made while sample volume is too small.
applying the Bernoulli equation to clinical situations
are as follows: (a) Flow acceleration and viscous friction Comparison of Echo Gradients with Those
are considered negligible; (b) The velocity proximal to Obtained During Cardiac Catheterization
the obstruction is negligible as compared to the distal
velocity. After making these assumptions, the formula is
(Fig. 72.16)
simplified to: pressure difference = 4 peak instantaneous Peak-to-peak versus peak instantaneous pressure
velocity. (c) The pressure gradients are not calculated in gradients: While interpreting Doppler flow gradients, it
tubular uniform structures. Although this formula has is important to remember that Doppler measures the
been validated for a number of situations, the assumptions peak instantaneous pressure difference, which is higher
made introduce important limitations to its applications than the peak-to-peak pressure difference measured by
in specific situations. These include serial obstructions cardiac catheterization. The relationship between peak
where it cannot be assumed that proximal velocity (VI) instantaneous pressure difference and peak-to-peak
is negligible. For measuring pressure differences across pressure difference is complex, possibly because it is
discrete obstruction within a tubular structure (such determined by a number of influences (such as heart rate,
as coarctation of the aorta), the VI should be measured severity of obstruction, dP/dt, etc.). Doppler gradients can
and VI should be subtracted from V2. Other examples exceed gradients recorded by cardiac catheterization by as
where V1 may be increased include combined stenotic much as 30 to 40 mm Hg.
Fig. 72.17: Two-dimensional echocardiography in a patient with

situs inversus. Subcostal short-axis view at the level of abdominal
vessels showing inferior vena cava and aorta in cross section with
Fig. 72.16: The graph demonstrates the comparison of peak aorta (red) to the right of spine, and inferior vena cava (blue) to the
instantaneous gradient with peak-to-peak gradient in left ventricular left of spine (), reversed position of the liver (toward the left of
and aortic pressure tracing. The instantaneous pressure gradient the spine) and gastric bubble (toward the right of the spine). (Ao:
is higher than the peak-to-peak gradient. Aorta; V: IVC; Li: Liver; GB: Gastric bubble).
Failure to Relate the Doppler • Identification of ventricular morphology and AV

connection.
Gradients to Flows • Identification of great vessels and ventricular arterial
Pressure gradients are influenced by flows. While interconnection.
preting gradients recorded by Doppler, the physiological • Identification of associated abnormalities and
state of the patient needs to taken into consideration. assessing their severity.
High output states, fever, anxiety, agitation, anemia can The initial step of evaluation starts with the deter-
all exaggerate gradients. For this reason, it is imperative mination of abdominal situs with IVC to the right of the
to record systemic blood pressure (BP) while trying spine and aorta to the left in situs solitus (Fig. 72.3) and
to predict PA pressures using gradients across VSDs. vice versa in situs inversus (Fig. 72.17). The atrial situs
Similarly, while interpreting the severity of stenotic lesions
generally corresponds with the abdominal situs. If the
by pressure gradients, the flow across the orifice needs to
IVC and aorta are on same side of the spine then possibly
be considered. Other pathophysiological states that can
we are dealing with isomeric heart (Fig. 72.18). The IVC
reduce blood flow include hypovolemia, hypotension, and
may be interrupted, which will suggest left isomerism
ventricular dysfunction.
(Fig. 72.18).
PRINCIPLES OF SEQUENTIAL
Identification of Atria and Their
CHAMBER ANALYSIS9–13
Arrangement
The cardinal principle of sequential chamber analysis states
that the morphology of a chamber should be determined The next step is to identify the atrial arrangement. Four
on the basis of its most constant component. Thus, one types of atrial arrangements can exist:
should not use a component to identify a structure if • Normal atrial arrangement (Situs solitus)—the RA is
that component itself is variable. The evaluation of any right-sided and the LA is left-sided.
congenital heart involves a systemic sequential analysis • Mirror image atrial arrangement (Situs inversus)—the
that involves the following steps: RA is left-sided and the LA is right-sided.
• Identification of abdominal situs. • Isomerism of the left atrial appendage.
• Identification of atria and atrial arrangement. • Isomerism of the right atrial appendage.
Fig. 72.18: Two-dimensional echocardiography. Subcostal short-

axis view at the level of abdominal vessels. Inferior vena cava and
aorta are shown in cross-section with aorta (red) and inferior vena
cava (blue) to right of spine. Spine (*). (Ao: Aorta; V: IVC).
A B
Figs 72.19A and B: Two-dimensional echocardiography in subcostal coronal view with superior tilt showing (A) broad-based right atrial
appendage (RAA); (B) narrow, finger-like left atrial appendage (LAA).
Identification of Atrium echocardiography. Unfortunately, altered hemodynamics

in complex CHD may not allow the appendage to have a
The atrial appendage: It is the most constant feature for
classical appearance.
atrial identification. The right atrial appendage is a broad
triangular structure with a broad junction with the rest The venous drainage: The opening of the suprahepatic
of the atrium (Figs 72.19A and B). It has coarse pectinate portion of the IVC to the RA is the most important marker in
muscle within the appendage, which extend all around identifying morphological RA and can be easily identified
the vestibule of the appendage and atrial junction. The on 2D echocardiography in the subcostal long-axis view.
morphological left atrial appendage is tubular with a The pulmonary venous connection is too variable to be
narrow junction with the rest of the atrium (Fig. 72.20). used as a marker for LA.
The posterior wall is smooth, and the pectinate muscle is The atrial septum: The septum primum overlaps the
confined only to the anterior quadrants of the vestibule. septum secundum from the left atrial side and if present,
Echocardiographically, the shape of atrial appendages is is an important feature in identifying the LA, best
best visualized by a combination of the subcostal coronal visualized in the subcostal sagittal view. Unfortunately,
view (right and left atrial appendages) and parasternal in some complex forms of CHD the atrial septum may
short axis at great vessel level (left atrial appendage). The be absent and thus this feature cannot be used as
pectinate muscles, however, cannot be well identified by a marker.
left- or right-sided atrium or sometimes in the midline

(for those who believe in the isomerism concept, both
atria are of same morphology, hence called the right or left
isomerism).
The atrial appendage morphology would be the most
specific marker for atrial identification. Unfortunately, the
shape of the appendage (as discussed previously) rarely
helps in complex CHD and identification of the atrial
pectinate muscle by 2D echocardiography is not usually
possible.
Left isomerism: Also called the polysplenia syndrome,
this can also be suspected on the abdominal scan of great
vessel. The aorta and a venous channel again lie on the
same side of the spine (left or right). The aorta is anterior
Fig. 72.20: Two-dimensional echocardiography. Subcostal sagittal and the venous channel lies posterior to it. This venous
view—bicaval view showing the attachment of suprahepatic channel actually represents the azygos/hemiazygos vein,
portion of inferior vena cava to right atrium. Right pulmonary which then continues posteriorly through the diaphragm
artery is shown in short axis posterior to superior vena cava (*). and joins the left (hemiazygos) or right (azygos) SVC.
(IVC: Inferior vena cava; LA: Left atrium; RA: Right atrium; SVC:
Superior vena cava).
The IVC is interrupted and the hepatic veins connect to
the atrium directly. IVC interruption is seen in 85% cases of
left isomerism. For those who do not believe in the concept
The coronary sinus (Fig. 72.5A): The coronary sinus always
of isomerism, the chamber that receives the coronary
traverses the floor of the LA and opens into RA. It is a useful
sinus/hepatic vein would become the morphological RA.
marker if present. The apical four-chamber view angled
There are other echocardiographic features by which
posteriorly profiles this feature well.
isomerism should be suspected although not diagnostic.
Aorta–inferior vena cava relation in the abdomen • Bilateral SVC.
(Fig. 72.3): This indirect method of inferring the atrial • Absent coronary sinus (in right isomerism or asplenia).
morphology is the most popular and easily performed • Single atrium.
technique by 2D echocardiography. It is very sensitive • Total anomalous pulmonary venous connection
and specific in patients of normal or mirror image atrial (TAPVC)—The pattern of TAPVC is different in right
arrangement but less so in the group with isomerism. It and left isomerism.
is based on the facts that, in patients of normal or mirror • Total anomalous systemic venous drainage.
image arrangements, the viscera and great vessels in the • Common AV valve.
abdomen are lateralized. The subcostal short-axis scan at • Discordance between abdominal and atrial situs.
the level of diaphragm shows the relation of great vessels
and the spine to each other. Thus, in normally arranged Ventricular Morphology and
atria, the aorta is always to the left of the spine and the IVC
to the right, at the level of the diaphragm. This relation is Atrioventricular Connection
reversed in mirror image arrangement. The connection of the atrial myocardium to the ventricular
mass is called the AV connection. The following types of
Echocardiographic Identification of connections are recognized (Table 72.3):
• Concordant AV connection (Fig. 72.21).
Isomerism
• Discordant AV connection (Fig. 72.22).
Right and left isomerism should be strongly suspected on • Univentricular atrioventricular connection (Figs
echocardiography when the following findings are present. 72.23A and B).
Right Isomerism: Also called the asplenia syndrome, this • Isomeric AV connection.
is strongly suspected on the abdominal scan of the great • Uniatrial and biventricular connection.
vessels. The aorta and IVC lie on the same side of the • Criss-cross AV connection.
spine (left or right) instead of on either side. The aorta To describe AV connection, one should identify the
lies posterior to the IVC. The IVC opens into either the ventricular morphology.
Table 72.3: Echocardiographic Identification of Atrioventricular Connection

The offsetting sign and moderator band are best identified by the apical four-chamber view
The chordal attachment of the morphological tricuspid valve to the interventricular septum is best seen in the subcostal
coronal views
The two discrete papillary muscles of left ventricle and the apical trabeculations of right ventricle are best appreciated in parasternal
short-axis views. The transducer is focused inferiorly starting at the base of the heart. The papillary muscles are first seen followed
by the apical trabeculations
Absence of an atrioventricular valve, common atrioventricular valve, and straddling are best seen in the apical views. The common
atrioventricular orifice is also well appreciated in the subcostal sagittal views
Fig. 72.21: Two-dimensional echocardiography. Apical four-cham- Fig. 72.22: Two-dimensional echocardiography. Apical four-
ber view showing all four cardiac chambers and offsetting of atrio- chamber view showing discordant atrioventricular connection, right
ventricular valves (attachment of tricuspid valve more toward apex atrium to left ventricle, and left atrium to right ventricle. Moderator
than mitral valve—arrow). (LA: Left atrium; LV: Left ventricle; RA: band on left side (anatomical right ventricle) and reverse offsetting
Right atrium; RV: Right ventricle). of atrioventricular valves are seen (arrow).
A B
Figs 72.23A and B: Two-dimensional echocardiography. Apical four-chamber view showing univentricular atrioventricular (AV)
connection. (A) Tricuspid atresia (arrow), hypoplastic right ventricle; (B) Mitral atresia (arrow), with hypoplastic left ventricle (LV).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Echocardiographic Identification of Identification of Great Vessels

Ventricular Morphology The PA is identified by its immediate bifurcation into left
The offsetting sign of atrioventricular valves: This sign, and right branches. This is best appreciated in the PSAX
if present, forms the single most important feature in view at great vessel level. Other clues are: (a) In the PLAX
identification of ventricular morphology. It takes into view if the posterior great vessel is seen coursing posteriorly
after its origin the vessel is the PA. The aorta always takes
account that the AV valve always follows its ventricle.
an anterior course. (b) Annular hypoplasia is often the
The tricuspid valve is attached more apically compared
rule in patients with pulmonary stenosis physiology (like
to the mitral valve. Thus, a ventricle which has its AV
tetralogy etc.). Then the vessel with a smaller annulus
valves attached more apically will be the morphological
and with turbulent flow (on color or pulsed Doppler) is
RV. The disadvantage with this sign is that when a VSD
often the PA. (c) The aorta does not give rise to any branch
extends to the inlet septum or in univentricular hearts, this
before the carotids across the coronaries and it gives rise to
“offsetting” sign is lost and therefore cannot be used as a the arch vessels. The coronary arteries are useful pointers
feature for ventricular morphological identification. to identify aorta, but because of their variable origin (e.g.
The ventricular septal and apical trabeculation: The the PA) they cannot be used as a criterion for a great vessel
morphologists consider this as the most constant feature of to be labeled as the aorta.
ventricular morphology. The RV apical trabeculations are
coarse. The moderator band contributes to this coarseness. Identification of Connection of Great Vessels
The RV side of the septum is seen to be coarser compared A combination of views are used to identify great vessels
to the LV side of the septum. Echocardiographically, this and their connection.
feature becomes difficult to identify if one of the ventricles Starting from the subcostal coronal scan, the
is severely hypoplastic or there is a true single ventricle. transducer is tilted superiorly in a gradual fashion. The
great vessels appear sequentially as the transducer is
Attachment of the chordae and papillary muscle to the
tilted superiorly, the posterior great vessel is seen first and
septum: The RV side of the septum gains attachment of the
then the anterior great vessel. During this process, the
chordae and papillary muscle. The left ventricular side of
great vessel connections with the ventricles can also be
the septum is always free of chordal attachment.
identified.
Papillary muscles: The LV has two discrete papillary Apical view—As the transducer is tilted superiorly from
muscles. The RV has three or more papillary muscles. the level of AV valves to the great vessel, the connection
with the ventricles can be identified.
Echocardiographic Identification of PLAX view—In this view if the two vessels can be clearly
seen in the same plane and have a side-by-side relation,
Atrioventricular Connection the two great vessels are likely to be transposed. Not
• Concordant AV connection: When the LA (whether only the continuity of the ventricle with the great vessels
right- or left-sided) connects to the morphological is identified in this view, but also the degree of override
LV and the RA (right- or left-sided) connects to the [to classify the anatomy, for example, double outlet right
morphological RV. ventricle (DORV)] can be assessed in this view.
• Discordant AV connection: When the LA (whether Univentricular atrioventricular connection: When both
right- or left-sided) connects to the RV and the the atria connect predominantly to one ventricle it is
morphological RA (right- or left-sided) connects to the called the univentricular connection. Most often the other
morphologic LV. ventricle can be identified echocardiographically and is
rudimentary. Rarely one can have true absence of another
Identification of Great Vessels and ventricle (true single ventricle). The atria can connect
to one ventricle by two valves (double inlet ventricle,
Ventricular Arterial Connection
common AV valve, or a single AV valve, the other valve
Echocardiographic identification of great vessel conne- being absent like in mitral or tricuspid atresia). When two
ction to the ventricular mass is evaluated in the following AV valves are committed to one main ventricular mass
steps. there can be a wide spectrum of commitment. At one end,
both AV valves are completely committed to one ventricle, L-Loop (left hand topology): It implies that the RV is to the
the other ventricle being devoid of an inlet. Then there left of the LV in normally arranged atria and vice versa in
can be a varying degree of commitment of one AV valve mirror imaged atria.
to the dominant ventricle. In such cases, the 50% rule is These features can be easily made out by 2D echo-
used, wherein, if one AV valve is committed more than cardiography.
50% to a ventricle, then it is ascribed to that ventricle. The
reason for commitment is due to straddling/overriding of Ventriculoarterial Connection
a valve through the VSD. Overriding refers to the AV valve
annulus and straddling occurs when the tensor apparatus The ventriculoarterial connections can be divided into the
crosses over to the contralateral ventricle. Usually both following types:
exist together but each can be independent of the other. • Concordant ventriculoarterial connection.
Tricuspid valve always straddles through an inlet VSD and • Discordant ventriculoarterial connection.
the mitral valve through an outlet VSD. • Double outlet ventricle (right or left).
• Single outlet—pulmonary atresia, aortic atresia.
Isomeric atrioventricular connection: This terminology • Common outlet.
is used by the group, which believes in the concept of
isomerism. In such cases, since both atria are isomeric
Concordant Ventriculoarterial Connection
(right or left), the connection cannot be concordant or
discordant as per definition. Subsequent description of The LV is connected to the aorta and the RV to the PA.
the ventricles is done by using the loop method (discussed Discordant ventriculoarterial connection (Fig. 72.24): The
later). LV is connected to the PA and the RV to the aorta. It is also
Uniatrial and biventricular connection: In these cases one called transposition of great arteries.
AV junction is absent but the other AV junction overrides Double outlet ventricle (Fig. 72.25): (1) Double Outlet Right
the ventricular septum with the valve of this junction Ventricle—When the PA and > 50% of the aorta [the VSD
straddling both ventricles, thereby creating a biventricular type] or the aorta and > 50% of the PA (the transposition
connection. type) arises from the RV, it is called DORV. Some authors
would define DORV only when > 90% of a vessel is
Atrioventricular Connections and the committed to the ventricle.
Loop Rule The present definition of DORV is based on connections
In the presence of concordant AV connection in normally of the great vessel to the ventricle. Some groups of authors
arranged atria, there will always be a D-loop (or right feel that double outlet ventricle should be defined only
hand topology), and in the presence of discordant AV
connection in normally arranged atria, it will always be
an L-loop (or left hand topology). The reverse applies in
patients of mirror image atrial arrangement (L-loop in
concordant AV connection and D-loop with discordant
AV connection). There are descriptions of hearts with
concordant AV connections with normally arranged atria
but with an L-loop and also vice versa. Thus, although in
the majority of cases the loop rule holds true and can be
inferred, it was felt necessary to mention the loop in the
sequential description of congenitally malformed heart
because of these rare examples. Also, in criss-cross AV
connections, there may be normally arranged atria and AV
concordance with L-looped ventricles.
D-Loop (right hand topology): It implies that the RV is Fig. 72.24: Two-dimensional echocardiography. Parasternal long-
to the right of the LV in normally arranged atria and vice axis view showing ventriculoarterial discordance. (AO: Aorta; LV:
Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
versa in mirror-imaged atria.
in terms of the conal morphology. Thus, these groups of ventricle and the other great vessel is atretic and cannot
workers would define DORV only when there is bilateral be traced to the ventricular mass. The commonest in
coni, that is, there is aortomitral and tricuspid pulmonary this group is pulmonary atresia. The echocardiographer
or mitral pulmonary and tricuspid aortic discontinuity. needs to distinguish between an absent connection and
(2) Double Outlet Left Ventricle (Fig. 72.26)—When the an imperforate valve. In the latter, there is a potential
aorta and >50% of the PA or PA and >50% of aorta arise communication between the great vessel and the ventricle
from the LV, it is called double outlet left ventricle (DOLV). and therefore can be designated as concordant or
In this condition, there is bilateral absence of the conus, discordant connection.
that is, both the semilunar valves are in continuity with the
Common outlet (Figs 72.28A and B): In this condition, a
AV valves.
common trunk arises from the ventricles and gives rise to
Single outlet (Figs 72.27A and B): This is recognized aorta, PA, and the coronaries. It is also called the truncus
when there is only one great vessel arising from the arteriosus.
Fig. 72.25: Two-dimensional echocardiography. Apical four-cham- Fig. 72.26: Two-dimensional echocardiography in subcostal coronal
ber view with anterior tilt showing double outlet right ventricle with view with anterior tilt in a case of double outlet left ventricle show-
nonrestrictive inlet ventricular septal defect (VSD; *). (AO: Aorta; ing both the great arteries coming off from the left ventricle. (AO:
LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle). Aorta; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
A B
Figs 72.27A and B: A case of ventricular septal defect and pulmonary atresia. Subcostal paracoronal view with color compare showing
the atretic right ventricular outflow tract (arrow) and ventricular septal defect (*). (AO: Aorta; PA: Pulmonary artery.
A B
Figs 72.28A and B: Two-dimensional echocardiography of an infant with truncus arteriosus. (A) Subcostal coronal view with anterior
tilt showing the single outflow-truncus arising from the ventricles; (B) Parasternal short-axis view showing the pulmonary arteries arising
from the common trunk and bifurcating into left and right pulmonary arteries. (LPA: Left pulmonary artery; LV: Left ventricle; MPA: Main
pulmonary artery; RPA: Right pulmonary artery; T: Truncus).
Connections and Spatial Relationship mass, whereas spatial relationship refers to the way the
One has to differentiate between connections and great vessels are related to each other. The two features
spatial relationship of great vessels. Connections refer to are independent of each other and should be separately
the way the great arteries are aligned to the ventricular defined echocardiographically.
PART 2: LEFT-TO-RIGHT SHUNTS: ATRIAL SEPTAL DEFECT, VENTRICULAR SEPTAL

DEFECT, PATENT DUCTUS ARTERIOSUS, AND AORTOPULMONARY WINDOW
In this section, echocardiographic findings of common (Fig. 72.29). If a lesion is beyond the tricuspid valve, it
shunt lesions are discussed. The addition of Doppler and would lead to the volume overloading of the LA and LV,
color flow mapping also gives physiological information often referred to as post-tricuspid shunts (Fig. 72.30).
about flow and pressures and enables the pediatric • The magnitude of the chamber enlargement depends
cardiologist to refer patients for surgical treatment without upon the magnitude of the shunt (in the absence of
cardiac catheterization, especially in neonates and infants. anemia). Thus, significant RA and RV enlargement
The commonly seen shunt lesions include ASD, VSD, would be a feature of pre-tricuspid shunt, while a
patent ductus arteriosus (PDA), and aortopulmonary significant LA and LV enlargement would be a feature
window (APW). of post-tricuspid shunt (Fig. 72.30). The dimension
of the chambers can be compared with standardized
GENERAL FEATURES: SHUNT LESIONS values to ascertain chamber dilation (e.g. by using
There are a few salient features of all the shunt lesions. z-scores).
• The shunt would lead to volume overload of the • The pressure of the investigated chamber can rise
chambers it feeds, generally described in relation on account of distal obstruction (obstruction of the
to the tricuspid valve. If a shunt is proximal to the outflow of the chamber) or it would be because of
tricuspid valve, it would lead to volume overloading of the transmitted pressure from the high pressure
the RA and RV, often referred to as pre-tricuspid shunt communicating chamber.
Fig. 72.29: M-mode echocardiography of a pretricuspid shunt Fig. 72.30: M-mode echocardiography at the ventricular level
showing enlarged right ventricle and volume overloading of the (post-tricuspid shunt) showing dilated left ventricle (LV) with
right ventricle with flattened interventricular septal motion in an normal septal motion in a neonate.
infant.
– Increase in RV pressures may be because of infun- – The size of the VSD may be compared to the size of
dibular or valvular pulmonary stenosis, obstruc- the aortic root for classifying the size of the VSD as
tion in branch pulmonary arteries, or obstruction large, moderate, or small.
in pulmonary vascular bed (as in elevated pulmo- – The size of defects like ASD, VSD, would help in
nary vascular resistance). deciding the size of the device, which may be used
– Increase in pressures in RV will also be seen in large to close these defects.
VSD; similarly, a large PDA would lead to significant • Echocardiography should focus not only on the
increase in PA pressures due to direct transmission characteristics of the primary lesion, but also on
of pressures. the structures adjacent of the defect, for example,
• The magnitude of the gradient from a chamber outflow distances from the adjoining valves.
would also be dependent on the magnitude of shunt – VSD—It is important to note the distances from the
aortic valve and tricuspid valve when considering
into the chamber (Fig. 72.29).
for device closure.
– This may lead to exaggerated gradients even in
– For ASD, the rims are seen not only for their
hemodynamically insignificant lesions, namely
adequacy to hold the device but also the adjoining
exaggerated pulmonary gradients in pre-tricuspid
structures which may be encroached whenever
shunts (like ASD), exaggerated mitral and aortic
contemplating a device closure (Figs 72.32A to C).
valve gradients in post-tricuspid shunts like VSD or – For APW, the distance of the defect from coronaries
PDA. and valves becomes important.
• The secondary manifestations of the shunt lesions may – The post-tricuspid shunt is known to mask
themselves lead to exaggerated secondary effects, for the manifestations of an anomalous LCA from
example, dilatation of LV leading to the mitral annular pulmonary artery (ALCAPA), and thus, one should
dilatation on account of post-tricuspid shunt may lead carefully look at the 2D anatomy and color flow
to mitral regurgitation and this may further lead to mapping to define the origin of the coronary
mitral annular dilatation and LV dilatation. arteries whenever investigating a post tricuspid
• Since a shunt lesion is a communication between two shunt lesion.
chambers, the gradient between the two chambers will • Whenever investigating a shunt at multiple sites or an
be dependent on the size of the defect (Figs 72.31A and B). associated lesions, one must remember that the shunt
• The size of the defect in two dimensions may be a flow may get exaggerated by the presence of distal
useful guide in deciding the degree of shunt. obstruction and also by the associated shunt.
A B
Figs 72.31A and B: (A) Continuous wave Doppler signal of a patent ductus arteriosus, showing peak gradient of 89 mm Hg, systemic
pressures were 100 mm Hg—estimated pulmonary artery (PA) pressures will be 11 mm Hg; (B) Shows the peak systolic gradient across
the ventricular septal defect (VSD) of 98 mm Hg, systemic pressures were 120 mm Hg. By these observations, the predicted right
ventricular (RV) systolic pressure would be 22 mm Hg.
A B
Figs 72.32A to C: Two-dimensional transesophageal echocardio-

graphy. (A) Four-chamber view showing atrial and atrioventricular
valve rims (arrows); (B) Basal short-axis view showing the atrial and
aortic rims (arrows); (C) Modified basal long-axis view, showing the
C superior vena caval and inferior vena caval rims (arrows).
– The associated post-tricuspid shunt may lead to • Determining the volume overload, pulmonary arterial
exaggerated manifestations of pre-tricuspid shunt pressures, associated lesion, and complications.
lesions (e.g. ASD). Thus, RA and RV may be unduly • Defining the lesions operability.
dilated even in the presence of small ASD, with the • Deciding the relevant modality of treatment.
associated presence of post-tricuspid shunt (VSD The essential approach to any lesion should not
or PDA) or associated presence of mitral stenosis. be directed at the shunt lesion, rather it should be a
– The associated aortic stenosis or coarctation of standardized sequential analysis, as it is not the shunt
aorta (CoA) may exaggerate the shunt across the lesion in isolation that exists and one needs to evaluate all
VSD. the structural heart defects. A few salient features of the
• The associated obstructive lesions distal to the shunt important shunt lesions—ASD, VSD, PDA, and APW—are
lesions may become masked and may manifest discussed below.
themselves only after the shunt lesion is closed.
– Mitral stenosis may not manifest itself in the ATRIAL SEPTAL DEFECTS
presence of ASD (although it may exaggerate the
shunt flow across it). Objectives (see Also Table 72.4)
– The manifestations of significant mitral regurgita- To diagnose ASD, assess the following:
tion may get unmasked after ASD closure. • Its anatomical site and size.
– High left ventricular end-diastolic pressure • The direction and quantum of flow.
(LVEDP) may not only exaggerate ASD shunt, but • The degree of pulmonary arterial hypertension.
also lead to pulmonary edema after ASD closure. • AV valve anomalies, pulmonary veins, and pulmonary
– VSD or PDA may mask the gradients across aortic valve stenosis.
stenosis or CoA, which may manifest after the
treatment of the underlying shunt lesion. Atrial Septal Defect Types1,2,14,15
• Certain systemic disorders and conditions may exag-
Echocardiography plays a major role in the evaluation of
gerate or confound the echocardiographic features of
ASD. Defects of atrial septum are classified as follows.
a shunt lesion.
– Anemia may exaggerate the gradients across any
shunt lesion or across valves. Anemia may lead to Patent Foramen Ovale
LV dilatation, thus confounding the assessment of Foramen ovale is a passage between septum secundum
post-tricuspid shunt lesion. on right side and septum primum on left side. Its patency
– Systemic hypertension may not only lead to
exaggerated shunt gradients, but also result in
secondary ventricular hypertrophy, thus leading to
high LVEDPs and exaggerating ASD shunt.
– Hyperdynamic states such as fever and thyroid
disorders may exaggerate the shunt gradients.
Thus, an assessment of a patient with a shunt lesion
does not mean an isolated evaluation by echocardiography;
it refers to complete clinical evaluation of the patient.
Now we will discuss individually the assessment of shunt
lesions seen commonly.
Step-wise Approach
(On Echocardiography)
Step-wise approach (on echocardiography) for evaluation Fig. 72.33: Two-dimensional transesophageal echocardiography.
of any shunt lesion involves: Basal long-axis view with color flow mapping showing left-to-right
• Determining the presence of shunt lesion (Fig. 72.33). shunt flow. No additional defects are seen. (LA: Left atrium; RA:
Right atrium).
• Defining its location and size of defect (Figs 72.32A to C).
Table 72.4: Stepwise Evaluation for an Atrial Septal Defect

Initial indication is the volume overload of the chambers [right atrium (RA) and right ventricle (RV)] in 4C view
Visualize the defect from subcostal view (sagittal and coronal views)
Determine the site of defect: fossa ovalis or other
Determine the direction of shunting
Assess associated structures, particularly pulmonary veins and atrioventricular (AV) valves
Assess pulmonary artery pressures: tricuspid regurgitation (TR) and pulmonary regurgitation (PR) gradients
Determine suitability for device closure
These limbic bands separate the defect from atrial wall.

These defects could be single, fenestrated mesh-like, or
multiple.
Sinus Venosus Atrial Septal Defect

(Figs 72.36A and B)
The hallmark of all of these defects is that the border of
the fossa ovalis should be intact, the defect is overlapped
by either SVC in SVC type and by IVC in IVC type sinus
venosus defect. There may be an associated anomalous
drainage of pulmonary veins. Defects extending from the
fossa ovalis superiorly or inferiorly should not be classified
as a venosus defect.
Fig. 72.34: Two-dimensional echocardiography. Subcostal bicaval
view with color view mapping showing fossa ovalis atrial septal Coronary Sinus Atrial Septal Defect
defect with left-to-right shunt. (SVC: Superior vena cava; RA:
Right atrium; LA: Left atrium).
(Fig. 72.37)
Coronary sinus ASD is a rare anomaly. It is located in the
is a must for fetal survival and for normal growth of the inferior most part of the atrial septum at the anticipated
fetal heart. After birth, the foramen ovale closes as left site of the coronary sinus ostium. The clue to the diagnosis
atrial pressure rises due to increased pulmonary venous of such a defect is the presence of a persistent left superior
return with fall in PVR. If pressure on either side of atria vena cava (LSVC) with evidence of RV volume overload.
rises, stretching of flap of foramen ovale occurs and leads
to shunting across foramen ovale. In most individuals, the Ostium Primum Atrial Septal Defect
foramen ovale is functionally closed shortly after birth; (Fig. 72.38)
however, patency of a competent foramen ovale has been
found in 25% of normal hearts on autopsy. This defect is present in the lower most part of the atrial
septum. This type of defect may be part of atrioventricular
Fossa Ovalis Atrial Septal Defect septal defect (AVSD) and may be characterized by the
absence, in part or whole, of the atrioventricular septum.
(Figs 72.34 and 72.35)
It is the commonest of the ASDs (69%) with varied sizes. Echocardiographic Imaging in
This type of defect occupies the central part of atrial
septum involving in part or whole flap valve of the foramen
Atrial Septal Defects
ovale. Septum ovale defects are bounded on either side by Enlarged RA, RV, and paradoxical septal motion of
the limbic bands (superior and inferior limbic bands). interventricular septal motion are indirect evidence of
Figs 72.35A and B: Two-dimensional echocardiography. Subcos-

tal bicaval view with color comparison showing fossa ovalis atrial
septal defect with adequate superior vena cava (SVC) rim (upper
arrow) and deficient inferior vena cava (IVC) rim (lower arrows) and
left-to-right shunt. (LA: Left atrium; RA: Right atrium; SVC: Superior
A B vena cava).
A B
Figs 72.36A and B: Two-dimensional echocardiography. Subcostal bicaval view with color comparison showing the superior vena cava
(SVC) type of sinus venosus atrial septal defect. SVC is overriding the defect with partial anomalous pulmonary venous drainage of
right upper pulmonary vein to SVC. (LA: Left atrium; RA: Right atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena cava).
left-to-right shunt at atrial level. The best views to directly the defect, it is IVC sinus venosus type. The defects in the
visualize the ASDs are subcostal coronal and sagittal views. centre of the atrial septum involving the fossa ovalis area
ASD can be diagnosed by a drop out in the interatrial are fossa ovalis defects. The defect in the lower most part of
septum with flow across the defect on color flow mapping. the interatrial septum with atrioventricular valves attached
When the defect is visualized, its relationship to the SVC at the same level are designated as ostium primum defects.
and IVC should be evaluated. If the SVC forms the roof of The imaging should also be done from apical four-
the defect, it is SVC sinus venous type. If the IVC straddles chamber and short-axis views. The four-chamber view will
Fig. 72.37: Two-dimensional echocardiography. Subcostal coronal Fig. 72.38: Two-dimensional echocardiography. Apical four-
view with posterior tilt showing coronary sinus type of atrial septal chamber view showing a large ostium primum atrial septal defect
defect (arrow). (LA: Left atrium; RA: Right atrium). (ASD) defect in lowermost part of the interatrial septum (arrow) with
absence of offsetting of the atrioventricular (AV) valves (arrow). (LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
also show the attachments of the AV valves. These may be than right atria. The second wave of left-to-right shunt
at the same level in ostium primum defect. The color flow occurs with atrial contraction. A small right-to-left shunt
mapping across the defect will show the direction of flow occurs during early systole when right atrial pressure
and also presence or absence of any atrioventricular valve exceeds left atrial pressure because of unequal activation
regurgitation. Doppler velocities across all valves should time of the two atria. A second wave of right-to-left shunt
be taken, in particular the pulmonary valve to look for any occurs with rapid filling phase in early diastole. During that
pulmonary stenosis. time, IVC flow tends to flow toward the LA. Respiration
The inflow velocities of the AV valves should be seen has some effect on direction of shunting; with inspiration,
to rule out any mitral valve obstruction. The associated the left-to-right shunt decreases, while the reverse occurs
mitral obstruction may be missed unless specifically seen during expiration.
on 2D echocardiography, as there may not be a significant For the estimation of pulmonary arterial pressure, the
gradient across the mitral valve even with significant peak gradient of tricuspid regurgitation gives the systolic
obstruction because of an associated ASD (true for all pressure of the RV in absence of RV outflow obstruction. If
Lutembacher cases). All pulmonary veins should be pulmonary regurgitation is present, the pressure derived
specifically imaged to see if they are abnormally connected from the peak diastolic velocity will reflect the pulmonary
or not and to look for any pulmonary vein stenosis. The arterial mean pressure.
pulmonary veins are best seen in subcostal coronal and Doppler echocardiography accurately depicts the
sagittal, apical four-chamber, PSAX, and suprasternal direction of shunting.
short-axis views. • Pulsed Doppler: Pulsed Doppler shows the typical flow
pattern as discussed earlier.
Direction of Shunt14–16 • Color flow mapping (Figs 72.33 and 72.39):
With an isolated uncomplicated ASD, pressure between – With 2D imaging, color flow mapping clearly shows
the two atria is similar with a variation up to 5 mm Hg, with the net direction of flow.
cardiac cycle and phases of respiration. Dominant shunt – M-mode—This is seldom used in daily practice but
occurs from left to right. Left-to-right shunt occurs mainly depicts best the direction of shunting during the
during mid to late systole as the “v”-wave of LA is larger various phases of a cardiac cycle.
A B
Figs 72.39A and B: A case of total anomalous pulmonary venous drainage in a 2-year-old child. Two-dimensional echocardiography
with color compare in subcostal sagittal view showing right-to-left shunt across atrial septal defect (arrow). (LA: Left atrium; RA: Right
atrium).
Doppler and CFI should always be performed for the Objective Calculation of Qp/Qs Ratios
following reasons:
• To confirm the presence of an echo dropout seen on Pulmonary and systemic flows can be calculated by the
2D imaging. The characteristic pulse Doppler tracing 2D and Doppler echocardiogram. The following formula is
and/or CFI confirms the echo dropout as a true defect. being used to calculate the flows:
• Interrogation of the AV and semilunar valves for V × CSA × RR
SV =
regurgitation and stenosis. In this regard, the presence 1000 mL /1
of trivial to mild tricuspid or pulmonary regurgitation
is a universal finding in large ASDs and is related SV : stroke volume.
to RV dilatation leading to tricuspid annulus and V : mean velocity (velocity time integral).
PA dilatation due to large pulmonary blood flow. CSA : Cross-sectional area of flow (cm2).
Increased forward velocity across the pulmonary RR : R-to-R interval (s/beat).
valves up to 3 to 4 m/s may be seen with large ASDs Cardiac output = stroke volume multiplied by heart rate
and does not always indicate pulmonary stenosis. The and the (heart rate equals 60,000/RR interval), then the
pulmonary valves in these cases are morphologically cardiac output will be
normal and there is no doming of the valve. V × CSA × 60s /min
• The tricuspid regurgitation velocity should always be
1000 mL /1
obtained to predict the RV systolic pressure and hence
indirectly the PA pressure. To calculate pulmonary blood flow (Qp), PA mean
• Interrogation of the pulmonary veins should be done velocity and diameter of pulmonary outflow can be
to rule out the association of pulmonary venous measured from PLAX view of right ventricular outflow
obstruction. tract (RVOT) or PSAX view.
To calculate the systemic blood flow (Qs), mean the cardiac cycle and in various phases of respira-
velocity across left ventricular outflow and the diameter of tion, particularly in the PA .
left ventricular outflow can be measured. To calculate the – Measurement in the direction of lateral resolution.
aortic mean velocity, apical five-camber view is used; the
sample volume is kept just above the aortic valve leaflets. Indication of Cardiac Catheterization
Other views that can be used to measure the aortic mean
velocity are subcostal and suprasternal views. Diagnostic cardiac catheterization in ASDs is indicated in
To measure the left ventricular outflow diameter, those instances where correct evaluation of PA pressure
PLAX view is the best view. Other views that can be used to is not possible by Doppler echocardiogram, pulmonary
measure the left ventricular outflow diameter are subcostal hypertension is suspected and information on PVR is
and suprasternal. required in decision making. This is because, although PA
While taking the mean velocity, the Doppler beam pressure may be reliably predicted by Doppler calculation,
should be positioned as parallel as possible to the flow, so calculation of flow data is fallacious and can introduce
that no correction for intercept angle needs to be made. error in the calculation of PVRs. In cases with elevated
To calculate the systemic and pulmonary blood flow, PVR, the reactivity of the pulmonary vascular bed can also
in place of left and RV outflow, we can use mitral and be tested during cardiac catheterization.
tricuspid mean velocity and annulus diameter in apical
four-chamber view assuming no regurgitation in patients Evaluation of Atrial Septum by
without shunt lesions. In patients with atrial shunt, the Transesophageal Echocardiography1,2,16
tricuspid flow will represent pulmonary blood flow and
mitral flow will represent systemic flow. But in ventricular In pediatric age group, transthoracic echocardiography
and aortopulmonary shunts, the mitral flow will represent using subcostal view provides a complete diagnosis and
the pulmonary blood flow + the systemic blood flow and good assessment of ASD for interventional or surgical
aortic flow will represent the systemic flow. closure. In adolescents and adults, as subcostal windows
After calculating the systemic and pulmonary blood do not provide adequate penetration, transesophageal
flow, left-to-right shunt can be calculated by subtracting echocardiography (TEE) is required for detailed profilation
systemic blood flow from pulmonary blood flow or the of the ASD.
ratio of pulmonary and systemic blood flows, Qp/Qs can Views that are most useful for evaluation of ASD on
be calculated. TEE are:
• Basal short-axis view.
• Bicaval or basal long-axis view.
Limitations of Technique of Estimation of • Four-chamber view.
Degree of Left-to-Right Shunt
Various Doppler methods to calculate Qp/Qs have been Basal Short-Axis View
described, but there are several possible sources of error This view can be obtained by keeping the endoscope in the
while Doppler is being used to calculate the flows; so we middle part of esophagus. This view provides imaging of
do not use this routinely. aortic valve and atrial septum. The aortic and atrial rims
• Errors in the measurement of mean velocity due to: can be best seen in this view. The fossa ovalis defect is seen
– Errors in determining the intercept angle. in middle part of atrial septum, sinus venosus defect SVC
– The lack of a uniform velocity profile across the type is seen in the upper part of the septum adjacent to
vessel lumen. SVC, and the sinus venosus IVC type of ASD is seen in the
– Variation caused by respiration or other physiologi- lowermost part of septum adjacent to IVC.
cal factors. During respiration, the variability in the
velocity time integral at mitral and tricuspid valve
are 14.5% and 13.2%, respectively.
Basal Long-Axis or Bicaval View
• Errors in measurement of cross-sectional area due to This view can be obtained by keeping the endoscope at the
– Inaccurate gain settings. same level and rotating the icon to 80° to 100°. This profiles
– Due to pressure, flow, and elasticity of vessel, the the ASD in relation to superior and IVC, fossa ovalis defect
cross-sectional area of vessel changes throughout is seen in middle part of septum. Sinus venosus defect
Table 72.5: Stepwise Evaluation for Ventricular Septal Defect

Initial indication is the volume overload of the chambers [left atrium (LA) and left ventricle (LV)]
Visualize the defect from all possible windows
Determine the presence of additional defects (screening the septum—septal sweep in subcostal, apical four-chamber, parasternal
short- and long-axis views in color and B-mode)
Determine the direction of shunting through the defect
Assess associated defects, particularly outflow obstructions and adjacent structures
Assess pulmonary artery pressures: ventricular septal defect (VSD) gradients, tricuspid regurgitation (TR), and pulmonary regur-
gitation (PR) velocities
Determine the volume overload of chambers (z-scores of LV, particularly in M-mode)
Determine suitability for device closure or surgery
is seen in relation to SVC with SVC type of defect or in view and apical four-chamber views. The aortic rim is best
relation to IVC with IVC type of defect along with partial seen in transthoracic echocardiography PSAX view. The
anomalous venous drainage of pulmonary vein. In case superior vena caval and inferior vena caval rims are best
of fossa ovalis ASD, superior vena caval and inferior vena visualized on transthoracic echocardiography in subcostal
caval rims can be accurately assessed in this view. sagittal (bicaval view).
Four-chamber View VENTRICULAR SEPTAL DEFECT

Four-chamber view can be obtained by keeping the Objectives (Table 72.5)
endoscope at the lower part of esophagus. This view
profiles the atrial septum with fossa ovalis defect in the • Confirmation of VSD.
middle of septum, atrial and AV valve rims, and volume • Determination of the size and morphological location
overloaded RA and RV. By rotating the endoscope, we of VSDs.
can see the attachment of right and left pulmonary veins • Ruling out associated lesions.
draining into LA. In this view mitral valve morphology and • Assessment of chamber size and wall thickness.
mitral regurgitation can also be assessed. • Estimation of shunt size (pulmonary/systemic flow
ratio).
• Estimation of RV and pulmonary arterial pressures.
Assessment of Fossa Ovalis Atrial Septal
Defect for Percutaneous Device Closure Morphological Location17,18
With the availability of various devices for the closure VSDs should be imaged from several planes. Artifactual
of ASD, the importance of detailed anatomy of the ASD dropouts may confuse the viewer using single plane
including rims around the defect and the relationship imaging about the presence or absence of a VSD,
with the surrounding structures has become of great particularly if it is small. The addition of color Doppler flow
importance. 2D echocardiography, both transthoracic imaging is useful to reconfirm the presence of VSDs. CFI
and transesophageal, clearly show the anatomy and is of VSDs has also radically improved the ability to detect
used for selecting the cases for device closure of the ASD. unusually located and/or very small VSDs.
During the procedure of device closure, TEE serves as the Morphological location of VSD is described as
most important landmark for the proper placement of the viewed from the RV. VSDs can be classified into following
“device.” We have proposed that for clarity, uniformity, and types1,2,18–22 (Fig. 72.40):
mutual communication, the rims should be designated • Perimembranous VSD
according to the structure they are related, for example, • Muscular VSD
superior vena caval, inferior vena caval, AV septal (rim at – Muscular inlet
the crux), aortic, and atrial rims (rim of the superior wall of – Muscular outlet
atrium near the right upper pulmonary vein). – Trabecular defect
The AV septal and atrial rims are best visualized on • Doubly committed VSD
transthoracic echocardiography in subcostal coronal • Inlet VSD
Perimembranous (Figs 72.41A and B) valve and tricuspid valve, with the membranous septum
completely excavated away. However, perimembranous
To identify a VSD as perimembranous, it is essential to
defects are not restricted to the membranous septum
demonstrate involvement of the membranous septal
but always extend into one or more of the neighboring
area. Cross-sectional echocardiography shows a discrete
subunits of the muscular septum. Such defects are then
area of septal dropout in the area of the interventricular
subdivided into three distinct groups on the basis of the
membranous septum adjacent to posterior semilunar
major extension of the defect.
Perimembranous outlet defect: These defects excavate
anteriorly from the area of interventricular membranous
septum into the subarterial portion of the muscular outlet
septum. They are called perimembranous subaortic
defects. Such defects are not seen in a scan through the
four-chamber plane and will only be seen when scanning
the four-chamber plus aortic root plane, that is, five-
chamber view or long-axis views. The defects are clearly
roofed by the roof of the posterior great artery, with the
trabecular septum and tricuspid valve forming their
inferior rim. Large perimembranous outlet defects, which
extend anteriorly below the whole great vessel root, are
consistently visualized in the precordial long-axis plane,
and are also visualized using serial short-axis scans.
Perimembranous inlet defects: In these defects, the area
of septal dropout associated with the defect extends
posteriorly into the four-chamber plane as the defect
excavates through the muscular inlet septum toward the
Fig. 72.40: Schematic diagram of the interventricular septum with
crux of the heart. The roof of this posterior extension is
removed right ventricular (RV) cavity from the RV side. Various
parts of the interventricular septum are shown: blue—muscular formed by the atrioventricular junction tissue enclosed by
septum, dark yellow—perimembranous region, purple—the inlet the septal aspects of the mitral and tricuspid valve annuli.
septum, and light brown—outlet septum. (Ao: Aorta; IVC: Inferior Anteriorly, this roof is formed by central fibrous body and
vena cava; Mod.: Moderator band; PB: Parietal band; RA: Right AV muscular septum and posteriorly by the AV fibrous
atrium; SB: Septal band; SVC: Superior vena cava).
plane. Perimembranous inlet defects will be consistently
A B
Figs 72.41A and B: Two-dimensional echocardiography. (A) Apical four-chamber view with anterior tilt showing the perimembranous
ventricular septal defect (VSD) (arrow) getting restricted by septal leaflet of the tricuspid valve in two dimensions; (B) Color flow map-
ping of the same patient showing the turbulent jet of the restricted VSD with small left ventricle (LV)-to-right atrium (RA) shunt (arrow).
visualized in a four-chamber plane roofed by AV valve body or an AV or arterial valve ring. Since it is impossible
septal leaflets, which insert into the central fibrous body to differentiate the subunits of the muscular septum
at a common level. Scanning anteriorly to the junction accurately using cross-sectional echocardiography,
of the four-chamber plane with the four chambers plus correct classification of any muscular defect depends on
aortic root plane will demonstrate the involvement of accurate knowledge of where each subunit of the muscular
the membranous septum by the defects. An isolated septum is visualized within the various planes available to
perimembranous inlet defect will not be visualized in any the echocardiographer. With this knowledge, it is possible
of the other standard echocardiographic planes recorded. to differentiate the muscular defects into three types.
Perimembranous trabecular defects: In the normal heart, Muscular inlet defects: These defects lie within the bounda-
the inferior border of the membranous septum merges ries of the smooth inlet septum and will be visualized by
imperceptibly into the trabecular septum. So it is not scanning through the four-chamber plane. They are not
surprising that cross-sectional echocardiography can visualized in any other of the echocardiographic planes,
neither determine the precise junction of these two and their characteristic feature, which allows differentia-
subunits, nor can it demonstrate involvement of the tion from the perimembranous inlet defects, is that the
trabecular septum in a perimembranous defect. However, upper muscular rim of the defect is separated from the AV
a correlation of echocardiography with morphological junction by a muscle bar. Thus, in muscular inlet defects,
findings suggest that two echocardiographic features will the AV junction morphology is normal compared to the
indicate inferior extension of a perimembranous defect abnormal morphology in perimembranous inlet defects.
into the trabecular septum: (a) a broad blunt upper Muscular outlet defects: These defects have entirely
end of the interventricular septum forming floor of a muscular rims and are located in the smooth muscular
perimembranous defect at the posterior aspect of a scan outlet septum below the anterior portion of the aortic root
through the four-chamber plus aortic root plane, and (b) and the subpulmonic infundibulum. This septal subunit is
where any such defect is seen to extend inferiorly for more a small and extremely elusive structure. It is only profiled
than half the aortic root diameter. accurately in one echocardiographic view, subcostal RV
outflow plane, but it can also be seen in PSAX view. In
Muscular Ventricular Septal Defects these planes, the defects are seen to be located mainly
in the anterior muscular outlet-septum separated from
(Figs 72.42 and 72.43) the pulmonary valve by the muscular infundibulum. By
The morphological feature diagnostic of a muscular defect definition, they never extend posteriorly to involve the
is that its rims are formed entirely by muscle and does membranous septum. Muscular outlet defects may occur
not include the fibrous tissue of either the central fibrous in isolation or may present part of a complex lesion.
A B
Figs 72.42A and B: Two-dimensional echocardiography in subcostal coronal view (A) with anterior tilt and color compare; (B) Showing
outlet muscular ventricular septal defect (VSD; arrow). (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
A B
Figs 72.43A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view showing the apical muscular
ventricular septal defect (VSD) with left-to-right shunt.
A B
Figs 72.44A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view showing the doubly committed
ventricular septal defect (VSD) with left-to-right shunt.
Muscular trabecular defect: These defects are divided into: on cross-sectional echocardiography. CFI is helpful also in
(a) Single trabecular defect—These are best visualized defining multiple muscular defects.
on short-axis scanning from the apex of the heart to the
great artery roots or long-axis scanning of septum in Doubly Committed Subarterial Defects1,23,24
different planes. Defect size is closely related to the ability
of the cross-sectional system to identify the defect. (b)
(Figs 72.44A and B)
Multiple trabecular defects (Swiss cheese defects)—These Doubly committed subarterial defect is roofed by conjoint
defects may not be directly visualized by cross-sectional aortic and pulmonary valve rings that appear to lie at
echocardiography. Multiple defects burrow through the same level. In these defects, the ventricular septum
the septum obliquely and may not produce a complete will appear intact throughout the four-chamber plane.
echocardiographic window across the width of the Doubly committed subarterial defects are seen from the
trabecular septum, so that the septum may appear intact four-chamber view with anterior tilt, paracoronal view,
parasternal long-axis, and PSAX views. Scanning anteriorly Inlet Ventricular Septal Defect
into the four chambers plus aortic root plane, the defects
will be visualized below the aortic and pulmonary trunks,
(Figs 72.45 and 72.46)
which take origin at the same level and are in a side-by- The features of the inlet VSD are essentially the same as
side relationship. The conjoint arterial valves roof the that of the muscular inlet VSD (described above) except for
defect, whose inferior margin is formed by the crest of the the absence of the muscular par below the inlet valves and
trabecular septum. there is continuity of both the inlet AV valves established
by the presence of the VSD; therefore, off setting of the AV
valve may not be present.
Size of VSD: The size of any defect is important to
comment but in practice, the judgment of size of defect
is generally made on hemodynamic grounds (degree of
left-to-right shunt, presence of volume overload, and
PA pressure). According to some authors, a VSD size is
defined in relation to aortic root size, a defect is defined
as a small VSD if it is less than one third of aortic root
diameter, one third to two thirds of aortic root diameter
is considered as a moderate-sized defect, and defects
that are more than two thirds of the aortic root size are
defined as large VSDs. With isolated defects, when there
is equalization of pressure between two ventricles in the
absence of pulmonary stenosis, they are then termed
large or nonrestrictive defects. Since right and LVs do not
Fig. 72.45: Two-dimensional echocardiography in apical four- contract exactly simultaneously, there is always some
chamber view showing the large inlet ventricular septal defect inequality in the ventricular pressures. A restrictive defect
(VSD) getting partially restricted by septal leaflet of tricuspid valve is one in which RV and PA pressures are lower than LV
(arrow).
with a pressure gradient of > 60 mm Hg (VSD peak velocity
A B
Figs 72.46A and B: Two-dimensional echocardiography. (A) Subcostal coronal view with anterior tilt showing the perimembranous
ventricular septal defect (VSD); (B) Parasternal long-axis view with posterior tilt toward the tricuspid valve and color compare showing
the perimembranous VSD.
> 4 m/s). Moderately restrictive VSDs have a pressure pulmonary stenosis, in case of anterior malalignment
difference of 25 to 60 mm Hg (VSD peak velocity 2.5–4 m/s). [as in tetralogy of Fallot (TOF)] or may be associated with
In all patients, the aortic valve should be carefully profiled posterior malalignment leading to subaortic obstruction.
in relation to VSD, to rule out any prolapse of aortic cusps The latter may be associated with other left ventricular
through the VSD making it appear artifactually small outflow tract (LVOT) obstructive lesions such as subaortic
(Fig. 72.47). These patients will need surgery if one third or membrane, bicuspid aortic valve, or even CoA.
more of the aortic cusp is prolapsing through the VSD, or if
it is associated with aortic regurgitation. There may be large Doppler Evaluation of
malaligned VSDs (Figs 72.48A to C) that are associated with
anterior or posterior malalignment of the outlet septum. Ventricular Septal Defect
These are generally very large and associated with either
Color Doppler
Use of color Doppler has proved a valuable addition to the
diagnosis of VSDs in several ways.
• The location of VSDs especially smaller ones and
multiple muscular ones can easily be determined
using Doppler color flow mapping technique.
• Size of VSD.
• Determination of shunt direction across the VSD.
• Color flow mapping is also useful for proper alignment
of jet flow for Doppler interrogation.
Color flow mapping has a major role in rapid detection
and localization of VSDs (Fig. 72.49).25–28 Small ventricular
septal defects, especially muscular, can be missed by
2D echocardiography. Color flow mapping is sensitive
in detecting small and multiple VSDs. The sensitivity of
color flow mapping is more with restrictive defects than
Fig. 72.47: Two-dimensional echocardiography with parasternal
long-axis view showing the doubly committed ventricular septal nonrestrictive ones, probably because of early detection of
defect (VSD) getting partially restricted by the prolapse of the right a turbulent jet with a restrictive VSD.
coronary cusp (arrow). (LA: Left atrium; LV: Left ventricle; RV: Color flow mapping has been used to define the
Right ventricle).
direction of shunt across a VSD whether it is left-to-right
A B C
Figs 72.48A to C: Two-dimensional echocardiography. (A) Parasternal long-axis view showing perimembranous ventricular septal defect
(VSD) with anterior malalignment of the septum (arrow); (B) Subcostal coronal view with anterior tilt showing the anterior malalignment
of the septum (arrow); (C) Parasternal long-axis view showing perimembranous VSD with posterior malalignment of the septum (arrow).
Continuous and Pulsed Wave

Doppler Examination25–28
Pulsed and continuous wave Doppler examination is used
to assess the direction of shunt across a VSD, pressure
gradient across it (difference of LV–RV systolic pressure),
RV pressure (by VSD gradient and peak gradient of
tricuspid regurgitation jet), and diastolic function of both
ventricles.
Direction of Shunt (Fig. 72.49)

With isolated uncomplicated nonrestrictive VSD, pressure
between the two ventricles is similar. In patients with
low PVR, dominant shunt occurs from left to right during
Fig. 72.49: A 25-year-old man with Eisenmenger syndrome. Two- systole, and with increase in LV, end-diastolic pressure
dimensional echocardiography with parasternal long-axis view
showing doubly committed ventricular septal defect with right-to- left-to-right shunt will persist during diastole also. A
left shunt. (LV: Left ventricle; RV: Right ventricle). typical “M”-shaped flow pattern is seen in patients
with nonrestrictive VSD, explanation for which is: as LV
contraction starts early and lasts longer than RV, so with
or right-to-left, and whether it is laminar (high RV systolic onset of systole flow occur from left to right, with decrease
pressure) or turbulent (low RV systolic pressure). With in degree of shunt during midsystole as pressure between
isolated restrictive VSD, the shunt is seen as a turbulent two ventricles equalize, and in later part of systole as RV
left-to-right jet during systole. With small or moderate size relaxes, left-to-right shunt dominates. With restrictive
defects, because the LV diastolic pressure is higher than RV VSD, left-to-right shunting occurs throughout systole. In
diastolic pressure, left-to-right shunt may persist during some small muscular VSDs, left-to-right shunt occurs only
diastole also. With a nonrestrictive VSD with low PVR, during the early phase of systole, presumably because
left-to-right shunt occurs during systole with a small right- of closure of the defect in midsystole with ventricular
to-left shunt occurring during the isovolumic relaxation contraction. Bidirectional or right-to-left shunting can
period. If in addition there is RV volume overload occur with both restrictive and nonrestrictive VSDs as
(associated significant tricuspid regurgitation, ASD, or described earlier.
anomalous pulmonary venous connection), right-to-left
shunt occurs throughout diastole. In a child with isolated Pressure Gradient across
nonrestrictive VSD with high PVR, direction of flow can Ventricular Septal Defect
be bidirectional or dominantly right to left depending
upon the severity of pulmonary vascular obstructive While taking continuous wave Doppler across a VSD,
disease. With associated pulmonary stenosis, there may be the cursor should be well aligned with the defect jet on
isolated right-to-left shunt depending upon the severity of color flow mapping. The velocity of the VSD shunt can
pulmonary stenosis. With severe RV outflow obstruction, be determined using the Bernoulli’s equation, p = 4V2,
a turbulent jet of right-to-left shunt may be seen in a small where p is the pressure difference and V is the maximum
VSD due to suprasystemic RV systolic pressure. recorded velocity. This gives the difference between the
With the use of color flow mapping with careful left and RV systolic pressures. The left ventricular systolic
interrogation, one should also identify if there is any LV to pressure is derived from the systolic BP (provided there is
right atrial shunt as the high velocity of LV to right atrial no left ventricular out flow obstruction), which should be
jet can be misinterpreted as elevated RV pressure. This can recorded at the time of Doppler study using appropriate-
happen particularly with VSDs, which are decreasing in sized BP cuffs.
size because of ingrowth of tissue from the septal leaflet of Right ventricular pressure = Systolic blood pressure–VSD
tricuspid valve. jet peak gradient (4V2)
Fig. 72.50: M-mode echocardiography in a case of ventricular Fig. 72.51: Two-dimensional echocardiography in apical four-
septal defect showing a dilated left ventricle (LV) in a 2-year-old chamber view with color compare showing the well-opened mitral
child. valve (arrow) and turbulence across mitral valve on color flow
mapping, because of increased flow across the mitral valve in
This equation has been found to have good correlation a patient with nonrestricted perimembranous ventricular septal
with cardiac catheterization-derived RV systolic pressure. defect. (LA: Left atrium; LV: Left ventricle).
However, sometimes the jet velocity may not reflect the
interventricular pressure gradient accurately because PA pressure if there is no pulmonary stenosis. Presence
proper alignment of the Doppler beam with the jet is of pulmonary stenosis and aortic regurgitation should
not possible, and that if the defect has some length to be evaluated. The velocities of flow across both AV valves
it, the viscous frictional forces make the application of and semilunar valves should be measured to rule out any
the modified Bernoulli’s equation inappropriate. Also, associated abnormality.
because the dP/dt of the RV is lower than that of the LV, a
falsely high jet velocity can be recorded in some cases with Pulmonary Blood Flow to Systemic
an anatomical large VSD. Determining the RV pressure
from tricuspid insufficiency jet velocity (which may be
Blood Flow Ratio
found in some cases) is more accurate in such instances. With regards to quantifying pulmonary and systemic
shunt flow using Doppler echocardiography, several
M-mode Echocardiography (Fig. 72.50) methods currently exist, although none is widely used due
to variable results.
M-mode echocardiography is used to assess left atrial and
left ventricular size, to provide an estimation of the degree
of shunt across the VSD and to evaluate RV size and wall
Assessment of Suitability for
thickness, which will reflect elevated RV systolic pressure. Device Closure
For direction of shunt, color M-mode is rarely used in daily Percutaneous closure for midmuscular VSDs and some
practice but depicts best the direction of shunting during perimembranous VSDs can be done. While assessing
the various phases of a cardiac cycle. the child for device closure, the defect should be at least
5 mm away from AV valves and semilunar valves and other
Interrogation of the Atrioventricular and defects requiring cardiopulmonary bypass should not
Semilunar Valves for Regurgitation and be present. The softer variety of Amptazer duct occluder
II (ADO II) series can be used to close defects in the
Stenosis (Fig. 72.51) perimembranous region (particularly a defect with an
Tricuspid regurgitation velocity should always be obtained aneurysm of the septal leaflet of the tricuspid valve) and
to predict the RV systolic pressure and hence indirectly the muscular VSDs.
Utility of Transesophageal – Pulmonary arterial pressure.

– Quantification of shunt.
Echocardiography • Associated defects.
TEE has helped in better visualization of VSDs when the
transthoracic window is poor, especially in adolescents Echocardiographic Views
and adults. Straddling and overriding of the AV defect can
be better detected by TEE. TEE is extremely useful during The following echocardiographic views profile accurately
percutaneous closure of these defects. the morphology of the duct.
Ductal view (Fig. 72.52): The transducer is placed in the
PATENT DUCTUS ARTERIOSUS high parasternal window just beneath the left clavicle.
After obtaining a short-axis cut of the great vessels
Anatomy visualizing the PA bifurcation, the transducer is rotated
The ductus arteriosus is a remnant portion of the sixth anticlockwise in gradual motion. At one point, the right PA
aortic arch that connects the left PA with the descending goes away from the view and the duct with the adjacent
portion of the aortic arch. The PA end of the PDA is descending aorta opens. This view in neonates and infants
usually immediately to the left of the PA bifurcation. also visualizes the origin of the left subclavian artery. In
The aortic connection is just distal to the origin of the patients with associated coarctation, the posterior shelf
left subclavian artery. The pressure differences across and coarctation can also be well visualized in this view.
the PDA is estimated by the Doppler velocity, which Suprasternal view: There are three views to visualize the
will predict the pulmonary arterial pressures, systolic duct from the suprasternal view.
as well as diastolic, by its difference with the BP of the
child. The flow across the PDA is estimated by the size (1) Suprasternal long-axis view—This is the best view for
of LA and LV. Spontaneous closure of the PDA usually visualizing the vertical duct arising from the undersurface
begins at the pulmonary end within 24 hours of birth. of the transverse arch in patients with pulmonary atresia.
Persistence of this fetal structure beyond 10 days of life in The origin of such ducti is well seen, but the insertion point
a term baby is considered abnormal. The ductus is funnel- at the PA requires further anterior tilt. This is because of
shaped in configuration in approximately two thirds the tortuous nature of such ducti.
of patients.27 In patients with discordant ventriculoarterial
connection (e.g. transposition of the great vessels), the
Echocardiography1,2,29–31 duct can be visualized well in its entire length in this view.
(2) Suprasternal short-axis view—This is the classical
Objectives (Table 72.6) short-axis arch view and can visualize those ducti which
• Demonstrate the presence of a duct. arise from the base of the left subclavian artery and
• Detailed definition of ductus. descend straight down to insert into the left PA. If the aortic
– Size of the duct. arch is right-sided and the patient has pulmonary stenosis
– Type of duct. physiology, the entire length of the duct can be seen in this
• The hemodynamic significance of ductus. view because unlike in patients with a vertical duct, it does
– Direction of shunt. not follow a tortuous course.
Table 72.6: Stepwise Evaluation for a Patent Ductus Arteriosus

Visualize the ductus ostium and aortic isthmus from the parasternal short-axis, high parasternal short-axis, and suprasternal views
Determine the direction of shunt by color flow mapping and Doppler
Take the peak velocity of the patent ductus arteriosus (PDA) signal, which will give the pressure difference between the aorta and
pulmonary artery, and obtain the aortic, right ventricular outflow tract (RVOT), and pulmonary artery (PA) velocities
Perform measurements of the left ventricle and left atrium as these will reflect the volume of the left-to-right shunt
Specifically look for associated defects like coarctation of aorta (suprasternal view), subaortic membrane, bicuspid aortic valve,
aortic interruption, and aortopulmonary window (communication between the ascending aorta and pulmonary artery)
The length of the duct needs to be measured to

determine the choice of coil/device/stent.
This view is particularly important for Amplatzer duct
occluder II (ADO II) devices as it may give a fair estimate of
the waist of the duct to decide for the device size. It is again
best determined by the modified ductal view.
In patients (older child, adolescent, and adults) where
it is not possible to evaluate detailed anatomy of the duct
because of inadequate windows, the size of the duct can be
determined by the narrowest width of the color flow across
the duct. This, however, always overestimates the ductal
size and gives only a rough estimate. In all cases where
nonsurgical intervention is planned, these measurements
performed by echocardiography will need to be confirmed
Fig. 72.52: Two-dimensional echocardiography in high parasternal
by the gold standard, that is, angiography.
view (ductal view) with color flow mapping showing patent ductus
arteriosus (PDA; arrow) with left-to-right shunt. (Ao: Aorta; LPA: Duct Morphology
Left pulmonary artery; PDA: Patent ductus arteriosus).
Usual Ductus
(3) Modified suprasternal long-axis view (Ductal view)— The usual ductus arises from the descending aorta just
This is a less well-described view to visualize the usual below the origin of the third branch of the aortic arch (left
duct. It has the advantage of visualizing the duct in its entire subclavian artery with left aortic arch) and inserts into
length and most closely mimics the lateral angiogram the PA immediately to the left of PA bifurcation. This type
performed during cardiac catheterization. From the usual of ductus is best defined from the ductal and modified
suprasternal long-axis view, the transducer is rotated suprasternal views. It narrows at the PA end and is of
anticlockwise. A slight anterior tilt then shows the duct “funnel shape,” has a straighter course, and the Doppler
from its ampullary part to its insertion and accurate signals can be very well aligned from the ductal view. For
measurements of the ductus size can be made. catheter interventions like ductal coiling/device closure
or ductal stenting, these ducti are easy to cannulate by the
Characteristics of the Ductus femoral artery route. Other uncommon configurations of
ductus include the following:
Size of Duct • Short duct with narrow aortic end
Because of frequent interventions being performed on the • Tubular connection with no narrowing
duct (closure/stenting), it has become important to make • Tubular connection with multiple narrowings
various measurements of the duct by echocardiography. • Calcified PDA
These include the following. • Aneurysm of the aortic end of the PDA.
Aneurysm of ductus arteriosus at the aortic end is a
Size of the Narrowest Part of the Duct rare complication in adult patients with ductus arteriosus
This should be done at the narrowest point (as this site and can be profiled from the ductal view.
would be hemodynamically most restrictive). In the
majority of cases, this would be at the site of PA insertion. Vertical Duct
In the usual duct, this can be accurately measured in the A vertical duct arises from the undersurface of the aortic
ductal view or the modified arch view. arch, has a tortuous course, and is commonly seen in
patients with VSD and pulmonary atresia because of
Size of the Ampulla of Duct in utero flow from the aorta to the pulmonary arteries.
To the interventionist, the size of the ampulla is important The best view to define a vertical duct is suprasternal
to determine the possibilities of coil placement. The amp- long-axis view. This view shows the duct arising from the
ulla can be best measured in the modified ductal view. undersurface of arch and having a “S” curve. Because
of the double curve, it is not possible to visualize the appears as predominantly red flow with minimal aliasing.
aortic and pulmonary ends in the same view. Anterior In patients with severe pulmonary arterial hypertension,
angulation of the transducer will show the PA insertion a bidirectional shunt is visualized on CFI. With supra-
of the duct. These ducti are difficult to cannulate from the systemic PA pressure as in duct-dependent systemic blood
femoral artery route and may have to be accessed from the flow, a turbulent high velocity right-to-left flow in systole
ascending aorta or from the upper limb arteries. and diastole in the descending aorta is observed. This can
mimic coarctation of the aorta.
Subclavian Origin
Subclavian origin of ductus occurs with duct-dependent Continuous Wave Doppler Examination of
pulmonary blood flow with right aortic arch. The Ductus Arteriosus
suprasternal view defines best this ductus. It has a With the use of continuous wave Doppler, the direction of
straighter course; hence the Doppler alignment is good. shunt in relation to cardiac cycle and pulmonary arterial
For catheter intervention, it is easy to cannulate this type pressure (systolic BP minus peak pressure gradient
of ductus arteriosus from the femoral arterial route. across duct = systolic pulmonary arterial pressure) can be
assessed. Systemic diastolic pressure minus the pressure
Hemodynamic Significance derived from the diastolic velocity of PDA signal gives the
Hemodynamic significance of ductus arteriosus can be diastolic pressure in the PA. With an isolated left-to-right
assessed by evidence of volume overload of LA and LV, shunt, and a small to moderate-sized duct normal or mildly
direction of shunt, and pulmonary arterial pressure. elevated PA pressures are seen. Doppler examination of
such a duct shows continuous flow toward the transducer
with the peak in late systole. A large duct with pulmonary
Chamber Dimensions
arterial hypertension will show bidirectional shunting
Left atrial enlargement signifies increased pulmonary on Doppler imaging of the duct, right to left in systole
venous return because of left-to-right ductal shunting. The and left to right in diastole. With increasing PVR and no
ratio of the LA to aorta (Ao) is measured at the level of the step-up in oxygen saturation in the PA, the peak of right-
aortic valve (the LA: Ao ratio) by M-mode echocardiography to-left shunt appears early in systole. With further rise
in PLAX view. The aortic root does not enlarge significantly in PVR, right-to-left shunt begins in systole extending to
with even an extremely large PDA. In general, LA: Ao ratio diastole. With duct-dependent systemic circulation and
> 1.3:1 indicates a significant shunt. The LV will enlarge severe pulmonary arterial hypertension, only right-to-left
as cardiac output increases with increased pulmonary shunting across the duct is visualized.
venous return. The best method to determine the presence
of volume overload of the LV is M-mode measurement of Evidence of Aortic Runoff
left ventricular diastolic dimension and comparing it with
(Figs 72.53A and B)
normal values for the patient’s age and weight.
Presence of aortic run off in a patient with a ductus is
indicative of low pulmonary diastolic pressure and blood
Direction of Shunt and Pulmonary
flow from aorta to PA occurs in diastole, which can be
Arterial Pressure31–33 detected by M-mode color flow mapping.
Thus, color flow mapping shows flow reversal in the
Color Doppler Imaging of Duct descending aorta in diastole up to the level of the duct. On
Color flow mapping increases the sensitivity of detection continuous wave Doppler examination of the descending
of a ductus. This includes a tiny duct, which may not be aorta from the suprasternal long-axis view below the
seen on 2D imaging and adolescent or adult patients ductus, forward flow signals in systole (below the baseline)
where absence of good windows (especially ductal view) and reverse signals in diastole (above the baseline) are
prevents visualization of the duct on 2D imaging. On color noted. The reverse signals indicate flow from descending
flow mapping, a small duct with normal PA pressure is aorta to PA. With the Doppler sample volume placed above
displayed as a mosaic flow from descending aorta to PA. the level of the duct, flow will be in a forward direction in
With a large duct, and low PVR, the duct jet generally both systole and diastole.
A B
Figs 72.53A and B: (A) Two-dimensional echocardiography in suprasternal view showing diastolic flow reversal in the arch of aorta; (B)
Same view with cursor across the flow on M-mode showing pan-diastolic flow reversal. (D. Ao: Descending aorta; TA: Transverse arch).
Limitations of Echocardiographic type A interruption of the aortic arch,36,39–41 TOF,39–42 and

anomalous origin of the right or LCA from the PA and right
Imaging of the Duct aortic arch.39,43,44 More rarely, it is associated with VSD,44–46
There are several limitations of profilation of a duct by 2D pulmonary39 or aortic atresia, d-transposition,47 and
imaging. tricuspid atresia.48
• Limited acoustic windows: The duct is profiled in the
direction of lateral resolution of the transducer, hence Types
it is difficult to visualize with certainty a very small
duct in small babies. A high frequency probe with an APW is classified into three types:37
excellent lateral resolution is helpful. Type I: Defect between semilunar valves and PA bifur-
• If the duct is long and tortuous, it may be difficult to cation.
profile the whole length of the duct. Type II: Distal type defect involving origin of right PA.
• Poor acoustic windows in adolescent and adult Type III: Large defect involving both type I and type II.
patients, deformity, hyperinflated lungs, very obese Echocardiography: Objectives are:
children. • Diagnosis
• Type of APW
Limitations in Hemodynamics • Associated heart defects
• Operability
The long length of a PDA results in underestimation of 2D echocardiography usually can accurately diagnose
pressure gradients across it. the aortopulmonary septal defect. Views most useful
for profiling an APW are subcostal coronal view, five-
AORTOPULMONARY WINDOW chamber view, PSAX plane at the level of great vessels,
and the suprasternal views. In all these views, the wall
(FIGS 72.54A AND B)
separating the aorta and PA is aligned in the direction of
Aortopulmonary window (APW) or aortopulmonary septal lateral resolution, so great care is needed to differentiate
defect accounts for 0.2 to 0.6% of patients with congenital a true defect from an artifactual dropout. A “T” artifact at
heart defects.34 Nearly half of all patients have associated the edges of the defect will distinguish it from a normal
cardiac lesions, including aortic origin of the right PA,35–39 dropout and CFI will confirm the defect.
A B
Figs 72.54A and B: Two-dimensional echocardiography with color comparison in parasternal short-axis view showing a large aortopul-
monary window (arrow). (Ao: Aorta; PA: Pulmonary artery).
A B
Figs 72.55A and B: Two-dimensional echocardiography with color mapping showing the Gerbode defect (arrow) with left-to-right shunt.
(Ao: Aorta; RA: Right artium; LV: Left ventricle).
Color Flow Mapping GERBODE DEFECT

Color flow mapping is used to demonstrate flow through (FIGS 72.55 AND 72.56)
the defect. With large defects, which usually is the case, In this entity, the shunt occurs from LV to RA through a
the flow appears laminar, low velocity, and bidirectional defect in the membranous ventricular septum and essen-
across the defect, and with smaller defects, a continuous tially leading to volume overload of the RA and the RV.
high velocity left-to-right jet is usually present. With low In these cases, RA dimensions and features of right
PVR, evidence of aortic runoff can be detected in both atrial and ventricular volume overload need to be
ascending and descending aorta in contrast to PDA in evaluated. The features are essentially same as ASD.
which normal aortic flow is seen in ascending aorta and The point to remember is that the pressure difference
the arch. The operability will depend on the degree of across the tricuspid valve taken in these circumstances
left-to-right shunt as assessed on color flow mapping, may be fallacious as one may mistake the high velocity LV
and the presence of left atrial and left ventricular to RA jet for the tricuspid regurgitation velocity resulting in
enlargement. an erroneous diagnosis of severe PA hypertension.
A B
Figs 72.56A and B: Two-dimensional echocardiography with color comparison in parasternal short-axis view showing Gerbode defect
(arrow) with left-to-right shunt (arrow). (Ao: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle).
PART 3: ATRIOVENTRICULAR SEPTAL DEFECTS
ATRIOVENTRICULAR SEPTAL DEFECTS long-axis view of LVOT defines the “goose neck” deformity
of LVOT (long LVOT with anterior position of the aorta;
Atrioventricular septal defects (AVSDs) account for 4 to Fig. 72.60). Apical four-chamber view will display the inlet
5% of CHD and an estimated occurrence of 0.19 in 1,000 VSD with loss of offsetting (Fig. 72.59). PSAX view shows
live births.49,50 About 40 to 45% of children with Down the trileaflet left AV valve, presence of cleft; presence
syndrome have CHD, and among these, approximately of common AV junction, and abnormal position of the
40% have an AVSD, usually the complete form.49 Complete papillary muscles in the LV. PLAX view shows discrepancy
AVSD also occurs in patients with heterotaxy syndromes in the left ventricular inflow and outflow measurements
(more common with asplenia than with polysplenia). and presence of LVOT obstruction (Fig. 72.58).
Common atrium has been associated with Ellis–van
Creveld Syndrome. The assessment of the AV junction is Types Of Atrioventricular
readily achieved by 2D echocardiography and since AVSDs
are primarily an abnormality of this region, delineation of
Septal Defect
detailed morphology is possible by this technique. Color Partial Atrioventricular
flow Doppler interrogation complements by demonstra-
Septal Defect (Fig. 72.61A)51–56,59,60
ting the sites of intracardiac shunting and AV regurgitation,
as well as defining any obstruction in the LVOT if present. Subcostal coronal and sagittal views supplemented with
Pulsed and continuous wave Doppler are used to assess apical four-chamber, PLAX and short-axis views can
PA pressure and severity of LVOT obstruction.51–58 define the anatomy with great accuracy. In addition, AV
The following basic views can define these anatomical valves need to be profiled in subcostal en face view by
features (Table 72.7). rotating the transducer 30 to 45° clockwise from subcostal
Subcostal coronal view shows the common AV junc- four-chamber view. From this view, with tilting the plane
tion, loss of offsetting of AV valves, scooped out inlet from anterior to posterior, all five leaflets, separate AV
septum, and inferior bridging leaflet of AV valve (Fig. 72.57). valve orifices, attachment of anterosuperior bridging
Subcostal sagittal view shows the common AV junction, leaflet to anterior muscular septum, and posteroinferior
in addition to both superior and inferior bridging leaflets septum to inlet septum can be defined. The opening of
and anterior unwedged position of aorta. Subcostal AV valves is seen as two separate openings created by the
Table 72.7: Common Features on Echocardiography in a Case of Atrioventricular Septal Defect

Loss of offsetting of atrioventricular valves
Deficiency of inlet portion of ventricular septum
Presence of common atrioventricular valve junction
Abnormal morphology of atrioventricular valve leaflets
Abnormal position of papillary muscles of left ventricle
Longer left ventricular outflow, and anterior unwedged position of aorta
Fig. 72.57: Two-dimensional echocardiography. Subcostal para- Fig. 72.58: Two-dimensional echocardiography. Parasternal
coronal view with anterior tilt showing the components of the com- long-axis view from an infant with complete atrioventricular septal
mon atrioventricular (AV) valve. (Ao: Aorta; IBL: Inferior bridging defect showing discrepancy in left ventricular inflow (line-a) and
leaflet; LV: Left ventricle; RV: Right ventricle; SBL: Superior bridg- outflow (line-b) measurements. (Ao: Aorta; LA: Left atrium; LV: Left
ing leaflet). ventricle; RV: Right ventricle).
Fig. 72.59: Two-dimensional echocardiography in apical four- Fig. 72.60: Subcostal apical four-chamber view with anterior tilt in
chamber view showing large inlet ventricular septal defect (VSD; diastole showing “goose neck deformity” of left ventricular outflow
arrow) in a case of atrioventricular septal defect. (LA: Left atrium; tract, that is, left ventricular outflow tract is elongated with anterior
LV: Left ventricle; RA: Right atrium; RV: Right ventricle). unwedged position of aorta (arrow). (Ao: Aorta; LV: Left ventricle;
A B
Figs 72.61A and B: Two-dimensional echocardiography. (A) Apical four-chamber view showing a large ostium primum atrial septal
defect (ASD; arrow), no ventricular septal defect (VSD), lack of offsetting of the atrioventricular (AV) valves (arrow); (B) Apical four-
chamber view showing a large ostium primum ASD and a large inlet VSD. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
presence of a bridging tongue of tissue. The characteristic apposition between superior and inferior bridging leaflets)
features of partial AVSD are two separate AV orifices within is seen toward the ventricular septum to differentiate this
the common AV junction, abnormal valve leaflets with anomaly from an isolated cleft of mitral valve, where the
or without cleft, and usually presence of ASD between cleft will be oriented toward the LVOT (Fig. 72.66). Also,
lower part of atrial septum and the crest of the ventricular other features of AVSD such as a longer LVOT, unwedged
septum. Leaflets are attached to the crest of ventricular and anterior position of aorta as described earlier will be
septum, so there will be loss of offsetting and usually there present. Less common variant is common atrium (virtual
is no VSD or it is restrictive, although this is not a universal absence of atrial septum), usually found in the setting of
finding. Rarely, only inlet VSD and intact interatrial septum left or right isomerism. Associated anomaly of AV valves
is present when the bridging leaflets are attached to the such as a dual orifice AV valve with or without stenosis
lower part of the interatrial septum. VSD can be profiled and Ebstein’s anomaly of right AV valve can sometimes be
from subcostal coronal and sagittal, apical four-chamber, present and should be looked for on 2D echocardiography
and PLAX with posterior tilt and short-axis views. Here the (Table 72.8).
trileaflet left AV valve guarding the left component of the
common AV junction, seen in PSAX and subcostal en face
Complete Atrioventricular Septal Defect
views, will be the hallmark feature to differentiate it from
an isolated inlet muscular VSD. Rarely, a left AV valve with (Atrioventricular Septal Defect with Common
three leaflets may be the only manifestation of an AVSD Valvular Orifice
with intact atrial and ventricular septum. There may be a (Fig. 72.61B)53–56,61–63
cleft of only left or right AV valve (more commonly of left
side). The cleft can be profiled in the subcostal en face view The typical echocardiographic features of complete
and PLAX and PSAX views. In PSAX view, the cleft (zone of AVSD seen in subcostal coronal and sagittal, and apical
Table 72.8: While Evaluating a Patient with Atrioventricular Septal Defect, the Following Needs to be Addressed
Type of defect—partial or complete atrioventricular septal defect
Extent of atrial shunting
Extent of ventricular shunting
Presence and degree of atrioventricular valve regurgitation
Commitment of atrioventricular valves to respective ventricles, is there balanced atrioventricular connection or unbalanced
atrioventricular connection, degree of ventricular hypoplasia if present
Presence of straddling
Potential for left or right ventricular outflow obstruction
Pulmonary artery pressures
Associated lesions
four-chamber views are ostium primum ASD, common Commitment of atrioventricular valve to ventricle and
AV valve guarding the common junction, and an inlet relationship of atrioventricular valve leaflets to the septal
interventricular communication of variable size. The VSD structure—balanced or unbalanced atrioventricular
can be small or large depending upon the attachment of the septal defect: Apical four-chamber, subcostal coronal, and
bridging leaflets. As described earlier, the inferior bridging subcostal en face views are required to profile commitment
leaflet is seen best in subcostal coronal view, and it may of AV valves to respective ventricles, and to look for presence
be firmly attached by a midline raphae to the septum; as of overriding. These views allow simultaneous visualization
a result, there will be no interventricular communication of all four chambers, AV valves, and atrial and ventricular
close to crux. The superior bridging leaflet is seen in septa. If the AV junction is shared equally, then there is a
apical four-chamber view with anterior tilt and most of balanced AV connection. When there is overriding of AV
the variations in ventricular component of shunting are valve to one of the ventricles and malalignment between
seen beneath the superior bridging leaflet. The subcostal atrial and ventricular septum, then the condition is termed
en face view shows both superior and inferior bridging as unbalanced AV connection leading to hypoplasia of left
leaflets. When the superior bridging leaflet is attached
or RV depending upon the degree of overriding (Figs 72.62
firmly to the septal crest, there is no defect beneath it;
and 72.63). One of the AV valves can be atretic, causing
more commonly, this leaflet is attached to a normally
hypoplasia of the respective ventricle. Abnormal relation
positioned medial papillary muscle and is attached by
between atria and ventricles can also occur when the
multiple cords to the crest of the septum. There are then
common AV junction is not equally shared between both
multiple interventricular communications through the
the ventricles but is committed exclusively to one or the
intercordal spaces, and the flow can be recognized on color
other atrium. This condition is termed as double outlet
flow mapping. This type of defect is called as Rastelli type
“A” under Rastelli classification. In the so-called Rastelli atrium or uniatrial biventricular AV connection and can
type B, the RV medial papillary muscle is positioned in be defined in subcostal and apical four-chamber views.64–66
midseptal position, the degree of bridging is greater, and Straddling of AV valves is also an issue that needs to
the bridging leaflet is less well attached to the ventricular be defined. Straddling of left AV valve is profiled in PLAX
septal crest, and becomes free-floating. When the papillary view while for right AV valve, the four-chamber view and
muscle is located still further in the RV, a so-called Rastelli subcostal en face view are required to profile the chordal
type C defect is produced. In this situation, almost always a attachments.
large VSD is present and is particularly frequent in Down’s Color flow mapping is required to define presence and
syndrome. direction of shunting across interatrial or interventricular
Rarely with complete AVSD, there will be absence of septum, presence of AV valve regurgitation or stenosis, and
any interatrial shunt when the superior bridging leaflet is presence of left or RVOT obstruction. Direction of shunt
attached to the lower end of atrial septum, and absence of across ASD or VSD can be profiled from views used to define
VSD when it is firmly attached to the ventricular septum as the defects. Subcostal, coronal, apical four-chamber, and
described with a partial AVSD. PSAX views are required to look for presence of AV valve
A B
Figs 72.62A and B: Two-dimensional echocardiography. Apical four-chamber view showing unbalanced atrioventricular canal defect
with left ventricle (LV) dominance (A) and right ventricle (RV) dominance (B). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
regurgitation, presence of LV–right atrial shunt, or RV— septum, infundibular hypertrophy), valvular, or supraval-
left atrial shunt. RV—left atrial shunt could be a cause of vular level.
cyanosis in a child with partial AV canal defect with normal
Complex atrioventricular septal defect: A complex AVSD
PA pressure. The quantitative assessment of valvular
can be defined as an AVSD morphology that precludes
stenosis is not accurate by Doppler echocardiography
two-ventricle correction.66-69 The following conditions can
when there is a large ASD. So it is important to evaluate
be the cause of such a situation:
valve anatomy by 2D echocardiography and look especially
• The most frequent is the association of AVSD with het-
for dysplasia, tethering of leaflets, and valve orifice. In such
erotaxy/isomerism. Anomalous systemic/pulmonary
cases, valve stenosis may manifest after closure of the ASD
venous connection and hypoplasia of ventricles fre-
if not addressed at surgery.
quently precludes biventricular correction.
The outflow tract of both LV and RV should be assessed,
as subvalvular obstruction of any of the outflows can occur. • Abnormalities of ventricular arterial connections.
• AVSD can be associated with DORV, making it difficult
Left ventricular outflow tract obstruction (Table 72.9):62–68 or impossible to route the LV to aorta as the VSD, thus
LVOT is longer and narrower than normal in AVSD,
precluding a two-ventricle repair.
although in most cases there is no overt stenosis. Any
• Right/left ventricular dominant AVSD.
factor that causes further narrowing of LVOT causes LVOT
Because of extreme straddling/overriding of the
obstruction. Causes of LVOT obstruction are highlighted
common AV valves across the VSD, one of the ventricles
in Table 72.9.
may be hypoplastic. This will prevent two-ventricle repair.
Right ventricular outflow tract obstruction: Pulmonary In most cases, quantification of hypoplasia is subjective,
stenosis can occur at subvalvular (malalignment of outlet based on echocardiography (Figs 72.62A and B).
Table 72.9: Left Ventricular Outflow Tract Obstruction in Atrioventricular Septal Defect
Tight adherence of superior bridging leaflet to septal crest causing left ventricular outflow tract to be longer and narrower. Left
ventricular outflow tract obstruction is more common in partial atrioventricular septal defect and with primum ASD
Discrete subaortic membrane
Ventricular septal hypertrophy
Abnormal chordal attachment of superior bridging leaflet
Prominent anterolateral muscle bundle
Left ventricular outflow tract can be profiled from subcostal coronal view with anterior tilt, subcostal sagittal view, and paraster-
nal long-axis view. Color flow mapping shows turbulence beginning in subaortic area
Careful Doppler interrogation shows site of obstruction and severity of obstruction of left ventricle outflow tract
A B
C D
Figs 72.63A to D: (A) Subcostal en face view profiling the common atrioventricular (AV) valve completely; (B) Tracing the common
AV valve orifice during end diastole, averaged over three cardiac cycles (area B); (C) This circumference is then divided by a line drawn
over the interventricular septum from the tip of the infundibular septum to the crest of the muscular septum, thus dividing the AV valve
into left and right components (D) Take the area of the left component. For a balanced atrioventricular septal defect, AVVI (“A” area/“B”
area in figure) should be > 0.67.
The area of the common AV valve and left component can Complete atrioventricular septal defect: This is asso-
be measured and the ratio calculated as shown in Figs ciated with large VSD and pulmonary arterial hyper-
72.63 A to D to quantitate the hypoplasia. tension. Thus, congestive heart failure develops in
Associated defects: TOF and DORV with malposed great the first few months of life. Also, rapid progression
vessels are frequently associated with complete AVSD, (6 months of life) of pulmonary vascular disease occurs
in this condition. Thus, there is an urgent need to correct
especially in the setting of isomerism.69 With unbalanced
these lesions early in life. If correction is performed at
AV connection leading to hypoplastic LV, aortic arch should
the appropriate age, then echocardiography alone is
be carefully profiled in the suprasternal long-axis view to
enough for assessment of this lesion as the morphology
rule out arch anomalies like CoA and arch interruption.
is well delineated by this technique and there is no need
With hypoplastic RV, pulmonary stenosis and pulmonary
for invasive determination of PA pressure and vascular
atresia may be associated and need to be profiled carefully.
resistance. Late presentation, however, may need more
detailed evaluation with cardiac catheterization.
Hemodynamic Assessment of
Atrioventricular Septal Defect With Pulmonary Stenosis
Patients with pulmonary stenosis present as TOF.
Without Pulmonary Stenosis However, the morphology is much more complicated. The
Partial atrioventricular septal defect: These defects VSD, which is predominantly of the inlet type, also extends
behave like an ASD. In the absence of significant AV into the outlet septum. Anterior malalignment of the
valve regurgitation and normal PA pressure, the lesion outlet septum causes RV outflow obstruction and other
is well tolerated and patients may present late like fossa morphological abnormalities associated with TOF.
ovalis ASDs. Significant AV valve regurgitation, however,
may cause early congestive heart failure. Accurate Mechanism of Atrioventricular
assessment of PA pressure and AV valve regurgitation is
critical in decision-making for timing of surgery. Doppler
Valve Regurgitation
assessment of PA pressure is usually performed by In the majority of cases, AV valve regurgitation occurs
assessing tricuspid regurgitation velocity. Care should be through the cleft in the left AV valve. This is well-appre-
taken in not confusing LV–right atrial shunt for tricuspid ciated in the PSAX views and subcostal paracoronal
regurgitation as the former (LV–right atrial shunt) will (en face) view. Regurgitation can also occur through the
invariably produce high velocity signals which will not be commissures of the left AV valve or through the right
reflecting pulmonary arterial pressures. AV valve.
PART 4: CONGENITAL LEFT VENTRICULAR AND RIGHT

VENTRICULAR INFLOW ANOMALIES
CONGENITAL ANOMALIES OF MITRAL Echocardiographic Views to Define

VALVE (TABLE 72.10)70 Mitral Valve Lesions
Mitral stenosis or mitral regurgitation forms the predomi- Mitral valve is best visualized in parasternal long-axis,
nant manifestation of mitral valve anomalies. Other as- apical four-chamber and two-chamber views, and in PSAX
sociated cardiac anomalies are present in 90% of them. view. Addition of color Doppler further helps to diagnose
Echocardiography can assess the degree of severity of the the abnormalities. PLAX view shows the motion of mitral
lesion and define the varied anatomy preoperatively for valve leaflets and any evidence of doming or prolapse can
planning the surgery. It is useful intraoperatively to guide be noted. The chordal length, chordal thickening, and
the surgery and postoperatively to assess the outcome.70–74 chordal insertion are also well seen in this view. Additional
Table 72.10: Common Mitral Valve Abnormalities

Supramitral membrane or ring
Hypoplasia of the mitral apparatus
Dysplasia of the mitral valve
Parachute mitral valve
Cleft mitral leaflet (anterior or posterior)
Abnormal mitral arcade
Double orifice mitral valve
Accessory mitral valve tissue/orifice
Ebstein’s anomaly of the mitral valve
Mitral valve prolapse
Mitral regurgitation secondary to other causes, some of which
are congenital
• Infective Fig. 72.64: Two-dimensional echocardiography in subcostal
– Myocarditis short-axis view with color flow mapping from a child with partial
atrioventricular septal defect showing left atrioventricular valve
– Kawasaki disease
regurgitation through the cleft (arrow). (LV: Left ventricle; RV:
– Infective endocarditis Right ventricle).
• Rheumatic heart disease
– Mitral regurgitation and mitral stenosis with stenosis, to see the level of stenosis. Doppler is also
• Papillary muscle dysfunction useful in estimating gradients and valve area. Gradients
– Ischemia by Doppler may be underestimated due to associated
– Anomalous origin of the left coronary artery from the interatrial defect or due to poor alignment secondary to
pulmonary artery multiple levels of obstruction to left ventricular inflow.
• Cardiomyopathy Pressure half-time method is often not reliable in this
– Dilated cardiomyopathy setting. Planimetry of the mitral valve orifice in PSAX view
– Hypertrophic cardiomyopathy
may be the best method for assessment of mitral valve
area.
– Storage disease/infiltration
The normal mitral valve area is 2.4 to 3.6 cm2/m2. In
– Hurler disease
mild mitral stenosis, the valve area is reduced to 1.2 to 2.4
– Amyloidosis
cm2/m2; in moderate mitral stenosis, mitral valve area is
• Connective tissue disease 0.6 to 1.2 cm2/m2; and in severe mitral stenosis, the valve
– Marfan's syndrome area is < 0.6 cm2/m2.
– Ehlers–Danlos syndrome, etc.
The Echocardiographic Evaluation of
abnormalities of LVOT like subaortic membrane, tubular
Individual Mitral Lesions
narrowing, and so on can be assessed. Apical four-chamber
view shows ventricular inflow region and any obstructive Supramitral Ring or Membrane75,76
membrane or ring in the atrium, like a supramitral ring.
The valve annuli can be measured. Apical four-chamber
(Figs 72.68 and 72.69)
view is good for showing dilatation of atria, which may It is a circumferential ridge of fibrous tissue on the atrial
occur secondary to atrioventricular valve stenosis surface of mitral valve attached to the base of the atrial
and/or regurgitation. PSAX view shows orientation of surface of mitral leaflets. True incidence, although not
commissures, chordae, and papillary muscles including well described, varies from 9 to 20% of reported cases of
the number of papillary muscles. Cleft mitral valve congenital mitral stenosis. In approximately 4% of cases, it
(Fig. 72.64) and double orifice mitral valve are also best is an isolated anomaly. The opening of the central orifice
diagnosed in this view. Addition of color flow mapping is of the shelf-like projection decides the severity of mitral
necessary for quantifying the regurgitation and in cases obstruction.
Echocardiographically, supramitral ring (Figs 72.65A The second variety is difficult to image in real time
and B) is well seen in parasternal long- and short-axis and is best visualized by slow frame-by-frame playback
views, and apical and subcostal four-chamber views. There examination of a held image. Majority of supramitral
are two variants of supramitral ring: rings are nonobstructive. The obstruction is produced
• Small separation between mitral annulus and the ring when their central lumen is small or when associated
during diastole—supra-annular variant. with a small mitral annulus, fusion of valve commissures,
• Membrane firmly adhered to the mitral leaflets— parachute mitral valve, or accessory mitral tissue.
annular variant. Color Doppler examination shows the site of actual
obstruction by turbulent flow and spectral Doppler
interrogation will provide the gradient across the mitral
valve. Supramitral ring needs to be differentiated from
cor triatriatum sinistrum (Figs 72.66 and 72.67). In cor
triatriatum sinistrum, LA is divided into two chambers—
superior and inferior by an abnormal diaphragm. The
superior chamber receives pulmonary veins and inferior
chamber communicates with left atrial appendage and
mitral inflow. With supramitral ring, the stenosing ring
is located much closer to mitral valve, lying between
the mitral valve and left atrial appendage; another
differentiating feature between the two is the movement of
the diaphragm during systole and diastole. The best views
to detect cor triatriatum are apical four-chamber, subcostal
A B coronal, and PLAX views. In apical four-chamber view,
the shelf is seen horizontally. The side attachment is to
Figs 72.65A and B: (A) Two-dimensional echocardiography in
apical four-chamber view showing annular type of supramitral atrial septum and on left side to lateral wall of LA above
membrane; (B) Color flow mapping of the same showing turbu- the left atrial appendage. In PLAX view, the shelf stretches
lence starting from the supravalvular membrane. (LA: Left atrium; superiorly to the posterior aortic root and inferiorly to
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
posterior left atrial wall. Color flow mapping shows that
Fig. 72.66: Two-dimensional (2D) echocardiography from an in- Fig. 72.67: Two-dimensional echocardiography. Apical four-cham-
fant with cor triatriatum. Zoomed up apical four-chamber view on ber view with color flow mapping showing turbulence (arrow) in
2D echocardiography, showing a shelf in left atrium stretching a case of cor triatriatum. (LA: Left atrium; LV: Left ventricle; RA:
from atrial septum on the right side to lateral wall of left atrium on Right atrium; RV: Right ventricle).
the left with a narrow communication (arrow). (LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).
with cor triatriatum, turbulence starts in mid left atrial Parachute Mitral Valve87,89
cavity, but with supramitral ring, mosaic jet forms at just
above the mitral valve. In this anomaly, all chordae tendineae of the mitral leaflets
Supramitral ring is known to be associated with CoA, are attached to a single papillary muscle. The anterolateral
VSD, double-outlet RV, TOF, subaortic stenosis, bicuspid papillary muscle is usually absent. Sometimes, there may
aortic valve, valvular aortic stenosis, complete AVSD, be two papillary muscles adjacent to each other, producing
PDA, bicuspid pulmonary valve, ASD, abnormal tricuspid a functional single papillary muscle.
valve, persistent LSVC draining into coronary sinus, partial Echocardiographically, papillary muscles are best
evaluated in PSAX view. Parachute mitral valve results
anomalous pulmonary venous connection (PAPVC),
in mitral stenosis due to reduced interchordal spaces.
endocardial fibroelastosis, double aortic arch, hypoplastic
Chordae may also be short and thickened. Severity of
LV, and coronary anomalies.
stenosis is best evaluated by measurement of gradients
across the mitral valve. This condition is often seen as part
Hypoplastic Mitral Valve77–84 of the Shone’s complex. Other associations include VSD,
A hypoplastic mitral valve is nearly always associated with double-outlet RV, and ASD.
hypoplastic left heart syndrome (HLHS) or its variant. LA
may be small in size. Mitral valve is often dysplastic with Cleft Mitral Leaflet (Fig. 72.66)89–93
a small annulus, thickened leaflets, and short chordae,
Isolated cleft in mitral valve is rare with a reported
attaching directly into the left ventricular wall. The papillary
incidence of 15% of all congenital anomalies of the mitral
muscles are poorly developed or rudimentary, with a very valve. This is one of the few lesions of mitral valve, which is
small left ventricular cavity. The LVOT including aortic readily amenable to successful surgery. Generally, the cleft
arch is very small or atretic. An ASD is mostly present is in the anterior mitral leaflet, but a cleft in the posterior
and LA is decompressed via the ASD. With restrictive mitral leaflet has been reported. This condition should be
ASD, left atrial pressure remains very high leading to differentiated from cleft in mitral valve associated with
severe pulmonary venous and arterial hypertension. partial or complete AVSD.
During echocardiography in patients with hypoplastic left The cleft extends from the free margin to the annulus
heart, mitral atresia, or severe mitral stenosis, interatrial for a variable length and divides the leaflet into two
communication should be carefully assessed for adequacy equal parts. The leaflets may be normal or may be mildly
from subcostal views. dysplastic or thickened, producing variable degree of
Echocardiographic measurements suggesting the mitral regurgitation. The parasternal short-axis of the cleft
diagnosis of HLHS include an aortic valve annulus is directed toward the LVOT. Cleft in mitral leaflet produces
< 5 mm, mitral valve annulus < 9 mm, left ventricular mitral regurgitation and color flow clearly shows the
inflow diameter < 21 mm, and end-diastolic left ventricular regurgitation originating from the cleft. The abnormality
volume of < 20 mL/m2. is best visualized in PSAX, apical four-chamber, and
subcostal short-axis views.
Dysplasia of Mitral Valve (Typical Congenital Cleft in anterior mitral leaflet is very commonly
Mitral Stenosis)77,84 associated with AVSDs. However, it is not a true cleft but
a commissure between the anterior and the posterior
This condition is characterized by thickening of leaflets bridging leaflets. The major axis of this so-called cleft in
with rolled edges, fused commissures, short chordae AVSD is directed toward the interventricular septum.
with reduced interchordal spaces and poorly developed
papillary muscles. The leaflets are thickened with limited Abnormal Mitral Arcade (Hammock
mobility and show typical doming during diastole. They
may also show nodularity on both atrial and ventricular
Mitral Valve)87–89 (Figs 72.68 and 72.69)
aspects of the valve. Echocardiography from a combination This is best seen on echocardiography from the atrial
of views, apical four-chamber, PLAX and short-axis views, side of the mitral valve in PSAX, subcostal short-axis, and
reveal the detailed anatomy of mitral valve. PLAX views. The valve has the shape of a funnel without
Severity of the obstruction can be assessed by commissures with a central orifice of variable size. Chordae
cross-sectional echocardiography along with Doppler are seen to cross the orifice, giving the appearance of a
interrogation of the gradient across the stenotic valve. hammock. Thickened papillary muscles of LV may also
Fig. 72.68: Two-dimensional echocardiography in a child with a Fig. 72.69: Two-dimensional echocardiography in a child with
hammock mitral valve showing thickened leaflets attached directly hammock mitral valve. Parasternal long-axis view showing
to the papillary muscle without intervening chordae with left atrium the hammock mitral valve with mild mitral regurgitation and
(LA) enlargement. (LA: Left atrium; LV: Left ventricle; RA: Right turbulence in left ventricular outflow tract (LVOT). (LA: Left
atrium; RV: Right ventricle). atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
A B
Figs 72.70A and B: Two-dimensional echocardiography in apical four-chamber view (A) showing double orifice mitral valve with sepa-
rate subvalvular apparatus; (B) Parasternal short-axis view at the level of the mitral valve showing the two separate orifices of equal
size. (LA: Left atrium; LV: Left ventricle).
result in partial obstruction to left ventricular inflow. Double Orifice Mitral Valve94–96
When examined from the left ventricular side in apical
or subcostal four-chamber view or apical two-chamber
(Figs 72.70A and B)
views, one can see either direct insertion of the leaflets In this condition, there are two mitral orifices with separate
into the papillary muscles or insertion through short, thick leaflets, chordae tendineae, and papillary muscles. In
chordae. Bridge of fibrous tissue adherent to the inferior 85% of cases, the orifices are of unequal size with smaller
aspect of anterior mitral leaflet may also be seen. orifice situated close to the anterolateral commissure
This abnormality usually results in both mitral stenosis in 41% and close to the posteromedial commissure in
and mitral regurgitation. Abnormal mitral arcade may be 44%. Less commonly, there is a bridge of fibrous tissue
associated with ASD, PDA, valvular and subvalvular aortic between the two leaflets making two openings. The
stenosis, and CoA. number of papillary muscles may vary from two to four.
Three varieties of double orifice mitral valve have been The reported associations include Ebstein’s anomaly of
described: an incomplete bridge type, in which a small tricuspid valve, Marfan syndrome, double-outlet RV, ASD,
strand of tissue connects the anterior and the posterior PDA, CoA, hypoplasia of ascending aorta, and valvular
leaflets at the leaflet edge level; a complete bridge type, in aortic stenosis.
which a fibrous bridge divides the atrioventricular orifice
completely into equal or unequal parts; and a hole type, Mitral Valve Prolapse98–101
in which an additional orifice with subvalvular apparatus
Mitral valve prolapse (MVP) results from myxomatous
is present in the posterior commissure of the mitral valve.
degeneration of the mitral valve, more commonly affecting
They could be distinguished by sweeping the transducer the posterior leaflet; however, anterior or both leaflets can
in cross-sectional view from the apex toward the base of be effected, which balloons into the LA during systole,
the heart. resulting in noncoaptation of mitral leaflets producing
Variable extent of mitral stenosis and regurgitation is the typical click and murmur. The mitral leaflets show
often present. The type of the defect does not predict the degenerative changes with elongated chordae. The
presence or severity of stenosis or regurgitation. Double chordae may sometimes get ruptured, producing severe
orifice mitral valve is commonly associated with partial or mitral regurgitation.
complete AVSD, CoA, aortic stenosis, PDA, VSD, and ASD.
Diagnostic criteria are as follows:
• Perloff et al. set the stage for accurately diagnosing
Accessory Mitral Orifice MVP by expanding the diagnostic standards to include
This abnormality results from a circular deficiency of clinical and echocardiographic criteria98,99
mitral leaflet tissue. The size of the orifice can vary and the • In a Framingham Heart Study, Freed et al. historically
border of the accessory orifice is usually devoid of chordae described echocardiographic criteria for MVP as
tendineae. In some cases, chordae may insert into an classic versus nonclassic (see below)100
independent papillary muscle. Accessory mitral orifice is • Use of the PLAX view increases the diagnostic accuracy
best visualized in PSAX and subcostal four-chamber view of MVP.101
with color Doppler interrogation. Apical four-chamber Findings are as follows:
view may show the abnormality of the subvalvular • Classic MVP—The PLAX view shows > 2 mm superior
apparatus. An abnormal position and orientation of a displacement of the mitral leaflets into the LA during
mitral regurgitant jet may help suspect this condition systole, with a leaflet thickness of at least 5 mm
and warrants further evaluation in different views. This • Nonclassic MVP—Displacement is > 2 mm, with a
condition is sometimes associated with transposition of maximal leaflet thickness of < 5 mm
great arteries, partial AVSD, and interrupted IVC. • Other echocardiographic findings that should be con-
sidered as criteria are leaflet thickening, redundancy,
Ebstein’s Anomaly of Mitral Valve annular dilatation, and chordal elongation.
Izumo et al. describes the superiority of using
This is a rare anomaly with very few published case three-dimensional (3D) TEE (en face view) versus 2D
reports.97 Here, the LA is dilated and the posterior leaflet TEE (commissural view) in patients with severe mitral
of mitral valve, which is dysplastic, is displaced downward regurgitation due to prolapse or flail mitral valve to assess
with normal insertion of anterior mitral leaflet into the the etiology with respect to quantification of prolapse
ventricular septum (above the septal tricuspid leaflet). Few segment and width. Based on the complex mitral valve
case reports have shown associated thin left ventricular anatomy, 2D TEE could not detect the largest prolapse gap
wall. This abnormality is best visualized in apical, subcostal and width, thus concluding 3D TEE superiority.101
four-chamber views and in PLAX view. The severity of MVP may be associated with connective tissue
mitral regurgitation can be assessed by color and spectral disorders or be idiopathic. The prolapse is best visualized
Doppler interrogation. in PLAX view, apical and subcostal four-chamber views,
Ebstein’s anomaly of mitral valve should not be and apical two-chamber view. Severity of the prolapse
confused with Ebstein’s anomaly of left atrioventricular can be graded by cross-sectional echocardiography and
valve in association with corrected transposition, where severity of regurgitation can be assessed by color Doppler.
septal leaflet of morphological tricuspid valve is apically MVP is often associated with ASD secundum and rarely
displaced. with VSD.
Table 72.11: Various Congenital Lesions of the Tricuspid Valve from a mild displacement to a severe one and produces
varying degree of low-pressure tricuspid regurgitation
Ebstein’s anomaly
and rarely stenosis of the tricuspid valve. The commissure
Tricuspid valve dysplasia
between the septal and posterior leaflet is the point of
Tricuspid valve prolapse maximal displacement. These two leaflets are mostly
Double orifice tricuspid valve dysplastic and of variable size. The RA dilates to a variable
Parachute deformity extent depending on the severity of tricuspid regurgitation.
Congenitally unguarded tricuspid orifice Tricuspid valve annulus is also enlarged. Because of the
displacement of the leaflets, the RV is divided into two
Tricuspid atresia
parts—the inlet portion of atrialized ventricle, which is
thin-walled and often aneurysmal, and the trabecular and
CONGENITAL ABNORMALITIES OF outlet portions, called functional RV.
TRICUSPID VALVE (TABLE 72.11) Few cases of Ebstein’s anomaly have an imperforate
tricuspid valve with a muscular partition between the
Apart from Ebstein’s anomaly, other congenital anomalies inlet and the trabecular portion of the RV. Presence of
of the tricuspid valve apparatus (valve annulus, valve an ASD is seen in 65 to 93% of cases and in majority, it is
leaflets, chordae tendineae, and papillary muscles) are not either a stretched patent foramen ovale or a small ostium
very common. Dysplastic valve with varied abnormalities secundum type of defect.
can occasionally be seen. Apical four-chamber view shows sail-like anterior
leaflet of tricuspid valve and is the view of choice for
Ebstein’s Anomaly of the Tricuspid assessing the degree of septal leaflet displacement, and
the apical four-chamber view with posterior tilt will
Valve102–111 (Figs 72.71A and B) profile displacement of posterior leaflet. The PLAX view
Ebstein’s anomaly is characterized by apparent apical when tilted toward RV inflow also shows the abnormally
displacement of septal and posterior tricuspid valve leaflet placed tricuspid valve. A septal leaflet displacement of >
insertion. The anterior leaflet is attached normally, but 8 mm/m2 has been found to be a sensitive indicator of the
is elongated and is “sail-like.” The displacement of septal diagnosis. Also, an absolute value of displacement of > 15
and posterior leaflets is caused by partial or complete mm in children of < 14 years, or > 20 mm in adults helps
adherence of these leaflets to the underlying myocardium. in echocardiographic diagnosis of Ebstein’s anomaly,
The incidence of this malformation varies from 0.03 to 0.6% discriminating it from the normal variations and position
of all CHDs. The disease comprises a spectrum of severity of tricuspid valve with marked right atrial enlargement.
A B
Figs 72.71A and B: Two-dimensional echocardiography in a patient with Ebstein’s anomaly of tricuspid valve. (A) Apical four-chamber
view with slight leftward tilt showing the apical displacement of the septal leaflet of tricuspid valve (arrow), (+) shows the normal site
of attachment of TV, enlarged right atrium, atrialized right ventricle, and reduced size of the functional right ventricle; (B) Apical four-
chamber view with posterior tilt (at the plane of coronary sinus) showing displaced posterior leaflet of tricuspid valve. (ARV: Atrialized
right ventricle; CS: Coronary sinus; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Anterior tricuspid leaflet, although normally attached the following ratio: RA + aRV/RV + LV + LA (RV = area of
at the tricuspid annulus, is rarely completely normal. the RV, RA = area of the RA, aRV = atrialized portion of the
Because of its sail-like nature and abnormal attachment to RV at end diastole, LA = area of the LA, LV = area of the
the ventricular wall and papillary muscle, it may cause RV LV); all the measurements are made in end diastole. These
inflow obstruction. The anterior leaflet may be joined to are graded as Grade I (ratio < 0.5), Grade II (ratio from
the posterior or septal leaflet as a hammock-like structure 0.5–0.99), Grade III (ratio from 1.0–1.49), and Grade IV
producing functional tricuspid stenosis. (ratio > 1.5), with Grade I having the best prognosis while
Ebstein’s anomaly may be associated with significant Grade IV, the worst prognosis.
RVOT dilatation and RV dysfunction. It may also be
associated with left ventricular dysfunction and variable Tricuspid Valve Prolapse111
degree for left ventricular fibrosis and hypertrophy. As an isolated anomaly, tricuspid valve prolapse is rare. It
Adequacy of functional RV determines the treatment is more commonly seen in association with MVP. There is
strategy of future single or two-ventricle repair. In a subset similar myxomatous degeneration of the tricuspid valve
of patients with Ebstein’s anomaly with small functional leaflets with thinned leaflets and elongated chordae. PLAX
RV and significant desaturation, a Glenn or Fontan type of view permits evaluation of septal and posterior leaflets.
surgery is indicated. The anterior and septal leaflets are best visualized from the
The usual Ebstein’s anomaly is always associated with PSAX and from apical and subcostal four-chamber views.
situs solitus and atrioventricular and ventriculoarterial In approximately 40 to 48% of cases, there is associated
concordance. However, Ebstein’s anomaly may involve MVP.
the left atrioventricular valve in atrioventricular and
ventriculoarterial discordance. Here, the nature of the Congenitally Unguarded
displacement and formation of the septal and posterior Tricuspid Orifice1,2,112
leaflets are similar, but the anterior leaflet is smaller and Here, the orifice between the RA and the RV is normal,
not elongated or sail-like. Also, the left atrioventricular but there is no tricuspid valve apparatus. There is either
valve regurgitation is of high pressure in comparison to complete absence of valve or only remnants of valvular
low-pressure tricuspid regurgitation in Ebstein’s anomaly tissue are present. The close differential includes dysplastic
of usual type. tricuspid valve (Figs 72.72A and B). Usually, the RA is
Associated defects in Ebstein’s anomaly are rare and dilated and the RV is hypoplastic.
include VSD, PDA, partial AVSD, pulmonary stenosis, This anomaly is best visualized in apical and subcostal
pulmonary atresia, and MVP. four-chamber views as well as in long-axis parasternal
In order to grade the severity of the Ebstein deformity inflow view. Severe low-pressure tricuspid regurgitation
using echocardiography, Celermajer et al.107 described is invariably present. Associations include pulmonary
A B
Figs 72.72A and B: Two-dimensional transthoracic echocardiography. Apical four-chamber view with color compare in a case of
severely dysplastic tricuspid valve showing markedly enlarged right atrium and severe tricuspid regurgitation. (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).
A B
Figs 72.73A to C: Two-dimensional transthoracic echocardio-

graphy in a case of Uhl’s anomaly. (A) Apical four-chamber view
showing dilated thin walled RV (arrow) and dilated tricuspid orifice
with tricuspid valve (arrow). (B) Parasternal short-axis view with
Uhl’s anomaly showing dilated right atrium with thinned out tricus-
pid valve (arrow). (C) Subcostal sagittal view of the same patient
showing thin tricuspid valve and the lack of apical trabeculations of
C right ventricle (arrow).
atresia with intact ventricular septum, ASD, PDA, double- is characterized by the apposition of the epicardium
chambered RV, cor triatriatum, VSD, Uhl’s anomaly113 and endocardium essentially because of the absence
(Figs 72.73A to C), and LSVC draining into RA. of the myocardial layer. Closest differential diagnosis is
arrythmogenic right ventricular dysplasia but Uhl's is
Uhl's Anomaly113 not familial unlike latter. Echocardiography would show
thinned out parchment-like appearance of the ventricular
Though not a disease of the tricuspid valve it is discussed wall (Figs 72.73A to C). Diastolic opening of the pulmonary
here because it mimics the presentation of Ebstein valve may be seen in some cases. MRI remains the
anomaly of TV or dysplastic TV closely. Uhl’s anomaly investigative modality of choice.
PART 5: LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
LVOT obstruction can occur at various levels: VALVULAR AORTIC STENOSIS

• Valvular aortic stenosis
• Subvalvular aortic stenosis Congenital bicuspid aortic valve occurs in 1.3% of the
• Supravalvular aortic stenosis. population114–118 and, therefore, is one of the most common
congenital heart malformations. Valvar aortic stenosis is particularly useful to define the valve motion. PSAX view
the most common type of LVOT obstruction accounting is the best view to define the number of cusps and cusp
for 70 to 91% of aortic obstructions. It is caused by cusp morphology.
deformities, either with or without narrowing of the Following variations occur in the aortic valve
“annulus.” It may manifest in the neonate or progressive morphology:
obstruction may develop in an inherently abnormal • Unicuspid aortic valve is separated pathologically
valve. into two types—acommissural and unicommissural.
The echocardiographic study of valvular aortic stenosis The acommissural valve is a rare anomaly and has a
should include the following:114-119 single membrane-like leaflet with a central circular
• Morphology of the stenotic valve orifice. The orifice is typically eccentric and circular
• Dimensions of the aortic root in systole. In diastole, an eccentrically located valve
• Severity of valvular obstruction closure is seen with raphae. Unicommissural valve is
• Left ventricular hypertrophy and function, and frequently seen in symptomatic neonates with aortic
• Associated anomalies. stenosis.In systole, the opening of the valve is eccentic
and circular while in diastole raphe is seen and valve
Morphology of Stenotic Aortic Valve closes eccentrically. The unicuspid valve is generally
stenotic in neonatal period, although occasionally it
The PSAX view at the base of the heart and long-axis view has sufficient redundancy and may be the cause of
are best views to determine the morphology and number obstruction in later life.
of cusps. • Congenital bicuspid aortic valve (Figs 72.74A and B)
Normally, the tricuspid aortic valve has three cusps occurs in about 2% of the general population. It is
of nearly equal size. Three commissures form a Y-shaped formed by the fusion of two cusps. The fusion of left
pattern in diastole. In systole, the leaflets open along these and right coronary cusps results in a bicuspid valve
commissures to create a wide-open triangular orifice. The with two cusps positioned anteroposteriorly with
congenital anomalies of aortic valve comprise a spectrum the commissures to the right and left. The fusion of
of deformities, which include a decrease or increase in right and noncoronary cusps results in two cusps
the number of valve cusps, their form, and size. Normal positioned right and left, and the two commissures
aortic valve leaflets are thin with unrestricted mobility. have an anteroposterior orientation. In some
In stenotic valves, leaflets are thickened and domed in cases, fused commissures called raphe are seen on
systole. Doming of valve leaflets during systole occurs due echocardiography and in the closed position may
to limited cusp separation leading to restricted mobility give the appearance of a tricuspid aortic valve. It is
of valve cusps. Subcostal coronal with anterior tilt, apical only in systole that the valve does not open along the
four-chamber with anterior tilt, and PLAX views are fused commissure. The development of aortic valve
A B
Figs 72.74A and B: Two-dimensional transthoracic echocardiography. (A) Parasternal short-axis view showing bicuspid aortic valve in
diastole with single closure line of fusion; (B) The same patient in systole showing the fused right and noncoronary cusps of the aortic
valve (arrows). (LA: Left atrium; RA: Right atrium; RV: Right ventricle).
A B
Figs 72.75A and B: (A) M-mode cut across the aortic valve and left atrium showing eccentric closure of the thickened aortic valve; (B)
Showing the same across the normal valve for comparison.
In PLAX view, an abnormal eccentric coaptation

line (best seen on M-mode) with systolic leaflet
doming and an abnormal pattern of systolic
opening is seen (Figs 72.75A and B).
– Although there is no fixed pattern of coronary
artery origin with a bicuspid aortic valve, usually
the coronary arteries emerge from the anterior
sinus in case of anterior and posterior cusps with
normally related great vessels. In presence of right
and left cusps, LCA arises from anterior part of left
sinus, and right coronary from anterior part of right
sinus.
• Congenitally stenotic tricuspid aortic valve has three
aortic cusps. The edges of the cusps are rolled or
Fig. 72.76: Two-dimensional echocardiography with parasternal gnarled with varying degrees of commissural fusion.
short-axis view showing a quadricuspid aortic valve. (Ao: Aorta; This abnormality is often associated with a narrowed
LA: Left atrium; RA: Right atrium; RV: Right ventricle).
aortic annulus.
stenosis is variable and may be related to valvular • Quadricuspid aortic valve (Fig. 72.76) is a very rare
characteristics. Patients with anteroposteriorly (as (0.013%) congenital anomaly. No correlation has been
opposed to right-left) and eccentric (vs symmetric) found between anatomical variation in the size of cusps
valve leaflets have faster rate of progression of aortic and functional status. Although aortic stenosis is rare,
obstruction. Patients with fusion of right coronary and approximately 50% of cases have aortic insufficiency.
noncoronary leaflets are more likely to have aortic Aortic regurgitation is more common with a small
regurgitation. Combination of bicuspid aortic valve accessory cusp.
and aortic coarctation is usually associated with milder – In the PSAX view, four diastolic closure lines are
aortic valve disease. present forming a characteristic “X” pattern, and
– The echocardiographic diagnosis of a bicuspid in systole, four cusps open and form a rectangular
aortic valve is based on demonstration of two cusps configuration. Color flow mapping will show
and two commissures on PSAX view. Additional presence of aortic regurgitation if present.
features that support the diagnosis include leaflet Pentacuspid aortic valve and hexacuspid aortic valves
redundancy, infolding, and eccentric valve closure. have been described in case reports.
Sinotubular Junction
Sinotubular junction is the point of the union of the aortic
sinuses and the tubular portion of the ascending aorta.
The apex of the aortic valve commissures correspond to
the sinotubular junction.
PLAX view is the best view to measure the aortic
valve annulus, the thickness and mobility of the leaflets,
the plane of the valvular orifice, the sinuses of Valsalva,
the sinotubular junction, and the proximal portion of the
ascending aorta.
Severity of Aortic Stenosis119–121

Direct quantitative assessment of the severity of aortic
long-axis view of left ventricular outflow showing measurements. valvular stenosis can be obtained using Doppler
(A: Annulus; B: Aortic sinuses; C: Sinotubular junction; LA: Left echocardiography. At the same time, one should also
atrium; LV: Left ventricle; RV: Right ventricle). look for associated left ventricular hypertrophy and left
ventricular diastolic and systolic dysfunction.
Normal aortic valve blood flow is laminar and peak
Aortic Root (Fig. 72.77) systolic velocity of blood flow across the aortic valve rarely
exceeds 1.5 m/s. In aortic valve stenosis, the LV generates
The aortic root is the portion of the ventricular outflow high pressures to overcome the obstruction, resulting
tract that supports the leaflets of the aortic valve delineated in both turbulent flow and increased velocity across the
superiorly by the sinotubular junction and inferiorly by the valve. The pulsed wave (PW) Doppler helps in localizing
ventricular junction. The aortic root acts as an individual the site of obstruction by demonstrating low velocity in
hemodynamic entity; integrity of all its components is the LV outflow and increased velocity across the aortic
essential for normal function. Aortic root dimensions are valve. However, continuous wave Doppler is required to
assessed at four levels: the annulus, the sinuses of Valsalva, quantitate the valvular obstruction.
sinotubular junction, and the proximal ascending aorta. The jet velocities distal to the stenotic aortic valve orifice
The aortic root dimensions should be routinely measured, are recorded from multiple views—subcostal, apical, right
as it is often dilated in the presence of bicuspid aortic valve, parasternal, and suprasternal views. The velocity of the
irrespective of associated hemodynamic disturbances. aortic stenosis jet is defined as the highest continuous
This progressive dilatation of aortic root is not prevented wave Doppler signal obtained from any window. Only
even after aortic valve replacement; as such cardiologists well-defined envelopes should be used for quantification
recommend reconstruction and remodeling of dilated of velocities to obviate significant errors. The ultrasound
aorta at surgery for bicuspid aortic valve patients. beam must be aligned parallel to the flow for accurate
velocity recording guided by 2D image and color flow.
Aortic Valve Annulus Angle correction should be avoided. Underestimation of
stenosis severity can occur due to a nonparallel intercept
Ventriculoarterial junction anatomically corresponds to angle. At higher velocities, a small error may lead to
the insertion of the arterial trunk into the ventricular mass significant errors of gradients because of the quadratic
that supports it. In the LV, this insertion acquires the shape relation between velocity and pressure gradient.
of a fibromuscular ring and is described as aortic annulus. The usual cause of overestimation of aortic stenosis
The muscular portion corresponds to the left ventricular is if one interrogates the mitral regurgitation signal
myocardium, which supports the valve, and the fibrous mistakenly. Both jets occur in systole and in the same
portion corresponds to the insertion at the level of the direction. A difference in timing may be helpful as the
fibrous continuity between the aortic and mitral valve mitral regurgitation signal velocity starts during isovolumic
leaflets. contraction and continues through isovolumic relaxation,
and the aortic stenosis signal start after the isovolumic • Doppler mean gradient is comparable to mean pressure
contraction during aortic ejection. gradient measured at the cardiac catheterization
The velocity determination across the aortic valve is • As Doppler mean gradient is the average of all the peak
flow-related. Hence, conditions causing increased flow instantaneous gradients throughout the systole, and
such as aortic regurgitation and elevated cardiac output not on single peak velocity, it can be obtained with
as seen in anemia, anxiety, pregnancy, and exercise will greater accuracy and reproducibility
increase the flow velocity across the aortic valve. Hence, it • Mean gradient is less affected by transvalvular flow.
is also necessary to determine the velocity proximal to the • Mean gradient is the basis of calculation of valve area
aortic valve and do the necessary correction in Bernoulli’s using the Gorlin equation.
equation. Conditions associated with low cardiac output
such as left ventricular failure commonly seen in neonatal Aortic Valve Area
or elderly aortic stenosis preclude the use of valve gradient
as an indicator of severity of valvular stenosis. Another The calculation of aortic valve area is a useful method for
physiological issue that needs to be considered in the determining the severity of the stenosis independent of
Doppler assessment of pressure gradients in patients transvalvular flow in contrast to pressure gradient across
with aortic stenosis is the phenomenon of distal pressure the valve. In children where the decision regarding severity
recovery. The fluid dynamics of valvular aortic stenosis of aortic stenosis remains unanswered in patients with
are characterized by a laminar high-velocity jet in the intermediate pressure gradients, determination of aortic
narrowed orifice, with the narrowed segment of the flow valve area should also be performed. Aortic valve area can
stream (the vena contracta) occurring downstream from be measured by the following methods:
the anatomical valve orifice. As the jet expands and • Planimetry—The aortic valve area can be measured
decelerates beyond the vena contracta, the associated by direct tracing from PSAX view at the level of great
turbulence results in an increase in aortic pressure vessels on 2D echocardiography. There are some
“pressure recovery” such that when aortic pressure is limitations in pediatric patients as
measured in the distal ascending aorta, the left ventricular – Fast heart rate leading to limitation of frame rates.
to aortic pressure difference is less than if aortic pressure is – Error in measurement of small orifice.
measured in the vena contracta. – Irregular valve opening that is difficult to trace.
Aortic valve area by continuity equation:
122–128
Pressure Gradients
CSALvot × VTILvot
Transvalvular pressure gradients are usually calculated CSAav =
VTIav
from Doppler aortic velocity profiles. The peak gradient
and the mean gradient are measured. The peak gradient CSA = Cross sectional area of aortic valve.
is determined from the peak velocity using the modified V TI = Velocity time integral.
Bernoulli equation (p = 4V2), and mean gradient by av = Aortic valve.
averaging all the peak gradients in a systolic ejection Lvot = Left ventricular outflow tract.
period. In general, the Doppler-measured peak gradient
may not correspond to the catheter measured peak- Critical Neonatal Aortic Stenosis129–139
to-peak pressure gradient, because Doppler measures
instantaneous peak-to-peak gradient, which is In infants presenting with signs of aortic stenosis in the
fundamentally different from the peak-to-peak catheter first few months of life, echocardiography provides a rapid
“gradient” usually calculated in the cardiac catheterization noninvasive diagnostic method.
laboratory. In some children, especially with moderate • The aortic valve leaflets are thickened and domed.
degree of stenosis, two measurements can differ by In many cases, the leaflets are immobile and a clear
as much as 30 mm Hg. Mean gradients, measured by systolic opening may not be visualized. The annulus
averaging the instantaneous catheter or Doppler gradients usually measures 5–8 mm
over the systolic ejection period, correspond more closely • Usually, there is post-stenotic dilatation of the
to each other. The Doppler mean gradient has several ascending aorta and the ratio of the ascending aorta
advantages over the Doppler peak instantaneous gradient. to the annulus is more than 1.0. This phenomenon
characterizes a LV, generating a pressure gradient for having an adequate mitral valve by acquiring
across the aortic valve leading to release of energy in the mitral valve area (calculated by assuming the
the ascending aorta and post-stenotic dilatation morphology was that of an ellipse with radii measured
• The LV is thickened and hypertrophied from the PLAX and apical four-chamber views) to
• Increased echogenicity of the mitral valve and papillary have a z-score of > −2. Only then can the new criteria
muscles is seen in PSAX, parasternal long-axis, and be applied. These criteria consist of (a) aortic annulus
apical four-chamber views z-score measured from the PLAX view, (b) ratio of
• Redirection of fetal flow patterns and delayed the long axis of the LV to the long axis of the heart, (c)
regression of PVR results in the RV and the main PA endocardial fibroelastosis grade: none, mild (affecting
being enlarged. papillary muscles only), moderate (affecting papillary
If the ventricular function is normal, the peak velocity muscles and some of the endocardium), and severe
across the valve is increased. More often, neonates present (affecting papillary muscles and extensive portions
with severe left ventricular dysfunction, and differentiation of the endocardium). A new regression equation was
from dilated cardiomyopathy becomes important. developed:
In the latter case, the aortic valve is normal with no Score = 10.98 (BSA) + 0.56 (Aortic annulus z –score) +
evidence of post-stenotic dilatation. However, associated 5.89 (LAR) – 0.79 (EFEgrade) – 6.78
cardiomyopathy is difficult to rule out in some cases where
and is only diagnosed retrospectively if the ventricular EFE grade is 0 for none or mild, and 1 for moderate or
contractility fails to improve after relief of aortic stenosis. severe.
The threshold score for survival to be better with
Aortic Stenosis Versus Hypoplastic biventricular versus univentricular repair is > −0.65.
Left Ventricle
In some infants dilated RV may dwarf the LV, which Hemodynamics
appears small or even hypoplastic. The following features The LV becomes hypertrophied with increasing left ven-
help to differentiate and indicate small LV: tricular outflow obstruction. Severe unrelieved obstruc-
• The evaluation of shape of the LV may be useful in these tion may lead to an oxygen demand/supply mismatch
cases. Normal LV is usually ellipsoid and extends to the leading to subendocardial ischemia and fibrosis.
cardiac apex in four-chamber view. But the hypoplastic Ventricular systolic function is assessed by the
LV is globular and does not extend to cardiac apex conventional methods of calculating shortening fraction
• LV inflow dimension (hinge point of posterior mitral and ejection fraction. This is an important parameter in
leaflet to cardiac apex of < 25 mm) the echocardiographic assessment of an aortic stenosis
• Mitral valve annulus diameter of < 9 mm patient as the assessment of severity by pressure gradients
• Ventriculoaortic junction of < 5 mm, (all measured depends upon ventricular function. A decrease in function
from apical four-chamber or long-axis view at end- decreases the transvalvular flow and the gradients no
diastole will indicate hypoplastic LV) longer reflect the severity of obstruction.
• Left ventricular cross-sectional area measured in Diastolic ventricular function is assessed by the filling
the PLAX view that included the mitral valve, aortic abnormalities of the LV. From the mitral valvular Doppler
valve, and left ventricular apex at end diastole of recording peak flow velocities, filling rates and proportion
< 2 cm, usually predicts hypoplastic of LV and of flow in various phases of diastole may be assessed.
nonsurvival after balloon aortic valvotomy Comparative studies of these subjects with normal controls
• Predominant or total antegrade flow in the ascending have revealed higher E-velocity, a much higher A-velocity,
aorta and transverse arch is indicative of an therefore an inverse E/A ratio. The percentage of total
adequate LV Doppler area in the first third of diastole was significantly
• A new discriminant analysis was found to more lower and the percentage of the total Doppler area under
accurately predict survival with a biventricular the A-wave was higher. M-mode tracing in PLAX view at
circulation than with the model using the traditional the mitral valve may show a b-bump or a c-interruption if
criteria. The new criteria first emphasized the need the LVEDP is elevated.
Associated Anomalies129-135 obstruction. Dynamic obstruction is part of hypertrophic

cardiomyopathy and systolic anterior motion of mitral
A PDA is seen in 20 to 65% cases of valvular aortic stenosis. valve, not being discussed in this chapter.
CoA is found in 11 to 53% cases and stenosis of the mitral Echocardiographic evaluation of fixed subvalvular
valve in 25% cases. Other anomalies like VSD and mitral aortic stenosis includes:
valve abnormalities are also common and should be • Cause of subvalvular stenosis.
looked for. • Presence of aortic regurgitation.
• Associated anomalies.
SUBVALVULAR AORTIC STENOSIS • Severity of obstruction.
Subvalvular aortic stenosis is responsible for 8 to 30% of Cause of subvalvular aortic stenosis.140–150 There are
cases of LVOT obstruction and corresponds to 1.2% of several types of fixed subaortic obstructions:
all cardiac anomalies.135-140 Classically, the subvalvular • Discrete fibrous membrane (Fig. 72.78).
aortic stenosis has been divided into fixed and dynamic • Fibromuscular collar.
• Tunnel subaortic stenosis.
• Posterior displacement of infundibular septum with
discrete narrowing of the LVOT (Fig. 72.79).
• Other less common causes are accessory mitral
valve tissue or tissue arising from membranous
septum protruding into LVOT, systolic anterior
motion of anterior leaflet of mitral valve as in cases of
hypertrophic cardiomyopathy (Figs 72.80 to 72.82).
Most commonly, a discrete fibrous membrane
or fibromuscular shelf encircles the LVOT. Rarely, it
extends for a longer distance (more than one third of
aortic diameter) and forms a tunnel-shaped obstruction.
Abnormal tissue may extend and tether the aortic valve or
Fig. 72.78: Two-dimensional echocardiography in parasternal the anterior mitral leaflet.
long-axis view showing subvalvular aortic stenosis with accessory Fixed subaortic obstruction usually occurs in
tissue of mitral valve (arrow). (Ao: Aorta; LV: Left ventricle; RV: association with other defects in 64–70% of the cases and
Right ventricle). is often diagnosed when the child is investigated for them.
Fig. 72.79: Two-dimensional echocardiography in parasternal Fig. 72.80: Two-dimensional echocardiography. Parasternal long-
long-axis view showing the ventricular septal defect (VSD; star) axis view in a child with discrete subaortic membrane showing
with posterior malalignment of the outlet septum (arrow). (Ao: anterior insertion of the membrane to the ventricular septum below
Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). the right aortic cusp (arrow). (Ao: Aorta; LA: Left atrium; LV: Left
ventricle; RV: Right ventricle).
Fig. 72.81: Two-dimensional echocardiography. Parasternal long- Fig. 72.82: Two-dimensional echocardiography. Parasternal long-
axis view in a 2-year-old child with discrete circumferential subao- axis view in a child with hypertrophic obstructive cardiomyopathy
rtic membrane (arrows) close to aortic valve. (Ao: Aorta; LA: Left showing the severely hypertrophied interventricular septum and
atrium, LV: Left ventricle; RV: Right ventricle). systolic anterior motion of the anterior leaflet of the mitral valve
(arrow). (LA: Left atrium; LV: Left ventricle; RV: Right ventricle
Ao: Aorta).
Echocardiographically, subaortic stenosis can be of aortic regurgitation. In contrast to bicuspid aortic valve,
studied from the parasternal long-axis, the apical four- there is no post-stenotic dilatation of the ascending aorta.
chamber, and subcostal coronal views with anterior tilt. The fibromuscular collar is a thick muscular ring that
Membranous subaortic stenosis appears as a discrete forms at a lower level in LVOT than the subaortic membrane
shelf adherent to the interventricular septum beneath and is seen easily in parasternal long-axis, four-chamber
the aortic valve or up to 2.5 cm below it. The PLAX view (apical and subcostal coronal) with anterior tilt as a thick
is highly sensitive in detecting discrete subaortic stenosis; fibrous shelf projecting into LVOT. A fibromuscular tunnel
however, in most cases, the anterior insertion alone is is diagnosed by the same views and gives the appearance
visualized because the membranous diaphragm being of a long narrow tract of subaortic narrowing.
directed posteriorly toward the left ventricular posterior Posterior displacement of outlet septum: Subaortic
wall and mitral valve is aligned parallel to the ultrasound stenosis due to posterior malalignment of outlet septum
beam in the PLAX view. In some cases, however, with occurs with a nonrestrictive perimembranous VSD.
careful interrogation, anterior insertion to ventricular This subgroup usually has associated arch anomalies
septum below the right aortic cusp and posterior insertion such as CoA or arch interruption. Best view to profile
to the base of anterior mitral leaflet insertion can both malalignment of outlet septum is parasternal long-axis,
be seen. In four-chamber subcostal coronal views with although four-chamber views with anterior tilt also define
anterior tilt, the ultrasound beam is perpendicular to the the posterior malalignment of outlet septum leading to
subaortic ridge and profiles the anatomy of membrane subaortic narrowing. Arch anomalies should be defined in
better. Careful PSAX scans of the LVOT are more likely to detail from suprasternal views in these cases.
visualize the extent of attachment of the membrane. The With subaortic stenosis, on M-mode trace the aortic
membrane may come in and out of the plane of PSAX valve leaflets shows a slight flutter and early to mid
section very quickly because of rapid cardiac motion and, systolic closure along with other evidence of significant
frame-by-frame analysis is required to assess it adequately. left ventricular outflow obstruction such as left ventricular
In the majority of cases, it has the shape of a horseshoe; hypertrophy. Doppler echocardiography quantitates
rarely is it a complete ring and in most cases only one the degree and site of obstruction. Color Doppler
insertion is visualized at a given time. Luminal diameter illustrates the flow acceleration at the site of stenosis and
is not a criterion for severity of obstruction. With discrete aliasing velocities beyond that. It helps in positioning
subaortic stenosis, the jet of stenosis often damages the ultrasound beam parallel to the left ventricular
the aortic valve leading to thickened aortic valve on 2D outflow in various views. The total gradient across the left
echocardiography. Color flow mapping reveals presence ventricular outflow is quantitated by continuous wave
Doppler using the modified Bernoulli’s equation. The right include VSD, CoA or arch interruption, bicuspid aortic
infraclavicular view with the patient turned toward the valve, supravalvular mitral stenosis, and persistence of the
right usually records the highest velocities. PW Doppler is LSVC with dilated coronary sinus causing restrictive left
used to localize the site of outflow obstruction by placing ventricular filling.
the pulsed Doppler sequentially and noticing the site of Discrete subvalvular aortic stenosis is a progressive
increase in outflow velocity. lesion. Fixed subaortic stenosis has been noticed to
In diffuse tunnel obstruction, the maximal velocity progress more rapidly in the presence of associated lesions
is produced inside the tunnel and this can be missed than isolated subaortic stenosis. This implies that careful
altogether by Doppler and catheter techniques, at times. screening should be done for associated lesions in all
In these latter cases, the pressure drop caused by viscous cases of discrete subaortic stenosis. Tethering of mitral
friction along the tunnel may cause a lower Doppler and aortic valves is also known to develop subsequently in
velocity to be recorded, resulting in an underestimation serial echocardiograms.
of the true gradient. The tunnel subaortic stenosis may be Even after adequate surgical relief, this lesion is known
associated with a narrow aortic annulus and often causes
to recur and patients require long-term follow-up.
significant concentric left ventricular hypertrophy. In
Aortic insufficiency can also progress and should be
infants with CoA or arch interruption, reduced aortic valve
carefully evaluated in follow-up of cases of discrete subaortic
diameter and increased mitral–aortic separation could
stenosis. The distance between the diaphragm and the aortic
be precursors to subaortic obstruction. The extension
valve should be noted in the initial echocardiogram. The
of subaortic fibroelastic tissue to involve the aortic
mechanism responsible for aortic regurgitation is believed to
root at the site of insertion of the aortic valve cusps and
increasing fibrosis of the aortic root may be responsible be repetitive trauma caused by the high velocity jet through
for this discrepancy. The size of the aortic root has a the subvalvular stenosis as well as extension of the fibroelastic
marked effect on the optimal relief of the fixed subaortic tissue of discrete subaortic stenosis toward the base of one or
stenosis. In patients with fixed subaortic stenosis, the more aortic cusps.
aortic root can be small (25%) and should be measured
preoperatively because in the presence of small aortic SUPRAVALVULAR AORTIC STENOSIS
root, a special surgical technique is required. There is a
(FIGS 72.83 AND 72.84)151–154
13% incidence of left ventricular outflow abnormalities in
immediate family members. Hence, their screening is an Supravalvular aortic stenosis is the rarest of left ventricular
essential part of patient evaluation. In 65 to 70% of cases of outflow obstructions (2–11%). It occurs at the sinotubular
subaortic obstruction, associated defects are present and junction and produces a localized or diffuse narrowing.
Fig. 72.83: Two-dimensional echocardiography. Apical four- Fig. 72.84: Two-dimensional echocardiography. Parasternal
chamber view with anterior tilt and color flow mapping in a case long-axis view of the left ventricular outflow tract showing severe
of supravalvular aortic stenosis. The turbulence (arrow) begins stenosis at the level of the sinotubular junction (arrow) in a patient
above the level of the aortic valve (arrow). (AA: Ascending aorta; with supravalvular aortic stenosis. (Ao: Aorta; LA: Left atrium; LV:
LV: Left ventricle). Left ventricle; RV: Right ventricle).
This is a group of lesions with varying anatomy and is in diameter with catheter peak gradients. In the diffuse
characterized into three types: type of supravalvular aortic stenosis, the aortic hypoplasia
• Membranous type (<25%) extends from the sinotubular junction to the innominate
• Hourglass type (50–75%) artery. Usually, the aortic arch and arch vessels are also
• Uniform hypoplasia of the ascending aorta (<25%). involved. A severely hypoplastic and narrowed segment
may have retrograde flow from PDA supplying the arch
During an echocardiographic diagnostic study, the
vessels and the descending aorta. When the aorta is
following anatomical features need to be defined: diffusely hypoplastic, the annulus is frequently also
• Morphology and severity of supravalvular aortic involved and can no longer be used as a reference for
stenosis estimation of severity.
• Anatomy of the aortic valve The membranous lesion generally appears as a discrete,
• The coronary anatomy linear echo extending inward from the walls of the aorta
• Associated anomalies. and encroaching on the vascular lumen. The membrane
is typically located at the sinotubular junction just above
Morphology the insertion of the superior LA wall into the posterior
aortic root. The separation between the inner margins of
The obstruction is best imaged in parasternal long-axis, an obstructive membrane is less than the diameter of the
apical five-chamber and subcostal views of the LVOT, or outflow tract at the annulus.
suprasternal long- and short-axis views and high right Doppler interrogation aids in confirmation of diagnosis.
parasternal views. In the parasternal and apical five- The site of stenosis is confirmed both by color flow and
chamber views in the normal heart, the aortic diameter pulsed Doppler, while maximal velocity is obtained by
increases at the level of the sinuses of Valsalva and then continuous wave Doppler. The jet flow of supravalvular
decreases at the superior border of the aortic sinuses. The aortic stenosis is often directed toward the innominate
diameter of the ascending aorta above the aortic sinuses is, artery; consequently, the highest value of the peak velocity
however, the same as the diameter of the ventriculoaortic is often obtained by aligning the Doppler beam parallel
junction or aortic root in the normal heart. with the innominate artery in the suprasternal views.
The detection of obstruction in hourglass deformity Pressure gradient across the aortic root is calculated
depends upon visualization of an obvious decrease from the modified Bernoulli’s equation and correlates
in the caliber of the vessel relative to the surrounding with catheter-measured peak-to-peak gradient across
normal areas, namely at sinotubular junction, then the fibrous diaphragm. However, in hourglass deformity,
some dilation, and narrowing again. The following gradual re-expansion of the aorta distal to stenosis leads to
measurements are taken in the PLAX view: (a) aortic pressure recovery and discrepancy between Doppler and
annulus, (b) maximal diameter at sinuses of Valsalva, (c) catheter-measured gradients.
sinotubular junction, (d) narrowest part, and (e) aorta
distal to the obstruction. Aortic Valve Anomalies
Measurements are taken from the inner aspect of
the aortic root echo to the inner aspect of the posterior Aortic valve abnormalities have been reported in 25 to 45%
root echo. Careful attention should be paid to aligning of the cases. It is important to assess the dynamics of aortic
the probe so that long axis of the vessel and adequate valve motion throughout the cardiac cycle. In diastole,
visualization of the lumen both proximal and distal to the the valve may function normally; however, in systole its
area of obstruction is obtained. Oblique angulation of the motion can be limited and the opening incomplete due to
cross-sectional scan plane to vascular lumen may give spatial restriction; this has been called “pseudovalvular”
the appearance of the aorta being cut-off as beam passes stenosis. The presence of bicuspid aortic valve, valve
obliquely through the lateral wall of the vessel. thickening, and commissural fusion have been reported
Normally, the diameter of the sinotubular junction is in a high percentage of cases. Aortic valve regurgitation
either equal or slightly (12.5%) more than the diameter has been reported in 13 to 66% cases of supravalvular
of the aortic annulus. In supravalvular aortic stenosis, a aortic stenosis and needs careful evaluation. Anomalies of
percentage decrease of > 25% from the annulus is noted. aortic valve can be seen in the same view as described for
There is a rough correlation in the percentage decrease valvular stenosis.
Coronaries
Coronary artery abnormalities are invariably associated
and may be:
• Dilated and tortuous coronary arteries due to
exposure to high systolic pressures proximal to the site
of obstruction (rarely coronary artery aneurysms have
been reported)
• Coronary orifice can be narrowed by the overhanging,
fibrous bridge.
• Rarely, the orifice of coronary artery can be completely
obstructed as the valve cusps become adherent to the
aortic wall.
With the patient in the left lateral position, the
transducer is placed in the left parasternal position
to obtain a short-axis view of the great vessels. Slight
adjustment of the imaging plane, so that it traverses the
heart below the pulmonary trunk, allows for visualization
of the origin and proximal portion of coronary arteries.
The ostia and the coronaries should be carefully assessed Fig. 72.85: Two-dimensional echocardiography high parasternal
short-axis view with color flow mapping, in a 2-year-old child with
for dilatation, tortuosity, or narrowing. The apical four-
William’s syndrome, showing bilateral pulmonary artery stenosis
chamber view demonstrates RCA coursing along the right with turbulent flow. (LPA: Left pulmonary artery; MPA: Main pulmo-
AV groove and aiming the transducer superiorly toward nary artery; RPA: Right pulmonary artery).
the left ventricular outflow, the branching of left anterior
descending (LAD), and left circumflex may be seen
other congenital heart defects, aortic root dilatation,
besides their profiling in the PSAX view. In severe cases,
and infectious processes of the aorta. Causes of aortic
myocardial ischemia and infarction have been reported
regurgitation are highlighted in Table 72.12.
as such regional wall motion abnormalities should be
The primary cardiac abnormalities are the commonest
assessed at echocardiography.
cause of congenital aortic regurgitation such as a
bicuspid or quadricuspid aortic valve. Significant aortic
Presence of Stenosis of the Central or regurgitation presenting in neonatal period can be due to
Branch Pulmonary Arteries (Fig. 72.85) two causes:
Stenosis of pulmonary arteries proximally at the bifurcation • Aortic–left ventricular tunnel.
or its branches can be associated with supravalvular • Unguarded aortic valve.
aortic stenosis, particularly with William’s syndrome. It While evaluating the patient with aortic regurgitation,
can be multiple sites along the branches or a generalized the following things should be assessed on echocardio-
hypoplasia. Central pulmonary arteries can be evaluated graphy:
by echocardiography, but if peripheral branches seems to • Left ventricular outflow abnormality-cause of aortic
be involved, other modalities such as spiral CT/MRI will regurgitation.
be needed. • Severity of regurgitation.
• Left ventricular dimensions—end-systolic and end-
Serial Echocardiograms diastolic.
• Left ventricular systolic and diastolic function.
The condition is usually progressive in nature and cases
with mild narrowing need careful long-term follow-up.
Left Ventricular Outflow Abnormality-
AORTIC REGURGITATION Cause of Aortic Regurgitation
Congenital aortic insufficiency is a rare entity as an PLAX view and PSAX view at the level of great vessels are the
isolated lesion. It frequently occurs in association with best views to define the LVOT abnormalities as described
Table 72.12: Causes of Aortic Regurgitation severity of regurgitation, ventricular dimensions, and
Primary congenital cardiac abnormality
ventricular function is very important before taking the
decision about management, whether the patient will
• Aortic valve abnormality
require medical follow-up, valve repair, or will need aortic
– Bicuspid aortic valve
valve replacement. Aortic valve annulus and aortic root
– Quadricuspid aortic valve
size should be measured in patients undergoing aortic
– Absence of aortic valve cusps (unguarded aortic orifice)
valve replacement in PLAX view. If a patient is planned
• Aortico-left ventricular tunnel (Figs 72.86A and B) for the Ross procedure, then pulmonary root should be
• Annulo-aortic ectasia (Figs 72.87 A and B). measured in addition to aortic root measurement.
Connective tissue disorders with aortic root dilatation Etiology of aortic regurgitation present in other heart
• Marfan syndrome, Ehlers–Danlos syndrome defects such as VSD with aortic valve prolapse (commonly
• Turner syndrome with aortic ectasia (Figs 72.87A and B) doubly committed, outlet muscular, or perimembranous),
Association with other forms of congenital heart defects subaortic stenosis, and truncus arteriosus has been
• Aortic valve prolapse into ventricular septal defect (doubly discussed in respective sections.
committed, outlet muscular, or perimembranous)
• Dilatation of aortic root as in tetralogy of Fallot physiology Aortic Root Dilatation
• Truncus arteriosus
Aortic root dilatation occurs in connective tissue disorders
Rheumatic fever (mostly associated with mitral valve disease)
like Marfan syndrome, Ehlers–Danlos syndrome,
Infectious processes of the aortic valve
Turner syndrome, and multivalvular heart diseases.
• Bacterial endocarditis
With connective tissue disorders, aortic root dilatation
in an earlier section. Aortic valve cusp number and is progressive; initially it involves sinuses of Valsalva,
anatomy, rolled, gnarled, and inadequate, or redundant ascending aorta, then dilatation progresses to involve
and prolapsing should be defined. Determination of aortic annulus leading to distortion of aortic valve, and
A B
Figs 72.86A and B: Two-dimensional echocardiography. Parasternal long-axis view with slight anterior tilt and color comparison
in a 7-day-old neonate with aortico-left ventricular tunnel (arrow). Color flow mapping shows aortic regurgitation. (Ao: Aorta; RV: Right
ventricle; LV: Left ventricle; T: Tunnel).
A B
Figs 72.87A and B: Two-dimensional echocardiography a case of annulo-aortic ectasia in a 2-year-old girl. (A) Parasternal long-axis
view showing the dilated (ectatic) aortic root; (B) Subcostal coronal view with anterior tilt showing the left ventricular outflow tract with
ectatic aortic root in the same patient. (Ao: Aorta; RV: Right ventricle; LV: Left ventricle; LA: Left atrium).
Table 72.13: Checklist of Aortic Insufficiency in Pediatric Patients mapping and pulsed Doppler interrogation, severity of
Look for the etiology of aortic valve insufficiency and assess aortic regurgitation can be evaluated. Parameters of AR
the need for surgical intervention are not discussed in detail as these are covered in chapters
Assess for the following: on acquired heart diseases. A reference checklist is given
• Thickening of the aortic valve cusps in Table 72.13.
• Morphology of the commissures, presence of a raphe
• Cusp prolapse
SINUS OF VALSALVA ANEURYSM
• Detached or flail valve cusps (FIGS 72.88A AND B)155–167
• Vegetations Sinus of Valsalva aneurysm is a rare cardiac anomaly. It
• Aortic root dilatation is thought to result from absence of normal elastic tissue
• Left ventricle (LV) wall thickness and abnormal development of the bulbus cordis leading
• LV end-diastolic dimension to a separation between the aortic media and the annulus
• LV end-systolic dimension fibrosus, which in turn leads to thinning of the wall of
• LV shortening fraction and LV ejection fraction the aortic sinus. Other diseases that involves the aortic
root may also lead secondarily to the sinus of Valsalva
aortic regurgitation. While evaluating the patient with aneurysm. This malformation occurs more frequently in
connective tissue disorders such as Marfan syndrome, males than females (3:1) and in patients of Asian (India in
aortic root measurements should be taken at four levels— Asia) origin. It can be congenital or occurs as an acquired
aortic annulus, sinus of Valsalva, sinotubular junction, lesion in patients with connective tissue disorders such as
and ascending aorta 1 cm above the sinotubular junction, Marfan syndrome, Ehlers–Danlos syndrome, annuloaortic
compared with age-related norms and should be followed ectasia, ankylosing spondylitis, or with endocarditis. The
up serially. Undue dilatation or rapid increase in these weakened wall of sinuses of Valsalva may progressively
parameters will identify the patient who is at a risk of dilate under systemic pressure and eventually rupture into
development of aortic dissection and needs elective aortic a low-pressure chamber.
root replacement procedure. Echocardiographic evaluation of aneurysm of sinus of
Valsalva includes:
Severity of Regurgitation • Sinus involved showing characteristic of aneurysm
With the use of 2D echocardiography, left ventricular • Presence of obstruction caused by the aneurysm or
dilatation and function can be assessed, and on color flow rupture of the aneurysm
A B
Figs 72.88A and B: Ruptured sinus of Valsalva in a 30-year-old male patient. (A) Two-dimensional echocardiography with color com-
pare in parasternal short-axis view showing aneurysm of noncoronary sinus rupturing into the right atrium (arrow). (Ao: Aorta; LA: Left
• Cavity where the aneurysm protrudes or ruptures addition, the runoff produces a Bernoulli’s effect, due to
• Aortic regurgitation which the cusp is pulled away from its line of apposition
• Associated anomalies. leading to increased incompetence. Aortic regurgitation
Any of the aortic sinuses may be involved, and more is more common when aneurysm of sinus of Valsalva
than one sinus may be rarely involved (0.6%). Right is associated with VSD (43.3%) as compared to intact
coronary sinus is most commonly involved (73%), ventricular septum (25.9%).
followed by the noncoronary sinus (20%); left coronary
sinus (6%) is the least commonly involved. Enlargement Associated Anomalies
of the aneurysm is in the direction of least resistance, so
most commonly it bulges into the right-sided chambers. Deformation and rupture of the sinus of Valsalva is
Unruptured aneurysm can cause obstruction of the commonly associated with VSD, so it has to be looked
surrounding structures like the RVOT, right PA and, for carefully (Fig. 72.91). The right sinus may prolapse
descending aorta. Tricuspid valve distortion leading through a doubly committed VSD towards RVOT, causing
to tricuspid regurgitation can occur with aneurysm RVOT obstruction and subsequent rupture. Both the right
bulging into RV inflow. Rarely it can burrow into the coronary and noncoronary sinuses may prolapse through
interventricular septum resulting in complete heart a perimembranous VSD into RV inflow, which may lead to
block. The aneurysm can also get calcified. The aneurysm distortion of tricuspid valve and tricuspid regurgitation.
may rupture into a neighboring chamber, leading to Other associated anomalies are pulmonary stenosis, TOF,
development of symptoms acutely. Bulging or rupture of ASD, bicuspid aortic valve, CoA, and LSVC, which needs to
the right sinus can occur into RV (commonest), RA, PA, LV, be scanned carefully.
or into the pericardium. Aneurysm of noncoronary sinus Aneurysm of sinus of Valsalva can be best defined
can bulge or rupture into RA (most commonly), RV, LA, from parasternal long-axis, subcostal coronal and apical
LV, or pericardium. Aneurysm of left coronary sinus can four-chamber views with tilting the transducer toward RV
extend into LA, RA, LV, RV, PA, or pericardium. inflow and outflow. PSAX view also defines the anatomy
with the sinus protruding into RV inflow, outflow, RA,
LA, or into the PA. Combination of 2D echocardiography
Aortic Regurgitation with color flow mapping and pulsed/continuous wave
Aortic regurgitation is a frequent occurrence in patients Doppler interrogation is helpful in detailed profilation
with an aneurysm of sinus of Valsalva with or without of the lesion. With rupture of aneurysm into right-sided
rupture. Aortic regurgitation occurs due to loss of support chambers, there will be dilatation of right-sided chambers
of the aortic sinus and its annulus and its distortion. In with large left-to-right shunt and rise of pressures. With
rupture of aneurysm into LA or LV, these chambers will be communication noted anteriorly with the RVOT ending
volume-loaded with rise in left atrial and LVEDPs. Color distal to right coronary sinus of aorta. In the PSAX view, an
flow mapping demonstrates rupture of aneurysm and echo dropout can be seen at the level of aortic valve. There
the cavity into which it opens. Rupture into RA, LA, RV, or is a crescent-shaped structure wrapping around the right
PA will result in continuous flow from aortic sinus to the coronary cusp anteriorly, clearly distinct from aortic root.
receiving cavity, while with rupture into LV, there will be The aortic origin can be shown to be above the sinus and
only diastolic flow. Color flow mapping also defines the separate from the origin of both coronary arteries enabling
aortic regurgitation. M-mode echocardiography is the differentiation of tunnel from sinus of Valsalva fistula.
best way to demonstrate direction of shunt and its relation Occasionally, a coronary artery may arise from the tunnel.
to cardiac cycle, with ruptured sinus of Valsalva (RSOV), In neonates and infants, it is possible to demonstrate the
a continuous flow from aorta to receiving low-pressure coronary artery origin on 2D echocardiography; however,
chamber can be demonstrated. in older patients it may be difficult.
The measurements of aortic annulus, sinotubular In apical four-chamber and subcostal coronal views
junction, and ascending aorta should be made. Also, the with anterior tilt, the tunnel is seen to protrude into RVOT.
exact sinus involved by the aneurysm and size of its final Subcostal sagittal view also shows bulging of tunnel into
opening and distance from the coronary orifice should be RVOT , which can cause subpulmonary stenosis.
measured when contemplating RSOV device closure. Both pulsed Doppler and CFI show systolic antegrade
RSOV aneurysm should be differentiated from flow and diastolic retrograde flow within the tunnel. CFI
aorticocameral tunnel, the most important feature being is particularly useful in assessing coexisting central aortic
the origin of tunnel will be above the sinus of Valsalva. regurgitation. Presence of subpulmonary stenosis should
also be defined with the use of color flow mapping and
AORTOCAMERAL pulsed Doppler interrogation. Associated anomalies have
COMMUNICATIONS168–174 been described in several studies and include bicuspid
aortic valve, aortic stenosis, PDA, pulmonary stenosis,
Aortocameral communications are abnormal communi- VSD, and sinus of Valsalva aneurysm. These anomalies can
cations between the root of aorta and one of the cardiac also be demonstrated on 2D echocardiography.
chambers, the commonest being aortico-left ventricular
tunnel, followed by aortico-right atrial tunnel, aortico-RV
tunnel, and least in frequency is aortico-LA tunnel.
Aortico-Right Ventricle Tunnel
Aortico-RV tunnel is another rare congenital malformation
Aortico-Left Ventricular Tunnel in which the tunnel connects aorta above the level of sinus
of Valsalva to the RV. PLAX view on 2D echocardiography
(Figs 72.86A and B) defines the tunnel as a communication starting above
Aortico-left ventricular tunnel is a rare congenital the sinus of Valsalva, and with anterior tilt from this view,
malformation characterized by abnormal paravalvular opening of this tunnel into RV can be defined. Color flow
communication between anterior aspect of aorta and LV. It mapping will reveal high velocity flow into the RV during
causes progressive left ventricular failure and aneurysmal systole as well as diastole. This needs to be differentiated
dilation of aorta. from RSOV into RV. In RSOV, the aortic end of tunnel is
Incidence of aortico-left ventricular tunnel has been situated below the coronary arteries. RV is usually dilated
estimated to be around 0.1% of congenitally malformed and hypertrophied, as it is volume- and pressure-loaded.
hearts from review of clinical and pathological material
and 0.46% of cardiac malformation identified on fetal
Aortico-Right Atrial Tunnel
echocardiography. About twice as many cases have been
reported in males as in females, but it is rarely seen in Aortico-right atrial tunnel, which connects aorta above the
patients of Asian, Oriental, and African descent. level of sinus of Valsalva to the RA, is very rare. The tunnel,
Echocardiography in neonate and young infant more commonly from left sinus, runs posterior to the aorta
helps demonstrate the entire course of tunnel as well and then opens into the RA. PSAX view at the level of great
as its relationship to aortic root, sinuses, and coronary vessels defines the length of tunnel joining RA and aorta.
ostia. The PLAX view shows a septal dropout at the Color flow mapping will show high-velocity, continuous
anterosuperior part of interventricular septum, with a free flow from aorta to the RA.
PART 6: ECHOCARDIOGRAPHIC ANATOMY OF TETRALOGY OF

FALLOT WITH PULMONARY STENOSIS
Prevalence of TOF (regardless of pulmonary valve mor- Ventricular Septal Defect

phology) ranges from 0.26 to 0.48 per 1,000 live births.
TOF consists of anterior and cephalad deviation of the Commonest VSD seen in 74% of cases of TOF is per-
outlet septum leading to a large malaligned (mostly perimembranous outlet VSD, since the tricuspid valve forms
one of the margins of the defect. Echocardiographically,
imembranous type) VSD with narrowing of the RVOT.175–179
the subcostal sagittal and coronal views show tricuspid–
Echocardiography is the most important investigation for
aortic–mitral continuity, which is a differentiating feature
any such case and has replaced invasive angiography for
from DORV. The aortic valve forms the roof of the defect;
preoperative evaluation of TOF patients. The evaluation
the posterior-inferior margin is the area of tricuspid–
of TOF has to be done in sequential analysis as for any
aortic–mitral continuity and the anterior and anterior-
other case of CHD. It is extremely rare to have TOF with
inferior margins are muscular. The PSAX view at the level of
isomeric hearts. PLAX view (Figs 72.89A and B) shows
the aortic valve shows that the VSD extends from the area
the defect well. Subcostal paracoronal view (Fig. 72.90)
of the tricuspid valve anteriorly to the area of the muscular
also identifies the anatomy of the tetralogy well, showing septum (Fig. 72.91). The outlet septum itself separates the
the VSD, infundibular septum, RVOT, and even branch aortic valve from the pulmonary valve.
pulmonary arteries, particularly right pulmonary artery Muscular outlet VSD is seen in 20% of cases of TOF. A
(RPA). The important salient features of the lesion are muscle bar is present in the posterior-inferior margin of
discussed below. the defect and this separates the tricuspid valve from the
aortic valve. Thus, except for the superior margin, which is
AORTIC OVERRIDE formed by the aortic valve, the VSD in rest of the margins
is entirely muscular. Echocardiographically, this feature is
The presence of the large malaligned VSD and dilatation of well seen in the subcostal coronal view as a bar of mus-
the aortic root can result in aortic override. This can also be cle separating the aortic valve from the tricuspid valve. In
seen with an isolated large VSD without anterocephalad the PSAX view also the aortic valve is separated from the
deviation of the outlet septum. Echocardiographically, tricuspid valve by a muscle bar. The importance of recog-
aortic override is best documented in the PLAX view nizing this entity is that this bar of tissue separates the con-
(Fig. 72.91). The aortic mitral continuity is also best seen duction tissue from the margins of the defect making the
in this view. chances of heart block unlikely when the VSD is closed.
A B
Figs 72.89A and B: Two-dimensional echocardiography in parasternal long-axis view with color comparison in a 2-year-old child with
tetralogy of Fallot showing a large perimembranous ventricular septal defect (VSD), aortic override ( ), and aortic mitral continuity
(arrow). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Fig. 72.90: A 2-year-old child with tetralogy of Fallot. Two-dimen- Fig. 72.91: Two-dimensional echocardiography. Parasternal
sional echocardiography with subcostal paracoronal view showing short-axis view showing a large ventricular septal defect with
the large perimembranous ventricular septal defect (VSD; arrow) anterior malalignment of the septum (arrow) leading to subvalvular
with anterior malalignment of the septum, causing subvalvular pul- pulmonary stenosis. (Ao: Aorta; PA: Pulmonary artery; RA: Right
monary stenosis. (Ao: Aorta; RVOT: Right ventricular outflow tract; atrium; RV: Right ventricle).
In 2 to 3% of the case of TOF, the VSD is juxta-arterial or low scale of color flow mapping to scan the entire part of
doubly committed VSD (Figs 72.92 and 72.93).180 The outlet the interventricular septum in more detail increases the
septum is absent and the roof of the VSD is formed by the chances of detecting additional VSDs more reliably.
aortic and pulmonary valve in continuity. The inferior Restrictive VSD (Hoffmann’s variant; Figs 72.95, 72.96,
margins are muscular unless the VSD is large enough and 72.97): This occurs in 1.5% of the cases of TOF. The
to extend to the area of tricuspid aortic continuity (i.e. hallmark of the VSD in TOF is its unrestrictive nature
perimembranous). The aortopulmonary continuity in this causing equalization of the right and left ventricular
type of VSD is best visualized in the subcostal paracoronal pressures irrespective of the degree of pulmonary stenosis.
and PSAX views. Very rarely, however, the VSD can become restrictive.
Inlet extension of VSD is recognized on echocardio- The mechanism of the restriction is best recognized
graphy from the apical four-chamber view. The important echocardiographically. The most common mechanism
echocardiographic features in this case will be dropped is the presence of accessory tricuspid valve tissue or the
out at the level of the AV valve. TOF can also be associated normal tricuspid valve, prolapsing through the VSD, which
with a complete AVSD. This is differentiated from the narrows the interventricular communication.
inlet extension of a perimembranous VSD by (a) absence
of offsetting, (b) presence of a common AV orifice (best Pulmonary Stenosis181–185
visualized in the subcostal sagittal scan, and (c) ASD of the
ostium primum variety. RVOT narrowing in a case of TOF is a consequence of
Additional VSDs have to be evaluated in any case the anterior and cephalad deviation of the infundibular
of TOF (Fig. 72.94). The most common site is in the septum. The hallmark in TOF is infundibular stenosis
trabecular septum. Because of the large proximal with variable degree of valvular stenosis (Figs 72.97
unrestricted perimembranous VSD and equal right and and 72.98). Thus, patients with large VSD and isolated
left ventricular pressures, these smaller defects can be valvular stenosis should not be classified under TOF.
missed even on the color scan unless great care is taken Anterocephalad deviation of the outlet septum is
to scan the interventricular septum in detail. The best seen best echocardiographically in the subcostal
views to visualize these defects on echocardiography are sagittal, coronal views and PSAX views at the level of
the subcostal sagittal and the apical four-chamber views. the great arteries. In patients of TOF and pulmonary
Use of the zoom feature in inverted view of the septum and atresia, the deviation is so extreme so as to produce
Fig. 72.92: Two-dimensional echocardiography. Parasternal short- Fig. 72.93: Two-dimensional echocardiography. Parasternal
axis view in a 3-year-old child with tetralogy of Fallot and doubly short-axis view in a patient of tetralogy of Fallot with color flow
committed ventricular septal defect showing the point of continuity mapping showing doubly committed ventricular septal defect. (Ao:
between aortic and pulmonary valves (arrow). (Ao: Aorta; PA: Aorta; PA: Pulmonary artery; RV: Right ventricle).
Pulmonary artery; RV: Right ventricle).
Fig. 72.94: Two-dimensional echocardiography. Modified para- Fig. 72.95: Tetralogy of Fallot—Hoffman’s variant. Two-dimensional
sternal short-axis view in a case of tetralogy of Fallot showing the transthoracic parasternal long-axis view with color comparison in
additional muscular ventricular septal defect (VSD) tract (arrow) a case of tetralogy of Fallot VSD getting partially restricted by the
with left right shunt. (LV: Left ventricle; RV: Right ventricle). septal leaflet of the tricuspid valve (arrow) leading to suprasystemic
right ventricle (RV) pressures with turbulent right-to-left shunt. (Ao:
Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
Fig. 72.96: Tetralogy of Fallot—Hoffman’s variant. Parasternal Fig. 72.97: Two-dimensional echocardiography. Parasternal
short-axis view with color compare showing large ventricular short-axis view with color compare showing severe infundibular
septal defect getting partially restricted by tricuspid valve leaf- pulmonary stenosis (arrow) in a patient of TOF. (Ao: Aorta; PA:
let (arrow) with severe infundibular pulmonary stenosis (arrow). Pulmonary artery; RV: Right ventricle).
(Ao: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle;
RVOT: Right ventricular outflow tract).
A B
Figs 72.98A and B: Two-dimensional echocardiography in sub- Fig. 72.99: Two-dimensional echocardiography in parasternal
costal paracoronal view with color compare showing severe short-axis view showing short segment pulmonary atresia (arrow)
infundibular pulmonary stenosis (arrow) with turbulent flow in in a patient of TOF. Branch pulmonary arteries are confluent. (Ao:
right ventricular outflow tract (RVOT), in a patient with tetralogy of Aorta; LPA: Left pulmonary artery; RPA: Right pulmonary artery;
Fallot. (PA: Pulmonary artery; RV: Right ventricle). RV: Right ventricle).
atresia (Fig. 72.99). Progression of the obstruction occurs demonstrable echocardiographically as doming of the
due to (a) failure of the subpulmonary infundibulum to pulmonary valve. It is important to measure the annular
grow with increase in somatic growth, (b) progressive size in all cases and compare to normal expected for the
acquired infundibular septal hypertrophy, and (c) patient size (Fig. 72.100). Normal pulmonary valve annulus
progressive acquired RV free wall hypertrophy. All these has surgical implications, because the surgeon can correct
features can be documented echocardiographically. the abnormality without using a transannular patch. The
Pulmonary valve is bicuspid in 51% of cases of TOF.2 annular size is best measured in PSAX views or anteriorly
Valvular stenosis is almost always present in TOF and is tilted long-axis view.
Fig. 72.100: Two-dimensional echocardiography. Parasternal Fig. 72.101: Two-dimensional echocardiography. Modified para-
short-axis view showing the measurement of the pulmonary sternal short-axis view with color compare in a patient with
annulus from the hinge points (+) and RCA. (Ao: Aorta; PA: Pul- tetralogy of Fallot showing absence of pulmonary valve (arrow)
monary artery; RCA: Right coronary artery (arrow); RV: Right ven- with hypoplastic pulmonary artery annulus and free pulmonary
tricle). regurgitation (on color mapping) across the pulmonary annulus.
(PA: Pulmonary artery; RV: Right ventricle).
arteries may be dilated to variable degrees. The physiology

is essentially same as that of TOF. The RV obstruction is
usually at the level of pulmonary annulus; associated
infundibular stenosis may sometimes be present. Very
rarely, there may be an absence of pulmonary valve in the
absence of VSD.
The Pulmonary Arteries

(Figs 72.102 to 72.105)
Echocardiographically, the pulmonary arteries need to
be visualized in multiple views. The paracoronal view not
only identifies the RVOT, but also well visualizes the right
PA. The subcostal coronal view shows the entire length of
Fig. 72.102: Two-dimensional echocardiography in high paraster- the right PA from its origin to its branching at the hilum.
nal short-axis view showing a narrow origin (arrow) of left pulmo- The PSAX view shows the pulmonary trunk, confluence,
nary artery in a case of TOF. (Ao: Aorta; LPA: Left pulmonary ar- and the origins of the right and left pulmonary arteries and
tery; MPA: Main pulmonary artery).
a considerable length of the right PA. The left PA, which
is most difficult to visualize, is occasionally seen in its
Tetralogy of Fallot with Absent entire length from the high left parasternal view and the
suprasternal oblique long-axis view with anterior tilt. The
Pulmonary Valve (Fig. 72.101) suprasternal short-axis view opens the entire length of
This is a rare variant of TOF seen in 2% of cases. The the right PA. Systematic delineation of the PA anatomy is
pulmonary valve is absent and replaced by nubs of crucial in the management of TOF. The size of pulmonary
fibrous tissue, which is best seen echocardiographically in arteries is measured at the hila, that is, before the first
short-axis views as bright echoes. This is associated with division. The optimum size of the pulmonary arteries may
significant pulmonary regurgitation clearly seen on color be seen from the standardized charts for the weight of the
flow mapping. The main/right/left proximal pulmonary baby. Various ratios used for this include: Mc Goons ratio,
A B
Figs 72.103A and B: Two-dimensional echocardiography with color compare in high parasternal short-axis view showing confluent
branch pulmonary arteries with diffuse narrowing in left pulmonary artery (LPA, arrow). (MPA: Main pulmonary artery; RPA: Right
pulmonary artery).
Fig. 72.104: Two-dimensional echocardiography in suprasternal Fig. 72.105: Two-dimensional echocardiography in suprasternal
short-axis view with anterior tilt showing both the branches short-axis view showing the right pulmonary artery and the adja-
of pulmonary artery. (LPA: Left pulmonary artery; RPA: Right cent structures. (AO: Aorta; Innom: Innominate vein; LUPV: Left
pulmonary artery). upper pulmonary vein; LA: Left atrium; RUPV: Right upper pulmo-
nary vein; SVC: Superior vena cava).
wherein the ratio of the sum of the size of branch pulmonary and left PA, (b) the site of insertion of the patent ductus
arteries to the descending aorta is measured. This should arteriosus into the left PA, and (c) supravalvular narrowing
exceed 1.5 for optimum outcome. The pulmonary arteries of the main PA. Diffuse hypoplasia can involve the left or
in TOF frequently show abnormalities of size, confluence, right pulmonary arteries or both. Multiple, peripheral PA
or obstruction. The most important step is to determine narrowing within the lung parenchyma is very rare and
whether the pulmonary arteries are confluent. Once this cannot be detected by echocardiographic scans.
is done, the individual pulmonary arteries are scanned to
look for narrowing. Narrowing can be of two varieties: (a)
Aortic Arch
discrete stenosis and (b) diffuse hypoplasia. The common
sites for discrete stenosis are (a) the point of bifurcation The suprasternal view is used to differentiate left and right
of the main PA, thus involving the origin of the right aortic arches. Right aortic arch is seen in 25% of patients
with TOF (Fig. 72.106). Two methods are used for their relationship of trachea and the ascending aorta in this view
differentiation: (a) In the suprasternal short-axis view, also helps in deciding the side of the arch. The ascending
the transducer is tilted posteriorly. In right aortic arch, aorta is to the left of the trachea in left-sided and on the
the descending aorta mostly falls to the right and in left right side in right-sided aortic arch.
aortic arch the descending aorta mostly falls to the left.
(b) The branching pattern of the head and neck vessels is Patent Arterial Duct and
most important. In left aortic arch, the normal branching
pattern of the first branch of the aortic arch (innominate
Aortopulmonary Collaterals
artery) can be seen dividing into two branches to the (Figs 72.107A and B)
right. The opposite is true in patients with right aortic arch Patent arterial duct is easily defined with color Doppler
with mirror image branching. The fallacy of the second flow as continuous flow in the left PA at its origin. The
method is when patients of right arch have an aberrant duct originates from its usual position in patients with
left subclavian (3%) artery and vice versa (<1%). (c) The TOF with pulmonary stenosis. In patients with pulmonary
atresia, however, the duct is “vertical”, that is, it arises from
the under surface of the arch and descends down, and
then takes a tortuous S-shaped course before joining the
left PA. Echocardiographically, patent arterial ducts in
TOF with pulmonary stenosis are best seen from the high
parasternal views (ductal view), whereas the vertical ducts
are best seen in the suprasternal long-axis views.
Right-sided duct can rarely be seen in patients with
TOF. In such cases, the continuous flow on color flow
mapping is seen in the right PA.
Aortopulmonary collaterals are most often present in
cases of TOF with pulmonary atresia, although they can be
present in patients with TOF with pulmonary stenosis. The
suprasternal views show the collaterals arising from aortic
arch or descending aorta on color Doppler mapping using
Fig. 72.106: Two-dimensional echocardiogram in suprasternal
view showing right aortic arch in a patient with tetralogy of Fallot a low scale as continuous turbulant signal. The presence
showing the division of the first branch toward left side. (LSA: Left of collaterals can also be suspected by profiling the
subclavian artery; LICA: Left internal carotid artery). descending thoracic aorta in its long axis from subcostal
A B
Figs 72.107A and B: Two-dimensional echocardiography. (A) Suprasternal long-axis view showing the origin of a collateral from the
ductal area (arrow); (B) Subcostal sagittal view showing collaterals arising from the descending aorta (arrow). (Des Ao: Descending
aorta; TA: Transverse arch).
window on color flow mapping with a low scale. It is left to right (pink tetralogy). However, the major advantage
usually not possible to further delineate the course of these of the color Doppler is in the evaluation of small additional
collaterals in the lung parenchyma by echocardiography. muscular VSD. In nearly all patients of TOF, the VSD does
not reveal any gradients because of equal right and left
Coronary Artery Anomalies186 ventricular pressures. The only exception is in patients
in whom the VSD becomes restrictive. The gradients
Surgically important coronary artery anomalies are seen in
then will be determined by the severity of the pulmonary
3 to 15% of patients with TOF. The PSAX view is the best to
stenosis. If the pulmonary stenosis is severe, then there
detect these anomalies. Because of the clockwise rotation
would be reverse gradients (from RV to LV) because of
of the aortic root, the right coronary sinus occupies a more
suprasystemic RV pressures.
leftward and anterior location, and left sinus becomes
more posterior. The origin of the right and left coronary Evaluation of pulmonary stenosis (Fig. 72.108): It is difficult
arteries from these sinuses can be seen clearly in PSAX to differentiate by Doppler, the relative contribution of
view. Thus, in patients with normal origin of coronary the infundibular, valvular, supravalvular, and peripheral
arteries, the left main stem is seen to course from a more pulmonary component of the obstruction. It is also
left/posterior location and the right main stem from a more difficult to align the Doppler signal in order to obtain
leftward position. The most common anomalies seen are: the total gradient across the RVOT and pulmonary valve
(a) origin of the LAD coronary artery from the RCA. In the because of their different spatial orientation. Subcostal
PSAX view, this is seen as a large branch arising from the sagittal view of RV outflow is the only view that opens both
RCA and coursing leftward and anterior toward the RVOT. the RVOT and pulmonary valve, and in some patients one
A large coronal branch can also be mistaken for the LAD. may be able to align the Doppler signal accurately. It is
However, in such cases the LAD is seen arising from the important to determine whether the pulmonary circulation
left main branch. (b) Single coronary artery arises from is protected from the high RV pressures.
the right sinus or left sinus, and (c) dual LADs arise from Color flow mapping shows that the turbulence starts at
the left and the right sinuses. In this condition, it may be the subvalvular level and continues to the PA level. Again,
difficult echocardiographically to differentiate the branch color flow fails to differentiate the relative contribution of
arising from the right main stem from a large coronal the various levels of obstruction in TOF.
branch. Poor echocardiographic window, very severe infun-
dibular obstruction, and severe polycythemia are some
causes of inability to document antegrade flow across the
Echocardiographic Measurements in RV outflow by color or pulsed Doppler accurately.
Tetralogy of Fallot1,2,187
The following measurements are routinely performed and
compared to a nomogram based on weight of the patient.
• The pulmonary annulus—This is measured at the site
of insertion of the pulmonary valve leaflets.
• The right and left PA at the hilum just before their
first branching—The suprasternal short-axis view
is selected for measuring the right PA and the high
parasternal or the oblique suprasternal long-axis views
for the left PA.
The Utility of Doppler in Evaluation of

Patients of Tetralogy of Fallot
Evaluation of ventricular septal defect: Ventricular septal Fig. 72.108: Continuous wave Doppler signal showing right ven-
defect (VSD) is usually seen to shunt right to left. If the tricular outflow gradient in a case of tetralogy of Fallot (TOF). Note
pulmonary stenosis is mild to moderate, the VSD shunts the sickle-shaped signal of the infundibulum stenosis (arrow).
Patent arterial duct and aortopulmonary collaterals: example, anomalous origin of one of the PAS from the
These are seen as continuous signals in the PA on color ascending aorta may also need additional imaging
flow mapping. Pulsed Doppler shows continuous high modalities.
velocity signals on the spectral waveform. The PDA is seen In older children, adolescents, and adults with TOF,
opening at the junction of the main PA, left or rarely right inability to delineate anatomical details is an indication
PA. Aortopulmonary collaterals usually open more distally for using other imaging modalities like angiography
beyond the hilum into the PA. MRI/CT. They are also indicated in evaluation of patients
for complete repair of TOF following shunt surgery for
Aortic regurgitation (Fig. 72.109): It is common to see mild
accurate delineation of the PA anatomy and determination
aortic regurgitation in TOF, especially in adolescents,
of PA pressure. It is difficult to predict the PA pressure
adults, and patients who had undergone shunt surgery
accurately by Doppler even if the signals are good.
or have had endocarditis. Associated anomalies include
In the immediate postoperative state, cardiac
presence of ASD in 38% and LSVC in 9% of cases of
catheterization is indicated for accurate delineation of the
TOF. One should keenly look for pulmonary venous
severity and extent of peripheral PA stenosis, residual VSD,
anomalies particularly on 2D echocardiography, because
and for coil occlusion of aortopulmonary collaterals.
with decreased pulmonary blood flow conditions, the
pulmonary veins may not be well visualized on color flow
mapping. Aortopulmonary window (APW) (Fig. 72.110), Postoperative Evaluation of
LVOT obstruction, mitral valve anomalies (Fig. 72.111), Tetralogy of Fallot
have been reported, but these left-sided lesions are very
rare in TOF patients. The postoperative evaluation of a case of TOF includes
evaluation for the residual lesions such as residual RVOT
Indications for Cardiac Catheterization in obstruction (Fig. 72.112), branch PA stenosis, and residual
VSD (Fig. 72.113). Branch PA stenosis is particularly
Patients with Tetralogy of Fallot common at the site of ductal insertion or at the site
In neonates and infants, the suspicion of major of previous BT shunt. It is important to take tricuspid
aortopulmonary collaterals (MAPCAs) and discontinuous regurgitation jet velocities to determine the RV pressure,
pulmonary arteries forms the most important indication as it would reflect any obstructive component in both
for cardiac catheterization. Rarer variations of TOF, for RVOT and branch pulmonary arteries.
Fig. 72.109: Two-dimensional echocardiography in parasternal Fig. 72.110: Two-dimensional echocardiography in parasternal
long-axis view with color flow mapping showing moderate aortic short-axis view at the level of the aorta, profiling a large aorto-
regurgitation and dilated aortic root in a case of grown up tetralogy pulmonary window (arrow) in a neonate with pulmonary atresia
of Fallot. (AO: Aorta; LV: Left ventricle; RV: Right ventricle). (*), and ventricular septal defect. (Ao: Aorta; PA: Pulmonary artery;
perimembranous ventricular septal defect (VSD). valvular pulmonary atresia).
Fig. 72.111: Two-dimensional echocardiography in parasternal Fig. 72.112: Two-dimensional echocardiography with color flow
long-axis view in a case of tetralogy of Fallot showing a large ven- mapping in a patient with tetralogy of Fallot following surgery.
tricular septal defect with overriding aorta. Mitral valve is thickened Parasternal long-axis view with anterior tilt shows significant
and doming is present consistent with mitral stenosis. Also note turbulence in right ventricular outflow tract. (LV: Left ventricle; PA:
the presence of short mitral chordae. (Ao: Aorta; LA: Left atrium; Pulmonary artery; RV: Right ventricle).
LV: Left ventricle; RV: Right ventricle).
A B
Figs 72.113A and B: Two-dimensional echocardiography in parasternal long-axis view with color flow mapping in cases of tetralogy of
Fallot after total correction showing significant residual ventricular septal defect (VSD) from the upper end of the VSD patch (VSD patch
marked by the arrow). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
Small residual VSD may be seen in the region of the to be carefully evaluated in the follow-up of this subset
patch at the site where the sutures are placed far apart to of patients. Another most important aspect to evaluate is
avoid damage to the AV node and the conduction system. the RV function, both systolic and diastolic (Figs 72.116 to
As endothelium grows over the patch, these shunts usually 72.119), which needs to be monitored at each follow-up
disappear generally by the third month, and rarely they visit. The presence of pulmonary regurgitation leads to
may be significant (Figs 72.113A and B). Other types of progressive dilatation of the right sided structures: RVOT, right
shunt that may be seen in postoperative patients include ventricle and right atrium, hence one needs to look keenly at
small insignificant coronary artery fistulas. Significant the RV function parameters (Figs 72.114 and 72.115).
pulmonary regurgitation may be present in a subset of Systolic function may be measured by TAPSE (tricuspid
patients where a transannular patch has been sutured. annular systolic excursion (Fig. 72.117), RV fractional area
This leads to progressive RV dilatation and in some cases, change (Figs 72.118A and B) and systolic indices on tissue
aneurysm of RVOT (Figs 72.114 and 72.115). This needs Doppler (Fig. 72.116).
Fig. 72.114: Two-dimensional echocardiography. Apical four- Fig. 72.115: Two-dimensional echocardiography with parasternal
chamber view in a patient with tetralogy of Fallot with transannular short-axis view in an operated patient with tetralogy of Fallot. A
patch showing dilatation of the right atrial (RA) and right ventricu- transannular patch is placed across the right ventricular outflow
lar (RV) cavity. (LA: Left atrium; LV: Left ventricle). tract. There is dilatation of the right ventricular outflow tract, right.
(PA: Pulmonary artery; RVOT: Right ventricular outflow tract; RA:
Right atrium; RV: Right ventricle).
Fig. 72.116: Tissue Doppler velocity of the annulus of the tricuspid Fig. 72.117: TAPSE: tricuspid annular systolic excursion is a
valve showing normal systolic velocity. measurement of the systolic function of the right ventricle. The
M-mode cursor is placed at the annular attachment of the anterior
septal leaflet.
Evaluation of the diastolic dysfunction must be With diastolic dysfunction there is resistance to right
done in any postoperative patient of TOF as the diastolic ventricular filling which exceeds pulmonary vascular
dysfunction may be present even in the absence of resistance, this leads to transmission of the transtricuspid
systolic dysfunction. Various parameters to assess the atrial flow to pulmonary artery, superior, inferior vena
right ventricular diastolic dysfunction include: (A) Inflow cava and hepatic veins; (C) Flow reversal in hepatic veins
parameters: Diastolic dysfunction can be measured by and SVC can be documented with Doppler signal.Reversal
measuring isovolumic relaxation time, deceleration time, in SVC is best interrogated from subxiphoid sagittal view
E and A wave velocities and EA ratio; (B) Inferior vena cava or suprasternal short-axis view; (D) Transmitted a wave
(IVC) : Normally there should be more than 50% collapse in main pulmonary artery: In parasternal short-axis view
of IVC with inspiration. With diastolic dysfunction IVC the Doppler cursor is placed in main pulmonary artery.
becomes dilated with decreased respiratory variation. The presence of “a” wave following “p” wave on ECG and
A B
Figs 72.118A and B: Right ventricle (RV) fractional area and fractional area change. Measuring the right ventricular area in diastole
(RVD) (A) and subtracting from it the right ventricular area in systole (RVS); (B) The difference divided by the right ventricle area in
diastole gives the fractional area change, a measure of the right ventricular systolic function.
A B
Figs 72.119A and B: Right ventricular diastolic dysfunction. Showing the profiling of inferior vena cava (IVC) from subcostal window:
(A) IVC is dilated; (B) M-mode cursor across the IVC shows no respiratory phasic variation.
present during all phases of respiration characterizes CHD. Like the varied spectrum of DORV, the definition is
atrial flow transmission to pulmonary artery; (E) Tissue also variable.188,189 The commonest definition by Neufield
Doppler diastolic parameters with cursor placed across et al.188 is as follows: Both great arteries and arterial trunks
the tricuspid valve. arise exclusively from the morphological RV, neither
semilunar valve is in fibrous continuity with either AV
DOUBLE OUTLET RIGHT VENTRICLE valve, and usually, a VSD is present and represents the
only outlet from the LV. Pulmonary valve or subpulmonary
Double outlet right ventricle (DORV) encompasses stenosis may be present or absent. Echocardiography has
features of various entities, ranging from simple VSD to a very important role in delineation of cardiac anatomy to
TOF to transposition of the great arteries (TGA). This is a enable planning of surgical strategy for DORV. DORV often
rare anomaly. Its frequency is approximately 0.09 cases occurs in association with a variety of complex situations
per 1,000 births, and it represents 1 to 1.5% of patients with that include atresia of one of the atrioventricular valves and
Classification and Terminology190–195

The determinants of individual subtypes of DORV are
based upon the location of the VSD and great artery
relationship.
Location of the Ventricular Septal Defect

in Relation to the Great Arteries
The location of the VSD determines whether it can be
routed to either of the two great vessels.
Subaortic (Fig. 72.120)

The VSD is located just below the aortic conus and thus
Fig. 72.120: A 5-year-old case of double outlet right ventricle
(DORV) with ventricular septal defect (VSD) and pulmonary
can be routed to aorta. In this case, saturations are better
stenosis. Two-dimensional echocardiography in parasternal long- because saturated blood from LV is directed to the aorta. In
axis view shows the mitral aortic large subaortic VSD, more than the event that the great artery relationship is normal with
50% aortic over ride discontinuity. (Ao: Aorta; LV: Left ventricle; no pulmonary stenosis, the condition may be corrected
like a perimembranous VSD. In presence of pulmonary
stenosis, it can be repaired like a TOF. In the majority of
discordant atrioventricular connection. For this section, cases in this group great vessels are normally related. In
we will confine ourselves to DORV in association with two rare cases, the aorta may be L-malposed.
functioning atrioventricular valves and atrioventricular
concordance.
Subpulmonic (Fig. 72.121)
Definition The VSD is located just below the subpulmonic conus
The diagnosis of DORV (Fig. 72.120) requires the following and thus can be routed to PA. In the majority of cases in
criteria to be fulfilled.188–193 this group the aorta is malposed (i.e. anterior to the PA).
Pulmonary arteries are often not “protected” by stenosis of
DORV infers that both great arteries arise from the RV
the valve or subvalvular region. These patients present like
as the name implies. There is a spectrum of the degree of
classical transposition-Taussing Bing Anomaly. However,
override of one great vessel and herein lies the controversy
pulmonic stenosis may occur if the conal septum between
as to when to classify an origin as double outlet versus
the aortic and pulmonary roots is deviated posteriorly
concordant ventriculoarterial connection. Some authors
and/or to the left.
follow the 50% rule, which means that if a vessel is 50% or
more committed to a chamber, it is considered originating
Doubly committed
from that chamber. However, it is difficult to determine
when exactly an override is 50% or more. To avoid this Here, the conal septum between the aortic and pulmonary
difficulty, some authors diagnose DORV if the override is outflow tract is deficient. As a result, the VSD is located
90% or more. below both outflow tracts and can be routed to either great
Some consider absence of fibrous continuity between vessel.
the posterior semilunar valve and the mitral valve
(Fig. 72.120A). According to some, this is not mandatory. Remote
One can have fibrous continuity of the mitral and one
semilunar valve with criteria of origin of great vessel being Remote VSDs are typically located in the inlet septum
fulfilled. We believe that both features are important for (Fig. 72.122). The tricuspid valve tensor apparatus comes
the diagnosis of DORV, that is, more than 50% override and in the path between the VSD and either of the outflow
mitral aortic discontinuity. tracts. Overriding/straddling of tricuspid valve can occur
Fig. 72.121: Two-dimensional echocardiography. Subcostal view Fig. 72.122: Two-dimensional echocardiography. Apical four-
with anterior tilt showing both great vessels arising from right chamber view in a case of double outlet right ventricle (DORV)
ventricle (RV). Subpulmonary ventricular septal defect (VSD) showing large inlet ventricular septal defect (VSD). (LA: Left
is routable to the pulmonary artery (arrow). (Ao: Aorta; LV: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
ventricle; PA: Pulmonary artery; RV: Right ventricle).
A B
Figs 72.123A and B: Two-dimensional echocardiography in a subxiphoid sagittal view in two cases, showing a ventricular septal defect
located in the inlet septum (arrow) and not related to either great artery origin. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
RV": Right ventricle).
in this type of VSD. This group also includes muscular VSDs • D-malposition of the aorta
that cannot be routed to either great vessel (Figs 72.123A • L-malposition of the aorta.
and B). Various combinations with the above give rise to 16
individual subtypes with 9 being commonly reported.
Additional descriptive features should include the state
Great Artery Relationship to one Another
of the outflow tracts to each great artery and associated
The following four possibilities exist in DORV: anomalies. The echocardiographic description should
• Normal great artery relation include a statement on whether or not a two-ventricle
• Side-by-side great artery relationship repair is feasible.
Establishing a Diagnosis of pulmonary annulus is commonly associated, and to

a varying degree, hypoplasia of the pulmonary trunk.
Double Outlet Right Ventricle by Echocardiographically, malalignment of outlet septum is
Echocardiography194 recognized by subcostal sagittal or coronal views. Apical
The following needs to be specifically evaluated: five-chamber and PLAX views show malalignment of outlet
• VSD septum very well in the group of patients with transposed
• Subpulmonary outflow tract great vessels. By Doppler echocardiography, the severity
• Subaortic outflow tract and arch of pulmonary stenosis is best assessed in the subcostal
• Coronary arteries. coronal view in the patients with normally related vessels
and in the apical views in those with d-malposed great
Subaortic Ventricular Septal Defect vessels.
In the subcostal sagittal view, the aorta will be seen to arise

predominantly from RV. In this view, the tricuspid valve is
Aortic Outflow Obstruction
seen to form the posteroinferior margin. Its attachments Stenosis of the subaortic region may result from anterior
can be variable. In some cases, some chordae can gain malalignment of the conal septum. This is seen in the
attachment to the outlet septum. The subpulmonary group of patients with malposed great vessels. They rarely
outflow tract will not be seen in this view as it is more produce any Doppler gradients. Typically, the aortic
anterior. This view also shows the size of the VSD (discussed annulus, ascending and transverse arches are smaller than
later). In the subcostal coronal view, both great vessels normal, and coarctation is a common association. The
can be seen arising from the RV, with the subpulmonary following measurements are important: aortic annulus,
outflow tract anterior and to the left of the aortic outflow ascending aorta, transverse arch, distal transverse arch,
like in TOF. aortic isthmus, and the narrowest dimension of the
The PLAX and apical five-chamber views also clearly coarctation site if present.
demonstrate the degree of override of aorta like in tetralogy
as well as aortic mitral discontinuity.
Subpulmonary Ventricular
Septal Defect (Taussig–Bing Anomaly)
In this condition, the aorta is anterior (malposed) in
majority of cases. In the subcostal sagittal view, the great
vessel overriding the septum is the PA. The aorta will not be
seen or will come under view in a more rightward sweep.
In the coronal section, both great vessels will be seen
having a parallel origin with the aorta to the right and PA
to the left. Pulmonary valve and mitral valve discontinuity
will also be obvious.
Stenosis of the Pulmonary Outflow Fig. 72.124: Two-dimensional echocardiogram from subcostal
short-axis view with color compare showing restricted subaortic
The commonest cause of obstruction is malalignment
ventricular septal defect (VSD) in a case of double outlet right ven-
of outlet septum. In normally related great vessels the tricle (DORV). VSD size is smaller (small white arrows) than the
outlet septum is anteriorly malaligned and in transposed aortic annulus (block yellow arrows) and routing will require VSD
great vessels the outlet septum is posteriorly malaligned, enlargement. Tricuspid valve () does not come in the pathway
from the left ventricle to aorta.
resulting in subpulmonary stenosis. Hypoplasia of
Table 72.14: The Complete Checklist for Preoperative Assessment of Double Outlet Right Ventricle
Visceral and atrial situs, sequential chamber relationship
Systemic and pulmonary veins, atria and atrial septum
Atrioventricular valve morphology, attachments of the valve tissue and function
The ventricles: size and function
The ventricular septum: location of ventricular septal defects, size of the ventricular septal defect as compared to the aortic annulus,
“routability” of ventricular septal defect, presence or absence of atrioventricular valve tissue in the way between the ventricular
septal defect and the great artery (at the site of the prospective patch), presence or absence of conal tissue that may obstruct the
prospective patch (which may therefore require resection)
The conotruncus: This includes the outflow tracts that lead to the great vessels and the great vessel origins including the semilunar
valves; the presence or absence of obstruction in either of the outflow tracts, the nature of obstruction if present, the severity of
obstruction; and the sizes of the aortic and pulmonary valve annuli
The status of the branch pulmonary arteries
The presence or absence of patent ductus arteriosus
The aortic arch: side, branching, and the presence or absence of coarctation
Feasibility of a two-ventricle repair (with reasons)
Likelihood for requirement of a conduit
Coronary artery anatomy
Restriction of the Ventricular mitral or tricuspid valve annulus should be determined

and compared to normal. A size of < 2 standard deviations
Septal Defect (Fig. 72.124) of either of the annuli merits concern.
Mild restriction in size of VSD is quite common but rarely • Straddling of either of the AV valves (Fig. 72.125).
severe to produce a Doppler gradient across the VSD Typically, straddling of the tricuspid valve is associated
(between left and RV). VSD is defined as small when its with an inlet defect and/or hypoplasia of the RV.
size is smaller than the aortic annulus. Straddling of the mitral valve may occur in patients
with outlet VSD
• Confluent adequate-sized pulmonary arteries: Meas-
Remote Ventricular Septal Defect urements of pulmonary annulus, pulmonary trunk, and
One should suspect a remote VSD if while visualizing the branch pulmonary arteries should be made like in TOF
great vessels, the VSD is not seen. Inlet VSDs are best seen • The VSD should be suitably located for intraventricular
in the subcostal views in a more posterior plane along with re-routing. If pulmonic stenosis is present, the VSD will
the tricuspid valve. In the apical 4C view, the VSD is seen be routable to the aortic root without AV valve tissue in
in the plane that visualizes the mitral and tricuspid valves the way. In the absence of pulmonic stenosis, the VSD
(crux). In this view, as one sweeps anteriorly toward the should be routable to either the aorta or the PA. This is
great vessels, the VSD disappears from view. Overriding where echocardiography provides critical information
that is often not available even on angiography
and straddling of tricuspid valve can be well visualized in
• Atrioventricular valve tissue in the way between VSD
this view.
and the aorta (Figs 72.126 and 72.127; or PA in the case
of a Taussig–Bing anomaly). Careful subxiphoid and
Role of Echocardiography in Planning PSAX sweeps help in evaluating the location of the
Surgical Strategy (Table 72.14)195–198 prospective baffle and whether the AV valves will come
in the way
The aim of evaluation is to ensure that a two-ventricle repair • Inlet VSD is not an absolute contraindication for two-
is carried out if and when possible. Both the long-term ventricle repair. It is possible to route remotely located
survival and functional status after two-ventricle repair VSDs with tricuspid valve tissue in the way using
are superior to patients who undergo the single ventricle innovative surgical methods such as the multiple
repair.196–198 Two good-sized ventricles: Hypoplasia of patch technique.
either of the ventricles clearly rules out a two-ventricle If the patient is a candidate for two-ventricle repair,
repair (Fig. 72.130). In borderline situations, the size of the additional specific issues need to be addressed. They include:
Fig. 72.125: Two-dimensional echocardiography. Subcostal sagi- Fig. 72.126: Two-dimensional echocardiography in subcostal
ttal view in a case of situs inversus with mesocardia showing a coronal view with anterior tilt in a case of double outlet right
large ventricular septal defect with hypoplasia of the right-sided ventricle, ventricular septal defect (VSD), and pulmonary stenosis.
left ventricle. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery; Note the presence of tricuspid valve tissue in the pathway from left
RV: Right ventricle). ventricle (LV) to aorta (arrow). (Ao: Aorta; LV: Left ventricle; RV:
Right ventricle).
A B
Fig. 72.127: Two-dimensional echocardiogram. Subcostal short-axis view with anterior tilt and color compare in a case of double outlet
right ventricle (DORV) with a large subaortic ventricular septal defect (VSD) and pulmonary stenosis. Presence of tricuspid valve (ar-
row) in the pathway from left ventricle (LV) to aorta resulting in restriction of the VSD. (Ao: Aorta; LV: Left ventricle; RV: Right ventricle).
• Origin of LAD coronary artery in the TOF type of • Other associated anomalies that may need to be
DORV (with subaortic VSD and normally related great addressed need proper evaluation.
arteries). If the LAD coronary artery is seen to cross in The postoperative evaluation of such cases is essen-
front of the RVOT, a conduit or a modified technique tially the same as that described under TOF repair. Post-
for relieving RV obstruction may be required. operative evaluation involves:
The origin of coronary arteries in the Taussig–Bing • Checking for residual lesions: residual VSD, additional
anomaly prior to an arterial switch operation. VSD
• The incidence of atypical coronary artery origin is • Checking for pulmonary stenosis and RVOT gradient
higher in the Tausig–Bing anomaly as compared with (or RV–PA conduit gradient; Fig. 72.128). The gradient
classical transposition across RVOT may be aligned in the subcostal sagittal
• Presence or absence of additional muscular VSDs that view with anterior tilt and in paracoronal and PSAX
may require to be closed at the time of surgery should views. Tricuspid regurgitation velocity may give a good
be looked for indication of the presence of distal obstruction
• Evaluation of branch pulmonary arteries TRUNCUS ARTERIOSUS

• Looking for RV systolic and diastolic function (as
explained earlier)
(FIGS 72.128, 72.131 TO 72.134)
• Looking for the presence of LVOT gradients (generally A common arterial trunk arising from the base of the
across the VSD) heart, and giving origin to aorta, pulmonary arteries, and
• In case of BT shunt, evaluate the patency of the BT coronaries is referred to as truncus arteriosus. A single
shunt (Figs 72.129 and 72.130) and look for distortion semilunar valve is found in truncus arteriosus, and this
of the pulmonary arteries valve differentiates truncus arteriosus from aortic and
• In case of a PA band, estimate the band gradients to pulmonary valve atresia, conditions in which a single
decide the adequacy of the band and PA pressures. arterial vessel also receives the entire output of both
One should also look for migration of PA band and ventricles but in which a second atretic semilunar valve
distortion of pulmonary artery branches. is present. Truncus arteriosus is usually associated with a
large VSD resulting from absent or deficient outlet septum.
In 25% of cases, the defect may extend to the membranous/
perimembranous area. Rarely, the VSD may be restrictive
or even absent. According to the initial classification given
by Collet and Edwards199 in 1948, truncus may be classified
as following:
Type I: Origin of a main pulmonary trunk from the
lateral aspect of the common trunk.
Type II: Left and right pulmonary arteries originate
separately from the common trunk. The origin is
close to each other, or from a common orifice,
usually located on the posterior aspect of the
common trunk.
Type III: Origin of only one PA from the common trunk,
the other PA can originate from the ductus
arteriosus or directly from ascending aorta
(MAPCA artery).
Fig. 72.128: Two-dimensional echocardiogram in parasternal Type IV: It is defined not by the pattern of origin of
short-axis view with color flow mapping in an operated patient with pulmonary arterial branch but rather by the
pulmonary atresia and ventricular septal defect (VSD) showing coexistence of an interrupted aortic arch or
conduit obstruction (arrow). (Ao: Aorta; RV: Right ventricle).
aortic hypoplasia or preductal aortic coarctation.
A B
Figs 72.129A and B: Two-dimensional echocardiography. Suprasternal short-axis view showing a patent left BT shunt (arrow) to left
pulmonary artery. (LPA: Left pulmonary artery); (B) Color Doppler flow mapping in the same patient showing flow through the left BT
shunt indicating its patency.
Fig. 72.130: Two-dimensional echocardiography with color flow

mapping suprasternal short-axis view showing the right BT shunt
(arrow) to right pulmonary artery. (RPA: Right pulmonary artery).
A B
Figs 72.131A to C: Two-dimensional echocardiography. (A) Sub-

costal coronal view with anterior tilt in a child with truncus arte-
riosus Type I, giving rise to aorta and pulmonary artery (arrow)
showing the presence of single outflow truncus (Tr) overriding the
ventricular septal defect (marked by star); (B) Parasternal long axis
view showing the presence of single outflow truncus (Tr) with main
pulmonary artery stump arising from the truncus ( arrow); (C) Par-
asternal short axis view with color flow mapping in the same patient
showing the common trunk (MPA) giving rise to both the pulmonary
arteries). (RV: Right ventricle; LV: Left ventricle; Tr: Single outflow
C truncus; Ao : Aorta).
In these cases, a well-documented correlation Type A signified the presence of VSD while type B signified
with 22q11 deletion syndrome is noted. the absence of VSD. Their type A1 corresponds to Type I
Van Praagh and van Praagh have proposed an of Collett and Edwards, and type A2 encompasses Types
expanded classification system that also includes two II and III. Type A3 includes cases with absence of truncal
commonly associated abnormalities of the great arteries. origin of one PA, with blood supply to that lung from the
Fig. 72.132: Two-dimensional echocardiography. Parasternal Fig. 72.133: Two-dimensional echocardiography. Subcostal coronal
short-axis view from an infant with persistent truncus arteriosus view with anterior tilt showing the origin of the main pulmonary artery
showing tricuspid truncal valve. (LA: Left atrium; RA: Right atrium; from the lateral aspect of the common trunk (arrow). There is flow
T: Truncal valve). acceleration in the main pulmonary artery consistent with flow re-
striction. (PA: Main pulmonary artery trunk; TR: Truncus; Ao: Aorta).
A B
Figs 72.134A and B: Two-dimensional echocardiography in high parasternal short-axis view with color compare in a case of persistent
truncus arteriosus type I with confluent branch pulmonary arteries. (LPA: Left pulmonary artery; RPA: Right pulmonary artery; T: Truncal
valve).
ductus arteriosus or from a collateral artery. Lastly, type Echocardiography

A4 is associated with underdevelopment of the aortic
arch, including tubular hypoplasia, discrete coarctation,
(Figs 72.131 to 72.134)
or complete interruption.200 Modified Van Praaghs Intracardiac anatomy usually reveals situs solitus and AV
classification was accepted by Congenital Heart Surgery concordance. Two balanced ventricles are usually present
Nomenclature Database Project 2000 and classified into and separated by a large VSD. Truncus arteriosus has
type A1-2, type A3( hemitruncus) and type A4. Dr Anderson been described with tricuspid atresia, hypoplastic double
et al. proposed a simplified classification classifying truncus inlet ventricle, and a very rare form with discordant AV
into Aortic dominant type and pulmonary dominant type. connection.
Two-Dimensional Echocardiography201 from the trunk. Truncal valve can be tricuspid (60–67%),
quadricuspid (25–31%), or bicuspid (8%). Valve leaflets
Subcostal coronal view with anterior tilt shows a common are usually thickened. The truncal valve is in continuity
trunk arising from the heart, and overriding the VSD. with the anterior mitral valve leaflet in the PLAX view. The
Subcostal paracoronal view can profile the pulmonary truncal valve may be normal, stenotic, and/or regurgitant.
artery arising from the trunk and bifurcating into right Rarely, there can be absence of the pulmonary arteries.
pulmonary artery and left pulmonary artery from lateral From suprasternal view, the aortic arch anatomy
aspect of the trunk. Apical four-chamber view with should be properly defined to ascertain the side of arch
anterior tilt will profile the common trunk arising from the (right aortic arch is reported in 25–36% of cases), branching
heart and overriding the VSD. PLAX view is the best view pattern, and associated coarctation or arch interruption.
to profile truncal anatomy. This view shows the common
trunk arising from the heart committed to both ventricles Doppler Imaging
and overriding the large VSD. Slight posterior tilt from a Color flow Doppler mapping shows regurgitation or
standard PLAX view shows the origin of main PA from the stenosis of truncal valve and any stenosis at the origin
posterolateral aspect of the common trunk. PSAX view at of pulmonary arteries. With continuous wave Doppler,
the base of heart shows the truncal valve in cross section, gradient across the truncal valve and pulmonary arteries
large outlet VSD, and pulmonary arteries as they arise can be recorded.
PART 7: COMPLETE TRANSPOSITION OF GREAT ARTERIES
TRANSPOSITION OF GREAT VESSELS (TGA) Anatomy

TGA means discordant ventriculoarterial connection, Among the characteristic features generally recognized
that is, origin of great vessels from inappropriate on echocardiography is the parallel orientation of ventri-
ventricles. TGA accounts for 5–7% of all congenital cular outflows (both the PA and the aorta) unlike normal
cardiac malformations. Aorta arises from morphological hearts where they cross (Figs 72.135A and B). The
RV and pulmonary arteries from the morphological LV. pulmonary valve is not wedged as deeply between the two
It is not based on the spatial interrelationship of the atrioventricular valves as is the aortic valve in the normal
great arteries.202–204 heart. The area of offsetting of atrioventricular valves and
A B
Figs 72.135A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view with anterior tilt in a neonate
with complete transposition of great vessels (TGA) showing ventriculoarterial discordance and the presence of parallel great vessels.
There is acute posterior angulation of the pulmonary artery as it arises from the left ventricle. (LV: Left ventricle; RV: Right ventricle; Ao:
Aorta; PA: Pulmonary artery).
perimembranous septum is less marked. LVOT is longer

than in a normal heart but not to the extent of AV canal
defect. In about 40 to 45% of cases there is an associated
VSD. The entire ventricular septum is a straight structure
rather than curved as in the normal heart.204,205
In the normal heart, aortic root is right and posterior to
pulmonary trunk with situs solitus and left and posterior
to pulmonary trunk with situs inversus. The most obvious
external abnormality is relation of great vessels with aorta
being generally anterior. The commonest relation is aortic
root right and anterior to pulmonary trunk (>80% of cases)
with situs solitus and left and anterior to pulmonary trunk
in situs inversus.202,203 Aortic valve is usually superior
to pulmonary trunk as it is supported by a muscular
infundibulum and the muscular infundibulum on the Fig. 72.136: Two-dimensional echocardiography in subcostal
left side is mostly absorbed leading to mitral–pulmonary view in a case of complete transposition of great vessel (TGA) with
continuity. However, conal anatomy can vary as a there color flow mapping, showing a restricted patent foramen ovale
(PFO; arrow). (LA: Left atrium; RA: Right atrium).
can be bilateral subarterial conus, subpulmonary conus
with absent subaortic conus, and rarely bilaterally absent
conus. Variations in great vessel relationship are reported arising from RV. Subcostal sagittal view in different planes
more commonly with a VSD, anterior-posteriorly related profiles the discordant ventriculoarterial connection,
great vessels, aortic root left and anterior, right and parallel course of great vessels, and conal anatomy.
posterior or left and posterior to pulmonary trunk. In patients with bilateral conus, fibrous discontinuity
between both semilunar valves and AV valves is present,
Associated Lesions202-205 usually there is associated VSD and variation in great
vessel relationship, and coronary pattern may coexist. This
Nearly half of the patients with d-transposition of great
conal anatomy can be well profiled in subcostal sagittal,
vessels have no other associated anomaly except a
coronal, and PLAX views. PLAX view demonstrates
persistent foramen ovale or ASD and PDA (Fig. 72.136).
parallel relation of great vessels and abrupt posterior turn
VSD is the commonest associated anomaly (40–45%);
of pulmonary trunk immediately after origin (Fig. 72.135).
combination of VSD and LVOT obstruction (pulmonary
The parallel arrangement of great vessels is suggestive
stenosis) are observed in 10%, and an isolated LVOT
of d-transposition of great vessels, but is not diagnostic,
obstruction in approximately 5% of cases. Less commonly
as it can be found in other ventriculoarterial connections
encountered anomalies are AV valve abnormalities, aortic
such as DORV with malposed great vessels. High PSAX
obstructions, arch anomalies, and anomalies of systemic
and modified suprasternal long-axis views also profile the
and pulmonary venous connections.
origin of aorta from the morphological RV.
In a normal heart, great vessels have a wrap around
Echocardiographic Evaluation relationship, that is, pulmonary trunk arises from RV and
takes a leftward turn while aorta after arising from the LV
Two-Dimensional Echocardiography turns to the right (circle sausage appearance; Figs 72.137A
The usual segmental analysis is essential as for any other and B). With normally related great vessels, in PSAX view,
congenital cardiac anomaly. After defining the situs, aorta is right and posterior to pulmonary trunk. With
subcostal coronal and apical four-chamber views provide complete transposition of great vessels, both outflows
the AV connection. Anterior tilt from subcostal coronal are in parallel as seen in PLAX (Figs 72.138A and B) and
and apical four-chamber views profiles first the posteriorly subcostal sagittal views. PSAX view profiles the spatial
placed great vessel that bifurcates into two, that is, PA arising relationship of great vessels, as double circles, aorta right
from LV with mitral–pulmonary continuity (Fig. 72.135). and anteriorly or directly anterior and less commonly
Further angulation profiles the anteriorly placed great aorta left and anterior and posteriorly placed pulmonary
vessel with muscular infundibulum (subaortic conus), trunk. As both outflows are at different levels, usually both
A B
Figs 72.137A and B: Two-dimensional echocardiography in parasternal long-axis view (Figure A) and parasternal short-axis view
(Figure B) at the level of the great vessels from an infant with transposition of great vessel (TGA) showing right and anterior position
of the aorta as compared to the pulmonary artery. Arrow shows the origin of the left main coronary artery. (Aorta: Ascending aorta; PA:
Pulmonary artery). (RV: Right ventricle; LV: Left ventricle).
A B
Figs 72.138A and B: Two-dimensional echocardiogram in subcostal coronal view with anterior tilt. (A) Shows the origin of the aorta from
the right ventricle; (B) Shows the origin of the pulmonary artery (and pulmonary artery branching) from the left ventricle. (Ao: Aorta; LV:
Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
semilunar valves cannot be profiled in the same plane. increase and left ventricular posterior wall thickness starts
Main PA and its branches are seen at level of a cross section regressing as the LV is supporting the pulmonary circulation
across aortic valve, and a cross section across pulmonary (Figs 72.140A and B). The RV is systemic ventricle and LV
valve provides an image of RVOT. Apical four-chamber is pulmonary ventricle in complete transposition of great
view may demonstrate the initial outflow tract dividing vessels with intact ventricular septum. Four-chamber and
into branch pulmonary arteries followed by opening of the subcostal coronal views show dilated RA and RV and the
aorta in a further anterior tilt (Figs 72.139A and B). interventricular septum bulges toward the LV, giving the
configuration of a banana to the LV (banana-shaped LV).
The motion of the upper part of the ventricular septum is
Chamber Size206–210
normal in 50% of patients, that is, moving posteriorly in
After birth in simple (or complete) transposition of great systole while in the remainder, septal motion is abnormal,
vessels with intact ventricular septum, with the fall in PVR remaining flat or moving anteriorly during systole and this
during first few weeks of life, left ventricular mass does not may also create a dynamic form of subpulmonary stenosis
A B
Figs 72.139A and B: Two-dimensional echocardiography in apical four-chamber view with anterior tilt showing the origin of pulmonary artery
(PA) from the left ventricle (LV). Further anterior tilt shows the origin of aorta (Ao) from right ventricle the (RV). Ventricular arterial discordance.
A B
Figs 72.140A and B: Two-dimensional echocardiography in parasternal long-axis view in a 1-year-old patient with transposition of great
vessel (TGA) with intact ventricular septum. Shows the thinned out posterior wall of the left ventricle (2.7 mm). M-mode echocardiogra-
phy of the same showing the regressed left ventricle (LV).
often associated with systolic anterior motion of the mitral • LV volume index
valve. These findings may be seen in apical four-chamber • LV mass/volume ratio.
view with anterior tilt and PLAX views. On M-mode, With intact interventricular septum, RA and RV
systolic anterior motion of the anterior leaflet of mitral are always dilated as seen in apical four-chamber and
valve is easily recognized. subcostal coronal views. In subcostal sagittal and PSAX
Features to be assessed by echocardiography while views, RV dominates with a circular configuration while
evaluating a child with complete transposition of great the LV takes on a crescentic shape.
vessels with intact ventricular septum for primary arterial In case of elevated left ventricular pressure as with
switch operation include:211,212 a nonrestrictive VSD, large PDA, large aortopulmonary
• LV geometry collaterals, significant left ventricular outflow obstruction,
• LV mass index (Table 72.15) or pulmonary arterial hypertension, the circular shape of
• LV posterior wall thickness index (<0.3 mm taken as LV will be maintained with normal posterior wall thickness
regressed) and LV mass.
Table 72.15: Left Ventricular Mass and Volume Indexed to Body Surface Area in m2.204
Volume (mL) End- Two-dimensional (2D) Mass M-mode (g) Mass/Volume Index
diastole (g) End-diastole End-diastole (g/mL)
Newborns 31.3 ± 6.1 47.7 ± 12.7 59.8 ± 21 1.57 ± 0.4
Infants 38.7 ± 5 48.8 ± 8 56.6 ± 15 1.27 ± 0.2
Children (1–4 years) 54.6 ± 7.6 58.6 ± 10.3 61.9 ± 11.5 1.08 ± 0.2
Children (4–8 years) 59.8 ± 6.9 61.9 ± 7.1 69 ± 24 1.05 ± 0.1
Children (8–12 years) 64 ± 6 66.9 ± 7.6 88.2 ± 20 1.04 ± 0.1
Children > 12 years 66.4 ± 7.8 74.1 ± 9.8 91.2 ± 40 1.1 ± 0.1
A B
Figs 72.141A and B: Two-dimensional echocardiography showing balloon atrial septostomy with echocardiographic imaging in a case
of transposition of great vessels (TGA). (A) Subcostal sagittal view showing the catheter in inferior vena cava (IVC). (B) Subcostal coro-
nal view showing the inflated balloon in left atrium. (B: Balloon; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Associated Defects mixing, and with large VSD or PDA to decrease left
atrial pressure and provide good mixing at the level of
Septal Defects atria. In case of restrictive interatrial communication,
echocardiography shows a dilated LA, interatrial septum
Interatrial Communication: Most commonly it is a patent bowing toward the RA, and turbulent flow across the
foramen ovale. Fossa ovalis ASD occurs only in 5% of cases interatrial communication with Doppler color flow
with complete transposition of the great vessels. Interatrial mapping. PW Doppler interrogation reveals gradient
communication as a site of bidirectional shunting results across the interatrial communication. Echocardiography-
in good intracardiac mixing if ASD is of adequate size. guided balloon atrial septostomy to enlarge the interatrial
Subcostal sagittal and coronal views are used to profile the communication can be done in the intensive care setting
atrial communication and color flow mapping is used to in neonates if the interatrial communication is restrictive
assess the direction of shunt, which is usually bidirectional and arterial switch operation is not planned immediately
(left to right during systole, i.e. with an atrial “v”-wave, (Figs 72.141A and B). 2D echocardiogram from subcostal
and right to left during diastole, i.e. with atrial “a”-wave). sagittal view is used to profile the course of balloon catheter
M-mode with color flow mapping across the ASD is the from IVC to RA and then to LA. Subcostal coronal view is
best method to profile the direction of shunting. It is very used to profile the catheter course during septostomy and
important to assess adequacy of interatrial communication to determine the size of ASD and flow across ASD after the
with intact ventricular septum or small VSD for better procedure.
Ventricular septal defect: Features of VSD to be assessed septum, there is potential for septal leaflet of tricuspid
include: valve to get prolapsed into the subpulmonary area and
• Location of VSD. cause subpulmonary stenosis. Other types of defects that
• Size of VSD. can be found are of AV canal type, multiple muscular,
• Direction of shunt. large apical muscular, and doubly committed VSDs. With
• Relation of AV valves to VSD. a doubly committed defect, aorta is usually left-sided and
• Relation of outflow tracts to VSD. there are increased chances of the presence of coronary
The most significant and frequently occurring defect in anomalies.
complete transposition is a VSD, which can be small, large, On echocardiographic evaluation, the following need
single, or multiple (Fig. 72.142). The most characteristic to be assessed in patients with TGA:
defects are those that open beneath the ventricular outlets • VSD
with the muscular outlet septum malaligned with the rest • Anomalies of AV valves
of the ventricular septum with overriding of one of the great – Attachment of tricuspid valve chordae to outlet
vessels, most commonly the PA (posterior great vessel). septum or to the crest of ventricular septum
These defects can have a posteroinferior muscular rim or – Overriding of AV valve
may extend to perimembranous area. Frequently, these – Straddling of AV valves
defects are crossed by the tension apparatus of tricuspid – Any evidence of a cleft in an AV valve
valve, which often inserts to a papillary muscle arising from • Outflow tract abnormalities
the outlet septum. The outlet septum can be malaligned – Outflow tract obstruction.
to LV causing pulmonary stenosis or anteriorly causing
Outlet trabecular defect: Scanning from subcostal sagittal,
subaortic narrowing. In cases of anterior malalignment,
subcostal coronal, and PLAX views would visualize a
arch anomalies such as CoA or arch interruption may be
defect in outlet septum with or without extension to
associated. The latter anomalies are more commonly seen
perimembranous area, intact subaortic infundibulum,
in DORV with subpulmonary VSD, a close differential
and overriding PA. In addition, malalignment of outlet
diagnosis of TGA. The VSD can extend to the inlet septum.
septum should be looked for. Outlet VSD with posterior
These defects are hidden beneath the septal leaflet of
malalignment (subpulmonary narrowing) can be profiled
tricuspid valve. When the defect extends into inlet septum,
from subcostal coronal view with anterior tilt, subcostal
there is potential for straddling and overriding of tricuspid
sagittal view of left ventricular outflow, and PLAX view.
valve. With perimembranous VSD extending to inlet
Because of the posterior deviation of outlet septum, there is
a direct route from LV to aorta making these children good
candidates for intraventricular re-routing from LV to aorta.
While evaluating for intraventricular re-routing of LV to
aorta, size of VSD in relation to aortic valve annulus needs
to be assessed in addition to relation of AV valves to VSD. If
the VSD is smaller in relation to aortic valve annulus, then
it needs enlargement during intraventricular repair. In the
presence of straddling of AV valve, biventricular repair will
not be possible and the patient would need to undergo a
Fontan procedure.
Anterior malalignment of outlet septum (subaortic
narrowing) can also be profiled from subcostal sagittal
view, subcostal coronal view of RV outflow, and PLAX
view. The anterior malalignment of outlet septum causes
subaortic narrowing and produces a long, oblique course
from the LV to aorta. This makes intraventricular repair
Fig. 72.142: Two-dimensional echocardiography from a one and
half-month-old child with transposition of great vessels (TGA). extremely difficult. With anterior malalignment of outlet
Parasternal long-axis view showing the presence of a large peri- septum, chances of arch anomalies are higher so a careful
membranous ventricular septal defect () and posterior malalign- evaluation of aortic arch from suprasternal long-axis view
ment of the septum (arrow). (AO: Aorta; LA: Left atrium; LV: Left is mandatory to rule out a hypoplastic transverse arch,
ventricle; RV: Right ventricle PA: Pulmonary artery). coarctation, or arch interruption.
With outlet VSD having perimembranous extension,

septal leaflet of tricuspid valve can prolapse across the
VSD into subpulmonary outflow leading to subpulmonary
narrowing, as mentioned previously. This can be profiled
from subcostal coronal and apical four-chamber views
with anterior tilt.
Inlet ventricular septal defect: Inlet VSD can be best
profiled from four-chamber views (apical or subcostal
coronal). Four-chamber (subcostal coronal and apical)
views are also useful to profile overriding of AV valves
and straddling of tricuspid valve if present. In addition, a
modified subcostal sagittal view at the level of AV valves
(en face view) can also define the presence of overriding
or straddling. Mitral valve straddling can also be profiled
Fig. 72.143: Two-dimensional echocardiography. Suprasternal
from PLAX view as the mitral valve chordae cross the VSD
view in a 6-day-old child with complete transposition of great ves-
and insert on the opposite side of septum. sels showing a large patent ductus arteriosus (PDA; arrow). (Des.
Ao: Descending aorta; PA: Pulmonary artery).
Doubly committed ventricular septal defect: Doubly
committed VSD is best profiled from a combination of
subcostal coronal and sagittal views, and PLAX and PSAX With restrictive PDA, color flow mapping reveals
view. These views show the defect in outlet septum and turbulent flow from aorta to PA. With continuous wave
presence of aortic and pulmonary valve continuity. Doppler interrogation of ductus, a high velocity continuous
Scanning from subcostal (coronal and sagittal), apical, signal peaking in late systole is obtained.
and parasternal (long-axis and short-axis) views are used
to define muscular VSDs.
Color flow mapping helps in defining the direction of
Left Ventricular Outflow Tract Obstruction213
shunt. With nonrestrictive VSD, direction of shunting is Left ventricular outflow obstruction can occur with an
left to right during diastole and right to left during systole. intact ventricular septum as well as in the presence of a
With restrictive VSD, a turbulent jet from right to LV will VSD. Such lesions can be found at the level of valve or can
be found. Continuous wave Doppler interrogation shows a be subvalvular. Isolated valvular stenosis is rare and usually
pressure gradient across the VSD as well as the direction of occurs in combination with subvalvular obstruction,
shunt. which can be dynamic, fixed, or both.
Patent ductus arteriosus (Fig. 72.143): Suspicion of Dynamic left ventricular outflow obstruction (Fig. 72.144):
presence of ductus arteriosus in complete transposition With intact ventricular septum or small VSD, the RV has
occurs when on color flow mapping, reverse flow is seen in systemic pressure; interventricular septum may bulge
PA from subcostal coronal with anterior tilt and subcostal toward LV in systole, causing dynamic subpulmonary
sagittal views. In complete transposition of great vessels, obstruction. In addition, this dynamic subpulmonary
because of the altered spatial relationship of great vessels, obstruction causes systolic anterior motion of anterior
the total length and anatomy of ductus is easily profiled leaflet of mitral valve, and midsystolic closure of
from suprasternal long-axis view. Color flow mapping and pulmonary valve. 2D echocardiography in apical four-
pulsed Doppler interrogation is used to define direction chamber, subcostal coronal, and PLAX views shows
of shunting and magnitude of shunt across the ductus. bulging of interventricular septum toward LV during
With a large PDA, as PA pressures are systemic, direction systole. M-mode through the mitral valve clearly reveals
of shunting is bidirectional, PA to aorta during systole systolic anterior motion of anterior leaflet of mitral valve
and aorta to PA during diastole if the PVR is lower than and across the pulmonary valve shows midsystolic valve
systemic. With the development of pulmonary vascular closure. Color flow mapping reveals mild turbulence
obstructive disease, direction of shunt becomes PA to across LVOT, and continuous wave Doppler usually
aorta during diastole too. reveals pressure gradients. In its severest form it behaves
Fig. 72.144: Two-dimensional echocardiography. Parasternal Fig. 72.145: Two-dimensional echocardiography with parasternal
long-axis view in a case of complete transposition of great vessels long-axis view showing posterior malalignment of the ventricular
(TGA) with intact interventricular septum showing systolic anterior septum leading to subvalvular left ventricular outflow tract obstruc-
motion of the mitral valve (arrow). This creates a dynamic left ven- tion (arrow) in a case of transposition of great vessels (TGA) with
tricular outflow tract obstruction. (Ao: Aorta; IVS: Interventricular ventricular septal defect (VSD) and pulmonary stenosis. (Ao: Aor-
septum; LV: Left ventricle; PA: Pulmonary artery). ta; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
like hypertrophic cardiomyopathy. The obstructive lesion

associated with septal bulging is more likely to occur with
anteroposteriorly related great vessels than side-by-side–
related great vessels. Milder form of septal bulging can be
exaggerated by presence of fibrous ridge located on the
septal bulge.
Fixed anatomical obstruction: Fixed LVOT obstruction,
if significant, results in LV becoming spherical and thick
walled.
Fixed obstruction occurs because of:
• Subpulmonary fibrous ridge/fibrous subpulmonary
membrane or ring. 2D echocardiography in four-
chamber (subcostal coronal and apical) views with
anterior tilt and PLAX view reveals presence of discrete
ridge/membrane in subpulmonary area. This fibrous Fig. 72.146: Two-dimensional echocardiography using subcostal
coronal view with anterior tilt in a child with complete transposition
ridge can be elongated giving a tunnel type lesion.
of great vessels (TGA) showing prolapse of accessory mitral valve
On color flow mapping, turbulence starts below the tissue into the left ventricular outflow tract (LVOT, arrow), causing
pulmonary valve, and on pulsed or continuous wave obstruction. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
Doppler, severity of stenosis can be graded. RV: Right ventricle).
• Valvar pulmonary stenosis is usually due to a bicuspid
pulmonary valve. 2D echocardiography in PSAX view
can demonstrate the bicuspid pulmonary valve. Color • All these lesions can occur with intact ventricular
flow mapping reveals that turbulence starts at the level septum as well as with VSD.
of pulmonary valve. • Malalignment of outlet septum: With nonrestrictive
• Anomalous attachment of the tension apparatus of outlet VSD, posterior malalignment of outlet septum
mitral valve across the outflow tract or accessory mitral may narrow the subpulmonary outflow tract. Subcostal
valve tissue can also cause LV outflow obstruction. coronal view with anterior tilt and PLAX view can
This can be profiled best in a five-chamber view profile the posterior malalignment of outlet septum
(Fig. 72.146). (Figs 72.145 and 72.146).
• Prolapse of septal leaflet of tricuspid valve or accessory • Imaging the bifurcation of LCA into LAD and circumflex
tricuspid valve tissue across VSD: In this condition, the vessels in PSAX view, PLAX view with anterior tilt, and
effective size of VSD becomes smaller due to prolapse subcostal coronal view with anterior tilt
of accessory tissue or septal leaflet of tricuspid • Absence of retropulmonary course of a coronary artery
valve. 2D echocardiography from subcostal coronal, (between mitral valve and the pulmonary trunk) in
subcostal sagittal, and PLAX views can profile the apical four-chamber, subcostal coronal, and PSAX
VSD and prolapse of tricuspid tissue across the defect views.
causing left ventricular outflow obstruction. Color
flow mapping is used to define level of obstruction and Circumflex Coronary Artery from Right
direction of shunt across the VSD.
Coronary Artery (from Sinus II), Left Anterior
Coronary Arteries Descending Coronary Artery from Sinus I
• Second most common pattern in complete trans-
The arterial switch operation for repair of complete
position
transposition requires the mobilization and reimplantation
• Demonstration of separate origin of coronaries from
of the coronary arteries. Whenever the position of the
respective sinuses in PSAX view at the level of great
aortic root is abnormal, the origin of the coronary arteries
vessels and subcostal coronal view with anterior tilt
deviates from those found in the normal heart. Thus,
• Demonstration of origin of circumflex coronary
identification of coronary artery anatomy becomes an
artery from RCA and then retropulmonary course
integral part of the preoperative echocardiographic
of circumflex vessel (between mitral valve and the
examination of a child with complete transposition
pulmonary trunk) in apical four-chamber, subcostal
of great vessels. Coronary anomalies can be correctly
coronal, and PSAX views
diagnosed preoperatively with echocardiography in 95%
• Absence of bifurcation of LCA into LAD and circumflex
of the patients. Sinuses are described from PSAX view and
in PSAX view and subcostal coronal view with anterior
labeled as left-facing sinus (left side-sinus I) and right-
tilt.
facing sinus (right side-sinus II). In most cases, the orifice
of the coronary artery is situated approximately in the
middle of the sinus of Valsalva just below the sinotubular Single Right Coronary Artery
junction; minor deviations from this central position are • Demonstration of single coronary ostium arising from
found frequently. sinus II (right-facing sinus) in PSAX view
In general, the origin and proximal course of the • Imaging origin of LCA from RCA and then its retropul-
coronary arteries can be profiled from modified PSAX view monary course between mitral valve and pulmonary
at the level of great vessels and apical four-chamber and trunk. This can be defined from modified PSAX (by
subcostal coronal views. From the PSAX view at the level moving the transducer superior and rightward), apical
of great vessels, relationship of great vessels and sinuses (I four-chamber, and subcostal coronal views
or left-facing, II or right-facing, and noncoronary) should • Demonstration of bifurcation of left main coronary
be defined. artery into LAD and circumflex branches
• Very rarely, LAD and circumflex coronary arteries have
Coronary Artery Patterns in separate origins from the right sinus. In this scenario,
Transposition of the Great Arteries214–219 LAD coronary artery courses anterior to aorta and
the circumflex artery has a retropulmonary course
Left Anterior Descending and Circumflex to reach the left AV groove. PSAX view at the level of
Coronary Arteries from Sinus I, and Right great vessels profiles origin of coronary artery from
sinus II, retropulmonary course of circumflex, and
Coronary Artery from Sinus II course of LAD artery anterior to aorta. Four-chamber
• Usual coronary pattern in complete transposition views (subcostal coronal and apical) in posterior
• Demonstration of separate origin of coronaries from plane profile retropulmonary course of circumflex and
respective sinuses in PSAX view at the level of great anterior tilt from this view profiles LAD artery coursing
vessels and subcostal coronal view with anterior tilt anterior to aorta.
Single Left Coronary Artery from left-facing sinus (sinus I). In this scenario, the
circumflex artery after arising from right courses
(Figs 72.147 and 72.148) retropulmonary to reach the left AV groove, and RCA
Less common than single RCA: Demonstration of single courses anterior to aorta after its origin from left.
coronary ostium arising from sinus I (left-facing sinus) in
PSAX view and origin of RCA from left. Its course anterior Intramural Coronary Artery
to aorta can be profiled in PSAX, apical four-chamber, and
subcostal coronal view with anterior tilt. The coronaries An intramural coronary is a potentially significant risk
can arise from a single stump, all three coronaries separa- factor for transfer of the coronaries as part of the arterial
tely or two coronaries with separate origin. switch operation for transposition of great arteries.
Rarely, circumflex artery arises from RCA and takes a Preoperative diagnosis is advantageous because it helps
retropulmonary course to reach left AV groove. Subcostal to prevent accidental injury to the intramural coronary
coronal view, apical four-chamber view, and PSAX view at artery during transection of the aortic root and excision of
the level of great vessels shows retropulmonary course of the coronary artery ostium from the aorta.
circumflex artery, while these views in anterior plane show • Origin of coronary artery from contralateral sinus, as
the RCA anterior to aorta. in case of left main or LAD artery origin from right-
facing sinus or RCA from left-facing sinus profiled in
Inverted Origin of Coronaries PSAX view. Their orifices are placed adjacent to the
• Demonstration of separate origin of coronaries in commissure (usually intercoronary).
PSAX view. • Identification of major coronary artery coursing between
• Imaging the retropulmonary course of LCA arising the two arterial roots in PSAX, apical four-chamber, and
from right-facing sinus (sinus II) and its bifurcation subcostal coronal views with posterior to anterior tilt.
into LAD and circumflex branches. This can be profiled After origin, the proximal portion of coronary artery
in PSAX, apical four-chamber, and subcostal coronal runs parallel to the aortic wall and perpendicular to
views. the intercoronary commissure, giving a “double border
• Demonstrating origin of RCA from left-facing sinus appearance” to the posterior aortic wall.
(sinus I), its course anterior to aorta in PSAX, and four- In approximately 10% of cases, the orifice of a coronary
chamber views with anterior tilt. artery will arise close to one of the valve commissures that
• Less commonly there can be inverted origin of can be defined in PSAX view at the level of great vessels.
circumflex only and origin of RCA and LAD artery With coronary ostia near the commissure, chances of
A B
Figs 72.147A and B: Two-dimensional echocardiography in parasternal short-axis views in two cases of complete transposition of great vessels
(TGA) showing the origin of the coronary arteries. (A) In case A, the coronaries arise from a single stump. In case B, all the three coronary arteries
arise separately. (Ao: Aorta; LAD: Left anterior descending artery; RCA: Right coronary artery; LCx: Left circumflex artery).

short-axis view in an infant with TGA showing both the coronary
artery systems arising from a common sinus (sinus I with two sep-
arate origin). (Ao: Aorta; LMCA: Left main coronary artery; RCA:
Right coronary artery).
A B
Figs 72.149A to C: (A) Two-dimensional echocardiography in par-

asternal short-axis view in an infant with TGA showing the origin
of dual right coronary artery (RCA). (A) RCA from sinus II; (B) Left
main and RCA from sinus I; (C) Apical four-chamber view with tilt
showing the presence of the left circumflex artery in the left atrio-
ventricular (AV) groove. (Ao: Aorta; LA: Left atrium; LAD: Left an-
terior descending artery; LCT: Left common trunk; LCx: Left cir-
cumflex artery; LV: Left ventricle; PA: Pulmonary artery; RA: Right
C atrium; RCA: Right coronary artery; RV: Right ventricle).
neopulmonary regurgitation are higher after arterial switch (higher origin of coronary). Occasionally, two ostia will
operation. Less commonly, ostia can also arise above arise from the same sinus (usually sinus II; Fig. 72.148).
the commissures in the tubular portion of the aorta Rarely there may be a dual RCA219 (Figs 72.149A to C).
PART 8: ATRIOVENTRICULAR AND VENTRICOARTERIAL DISCORDANCE
ATRIOVENTRICULAR AND Associated Defects221–232

VENTRICOARTERIAL DISCORDANCE The commonest associated anomaly is left AV valve
Congenitally corrected transposition of great vessels (tricuspid valve) abnormalities.220 The other lesions are
(CTGA) or L-transposition of great vessels is characterized VSD and pulmonary stenosis. However, any kind of
by discordant AV connections combined with discordant congenital cardiac malformation can be encountered.
ventriculoarterial connection. Thus, with situs solitus,
morphological RA on right side is connected to Coronary Artery Anatomy233–237
morphological LV on right side (L-looped ventricles), Usually coronary arteries arise from two aortic sinuses,
which is in turn connected to PA.220 On the other hand, which are adjacent to the pulmonary trunk. The anatomy
morphological LA is connected to morphological RV on is mirror image relative to the arrangement seen in the
left side, which gives rise to aorta (Figs 72.150 and 72.151). normal heart. Right side coronary artery exhibits the
This anomaly can be encountered with situs solitus or pattern of morphological LCA and left-sided coronary
with situs inversus. Anomalies having univentricular AV artery exhibits the pattern of morphological RCA. The
connection such as atresia of one of the AV valves, and most frequent coronary anomaly is single coronary artery.
double inlet LV with hypoplastic RV on left side should be Segmental analysis is crucial for the diagnosis of
excluded. corrected transposition and 5% of corrected transposition
The aorta usually arises left and anterior to pulmonary cases occur in the setting of situs inversus. The
valve (L-transposed aorta), but rarely aorta can be right discordance between the atrial (abdominal) situs and
and anterior to pulmonary trunk. There will be fibrous the cardiac position is itself a pointer to possibility of
continuity between mitral leaflet and the pulmonary valve. CTGA, for example, dextrocardia with situs solitus and
On the other side, aorta is separated from tricuspid valve levocardia with situs inversus, and 25% of patients with
by the muscular infundibulum. corrected transposition have cardiac malposition, either
Fig. 72.150: Two-dimensional echocardiography. Subcostal coro- Fig. 72.151: Two-dimensional echocardiography. Subcostal coro-
nal view in a patient of corrected transposition of great vessels nal view with anterior tilt in a child of corrected transposition of
(CTGA) showing atrioventricular discordance. (LA: Left atrium; LV: great vessels (CTGA) and ventricular septal defect (VSD; star)
Left ventricle; PA: Pulmonary artery; RA: Right atrium; RV: Right showing the L-looped ventricles with aorta arising from morpho-
ventricle). logical left-sided right ventricle and pulmonary artery arising from
morphological left ventricle (right-sided). Mitral and pulmonary
valves show continuity (arrow). (Ao: Aorta; LV: Left ventricle; PA:
Pulmonary artery; RV: Right ventricle).
dextrocardia or mesocardia. As with d-transposition, culoarterial connections should be defined from subcostal
echocardiographic diagnosis of corrected transposition coronal and apical four-chamber views (Fig. 72.151). To
of great vessels is based on sequential chamber analysis demonstrate ventriculoarterial connections, it is important
and demonstrating abnormal AV and ventriculoarterial to trace ventricular outflow tracts to the great arteries.
connections. After defining situs from subcostal short- In the subcostal view, after the ventricles are identified,
axis view, both atria should be defined from subcostal continuing superior angulation first shows the PA arising
coronal, and sagittal and apical four-chamber views, to from the LV. This artery is deeply wedged between the
profile discordant AV connection, that is, morphological mitral and tricuspid valves and the pulmonary valve is
RA connects to morphological LV. The presence of atrial seen to be continuous with the mitral valve. This view also
situs (which generally goes along with the abdominal profiles the LVOT and its abnormalities. Continuing the
situs) opposite to the cardiac position, that is, dextrocardia superior angulation shows the aorta arising from the RV
with situs solitus and levocardia with situs inversus, is in (Figs 72.151 and 72.152). It is seen to lie furthest to the left
itself generally a setting of corrected transposition of great and follows a straight course upward. It has no continuity
arteries. with the tricuspid valve. The two great vessels are seen to
In situs solitus and levocardia: From the subxiphoid have a parallel course. In those with suboptimal subcostal
view, as the transducer is angled superiorly from the windows the aorta may not always be visualized in this view
coronal view, the LV, which lies inferiorly and to the right is because the ribs prevent further anterior tilt. The next view
viewed. Further superior tilt brings the RV in view, which is the apical four-chamber view. This may be suboptimal
lies to the left of the morphological LV. in cases with mesocardia or dextrocardia. In these cases,
In situs inversus and dextrocardia: The morphological the transducer may have to be placed very close to the
RA is left-sided. On angulating the transducer superiorly sternum or some times over it. This view, however, shows
in the subxiphoid coronal view, the LV first comes under the features of discordant AV connection very well. In situs
view. It lies inferiorly and to the left. On further superior solitus and levocardia, the left-sided AV valve (tricuspid
tilt, the RV is viewed, lying to the right of the LV. valve) is seen to attach more apically compared to the
Four-chamber views show malalignment between right-sided AV valve (mitral valve; Figs 72.153 and 72.154).
interatrial septum and inlet part of interventricular This view also shows the moderator band of the left-
septum. This is seen as a rightward curve of atrial septum sided morphological RV very clearly. The apex of the RV
in situs solitus. After defining AV connections, ventri- (left-sided) frequently appears foreshortened in this view.
Fig. 72.152: A case of corrected transposition of great vessels Fig. 72.153: Two-dimensional echocardiography. Apical four-
(CTGA). Subcostal paracoronal view with slight tilt to the right chamber view from a 4-year-old child with corrected transposition
showing the pulmonary artery arising from the right-sided left of great arteries showing atrioventricular discordance, reverse
ventricle and its division. (LA: Left atrium; LPA: Left pulmonary offsetting of atrioventricular (AV) valves (arrow), and moderator
artery; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle; band in morphological right ventricle on left side (arrow). (LA: Left
RPA: Right pulmonary artery). atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
The membranous septum is clearly seen in this view Subcostal sagittal view also defines ventriculoarterial
situated between the morphological LV (right-sided) and connections, origin of aorta from RV, and PA from LV
LA (left-sided). and parallelly arranged great vessels. Parasternal long
Anterior tilt from the apical four-chamber view first axis is oriented in a much more vertical direction than
shows the PA and LVOT (Fig. 72.155). On further anterior usual. As ventricular septum in corrected, transposition
tilt, the aorta should be seen to the left of the PA. This is sagittally oriented, the long-axis view may be confusing,
view may not always be optimal, because on continuing particularly in the presence of large perimembranous
anterior tilt, transducer contact with the chest wall is VSD with inlet extension giving the impression of single
frequently lost. Loss of offsetting remains a characteristic ventricle. This occurs because the ventricles are side-by-
feature. (Figs. 72.156A and B). side with sagittally arranged interventricular septum, and
Fig. 72.154: Two-dimensional echocardiography with zoomed up Fig. 72.155: Two-dimensional echocardiography with four-
view of the crux of the heart showing atrioventricular discordance chamber view with anterior tilt in a case of corrected transposition
and reverse offsetting of the atrioventricular (AV) valves (arrow). of great vessels (CTGA) showing a regressed left ventricle; there is
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right no left ventricular (LV) outflow tract obstruction. (LV: Left ventricle;
ventricle). PA: Pulmonary artery; RV: Right ventricle).
A B
Figs 72.156A and B: Two-dimensional echocardiography. Apical four-chamber view in a case of corrected transposition of great vessels
(CTGA) showing the comparison of the normal heart with normal offsetting (arrow) (Figure A) of atrioventricular valves as compared to
the CTGA with reverse offsetting and the trabeculated right ventricle (RV) with a moderator band (Figure B). (LA: Left atrium; LV: Left
the plane of ultrasound passes through an AV valve and

semilunar valve without passing through any portion of
the interventricular septum.
PLAX view profiles parallel-arranged great vessels
and multiple chords attaching to interventricular septum
with tricuspid valve and aortic discontinuity. Because the
ventricles and great vessels are side-by-side, in long-axis
view of LV and PA the adjacent RV and aorta may also be
visualized. Minor posterior tilting from PLAX view shows
the posterior AV valve continuity with the posterior arterial
valve. The PSAX view at the level of great vessels defines
spatial relationship of great vessels. As great vessels have
parallel arrangement, these are seen as a double circle in
PSAX view, usually aorta to left and anterior to PA. However,
in corrected transposition, any spatial orientation of great Fig. 72.157: Two-dimensional echocardiography. Modified par-
asternal long-axis view showing the origin of the pulmonary artery
vessels is possible, as in d-transposition of great vessels.
from the left ventricle (LV) with prolapse of the mitral valve into
As both semilunar valves are not at the same level, cross the left ventricular outflow tract (LVOT; arrow) causing obstruction.
section through the aortic valve profiles main PA and its (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
bifurcation and cross section through pulmonary valve
profiles RVOT in long axis. PSAX view is oriented more VSD extending to inlet septum, offsetting of AV valves is
horizontally in corrected transposition and ventricular lost. Septal leaflet of tricuspid valve may prolapse through
septum is aligned in anteroposterior direction. VSD, causing it to become hemodynamically smaller, or it
Coronary artery can be defined from PSAX view; may prolapse into subpulmonary area through the defect
origin of RCA (anatomical left) can be profiled from left causing subpulmonary stenosis (Fig. 72.157). Muscular
coronary cusp, although it is more difficult to profile the VSD can be profiled from a combination of views (four-
left coronary cusp than in d-transposition of great vessels. chamber, parasternal long- and short-axis). These views
As aorta is left and anterior, ascending aorta ascends on define muscular boundaries of the defect and its extension
left border of the heart straight up on the left and then (Figs 72.158 and 72.159). Doubly committed VSD is best
arches, and descending aorta descends on the left behind profiled from subcostal coronal view and is roofed by the
the ascending aorta. High left parasternal view with continuity between aortic and pulmonary valve leaflets.
anticlockwise rotation (ductal view) profiles the ductus Color flow mapping shows direction of shunt, turbulent
and also the whole length of arch because of its leftward (restrictive) or laminar flow across the VSD (Fig. 72.159).
and anterior position. Usually, the aortic arch is left- Pressure gradient (difference in left and RV pressure) can
sided with normal arch branching; demonstration of arch be determined by use of continuous wave Doppler.
branches is important as right aortic arch occurs in 18% of
cases with corrected transposition. Tricuspid Value Abnormalities232 (Fig. 72.160)
These are almost an essential part of CTGA and are
Associated Malformations seen in 90% of cases in autopsy series, although they
Ventricular Septal Defect are hemodynamically significant in 30% of cases only.
The most common anomaly is valvular dysplasia, with
It is reported in 60–70% of patients with CTGA. The or without apical displacement of the septal and or
commonest is perimembranous defect (Fig. 72.151), which mural leaflet. Apical four-chamber, parasternal long-
is subpulmonary and extends posteriorly toward the crux of axis, and modified short-axis views are the best to define
the heart and erodes significantly the inlet septum. Fibrous tricuspid valve anomalies, including thickening of valve
continuity between mitral, tricuspid, and pulmonary valve leaflets and downward displacement of septal and mural
is present. It is important to define VSD relation to AV valves leaflets. Atrialization, with thinning and dilatation of RV
and document if there is any overriding or straddling of AV as seen in Ebstein’s anomaly with concordant AV and
valves, more commonly of tricuspid valve. In cases with ventriculoarterial connections is rare with corrected
A B
Figs 72.158A and B: Two-dimensional echocardiography. Apical four-chamber view with color compare with anterior tilt showing the
origin of the pulmonary artery from the left ventricle and the presence of supravalvular pulmonary stenosis (white arrow). A ventricular
septal defect is noted (yellow arrow) in the anterior muscular plane. (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
Fig. 72.159: Two-dimensional echocardiography with color com- Fig. 72.160: Two-dimensional echocardiography. Apical four-
parison showing a small anterior muscular ventricular septal chamber view with color flow mapping showing severe tricuspid
defect (arrow) in a case of corrected transposition of great vessels regurgitation (TR) in Ebstein’s anomaly of the left-sided atrioven-
(CTGA). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; tricular (AV) valve in a case of corrected transposition of great ves-
RV: Right ventricle; RA: Right atrium). sels (CTGA). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
transposition. With significant Ebstein’s malformation, to supratricuspid ring or membrane. From apical four-
hypoplasia of RV and occasionally subaortic obstruction chamber and PLAX view, this membrane is seen as thin,
can occur and should be properly defined. linear echoes just above the tricuspid valve, attaching
Other abnormalities can be short and thickened laterally to free wall of LA and medially to left atrial
chordae that insert directly into ventricular wall, de- surface just above the crux. Color flow mapping shows
formed and abnormal papillary muscles, dilatation of turbulence beginning above the level of valve, and on
tricuspid valve annulus, and straddling of the left AV valve pulsed or continuous wave Doppler interrogation, severity
(Fig. 72.161). By color flow mapping, valvular regurgitation of obstruction can be defined. Rarely cor triatriatum
(more commonly) or stenosis of tricuspid valve should sinister may be the cause of tricuspid inflow obstruction.
be defined. Less commonly, tricuspid valve stenosis Best views to profile cor triatriatum are PLAX and four-
can occur in congenitally corrected transposition due chamber views. In PLAX view, the membrane extends
from posterior wall of LA toward interatrial septum. In is obtained with the use of pulsed and continuous wave
apical four-chamber view, the membrane extends from Doppler interrogation. Often a PA band is placed in the
lateral wall of LA above the left atrial appendage toward restricted VSD or intact septum subgroup as a measure to
the interatrial septum. Cor triatriatum can be obstructive prepare the LV for the subsequent double switch surgery
or nonobstructive. Color flow mapping and pulsed (Fig. 72.164). Care should be taken to separate the jet of
Doppler imaging should be used to define any obstruction VSD from pulmonary stenosis.
to tricuspid inflow.
Mitral valve abnormality with CTGA: Dysplastic mitral Abnormalities of Right Ventricular
valve can occur, but is not common. Outflow
Abnormalities of RVOT are somewhat uncommon,
Left Ventricular Outflow Tract occurring in about 10% of patients. Obstruction or severe
Obstruction regurgitation of tricuspid valve is commonly associated
with outflow tract obstruction. In the setting of severe
Pulmonary stenosis occurs in approximately 30 to 50% of left AV valve regurgitation, one must be certain that the
patients, with 85% of these in association with a VSD. The obstruction is not simply functional. As aorta is supported by
anatomical nature of pulmonary stenosis varies. Valvular the muscular infundibulum, muscular outflow obstruction
stenosis is usually accompanied by one or the other may be dynamic. Other causes of obstruction can be
variety of subpulmonary obstruction. Subpulmonary anterior malalignment of outlet septum with VSD, subaortic
obstruction may take the form of fibrous diaphragm or membrane, valvular stenosis, and accessory mitral or
an aneurysm of fibrous tissue that protrudes into LVOT tricuspid valve tissue. The RVOT can be best imaged from
(see Fig. 72.157). The fibrous tissue tags may originate parasternal long-axis, subcostal coronal with anterior tilt,
from perimembranous septum, tricuspid or mitral valve, and subcostal sagittal views. Doppler interrogation can be
or even a leaflet of pulmonary valve (see Fig. 72.157). difficult due to improper alignment, and a high parasternal
Posterior malalignment of outlet septum associated with or subcostal coronal with anterior tilt views are used in an
VSD can also cause LVOT obstruction. The anatomical attempt to align the Doppler beam.
features of LVOT are best defined from subcostal coronal Coarctation of aorta is often associated with severe
and apical four-chamber views with anterior tilt, and tricuspid regurgitation, VSD, hypoplasia of RV, and sub-
PLAX view (Figs 72.162 to 72.164). Peak pressure gradient aortic obstruction.
Fig. 72.161: Two-dimensional echocardiography in apical four- Fig. 72.162: Two-dimensional echocardiography in subcostal view
chamber view in a case of corrected transposition of great vessels with anterior tilt and color flow mapping in corrected transposition of
(CTGA) showing Grade I straddling of the left atrioventricular great vessels showing turbulence across left ventricular outflow tract
valve (tricuspid) across the large inlet ventricular septal defect (LVOT) (arrow) because of hypoplastic pulmonary annulus and main
(arrow). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: pulmonary artery (MPA). (LV: Left ventricle; PA: Pulmonary artery;
Right ventricle). RV: Right ventricle; RA: Right atrium).
Fig. 72.163: Two-dimensional echocardiography in subcostal view Fig. 72.164: Two-dimensional echocardiography in apical four-
with anterior tilt and color flow mapping in corrected transposition chamber view with anterior tilt showing a pulmonary artery (PA)
of great vessels (CTGA) showing turbulence across left ventricular band in situ with flow acceleration across it (arrow). (LV: Left
outflow tract (LVOT) because of supravalvular pulmonary stenosis. ventricle; RV: Right ventricle; PA: Pulmonary artery).
(LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle)
(arrow).
PART 9: PULMONARY VEINS
Evaluation of the pulmonary veins forms an essential • Suprasternal short-axis view (Crab view): This is the
component of any segmental analysis of the congenital best view in the pediatric age group to demonstrate all
heart. The indicators for the abnormal connection include four pulmonary veins that are located below the right
dilated chambers to which the veins drain, right-to-left atrial PA (Figs 72.168A and B).
shunting, nonvisualization of the veins to the LA (bald LA),
and a small LA. In such a scenario it is equally essential to NORMAL FLOW PATTERN OF
screen the systemic veins (SVC, IVC, innominate veins) for PULMONARY VEINS
abnormal dilatation or increased flow. Echocardiographic
All pulmonary veins should be interrogated by color
views for demonstration of pulmonary venous connection
flow mapping followed by pulsed and continuous wave
to LA and their flow patterns are:238–241
Doppler with attention to intercept angle, wall filter, and
• The subcostal view (Figs 72.165A and B): The coronal
velocity scale. Pulsed Doppler sample volume should
section clearly demonstrates right upper and left
be placed distal to the orifice of the pulmonary vein as it
upper pulmonary venous connection to LA, while
enters the LA, so that Doppler velocity reflects the events
the sagittal section identifies the right upper and right occurring in pulmonary vein and not the LA. In normal
lower pulmonary veins. subjects, the pulmonary vein Doppler recording consists
• Apical four-chamber view (Figs 72.166 and 72.167): of biphasic forward flow, “S”-wave in systole due to atrial
This view gives a clear visualization of the left-sided relaxation and descent of floor of LA, “D”-wave in diastole
pulmonary veins and right upper pulmonary vein. due to fall in LA pressures with opening of mitral valve and
Posterior tilt in apical four-chamber view at level of rapid ventricular filling, and a small reversed “A”-wave
IVC shows the right lower pulmonary vein connection occurring during LA contraction.
to LA. The normal values for the S-, D-, and A-wave in
• Parasternal short- and long-axis views: PSAX and subjects under the age of 50 years are nearly constant,
PLAX views show both left and right upper pulmonary except in newborns who have nearly equal S- and D-waves.
veins. The descending aorta in short axis separates Continuous high velocity pattern present in pulmonary
right and left pulmonary veins. veins in the first few days of life may be due to relatively
A B
Figs 72.165A and B: Two-dimensional echocardiography in subcostal coronal view with color flow mapping showing right upper and
left upper pulmonary veins draining into left atrium (LA; arrows). LA: Left atrium; RA: Right atrium).
A B
Figs 72.166A and B: Two-dimensional echocardiography in sagittal subcostal view showing right upper and right lower pulmonary veins
draining to left atrium (LA; arrows). (LA: Left atrium; RA: Right atrium).
Figs 72.167A and B: Two-dimensional echocardiography in apical

four-chamber view showing the right upper and left lower pulmonary
veins connecting to left atrium (LA; arrows). (LLPV: Left lower
A B pulmonary vein; RUPV: Right upper pulmonary vein).
A B A B
Figs 72.168A and B: Two-dimensional echocardiography in Figs 72.169A and B: Two-dimensional echocardiography in
suprasternal short-axis view (crab view) with color compare suprasternal short-axis view with color compare showing left-
showing all four pulmonary veins connecting to the left atrium sided pulmonary veins (arrows) draining to the innominate vein.
(arrows). (LLPV: Left lower pulmonary vein; LUPV: Left upper (Innom: Innominate vein).
pulmonary vein; RLPV: Right lower pulmonary vein; RUPV: Right
upper pulmonary vein).
hypoplastic and underperfused pulmonary veins during on the other hand has been reported. Usually, left-sided
fetal life, which are suddenly exposed to increased pulmonary veins connect anomalously to the derivatives
pulmonary blood flow after birth with fall in PVR. of left cardinal system, that is, coronary sinus and the left
innominate vein. Right-sided pulmonary veins usually
ANOMALIES OF PULMONARY VEINS connect to derivatives of right cardinal system, that is, SVC
or IVC. However, as the developing splanchnic plexus is
Abnormal Number of Pulmonary Veins a midline structure, the possibility of cross connection
Normally there are two right and two left pulmonary veins. exists.
The most common variation is the presence of a single Most cases of PAPVC associated with an ASD with
pulmonary vein on either left or right side. There may only 20% of cases having intact interatrial septum. Other
be increased number of pulmonary veins. The number associated heart defects can be VSD, LSVC, and complex
of pulmonary veins on one side could vary from three to congenital heart defects associated with isomerism. After
five. The variation in number of pulmonary veins is of no sinus venosus ASD, the commonest type of PAPVC is left
clinical significance if they are connected normally to LA. upper lobe pulmonary vein to left innominate vein followed
by right pulmonary veins to IVC (Scimitar syndrome).
Anomalous Pulmonary Venous Return The latter group is associated with hypoplasia of right PA
Partial anomalous pulmonary venous connection and right lung, anomalies of bronchial system, horse-
(PAPVC) is used to describe the condition where one or shoe lung, pulmonary sequestration, and dextroposition
more but not all pulmonary veins connect abnormally of heart along with, on the chest X-ray, a Turkish sword-
(Figs 72.169A and B) and total anomalous pulmonary like shadow on the right heart border produced by the
venous connection (TAPVC) is a term used when all descending pulmonary venous channel formed by right-
four pulmonary veins connect abnormally to systemic veins sided pulmonary veins connecting to IVC.
or RA. When pulmonary veins are connected normally to While doing echocardiography, a high index of
posterior wall of LA but drain anomalously because of the suspicion is required to diagnose PAPVC. In any patient
orientation of the atrial septum, the condition is termed with right atrial and RV dilation, all pulmonary veins
as partial or total anomalous pulmonary venous drainage should be defined on 2D echocardiography and color flow
(PAPVD/TAPVD). mapping connecting to LA, and all possible drainage sites
Almost every possible connection between pulmonary should be interrogated with color flow, because in the
vein on one hand and various systemic venous tributaries case of supernumerary pulmonary veins, one can profile
four pulmonary veins connecting to LA but an accessory Steps in Diagnosis of Total Anomalous
pulmonary vein or a tributary of pulmonary vein may be
draining anomalously.
Pulmonary Venous Connection
In majority of cases with PAPVC, there will be evidence Features Common to all Forms of Total
of right-sided volume overload in the form of dilated RA Anomalous Pulmonary Venous Connection
and RV with paradoxical septal motion except in the case
of PAPVC of a single pulmonary vein or PAPVC associated Dilated right-sided structures (RA, RV, and PA; Fig. 72.170).
• LA and LV (may appear smaller)
with stenosis (small shunt).
• Interatrial septum bows toward left, with right-to-left
shunt at atrial level (Fig. 72.171)
TOTAL ANOMALOUS PULMONARY • RV appears to compress the LV and on M-mode,
VENOUS CONNECTION242–246 evidence of paradoxical ventricular septal motion
suggestive of RV volume overload is seen
All pulmonary veins form a confluence and that confluence • Echocardiographic evidence of pulmonary hyper-
drains to systemic venous system. The commonest site of tension may exist, in the setting of obstruction or flow-
drainage is left innominate vein followed by portal system, related PA hypertension
coronary sinus, SVC, RA, and rarely mixed type, that is, • Inability to image the pulmonary vein entrance to LA is
more than one site of abnormal connection. the first echocardiographic suspicion that one may be
dealing with TAPVC.
The goals of echocardiography are:
• To define individual pulmonary veins Pulmonary Venous Confluence (PVC)
• To ascertain that all pulmonary veins are draining
The common chamber to which all pulmonary veins
to pulmonary venous confluence (PVC) or draining
connect before finally draining to systemic venous channel
directly is referred to as PVC. PVC is profiled as an echo-free space,
• Relation of PVC to LA, PA, and airways which lies in a plane posterior to LA but is separated from
• Site of drainage of PVC/individual veins it. It is well seen in suprasternal short-axis and subaortic
• Any evidence of obstruction at the site or during the coronal views. In the subcostal sagittal view in its cross
course of the pulmonary venous channel section below the right PA and posterior to the SVC, giving
• Adequacy of interatrial communication the appearance of a double circle. The size and orientation
• PA pressures (horizontal and vertical) of PVC and its relation to LA is
• Any associated heart defect. important when planning for surgery.
Fig. 72.170: Two-dimensional echocardiography. Apical four- Fig. 72.171: Two-dimensional echocardiography with color
chamber view in a case of total anomalous pulmonary venous compare in subcostal coronal view in a 6-month child with total
connection (TAPVC) showing markedly dilated right atrium and anomalous pulmonary venous connection (TAPVC) showing right-
right ventricle. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; to-left shunt at the atrial level (arrow). (LA: Left atrium; RA: Right
RV: Right ventricle). atrium).
After identifying the PVC, the next step is to profile sinus), the PVC lies directly posterior to LA and all four
individual pulmonary veins for their course and diameter pulmonary veins join ipsilaterally to that confluence at
because obstruction can occur at any level. This is the the same level (Fig. 72.175). In intracardiac type of TAPVC,
strong independent predictor of survival in TAPVC. with connection to RA, the PVC can be imaged draining
directly to RA. Rarely the pulmonary veins can drain
individually to RA. The views most commonly used are
Orientation and Site of Drainage of subcostal coronal, and sagittal and PSAX views. In TAPVC
Pulmonary Venous Confluence to coronary sinus, the dilated coronary sinus receiving
all pulmonary veins bulges anterosuperiorly into LA and
In case of supracardiac TAPVC (to vertical vein/SVC/
can be imaged easily in subcostal coronal, apical four-
azygos vein), PVC lies horizontally oriented posterior
chamber, and PLAX views.
and superior to LA (Figs 72.172A and B). This can be best
In infracardiac TAPVC (to IVC/hepatoportal system),
visualized in suprasternal short-axis and subcostal sagittal
the pulmonary veins connect to vertical vein at a different
views. In supracardiac TAPVC to left innominate vein, the
level and the repair is more challenging (Fig. 72.176).
ascending channel connecting to left innominate vein can The PVC is often small, inferior and posterior to LA, the
be shown in suprasternal short-axis view (Fig. 72.173). descending venous channel (Des. PVC) passes through the
In this view, the anomalous pulmonary venous conne- diaphragm usually anterior to aorta, and joins a systemic
ction gives the appearance of a large vascular collar vein or the hepatoportal system (portal vein, ductus
surrounding the transverse arch. Color flow mapping venosus, left hepatic vein, and IVC). Three channels, two
in a vertical vein shows continuous low velocity flow descending (aorta and the Des. PVC) and one ascending
directed away from the heart. There will be evidence of (IVC, which is dilated) can be defined in subcostal views.
dilated innominate vein and SVC along with dilated right- The short-axis view shows these three vessels in short axis.
sided chambers. In TAPVC to SVC or azygos vein, the Subcostal sagittal view is the best view to define the origin
innominate vein will be of normal caliber and in TAPVC of the PVC, its course, and connection to hepatoportal
to azygos or to SVC, the SVC will be grossly dilated. In a system. Pulse Doppler examination shows continuous low
rare variety of TAPVC, that is, to azygos vein, the channel velocity flow signals or high velocity disturbed flow if there
ascends posterior to right PA and joins the azygos vein is obstruction, which is usually present in these cases. The
(Fig. 72.174). The best view to define this anatomy is flow in the descending pulmonary venous channel is away
subcostal sagittal, high PSAX, and suprasternal short axis from the heart and continuous low velocity flow signals in
views. In case of intracardiac TAPVC (to RA/coronary IVC are directed toward the heart.
A B
Figs 72.172A and B: Suprasternal short-axis view with color flow mapping in a case of supracardiac total anomalous pulmonary venous
connection (TAPVC). (A) Shows right- and left-sided pulmonary veins forming a pulmonary venous confluence; (B) Shows pulmonary
venous confluence continuing as vertical vein. (PVC: Pulmonary venous confluence; RPA: Right pulmonary artery; VV: Vertical vein).
Fig. 72.173: Two-dimensional echocardiography with color flow Fig. 72.174: Two-dimensional echocardiography with colour flow
mapping. Suprasternal short-axis view in a patient with supracar- mapping in suprasternal view in a case of supracardiac total
diac total anomalous pulmonary venous connection (TAPVC) with anomalous pulmonary venous connection (TAPVC) showing the
color flow mapping showing the ascending vertical vein (VV) from pulmonary veins from left and right sides ascending and draining
the pulmonary venous confluence draining into the innominate vein, to the azygos vein (arrows). (AZ: Azygos vein).
which together with the superior vena cava (SVC) is dilated. (In-
nom: Innominate vein; SVC: Superior vena cava; VV: Vertical vein).
Fig. 72.175: Two-dimensional echocardiography with color flow Fig. 72.176: Two-dimensional echocardiography with color
mapping. Subcostal coronal view showing the pulmonary venous con- compare. Subcostal sagittal bicaval view (modified) in a case of
fluence draining to the coronary sinus (CS), right-to-left shunt across infradiaphragmatic total anomalous pulmonary venous connection
the patent foramen ovale (PFO; arrow), dilated right atrium (RA), and (TAPVC) showing the pulmonary venous confluence continuing as
normal-sized superior vena cava (SVC). (CS: Coronary sinus; RA: the descending vertical vein (desc. VV) and then draining into the
Right atrium; PFO: Patent foramen ovale; SVC: Superior vena cava). inferior vena cava through hepatic sinusoids (RA: Right atrium).
Rarely, a mixed form of TAPVC occurs in which Defining the Obstruction247

pulmonary veins drain to two or more separate systemic
venous sites. In this subgroup, multiple windows using Color flow mapping and PW Doppler should be used to
2D and color flow mapping must be used to image the diagnose the presence of obstruction. Normal pattern
connection of all four individual pulmonary veins. The in a pulmonary vein is laminar flow on color Doppler
most common type of mixed drainage connection is to mapping and biphasic low velocity flow on pulse
coronary sinus and via vertical vein to left innominate Doppler. If there is obstruction at any level, there will
vein. be turbulent, aliased flow on color flow mapping and
high velocity disturbed flow on pulsed Doppler, which will • At junction of venous confluence to the site of drainage
not touch the baseline, and with more severe obstruction (Fig. 72.179)
it will be continuous. The level of obstruction can be the • In infradiaphragmatic TAPVC
following: • As channel passes through the diaphragm
• Long channel itself offers some resistance • Resistance offered by hepatoportal system.
• Restrictive interatrial communication (Fig. 72.177) The tricuspid regurgitation velocity helps to predict the
• In supracardiac TAPVC to left innominate vein, as the PA pressures. In obstructed TAPVC if a ductus is present,
ascending channel passes between left PA and left there may be right-to-left shunt indicating suprasystemic
bronchus (vascular ring), it gets compressed (so-called pressures in the pulmonary circuit.
hemodynamic vise; Figs 72.178A and B)
Anomalous Drainage of Pulmonary
Veins with Normal Connection
Anomalous pulmonary venous drainage may be partial
(PAPVD) or complete (TAPVD).248 Normally connected
pulmonary veins may have flow directed to RA in the
following conditions:
• Sinus venosus ASD.
• Large fossa ovalis ASD extending to posterior wall
of LA.
• Septum primum malposition defect.
Septum Primum Malposition Defect

Causing Partial or Total Anomalous
Pulmonary Venous Drainage
Fig. 72.177: Two-dimensional echocardiography with color flow (Figs 72.180 and 72.181)
mapping in subcostal sagittal view showing a restrictive interatrial
communication (arrow) with the turbulence across a patent This condition is usually associated with visceral
foramen ovale in a case of total anomalous pulmonary venous heterotaxy, commonly with left isomerism and rarely with
connection (TAPVC). (LA: Left atrium; RA: Right atrium).
right isomerism.
A B
Figs 72.178A and B: (A) Two-dimensional echocardiography with color flow mapping. Modified suprasternal short-axis view showing
turbulence in the ascending vertical vein in a case of supracardiac total anomalous pulmonary venous connection; (B) Continuous
wave Doppler tracing showing the continuous flow throughout the cardiac cycle with a mean gradient of 5 mm Hg. (Ao: Aorta; Innom:
Innominate vein; VV: Vertical vein).
Fig. 72.179: Two-dimensional echocardiography using subcostal

coronal view with anterior tilt and color flow mapping showing the
pulmonary venous confluence (PVC) draining to the coronary sinus.
Patent foramen ovale (PFO) shunting is right to left (white arrow).
There is flow acceleration at the junction of the pulmonary venous
confluence and the coronary sinus (black arrow). (CS: Coronary
sinus; RA: Right atrium).
A B
Figs 72.180A and B: Two-dimensional echocardiography. Subcostal coronal view with color compare in a case of total anomalous
pulmonary venous drainage showing a malaligned interatrial septum (arrow) resulting in the drainage of the pulmonary veins occurs into
the right atrium (arrows). (LA: Left atrium; RA: Right atrium).
Fig. 72.181: Two-dimensional echocardiography. Apical four-

chamber view showing malaligned interatrial septum (arrow) lead-
ing to total anomalous pulmonary venous drainage with normally
connected pulmonary veins. RA is markedly dilated. (LA: Left
Echocardiographic pointers to the diagnosis of septum or situs inversus). In contrast, the incidence of systemic
primum malposition defect are: venous anomalies in patients with heterotaxy syndrome
• Deviation of septum primum toward anatomical LA exceeds 90%. Systemic venous anomalies are usually
• Absence of septum secundum associated with other CHD.
• Normally attached pulmonary veins to anatomical LA Abnormalities of systemic veins may be classified into
• Interatrial communication in most posterior plane abnormalities involving:249–252
between posterior wall of LA and displaced septum • SVC.
primum • Retroaortic innominate vein
• On color flow mapping, drainage of pulmonary • IVC and hepatic veins
veins to anatomical RA can be visualized. Number of • Abnormalities of coronary sinus
pulmonary veins draining anomalously depends upon • Persistence of valves of embryonic venous sinus
degree of malposition of septum primum.
• Total anomalous systemic venous connection
The views that can profile these anomalies are
• Decompressive veins like levoatriocardinal vein.
subcostal coronal, apical four-chamber, and parasternal
long-axis.
Superior Vena Cava
Pulmonary Vein Stenosis Persistent Left Superior Vena Cava
Pulmonary vein stenosis may be seen with TAPVC and
The commonest abnormality of SVC is persistence of
needs to be keenly looked at but as an isolated case is a
LSVC draining to coronary sinus and then to RA. The
rare anomaly. It is usually associated with other lesions.
prevalence of LSVC is 0.3% in general population, and 2
Echocardiographic diagnosis is based upon a high index of
to 10% with congenital heart defects.253 LSVC exists with
suspicion in patients who present with features of primary
right superior vena cava (RSVC) in > 84% of cases and in
pulmonary hypertension or pulmonary hypertension out
>75% cases, a bridging innominate vein (“H” connection)
of proportion to the heart defect.
is present. LSVC is located anterior to aortic arch and left
After 2D demonstration of pulmonary venous conne-
PA, passes inferiorly, accepts hemiazygos vein, and then
ction, color flow mapping must be done. On CFI, turbulent
joins coronary sinus in the posterior AV groove.
or mosaic flow pattern is the first indication of pulmonary
Hemodynamic significance: Although in isolation, it is
vein stenosis in contrast to laminar flow in nonobstructed
of no hemodynamic significance, it poses difficulty with
pulmonary veins. Pulsed and continuous wave Doppler
transvenous insertion of pacemaker leads; during an open
interrogation reveals loss of phasic flow and/or increased
heart procedure, separate venous cannulation is needed
velocity. The change in flow pattern depends upon the
if the bridging vein is absent or very small and in patients
length of the obstructed channel and severity of stenosis.
undergoing univentricular correction. It has been noted
The condition has to be differentiated from increased
pulmonary venous flow velocity associated with a large that with persistent LSVC, the incidence of arrhythmias
left-to-right shunt. In that case, there will be increased is higher and chances of preexcitation are ten times
pulmonary vein velocity with preservation of phasic more than the general population, possibly because of
pattern, which touches the baseline. On 2D measurement, abnormality of AV node and SA node. Congenital cardiac
the stenosed pulmonary vein will be found to be of smaller malformations that show a high frequency of persistent
caliber than normal. LSVC are juxtaposition of right atrial appendage (34%), AV
canal defect (19%), mitral atresia (17%), and TOF (11%).
With persistent LSVC, there is increased association
ANOMALIES OF SYSTEMIC VEINS of left-sided obstructive lesions and hypoplastic LV as
Systemic venous anomalies are rarely symptomatic in the dilated coronary sinus impinges on mitral inflow
isolation, but are brought to notice on evaluation for during fetal life. An increase in the magnitude of left-to-
other associated lesions. Their visualization may have right shunt at the atrial level was found in patients with
a significant impact on the management of various associated ASD.
lesions either directly or indirectly. Clinically significant The presence of LSVC can be suspected on a chest
abnormalities of the systemic veins are infrequent when radiogram based on a shadow along the left upper border
the visceroatrial situs is lateralized (either situs solitus of mediastinum.
Echocardiography: 2D echocardiography in subcostal flow is seen away from the transducer in contrast to the
coronal, apical four-chamber, and PLAX views show the vertical vein receiving pulmonary veins where flow is seen
dilated coronary sinus as an oval structure, while subcostal toward the transducer.
coronal, and apical four-chamber views in posterior In cases of poor echo windows, injection of agitated
plane show the dilated coronary sinus in its whole length saline in left arm delineates the LSVC and coronary sinus.
(Figs 72.182A to C). Other causes of dilated coronary sinus
should be looked for if one does not find a LSVC, such as Left Superior Vena Cava to Coronary Sinus
anomalous pulmonary venous drainage to coronary sinus, with Interatrial Communication and Defect in
high right atrial pressure leading to passive congestion, and
Wall of Coronary Sinus (see Fig. 72.34)
increased LCA flow leading to increased coronary sinus
return. A dilated coronary sinus should be differentiated This is a rare anomaly, known as Raghib syndrome. If a
from pericardial effusion, which is not continuous with RA localized defect is present in the wall of coronary sinus,
and inferior to the level of anterior mitral leaflet. it is called partial unroofing of coronary sinus. More
Suprasternal short-axis view with left tilt shows LSVC, marked deficiency leads to LSVC draining to LA, also
with or without the bridging innominate vein (Figs 72.183A known as coronary sinus type of ASD. Here, the interatrial
and B). Coronary sinus in this view is seen as a crescent- communication is coronary sinus ostium, which is located
shaped structure receiving LSVC. On color flow mapping, below and posterior to fossa ovalis. Rarely there may be
A B
Figs 72.182A to C: 2D echocardiography in a case of left superior

vena cava (LSVC) to coronary sinus. (A) Apical four-chamber view
with posterior tilt shows a dilated coronary sinus; (B) Apical four-
chamber view shows a dilated coronary sinus in short axis; (C)
Parasternal long-axis view showing the dilated coronary sinus
(CS). (CS: Coronary sinus; LA: Left atrium; LV: Left ventricle; RA:
C Right atrium; RV: Right ventricle).
A B
Figs 72.183A and B: Two-dimensional echocardiography in suprasternal short-axis view with color compare showing a large left
superior vena cava (SVC) draining to coronary sinus. (CS: Coronary sinus; LSVC: Left superior vena cava).
associated fossa ovalis or ostium primum ASD. Depending Absent Right Superior Vena Cava253,254
upon pressure difference between two atria, left-to-right
or right-to-left shunt occurs. The patient may present with It is a rare anomaly of systemic veins and has been reported
complication of right-to-left shunt with no significant in 0.1% of patients undergoing cardiac catheterization.
murmur, such as cyanosis, paradoxical embolism, brain Either the right SVC is completely absent or represented by
abscess, and stroke. Rarely, the ostium of coronary sinus a fibrous vestigial cord. The right innominate vein drains
may be atretic, causing whole of the LSVC flow into the to LSVC and then to coronary sinus. Embryologically, it is
LA. Rare cases of this in association with Lutembacher due to complete involution of right cardinal vein.
syndrome have been reported.254
Echocardiography: 2D echocardiographic findings are Clinical Significance
similar to LSVC drainage to coronary sinus. Special caution
Sinoatrial junction is poorly developed in these cases and
should be taken to define integrity of the wall of coronary
patients, usually adults, may develop sick sinus syndrome
sinus in case of cyanosis, because localized fenestrations
requiring a pacemaker. Issues that make the diagnosis of
are difficult to detect. In these circumstances, contrast
absent right SVC important include: (a) implantation of
echocardiography with saline injection in left arm shows
transvenous pacemaker, (b) placement of transvenous
filling of coronary sinus and LA almost simultaneously.
pulmonary catheter for monitoring particularly without
the usage of fluoroscopy, (c) systemic venous cannulation
Left Superior Vena Cava to Left Atrium for extracorporeal membrane oxygenation (ECMO),
With completely unroofed coronary sinus, LSVC terminates (d) systemic venous cannulation for cardiopulmonary
in LA generally between left atrial appendage anteriorly bypass, (e) partial or total cavopulmonary anastomosis,
and left upper pulmonary vein posteriorly. This anomaly (f ) orthotopic heart transplantation, (g) endomyocardial
rarely occurs in isolation, and is usually associated with biopsies, and (h) cardiac catheterization particularly for
heart defects like right isomerism. interventricular procedures.
Echocardiography: Subcostal coronal, PSAX, and Special precaution should be taken at the time of open-
suprasternal short-axis views show connection of LSVC to heart surgery to avoid damage to area around coronary
LA. On color flow mapping, flow is seen toward the heart sinus, and ligation of coronary sinus must be avoided.
and away from the transducer. Contrast echo with saline Echocardiography: Suprasternal short-axis view is
injection in left arm demonstrates connection of LSVC especially important for determining whether right SVC is
to LA. present or not, in addition to profiling of LSVC to coronary
sinus. In cases with absent right SVC, systemic veins usually present with cyanosis, clubbing, easy fatigability,
appear to be mirror image of normal in suprasternal short- features of polycythemia, and generally have no significant
axis view. Subcostal sagittal view shows absence of right findings on cardiac examination. Some patients present
SVC connecting to RA. with cerebrovascular accident. A common association
Rarely, there may be absence of both the right and is anomalous drainage of right upper pulmonary veins
LSVC, and blood from arms, head, and upper torso is to the SVC1—an anomaly that should be actively looked
returned to the RA through the azygos vein and IVC for once anomalous RSVC drainage is diagnosed.
mimicking chronic SVC obstruction. Echocardiography as described above and contrast
echocardiography are good modalities for the diagnosis;
Right Superior Vena Cava to Left Atrium rarely, when in doubt, one may consider CT scan for the
diagnosis. Surgical correction is warranted to prevent the
(Figs 72.184 and 72.185) risks of chronic polycythemia and systemic embolization.
Anomalous drainage of the SVC to the LA is an extremely Surgery has had excellent short- and long-term outcomes.
rare congenital cardiac malformation scantily reported About 23 cases have been reported since the date of this
in the English literature. Patients with this condition anomaly.
A B
Figs 72.184A and B: Two-dimensional echocardiography. Subcostal sagittal view with color compare showing the drainage of right
superior vena cava (SVC; arrow) to left atrium. (LA: Left atrium; RA: Right atrium; SVC: Superior vena cava).
Fig. 72.185: Saline contrast study. Saline was injected into the
right brachial vein and contrast echoes appeared in the left atrium
followed by left ventricle. No contrast appeared in the right atrium
and right ventricle, clearly showing communication of superior
vena cava (SVC) to left atrium. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle; SVC: Superior vena cava).
Aneurysm of Superior Vena Cava echocardiography and color flow mapping, this is seen as
a horizontal channel crossing from left to right beneath
It occurs due to inherent weakness in the structure of the transverse arch superior to right PA. At times it can be
venous wall. 2D echocardiography in subcostal sagittal mistaken for right PA. But on pulsed Doppler interrogation,
and suprasternal short-axis views can demonstrate the the flow pattern in innominate vein will be venous and
aneurysmal dilatation of SVC. right PA will be arterial.
It is important to exclude the causes of undue dilatation
of the SVC in cases like anomalous drainage of pulmonary
Abnormalities of Inferior Vena Cava
veins (TAPVC/PAPVC to SVC) or abnormal fistulous
communication to SVC.255 Inferior Vena Cava Interruption
Embryologically, it occurs due to failure of right subcardinal
Retroaortic Innominate Vein (Fig. 72.186) vein to develop properly and to anastomose with vitelline
Abnormal course of left innominate vein beneath the vein, resulting in enlargement of supracardinal system.
arch is named as retroaortic innominate vein and Incidence of IVC abnormalities is 0.6% in patients with
embryologically it is due to persistence of lower venous CHD, with 86% left isomerism, and it rarely occurs in
plexus between both anterior cardinal veins. Retroaortic isolation or with right isomerism.
innominate vein joins right SVC below the insertion of Clinical significance: This anomaly is of no physio-
azygos vein. logical value but complicates cardiac catheterization,
Clinical importance: This anomaly is of no hemo- cardiac interventions, and single ventricular repair.
dynamic consequences but during surgery, mobilization of Interrupted inferior vena cava with azygos continuation
right SVC is difficult. It should be especially visualized and (Fig. 72.187):256 There will be absence of intrahepatic seg-
differentiated from right PA on echocardiography. Most ment of IVC. Hepatic veins connect directly to RA or form
patients of retroaortic innominate vein have associated a common hepatic vein to connect to RA. Venous return
congenital cardiac malformations including TOF, truncus from lower body is via azygos vein, which connects to right
arteriosus, AVSD, heterotaxy syndrome, HLHS, pulmonary SVC or hemiazygos which connects to innominate vein.
atresia with intact ventricular septum, and CoA. Echocardiography: Subcostal sagittal view at the level
Echocardiography: Suprasternal short-axis view is of diaphragm shows anterior aorta, and a venous channel
best to demonstrate retroaortic innominate vein. On 2D posterior and on right side of the spine. Subcostal sagittal
Fig. 72.186: Two-dimensional echocardiography. Suprasternal Fig. 72.187: Two-dimensional echocardiogram. Subcostal sagittal
short-axis view with color flow mapping in a child with tetralogy view with leftward tilt and color flow mapping showing an ascen-
of Fallot showing laminar flow in retroaortic innominate vein. (Ao: ding venous channel (azygos continuation) posterior to aorta.
Decesnding Aorta; As Ao: Ascending aorta; Innom V: Innominate (Ao: Aorta; AZ: Azygos; RA: Right atrium).
vein; RPA: Right pulmonary artery).
view shows ascending venous channel posterior to aorta Abnormalities of Coronary Sinus257,258
(see Fig. 72.187) and hepatic veins entering RA, and no
continuity is seen between hepatic vein and IVC. Partial or Completely Unroofed
Interrupted inferior vena cava with hemiazygos continu- Coronary Sinus
ation: In this defect, the abdominal or hepatic portion of It manifests itself as LSVC to LA. Rarely there is no LSVC.
IVC is absent, and hepatic veins connect to RA. Systemic
venous return from lower body returns via hemiazygos Atretic Coronary Sinus Ostium
vein, which connects to LSVC or coronary sinus or directly
to RA. Thin membrane-like tissue causes atresia of coronary
Echocardiography: Subcostal short-axis view at level sinus ostium. There is an alternate exit for coronary sinus
of diaphragm shows aorta anterior to spine, and venous blood to flow via LSVC, or at times an abberant connection
channel (hemiazygos vein) on left and posterior to the with IVC. If there is no alternate exit, then the condition
spine. will be fatal because of myocardial infarction.
Rarely, venous return from lower body is to both SVC Echocardiography on color flow mapping in supraster-
via azygos vein on right side and hemiazygos vein on left nal short-axis and PSAX views shows retrograde flow in
side, and only hepatic vein continues to drain to the RA. LSVC (away from the heart and toward the transducer) if
LSVC exists with normal connecting pulmonary veins.
Inferior Vena Cava to Left Atrium
Anomalous Connection of Hepatic Vein to
Embryologically, it is impossible for IVC to connect to the
morphological LA. It is the persistence of Eustachian valve Coronary Sinus
directing the IVC blood to LA through foramen ovale or This rare anomaly is of no hemodynamic significance. On
ASD. Clinically, the patient presents with features of right- 2D echocardiography with color flow mapping, hepatic
to-left shunt. Rarely, with IVC type of sinus venosus ASD, venous flow to coronary sinus can be demonstrated in
right atrial connection of IVC may get atretic leading to a subcostal coronal view in the posterior plane.
condition mimicking IVC connecting to LA. Clinical significance: It is characterized by progressive
Echocardiography: Subcostal sagittal view shows and severe cyanosis after Fontan completion if not
inferior vena caval blood directed to LA or IVC directly recognized preoperatively.
connecting to LA. Saline contrast injection in femoral vein
demonstrates filling of LA from IVC.
Coronary Sinus Aneurysm/Diverticulum
Clinical importance: Clinical importance of hepatic
veins connecting to LA lies in recognizing the condition This rare condition has been reported in up to 8 to 10%
prior to Fontan or Kawashima procedure, as these may of cases presenting with supraventricular tachycardia,
lead to arterial hypoxemia and pulmonary arteriovenous undergoing radiofrequency ablation. Gold standard for
fistulas. The visualization can be done by echocardio- the diagnosis is angiography, but diagnosis can be made
graphy, angiography, CT scan, or MRI. by echocardiography if aneurysm is not very small.
Echocardiography: Subcostal coronal and apical four-
Bilateral Inferior Vena Cava chamber views with posterior tilt show out pouching with
a distinct neck arising from coronary sinus.
Bilateral venous systems contribute to the formation of the
normal IVC and thus can explain the possibility of bilateral
IVCs. Bilateral suprahepatic IVC are a frequent finding Prominent Venous Valves
in cases of visceral heterotaxy syndrome with asplenia.
These can rarely occur in normal visceral situs in which
Venous Valves
case left-sided hepatic veins drain into a normal coronary Venous valves such as Eustachian and Thebesian are
sinus. Bilateral infrarenal IVCs can occur in normal or prominent during fetal life and tend to involute after
abnormal visceral situs. Their visualization is particularly birth. Persistence can be in the form of small remnants.
essential whenever contemplating the Fontan completion Pathological persistence of venous valves can cause partial
or interventional procedures. or complete division of RA, that is, venous sinus-accepting
systemic veins and true RA (right atrial appendage and bypass can be created. In type II, the IVC is interrupted,
vestibule of tricuspid valve), known as cor triatriatum and single cannulation of the SVC and conventional
dexter. Right-to-left shunt may occur across foramen ovale. cardiopulmonary bypass can be achieved. In this case,
There is high association with RV and PA hypoplasia in hepatic veins can be cannulated with a small cannula after
these cases. The incomplete regression of the embryonic excision of the atrial septum or an intracardiac extractor
right valve of sinus venosus may leave a fenestrated is used to manage the blood flow. Most of these cases
or unfenestrated membrane in the RA that should be including the three seen by us have been associated with
considered a normal benign variant of the so-called Chiari heterotaxy syndromes.
network.
Echocardiography in subcostal sagittal and coronal
Decompressive Venous Channels
views shows persistence of an echodense structure in
RA and on color flow mapping, right-to-left shunt across Levoatrial cardinal vein: This is a persistence of connection
foramen ovale may be seen. This anomaly needs to be of pulmonary veins to systemic veins, which occurs due
differentiated from right atrial myxoma. Sometimes a very to severe left-sided obstruction and restrictive interatrial
prominent Chiari network may cause difficulty during communication; rarely does it occur in isolation. The
atrial septal device closure. levoatriocardinal vein arises directly from LA or from a
pulmonary vein and connects to right SVC, innominate
Total Anomalous Systemic vein, or jugular vein.
Venous Drainage Echocardiography: Subcostal coronal PSAX, and supra-
It is an exceptional form of CHD scantily described in sternal views can demonstrate the levoatriocardinal vein.
the literature. All systemic venous flow, including the On color flow mapping, flow is seen away from the heart and
RSVC, persistent LSVC, IVC, and coronary sinus drain toward the transducer with normally connecting pulmonary
abnormally into the LA. This disorder is usually associated veins.
with AV canal defects, common atrium, ASD, VSD, and Decompressive venous channels are especially
heterotaxy. It is a very rare disorder and a total of only common after Glenn shunt surgery for a univentricular
11 cases have been reported so far including three of our pathway. These can be suspected on echocardiography
unpublished cases. Surgical correction is often difficult and well profiled either by angiography, CT, or MRI. They
and complicated. Total anomalous systemic venous can be tackled either by catheterization (vascular plugs or
drainage can be classified into two types; accordingly, coils) or surgically. It is important to occlude the channel
the type of vena cava cannulation will vary. In type I, the before determining the pulmonary pressures in pre-
IVC is not interrupted and conventional cardiopulmonary Fontan catheterization.
PART 10: IMAGING OF CORONARY AND PULMONARY ARTERIES
CORONARY ARTERY ANOMALIES with bicuspid aortic valves. Even though various coronary
anomalies cannot always be identified by echocardiogram,
Coronary anomalies cause 10 to 19% of sports-related it is imperative to identify the origin of coronaries on every
deaths in athletes and 1.2% of non–sports-related deaths in echocardiogram. Failure to identify normal coronary
young individuals.259–264 In 69% of the population, there is a origins from the mid-portions of the left and right coronary
dominant RCA system, while in 11%, the LCA is dominant, sinuses will alert one to the possibility of an anomalous
thereby giving rise to the posterior descending coronary coronary origin.
artery, and in 20% there is codominance.263 Interestingly, a The origin and proximal course of the coronaries are
significant number of patients with bicuspid aortic valves identified in the PSAX views recorded from a high left
or aortic stenosis (20–57%) have left-dominant systems parasternal window using a high frequency transducer
and a short left main coronary artery.265–267 Also, separate (Figs 72.188A to D). The origin of left main coronary
origins of the LAD and left circumflex coronary arteries artery from the left coronary sinus, bifurcation of left main
occurs in about 1% of population and is more frequent coronary artery to LAD, and left circumflex and origin
A B
C D
Figs 72.188A to D: Two-dimensional echocardiography with color flow mapping showing normal coronaries. (A) Parasternal short-axis
view showing the right coronary artery (RCA) and the left anterior descending artery (LAD, arrow); (B) Color flow mapping showing the
course of LAD; (C) Parasternal short-axis view showing the origin of the right coronary artery (arrow); (D) Color flow mapping showing
right coronary artery (RCA). (Ao: Aorta; PA: Pulmonary artery; RVOT: Right ventricular outflow tract).
of the RCA from the right coronary sinus will be noted (ARCAPA) from the proximal PA or more distally from the
in this view. On gentle clockwise rotation of the probe, proximal right PA is a rare congenital anomaly with serious
a longer length of the LAD artery and its branches will consequences. In such cases, symptoms appear within a
be noted. Similarly, a gentle counterclockwise rotation few days of birth, and death may follow within a few weeks;
of the probe should be able to show a longer length of it is compatible with life only if associated with pulmonary
the RCA. hypertension or significant collaterals from the other
Numerous coronary artery abnormalities are described coronary artery system.
in normal as well as abnormal hearts. We will only focus The heart develops normally in fetal life. After birth, as
on clinically and significantly relevant abnormalities. long as the pulmonary arterial pressure remains at or near
systemic levels, the left ventricular myocardium is supplied
Anomalous Pulmonary Origin of by the anomalous artery and remains well perfused. As
pressure in the PA falls postnatally, perfusion of the LV
Coronary Artery268–273 becomes inadequate, as the coronary artery pressure falls
Anomalous origin of the left main coronary artery (the below the left ventricle end-diastolic pressure (LVEDP).
LAD, circumflex branch) from PA (ALCAPA) or RCA The consequence of this circulatory handicap is a decrease
of left ventricular function, due to myocardial ischemia. ductus) may not lead to the above picture, and in fact
At the same time, the ischemic myocardium is being may even show a normal coronary flow pattern on color
perfused increasingly by a developing set of collateral Doppler echocardiography. Thus, the diagnosis can be
vessels from the RCA, which are present normally. Those easily missed if the origin of the coronary arteries is not
with perfectly distributed collateral arteries before the PA profiled properly leading to a catastrophic event after
pressure falls escape with minor ischemic damage of the closure of these defects.274,275 It is, therefore, imperative to
left ventricular myocardium, which is well perfused, and recognize the entity by imaging the coronary arteries on
normal in form and function; this type is often diagnosed 2D echocardiography. Echocardiographic features of the
in adolescence or adulthood. Any case of LV dysfunction anomaly include:
should be evaluated for this anomaly. It is important to • Dilated RCA and absence of LCA from left sinus
remember that any condition wherein the PA pressures (Fig. 72.189).
are high such as high pressure shunt lesions (VSD or • Anatomical delineation of the anomalous origin of LCA
from PA is done by 2D echocardiography in PSAX view
at the level of great vessels. The common site of origin
of LCA from PA is from the posterior-facing sinus. Very
rarely, the anomalous LCA may have its origin from the
other nonfacing anterior sinus of PA. Rare instances
of origin of LCA from main PA trunk or right PA have
been reported, often missed on echocardiogram
and diagnosed by other means. Echocardiographic
identification of the origin has a major relevance to
surgical strategy, since establishment of dual coronary
supply is easily achieved only if the coronary artery can
be mobilized to the aorta (Figs 72.190A and B).
• Detection of left-to-right shunt in PA.
• Color Doppler demonstration of reversal of flow from
Fig. 72.189: Parasternal short-axis view showing a dilated right LCA (Figs 72.190A and B) to PA.
coronary artery in a case of anomalous left coronary artery ori- • Left ventricular dilatation and reduced systolic
gin from the pulmonary artery. (Ao: Aorta; RCA: Right coronary function; surgical risk stratification depends on left
artery).
ventricular function.
A B
Figs 72.190A and B: Two-dimensional echocardiography. (A) Shows the origin of the left main coronary artery (marked by the arrow)
from the pulmonary artery; (B) Color flow mapping shows flow reversal in the coronary artery with left anterior descending artery (LAD)
showing blue color flow signals indicative of flow toward the pulmonary artery in a case of anomalous origin of left coronary artery from
pulmonary artery (ALCAPA). (Ao: Aorta; PA: Pulmonary artery).
A B
Figs 72.191A and B: Two-dimensional echocardiography. Apical four-chamber view showing the sclerosed papillary muscle (arrow)
in a case of anomalous left coronary artery from pulmonary artery; (B) Color flow mapping showing mild mitral regurgitation. (LA: Left
atrium; LV: Left ventricle).
• Ischemic mitral regurgitation (Figs 72.191A and B). In all

infants and children with unexplained left ventricular
dilatation and mitral regurgitation, anomalous origin
of LCA from PA should be considered as a possible
etiology and actively excluded.
• Scarring of papillary muscles of the LV as a result of
myocardial ischemia, which acts as a very strong clue
in infants with severe left ventricular dysfunction to
the etiology for the myocardial dysfunction.
• Color Doppler demonstration of myocardial collateral
flow in the interventricular septum through septal
collaterals from RCA to LAD artery (Fig. 72.192).
Anomalous Origin of Right Coronary Fig. 72.192: Two-dimensional echocardiography. Modified para-
sternal short-axis view with color flow mapping in a case of
Artery from Pulmonary Artery276–278 anomalous left coronary from pulmonary artery showing a dilated
right coronary artery (RCA; arrow) with multiple collaterals from
Right coronary artery (RCA) origin from the pulmonary
right coronary artery (arrow). (Ao: Aorta).
trunk (ARCAPA) is rarer than anomalous origin of
LCA from the PA. These patients can present with left
ventricular dysfunction at a later age as compared to
patients of ALCAPA (Fig. 72.193). The coronary flow to
Tangential Origin of Coronary Artery
the interventricular septum is compromised. In infants Normal coronary artery origin is from the mid-portions
and children presenting with left ventricular dysfunction, of the left and right coronary sinuses of the aorta. The
the origin of RCA should also be looked for carefully. If coronaries are given off perpendicular to the aortic wall. In
it is not seen from the aortic sinuses, its origin from the some cases, coronary arteries can arise from other areas,
PA is a possibility. The other clue could be the presence namely high origin or commissural origin, arising from
of collateral flow in the interventricular septum with the noncoronary sinus, or arising tangentially instead
normal LCA. of perpendicularly. The tangential origin is associated
Various coronary abnormalities of coronary origin with a slit-like coronary orifice. This can be suspected
have been described.279-287 Few important ones are dis- on careful echocardiographic profiling of coronary
cussed here. origin in PSAX view using a high frequency transducer.
The common sites of drainage are RV in 30%, RA in 25%,

and PA in about 20%, rarely into LA, LV, coronary sinus, or
SVC. The termination may be a single or multiple orifices.
Echocardiographic features of coronary arteriovenous
fistula include (a) dilated proximal coronary artery, (b)
continuous color Doppler flows with high velocity rather
than normal velocity diastolic flows seen in normal
coronary arteries, (c) delineation of the course of the
fistula to its termination, unless it is tortuous when it may
not be possible to track it fully, (d) high-velocity turbulent
flow at the site of drainage, and (e) volume overload of
cardiac chambers depending on the magnitude of left-to-
right shunt through the fistula. Rarely, the fistula opens
into a large sac (fifth cardiac chamber), which opens into
Fig. 72.193: Two-dimensional echocardiography. Parasternal one of the cardiac chambers. Echocardiography provides
short-axis view showing anomalous origin of the right coronary
artery from the pulmonary artery (arrow). (Ao: Aorta; PA: Pulmo- good details of fistula in many patients. Angiographic
nary artery). classification of surgical relevance is proposed by
Sakakibara.288 Type A (proximal type)—proximal coronary
segment dilated to the origin of fistula, distal end normal;
Pathologists consistently observe a fibrous ridge at the and type B (distal type)—coronary artery dilated over
ostium of tangentially oriented ectopic coronary arteries. its entire length, terminating as a fistula in the right side
Such ridges are often said to have potentially catastrophic of the heart.
consequences. Nevertheless, plaque activation or
rupture is seldom documented, even on examination of CORONARY ANEURYSMS
the histological anatomy. High origin and commissural
origin of coronary arteries are also sometimes associated Aneurysms are defined as dilations of a coronary vessel
with the same anatomy. In these anomalies especially 1.5 times the adjacent normal coronaries. The aneurysm
high origin of coronary arteries above the aortic sinuses, can be saccular or fusiform, fusiform being the most
abnormal fluid dynamics have been documented to cause common. Aneurysms may be congenital or acquired,
significantly attenuated blood flows. the latter may be further subdivided into atherosclerotic,
Kawasaki disease, traumatic, iatrogenic (surgical, after
CORONARY ARTERIOVENOUS angioplasty, catheterization, or endomyocardial biopsy),
infectious, and systemic diseases (polyarteritis nodosa,
FISTULA (FIGS 72.194A TO C)
syphilis, Ehlers–Danlos syndrome, Marfan disease, and
Coronary arteriovenous fistulas are present in 1 of 50,000 scleroderma).
live births (0.002% of the general population) and are
visualized in 1 of 500 patients undergoing catheterization
Kawasaki Disease
(0.2–0.25%).288,289 The etiology of these lesions may be
congenital or acquired; the latter may be broken down Kawasaki disease is the commonest disease associated
into infectious, traumatic, and iatrogenic. Iatrogenic with coronary aneurysms in the pediatric age group.
causes may be further subdivided as after surgery, Moderate coronary involvement with aneurysms is present
catheterization, angioplasty, or endomyocardial biopsy. in 12.8 to 25% of patients with untreated Kawasaki disease;
We will be discussing congenital coronary arteriovenous the incidence of coronary involvement reduces to one
fistulae only. fifth after timely intravenous gamma globulin therapy. The
Coronary arteriovenous fistula involves RCA in 60%, following definitions pertaining to Kawasaki disease are
LAD artery in 25%, and left circumflex artery in 15% of generally accepted in the literature: segmental stenosis—a
patients. Rarely it involves more than one coronary artery. braid-like lesion with multiple tortuosities of the vessel,
A B
Figs 72.194A to C: A 2-year-old child with coronary arteriovenous

(AV) fistulae from right coronary artery (RCA) to right atrium (RA).
(A) Parasternal short-axis view showing a dilated right coronary
artery (arrow) at its origin; (B) Apical four-chamber view with pos-
terior tilt showing the dilated and aneurysmal right coronary artery
in the atrioventricular (AV) groove (arrow); (C) Apical four-chamber
view showing the aneurysmal RCA with small opening (arrow)
in the right atrium. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
C PA: Pulmonary artery; RA: Right atrium; RV: Right ventricle).
which represents recanalization of an occlusion; localized artery in the left AV groove. The circumflex artery can also
stenosis—a discrete, wedge-like narrowing at the inlet be profiled from long-axis view with posterior tilt and
or outlet of an aneurysm; ectasia—1.5 times larger than subcostal five-chamber view.
normal adjacent coronaries; dilation/small aneurysm—
up to 3 mm (± irregular lumen); aneurysms—4 to 8 mm; Atresia of Left Main Coronary Artery290
and giant aneurysms—greater than 8 mm.
On echocardiography, proximal parts of left main, Atresia of left main coronary artery with lack of luminal
LAD artery, left circumflex, and RCA can be defined from continuity from aortic root to LCA is associated with
parasternal short- and long-axis view at the level of great collaterals from the RCA through septal branches. This
vessels as described earlier. Apical four-chamber view mimics the septal collaterals of anomalous origin of LCA
with posterior tilt to the right side defines the distal part of from PA on echocardiography, but there will be no reverse
RCA in right AV groove and to left side profiles circumflex flow pattern seen in LCA and no left-to-right shunt in PA .
PART 11: ECHOCARDIOGRAPHIC EVALUATION OF AORTIC ARCH AND ITS ANOMALIES
Aortic arch anomalies constitute an important subgroup ABNORMAL FORMATION OF ARCH

of cardiac lesions, which may occur in isolation or in
association with other intracardiac defects. The thrust in The subgroup of aortic arch abnormities resulting from
the present century is on precise, noninvasive imaging of abnormal formation of aortic arch includes:
the aortic arch and its principal branches in order to plan • Mirror image right-sided aortic arch
timely appropriate treatment. • Vascular rings
Echocardiography is an important noninvasive diag- • Cervical aortic arch
nostic modality in diagnosing, monitoring the progression • Double aortic arch.
of lesions, and planning timely intervention and follow-
up after intervention in these lesions. The suprasternal Right Aortic Arch292–297
notch291 views are the most important echocardiographic Left and right aortic arch refer to which bronchus is
views in diagnosis of aortic arch anomalies. In neonates crossed by the arch, not to which side of the midline the
and infants, subcostal, high parasternal and apical views aortic root ascends. Practically, the sidedness of the aortic
can also provide imaging of the aortic arch, but these arch is determined indirectly with echocardiography
views permit imaging only up to the origin of first branch or angiography by the branching pattern of the
and these windows are inadequate in older children and brachiocephalic vessels. As a rule, the first arch vessel
adults. PLAX and short-axis views can image only the gives rise to the carotid artery opposite the side of the
proximal aorta and hence have limited usefulness. arch. The very rare cases of retroesophageal or isolated
2D echocardiographic imaging of the arch: The innominate artery are exceptions to this rule. But by
arch of aorta is best imaged from suprasternal view.291 far the more common source of error in the use of this
For the suprasternal views, the transducer is placed in rule is the difficulty in deciding which of the two carotid
the suprasternal notch and aligned as closely parallel arteries the first one is. A more reliable rule but one that
as possible with the sternum. In order to gain access to may be difficult to apply with ultrasound imaging is that
the suprasternal notch, the patient is positioned supine retroesophageal vessels or isolated vessels, that is, arising
with a pillow beneath the shoulders to extend the neck only from a ductus or ligamentum (without connection to
without producing tension on the sternocleidomastoid the aorta), are always opposite the side of the aortic arch.
muscles. The patient’s head is turned to the left or right The incidence of right aortic arch among patients
so that the chin does not prevent adequate placement of with tetralogy of Fallot has been reported to be anywhere
the transducer in the suprasternal notch. The ascending from 13 to 34%.292 The incidence in truncus arteriosus is
aorta, transverse, and descending thoracic aorta are generally higher than in tetralogy. An overall incidence of
best visualized in the suprasternal long-axis view. To 8% in patients with d-TGA with intact septum, and 16% in
obtain good suprasternal long-axis view, the transducer those with transposition, VSD and pulmonary stenosis has
is placed in the suprasternal notch with the plane of been reported.293
ultrasound beam oriented between the right nipple and The normal left-sided arch is viewed in suprasternal
the left scapular tip. Suprasternal short-axis view is used long-axis view with the plane of ultrasound beam directed
to define the branching pattern and side of arch. To obtain between the right nipple and left scapular tip with the first
suprasternal short-axis view, the transducer is placed in branch being the right innominate artery, which bifurcates
horizontal position in suprasternal notch and should be into right subclavian and right carotid artery. The diagnosis
turned to right to define bifurcation of first arch vessel with of right-sided arch is suspected when the transducer has to
left aortic arch, and to left with right aortic arch. be rotated counterclockwise between the left nipple and
Aortic arch anomalies can be classified as follows: right scapular tip in order to visualize the arch. Tilting the
• Abnormal formation of aortic arch transducer anteriorly and posteriorly from suprasternal
• Coarctation and its most severest form, that is, long-axis view images the relation of transverse aorta with
interrupted aortic arch the tracheal rings and tracheal air column, that is, in right
• Aortic aneurysms. aortic arch the transverse aortic arch is seen to the right
of trachea. Right-sided aortic arch is further confirmed ligamentum arteriosum or an atretic segment of aortic
if the first vessel courses leftward and then bifurcates, arch which cannot be profiled by 2D echocardiography.
which is seen in suprasternal short-axis view. If the first The common type of vascular ring anomalies include:
arch vessel courses to left but does not bifurcate, then • Left aortic arch with aberrant right subclavian artery
there are chances of more complex malformations being (Fig. 72.195) and right duct or ductus ligament
present such as an aberrant left subclavian artery as well • Right aortic arch with aberrant left subclavian artery
as increased chances of vascular ring formation. and left duct or ductus ligament
• Right aortic arch with retroesophageal segment and
left descending aorta, and
Vascular Rings
• Double aortic arch.
A vascular ring is an aortic arch anomaly in which the On echocardiography, diagnosis of left aortic arch with
trachea and esophagus are completely surrounded by aberrant right subclavian or right aortic arch with aberrant
vascular structures. The vascular structures need not be left subclavian is possible if there is failure to demonstrate
patent. Vascular rings are formed when an aortic arch bifurcation of the first arch vessel. Demonstration of origin
abnormality forms a ring of tissue encircling the trachea of fourth arch vessel, that is, aberrant subclavian is difficult
and esophagus. The presence of vascular ring anomaly by echocardiography, and definition of detailed anatomy
is highly probable if the suprasternal views demonstrate requires spiral CT or MRI. Double aortic arch can be
arch anatomy other than a left arch with a normal right directly visualized by performing a standard long-axis view
innominate artery or a right arch with mirror image and rotating the transducer 30° to 45° counterclockwise
branching. These rings are recognizable by the presence (Fig. 72.196). In suprasternal short-axis view, evidence of
of one of three “d”s opposite the side of the aortic arch: a double circle with tracheal ring in center is suggestive of
diverticulum, dimple, or descending aorta. A diverticulum double aortic arch. In some cases, even subcostal views
is a large vessel arising from the descending aorta that may demonstrate aorta bifurcating into two arches a few
gives rise to a smaller caliber vessel with a sudden taper. centimeters above the aortic valve. Demonstration of both
A dimple is a tapered, blindly ending out pouching from arches requires use of views for left and right aortic arch.
the aorta. Descending aorta opposite the side of the aortic Usually, one arch dominates and the other is hypoplastic
arch refers to its location in the upper thorax. These three or atretic. Origin of arch vessels should be defined from
“d”s form the vascular ring only when connected by a suprasternal views.
Fig. 72.195: Two-dimensional echocardiography in suprasternal Fig. 72.196: Two-dimensional echocardiography. Suprasternal
long-axis view showing the aberrant right subclavian artery short axis in a case of double aortic arch showing the right and left
(arrow) arising distal to the origin of the left subclavian artery. (Ds components of the arch. (R: Right component of the double aortic
Ao: Descending aorta; TA: Transverse arch). arch; L: Left component of the double aortic arch).
Cervical Aortic Arch298–301 of coarctation anatomy in most patients. High quality ultra-
sound images can be obtained in infants but may be some-
In this anomaly, the arch is found above the level of the what difficult to obtain in larger children and adolescents.
clavicle. There are two main subcategories of cervical arch:
• Those with anomalous subclavian artery and vascular Echocardiographic objectives:
ring, with either descending aorta contralateral to the • Is there coarctation?
arch or retroesophageal diverticulum. • Is there discrete stenosis or long segment stenosis?
• Those with a virtual normal branching pattern. • Is there arch hypoplasia?
Cervical aortic arch presents as a pulsatile mass and • Branching pattern of arch.
may be identified from the suprasternal long-axis view. It • Are there associated cardiac anomalies, in particular,
requires placement of transducer onto the neck over the bicuspid aortic valve, left-sided obstructive lesions
pulsatile mass, and suprasternal long-axis view will then such as aortic stenosis, subaortic membrane, mitral
show a long ascending aorta. valve disease, or hypoplastic left heart?
• Left ventricular hypertrophy.
• Ventricular function.
COARCTATION OF AORTA (CoA)302,303 The echocardiographic examination using the supra-
CoA is narrowing of the aorta, most commonly at the sternal long-axis view provides an image of the entire arch,
junction (isthmus) of the arch of aorta and descending with the area of coarctation seen near the origin of left
thoracic aorta. CoA occurs in approximately 6 to 8% of subclavian artery, that is, juxtaductal CoA (Figs 72.200A
patients with CHD.301 True CoA results from a localized and B). On 2D echocardiography, it is seen as a prominent
thickening of aortic media, which protrudes into the posterior shelf with significant coarctation. When imaging
lumen of the aorta from the posterior and lateral walls the arch, one must be absolutely certain that the entire arch
and obstructs blood flow (Figs 72.197A and B). Although is imaged, particularly in the region of the left subclavian
usually a discrete lesion, coarctation may consist of a artery. The diagnosis can be missed with inadequate
long stenotic segment or tubular hypoplasia. Very rarely, imaging in this area.
the narrowing may be located in the abdominal aorta Type of coarctation may either be a long segment
(Fig. 72.198). narrowing or, more commonly short segment obstruction
2D echocardiography and Doppler studies (Figs caused by posterior endothelial shelf projecting into
72.199A and B) provide an accurate, noninvasive assessment the aorta (Figs 72.197A and B). There may be associated
A B
Figs 72.197A and B: Two-dimensional echocardiography with color compare. (A) Suprasternal long-axis view with color compare in a
child of coarctation of aorta showing tiny posterior (arrow) shelf with flow acceleration; (B) Suprasternal long-axis view with color com-
pare in another case of coarctation of aorta showing a prominent posterior shelf (arrow) with significant turbulence in the arch starting
from the coarctation region. (As Ao: Ascending aorta; Ds Ao: Descending aorta; TA: Transverse arch).
Fig. 72.198: Arch measurements. Suprasternal long-axis view of

the ascending aorta and aortic arch showing the measurements to
be made. (A: Ascending aorta; B: Transverse arch; C: Isthmus; D:
Narrowest segment; E: Descending aorta).
A B
Figs 72.199A and B: Continuous wave Doppler signals across the coarctation of aorta. (A) Demonstrates mild coarctation of aorta
with early diastolic spill and a gradient of 31 mm Hg; (B) Shows severe coarctation of aorta with pan-diastolic flow and a gradient of
61 mm Hg.
A B
Figs 72.200A and B: Two-dimensional echocardiography. Suprasternal long-axis view showing interruption of the aortic arch distal to
the left subclavian artery with arch hypoplasia (arrow); (B) Represents suprasternal long-axis view showing interruption of the arch of
aorta (arrow) between the carotid arteries (type C). (As Ao: Ascending aorta; Ds Ao: Descending aorta; TA: Transverse arch).
hypoplasia of transverse arch. In the region of the ductus signal, diastolic spill, lumen size at the coarctation site, left
arteriosus, an anterior shelf may normally exist just ventricular hypertrophy, and PA pressures help in the final
proximal to the aortic origin of ductus. It is important not diagnosis.
to mistake this for the coarctation shelf. The measurements The coarctation area can also be profiled from high
which should be taken in a case of coarctation are shown PSAX view with counterclockwise tilt. This view opens up
in Figure 72.198. the coarctation area and descending aorta below the left
Certain intracardiac findings suggest the possibility subclavian artery.
that a CoA may exist before the arch is imaged. Left In patients with inadequate suprasternal widow, pulsed
ventricular outflow obstructive lesions, significant right or Doppler echocardiographic recordings from the proximal
left ventricular hypertrophy without obvious explanation, abdominal aorta can be useful. It shows a continuous
and the absence or reduced pulsation in descending antegrade flow signal with no evidence of flow reversal or
aorta as imaged from the subcostal window suggest the cessation, the time of peak velocity is prolonged, and the
possibility of CoA. mean acceleration rate is decreased.
Doppler Feature of Aortic INTERRUPTION OF AORTIC ARCH

Coarctation303–311 Arch interruption represents the most severe form of
Doppler echocardiography can assist in determining the coarctation, which can be demonstrated from suprasternal
hemodynamic severity of coarctation. First, color flow views.312,313 Interruption of arch of aorta is generally
imaging of the descending aorta is performed, looking associated with complex CHDs like AV canal defect,
for a high-velocity (turbulent flow) jet in the region of d-transposition of great arteries with or without tricuspid
coarctation. Once such a jet is imaged on color Doppler atresia, Taussig–Bing anomaly, and congenitally corrected
examination, the continuous wave Doppler beam is transposition apart from VSD, PDA, AP window, subaortic
directed into the area of turbulent flow (Figs 72.199A and stenosis, bicuspid aortic valve, and mitral stenosis. The
B). The Doppler recording usually shows a high velocity interruption of arch of aorta may involve only a short
jet with antegrade flow extending well into diastole. This segment or a long segment with a long distance between
flow pattern is characteristic of severe obstruction in a the proximal and distal segments of the aorta. Celoria
vessel with a pressure gradient extending into diastole.
and Patton312 classified them as type A if the interruption
As the severity of obstruction increases, the pressure
was distal to the left subclavian artery, type B if between
above the coarctation remains elevated for a longer
carotid and subclavian arteries, and type C if between
period of the cardiac cycle, and thus, the jet extends into
carotid arteries. However, these types may be further
the entire diastole. From the peak velocity of the jet (V2)
subcategorized313 and definitions generalized to include
and the simplified Bernoulli equation (P = 4V22), the peak
both right and left arch patterns as in Table 72.16.
instantaneous pressure gradient across the coarctation
Type B arch interruption is the most common form
can be calculated. Many patients with CoA have multiple
left heart obstructive lesions (e.g. bicuspid aortic valve, and is usually associated with conotruncal anomalies
valvular aortic stenosis, subaortic membrane, etc.) that and normally aligned great arteries in which there is a
can lead to an increased peak flow velocity proximal to large malalignment type of VSD associated with posterior
the coarctation jet. If the peak velocity proximal to the displacement of the infundibular septum and subaortic
coarctation (V1) exceeds 1 m/s recorded by pulsed Doppler obstruction. Type B interruption is also commonly seen in
echocardiography from suprasternal long-axis view, then association with DiGeorge syndrome. Type A interruption
the expanded Bernoulli equation (P = 4[V22 − V12]) should tends to occur with aorticopulmonary septal defect and
be used to avoid a significant overestimation of the peak intact ventricular septum and in a large group of patients
gradient. Even then the gradient across the coarctation with transposition of great arteries.
may be unreliable. The gradient depends on the shape and On 2D echocardiography, suprasternal views show
length of the coarctation segment, the patient’s cardiac ascending aorta continuing to at least one of the arch
output, and the presence and extent of collateral flow. vessel. In type A arch interruption, all three branches are
Thus, the gradient can over- or underestimate the severity seen proximal to interruption; in type B interruption, first
of coarctation. If the left ventricular ejection fraction is low, and second branches are seen proximal to interruption;
there can be underestimation of severity of coarctation and in type C interruption, only the first branch, that is,
by Doppler. In such cases, a slow dP/dt of the systolic innominate artery is seen arising proximal to interruption.
Table 72.16: Classification of Interrupted Aortic Arch

Type A: Interruption distal to the left subclavian artery
• Without retroesophageal or isolated subclavian artery
• With retroesophageal subclavian artery
• With isolated subclavian artery
Type B: Interruption between second carotid and ipsilateral subclavian artery
• With retroesophageal subclavian artery (i.e. both carotid arteries proximal, both subclavians distal)
Type C: Interruption between carotid arteries
• With retroesophageal subclavian artery
Descending aorta is relatively dilated with ductal conti- recognized from the parasternal long-axis, PSAX, subcostal
nuation to descending aorta, which is well profiled in coronal, and suprasternal long-axis views. Aneurysmal
suprasternal long-axis and high PSAX views. Care should involvement of descending aorta usually occurs secondary
be taken to ensure that the ductus arteriosus connecting the to previous surgical or catheter intervention such as
main PA to descending aorta (ductal arch) is not mistaken surgical repair of CoA or balloon dilatation of coarctation.
for the true arch. Color flow mapping confirms the findings Chances of aneurysmal formation are much less after stent
and as systemic flow is duct-dependant, patency of duct deployment for CoA. Descending aorta aneurysm can be
or any restriction of duct should be defined by color flow defined from suprasternal long-axis, suprasternal short-
mapping. Pulsed Doppler is used to assess the gradient axis (aneurysm from lateral wall), and subcostal sagittal
across the ductus. Sometimes, restriction of ductus views in infants and children. The echocardiographic
arteriosus can mimic CoA, and that should be clearly measurements are helpful in diagnosing and assessing
defined. In patients with suprasystemic PA pressures, there the progression of the disease. Besides visualization of the
can be turbulent right to left flow across the ductus. aneurysm, echocardiographic examination should also
focus on detecting complications of aneurysms such as
AORTIC ANEURYSM compression of adjacent structures and fistula formation.
Transthoracic echocardiography is a useful modality
The third important subgroup of aortic arch anomalies
in diagnosis, monitoring, and planning appropriate non-
includes aneurysms of the aorta. Aneurysm of a vessel
surgical/surgical management and in postintervention
is defined as a dilated segment > 50% in diameter as
compared to the proximal segment. In pediatric age follow-up of these patients. Transthoracic echocardio-
group, in contrast to adults, aneurysm of ascending aorta graphy has limitations in grown-up children because of
is more common than descending aorta. Annuloaortic suboptimal acoustic windows and in the postintervention
ectasia is defined as aneurysmal involvement of annulus period. In such patients, transesophageal echocardio-
and aortic root, in addition to ascending aorta. In children, graphy spiral CT, or MRI can demonstrate these lesions.
aneurysmal dilatation of proximal aorta can be caused by
conditions associated with medial degeneration of the Anomalous Origin of Branch
aorta such as Marfan syndrome, Ehlers–Danlos syndrome, Pulmonary Artery from Ascending
Turner syndrome, in association with bicuspid aortic valve,
and idiopathic; however, some types of infectious disease
Aorta (Fig. 72.201)314,315
like bacterial endocarditis can also result in aneurysm Anomalous origin of one PA from ascending aorta used to be
formation. Aneurysm of proximal aorta is usually easily known as “hemitruncus” (This terminology is not used now).
Echocardiographic diagnosis of origin of PA from

ascending aorta depends upon demonstration of:
• Absence of normal bifurcation of main PA
• RVOT continuing as one PA
• Origin of one PA from ascending aorta.
Subcostal coronal view with anterior tilt, PLAX view,
and suprasternal long-axis and short-axis views show
origin of a branch pulmonary artery (PA) from ascending
aorta. PSAX view profiles main PA continuing as one PA
and with change in the plane of ultrasound, origin of
another PA from ascending aorta can be profiled. This is
differentiated from truncus arteriosus, by demonstrating
two separate semilunar valves, and from APW by
demonstrating PA bifurcation in the latter defect.
Fig. 72.201: Two-dimensional echocardiography in modified
suprasternal short-axis view showing the origin of the left Pulmonary Artery Sling316–318
pulmonary artery from the ascending aorta. (Ao: Aorta; LPA: Left
pulmonary artery; RPA: Right pulmonary artery). Pulmonary artery sling is a rare anomaly in which the left PA
arises from right PA and passes posteriorly between trachea
and esophagus to the left side. Due to this course, left PA
This anomaly needs to be differentiated from APW, truncus forms a sling, encircling trachea, anteriorly main PA, right
arteriosus, and discontinuous pulmonary arteries with one and posteriorly left PA, and on the left ductus arteriosus or
PA supplied from ductus or a collateral, as in the setting of ductus ligament. PA sling compresses the right bronchus
VSD with pulmonary atresia. By far, the more common form and trachea from the posterior aspect as it courses to left and
is anomalous origin of the right PA, seen in 82% of 108 cases posterior after its origin from right PA. The child presents with
excellently reviewed by Kutsche and Van Mierop.314 The respiratory distress and stridor in infancy.
anomalous right PA usually arises from the posterior aspect of This anomaly should be looked for in infants presenting
the ascending aorta close to the aortic valve. Less commonly, with stridor and respiratory distress. The anomaly could
first be suspected if PSAX view does not show normal
it originates from the lateral ascending aorta just proximal
main PA bifurcation, main PA seems to continue as right
to the innominate artery. PDA and aorticopulmonary septal
PA , and on tilting the transducer inferiorly and to left,
defect are commonly associated with anomalous origin
left PA can be profiled arising from right PA . After origin,
of the right PA; other cardiovascular anomalies are rare. In left PA passes posterior to echogenic shadow of trachea.
contrast, TOF and aortic arch anomalies, for example, right This finding can also be defined from high PSAX view
aortic arch and anomalous origin of the subclavian artery, are and modified suprasternal short-axis view. With the use
common in anomalous origin of the left PA. An association of color flow mapping, it becomes easier to define the
with DiGeorge syndrome, frequently noted with persistent anomaly. Associated defects such as VSD, ASD, and TOF
truncus arteriosus, is not seen with anomalous origin of a PA can occur in 40% of cases of PA sling and should be looked
from the ascending aorta. for on echocardiography.
PART 12: UNIVENTRICULAR HEART AND HETEROTAXY SYNDROME
INTRODUCTION directed to the pulmonary circulation either partially

Univentricular heart means a cardiac malformation in (bidirectional Glenn shunt) or totally (modified Fontan
which a biventricular repair is not feasible and hence these surgery). As per the unified reporting system of the Society
hearts will be subjected to the “surgical univentricular of Thoracic Surgeons-Congenital Heart Surgery Database
pathway,” where the systemic venous return will be Committee, this includes the following.
Hearts unsuitable for biventricular correction are: • Single inlet ventricle: One of the AV valves is atretic
• Univentricular AV connections and not patent. In this entity, either the left or the right
• Tricuspid atresia AV valve is atretic and it is better to use the term left
• Mitral atresia and HLHS AV valve atresia or right AV valve atresia rather than
• Pulmonary atresia with intact ventricular septum tricuspid and mitral atresia (Fig. 72.203).
• Heterotaxy syndromes • Common inlet ventricle: In this case, the AV connec-
• Cardiac malpositions tion is through a common AV valve; this condition is
• DORV with nonroutable VSD. commonly seen in heterotaxy syndromes (Figs 72.204A
and B). The AV valve has free-floating leaflets and is
UNIVENTRICULAR ATRIOVENTRICU- classified as Rastelli type C AVSD.
LAR CONNECTIONS319-324
Double Inlet Ventricle
The unifying feature of hearts previously described
as single, common, or univentricular is that the AV The classic form of single or common ventricle described
connection is completely or predominantly to a single as double inlet ventricle has been classified by Van Praagh
ventricular chamber. Two basic situations may be present into four types:
in univentricular physiology: • Double inlet LV
• Both AV valves are committed to one ventricular • Double inlet RV
chamber. • Double inlet ventricle of mixed morphology
• There may be only one AV valve permitting the access • Double inlet ventricle of indeterminate or undiffer-
of only one atrium to the dominant ventricle (with the entiated morphology.
second ventricle being rudimentary). The echocardiographic recognition of a double
The literature has varying names for these hearts, inlet ventricular with left or right morphology is based
namely cor triloculare biatriaum, cor biloculare, single on two well-defined morphological principles. The
ventricle, common ventricle, and univentricular heart. first morphological principle states that left ventricular
This group is subclassified into three groups based on the myocardium typically has a relatively smooth appearance
AV valve connections: with numerous fine oblique trabeculations, whereas
• Double inlet ventricle (Fig. 72.202): Common form of RV myocardium has an irregular surface with relatively
single ventricle where there are two patent AV valves. coarse straight trabeculations. The second principle states
Fig. 72.202: Two-dimensional echocardiography. Apical four- Fig. 72.203: Two-dimensional echocardiography. Apical four-
chamber view showing the two inlets into a ventricle with left ven- chamber view showing in a case of tricuspid atresia with hypo-
tricular morphology in a case of double inlet left ventricle (DILV). plastic right ventricle (RV), a large atrial septal defect (arrow) and
(LA: Left atrium; LV: Left ventricle; RA: Right atrium). a small muscular ventricular septal defect (VSD; star). (LA: Left
atrium; LV: Left ventricle; RV: Right ventricle; RA: Right atrium).
A B
Figs 72.204A and B: Two-dimensional echocardiography. (A) Apical four-chamber view showing complete atrioventricular septal defect
(AVSD) with a non–apex-forming small left ventricular (LV) cavity not suitable for biventricular pathway; (B) Shows a common AVSD
(unbalanced type) with hypoplastic right ventricle (RV) on a single ventricular pathway. (LA: Left atrium; LV: Left ventricle; RA: Right
that the ventricular chamber that has an infundibulum and not giving off any great arteries, it is morphologically
giving rise to a great artery whether it represents the main the LV.
ventricle or the hypoplastic outflow chamber represents To restate the above echocardiographic principles for
the morphological RV. As a corollary, the ventricular differentiation between the morphological right and LV
chamber having a direct arterial connection without an again, if there is a univentricular heart with a rudimentary
intervening infundibulum represents a morphological LV. chamber located posteroinferiorly, which does not give
A good echocardiographic view of the foramen off a great artery, and remains a blind pouch, then it is
(bulboventricular foramen) separating the main ventricle the left ventricular rudimentary chamber and so the main
into which both the AV valves connect with the outlet ventricle gets described as double inlet ventricle of RV
chamber should be obtained by using subcostal and morphology. Van Praagh describes such a morphological
parasternal windows. The differentiation of ventricular double inlet ventricle of RV morphology as a “hip pocket
morphology as smooth surface versus irregular surface rudimentary left ventricular chamber,” to indicate that
it is always posteriorly located. To summarize, double
is also best made on these views. A careful look at either
inlet ventricle is classified as double inlet ventricle of left
side of the bulboventricular foramen, (one side of which is
ventricular morphology (accounts for 80% of double inlet
main ventricle and other side is the rudimentary chamber)
ventricles) if the rudimentary outflow chamber is located
will show which of the sides has rough trabeculations and
anteriorly and gives off a great artery, and the rudimentary
which side has a relatively smooth surface and this will
chamber has relatively coarser trabeculations on its
define the main chamber and the rudimentary chamber
surface near bulboventricular foramen. In double inlet
as either the morphological right or the morphological ventricles of RV morphology (accounts for < 10% of double
left. inlet ventricles), the rudimentary chamber is a blind
Identifying on echo the infundibular chamber, which posteroinferior “hip pocket pouch”; it does not give off
always goes with morphological RV, is important. A any great artery and the main ventricle will have coarser
patent infundibulum always gives off a great artery and walls than the rudimentary ventricle. The other two
echocardiographic identification of an infundibulum is varieties of double inlet ventricle described by Van Praagh
done by noting the separation from the AV valves, namely namely double inlet ventricle of mixed morphology and
the AV valve–semilunar valve fibrous discontinuity. So a indeterminate morphology (account for < 10% of cases and
hypoplastic subarterial outlet chamber noted on echo- are very rare) have echocardiographically no rudimentary
cardiography is indicative of morphological RV. If the chamber and both great arteries arise from the same main
rudimentary chamber is a blind chamber located inferiorly ventricular cavity.
Double Inlet Left Ventricle Assessment of the Systemic Veins

This is the commonest type of double inlet ventricle In every patient with univentricular AV connection, since
accounting for almost 80% of the double inlet ventricles. the path of palliation is univentricular, it is mandatory
This group is subdivided into three subgroups based on to be precise about the systemic and pulmonary venous
the great artery relationship: anatomy before planning surgery. Systemic venous
• Left and anterior Aorta (L-malposed aorta): In 55% anomaly commonly present is bilateral SVC. With left
of double inlet left ventricle (DILV), the main LV isomerism, interrupted IVC should be looked for (seen in
gives off the PA , there is pulmonary valve–AV valve 85% of patients).
fibrous continuity, AV discordance, the rudimentary
chamber is located on the left and anterior side of the Assessment of the Pulmonary Veins
morphological main LV, and the anterior rudimentary
RV infundibular chamber gives off the aorta and the Anomalies of pulmonary venous return are also common
aorta is left and anterior to the PA . in single ventricle patients, especially in the setting of
• Right and anterior Aorta (D-malposed aorta): In 30% of heterotaxy syndromes. In single ventricular physiology
DILV, the main LV gives off the PA, there is pulmonary with reduced pulmonary flows, the anomalous pulmonary
valve–AV valve fibrous continuity, the anterior venous drainage and associated obstruction of pulmonary
rudimentary RV infundibular chamber gives off the veins may not show up clearly since the pulmonary venous
aorta, and the aorta is right and anterior to the PA. flow will be of low velocity. Unidentified anomalous
• Normally related great arteries (Holmes Heart): In 15% pulmonary venous drainage into the azygos vein may
of DILV, the main ventricular chamber gives off the inadvertently be ligated/clipped as a part of Glenn shunt
aorta, there is aortic–AV valve fibrous continuity, the with serious implications. The echocardiographer should
rudimentary anterior infundibular chamber gives off the make serious attempts to trace all the pulmonary veins
PA, the PA is located left and anterior to the aorta, and meticulously and ensure that no individual pulmonary vein
the aorta–PA relation resembles that of a normal heart. drains anomalously into a chamber other than the atria and
Apical view shows double inlet ventricle and PSAX view there is no obstruction in the pulmonary venous pathway.
shows normally related aorta–PA relationship; there is
narrowing of the subpulmonary infundibulum causing Assessment of the Atrioventricular Valves
subvalvular pulmonary stenosis.
Rare variations from this schema include double outlet It is imperative to assess the function of the AV valves
from the rudimentary chamber, atresia of one semilunar precisely, since the AV valve function has an important
valve often the pulmonary valve, and truncus arteriosus. bearing on the univentricular repair.
Stenosis of the AV valves is common and may mani-
fest with minimal gradients. Morphological features of
Double Inlet Right Ventricle these valves may be supramitral ring, hypoplastic AV valve,
The main RV gives off both great arteries, the semilunar double orifice AV valve, parachute mitral valve or dysplas-
valves of both aorta and PA are not in fibrous continuity tic AV valve. These have major effects on the univentricular
to the AV valves, and a well-discerned rudimentary hip pathway. Significant regurgitation of the AV valves too pos-
pocket (chamber) located posteriorly is of left ventricular es problems in univentricular surgical approach by increas-
morphology. Depending on the location of the posterior ing the atrial pressures and thereby impeding pulmonary
rudimentary LV, the segmental approach will vary as venous return. Such valves may need annuloplasty and
D-loop (when the rudimentary chamber is posterior and repair to minimize the degree of AV valve insufficiency.
to the left), and L-loop (when the rudimentary chamber is In patients with volume-overloaded heart, with increased
posterior and to the right of the main RV). Since the long- pulmonary blood flow, the ventricular dilatation and
term outcome after Fontan surgery for single ventricles of associated AV valve annular dilatation results in regurgi-
RV morphology is suboptimal compared to the single LVs, tation. Once the volume overload is arrested by surgical
echocardiographic identification of the precise ventricular intervention like PA banding, ventricular volume
morphology as right and left is not just of academic interest reduces immediately and the AV valve regurgitation of such
but has prognostic significance. patients will reduce in severity.
Bulboventricular Foramen and mitral atresia with hypoplastic right or left heart.
Morphological definition for a single ventricle with one
The bulboventricular foramen is the orifice through AV valve atresia is that the atretic or imperforate plate of
which the main ventricular chamber feeds blood to the
the valve annulus overrides the ventricular septum and is
rudimentary outflow chamber. If the chamber supports
predominantly (>50%) committed to the main ventricle.
the pulmonary flows like in Holmes heart, then restriction
This means there is malalignment of the atrial and
of the bulboventricular foramen results in restricted
ventricular septa produced by AV valve annular override.
pulmonary blood flow. If the rudimentary outflow
Echocardiographic recognition of this feature will be done
chamber supports the aortic circulation, restriction of
by demonstrating malalignment of the atrial and ventricular
bulboventricular foramen results in subaortic obstruction.
septum in a four-chamber, subcostal coronal and long-axis
To calculate the area of bulboventricular foramen, measure
view with anterior tilt. In tricuspid atresia and mitral atresia,
the maximum diameter of the bulboventricular foramen
there will be alignment of the interatrial and ventricular
in two orthogonal planes. If the dimensions are “a” and “b,”
septum and the atretic plate of the AV valve will be related to the
then the area of the bulboventricular foramen is calculated
assuming it to be an ellipse using the formula: hypoplastic RV or hypoplastic LV and will not override the
ventricular septum.
Area = a + b/4
When the area of the bulboventricular foramen is
TRICUSPID ATRESIA
> 2.0 cm2/m2, the foramen is considered nonrestrictive.
In patients with area < 2.0 cm2/m2, during the initial Tricuspid atresia is defined as complete agenesis of the
palliation, the restrictive bulboventricular foramen may tricuspid valve with no direct communication between the
need to be addressed using one of the surgical strategies RA and RV. Morphologically, this entity is classified as in
like Damus–Kaye–Stansel procedure (end-to-side ana- Table 72.17.
stomosis of the main PA to the ascending aorta) or Echocardiographic differentiation between tricuspid
enlargement of the bulboventricular foramen. In patients atresia and single ventricle with right AV valve atresia has
in whom the bulboventricular foramen is anatomically already been highlighted in the previous discussion. We
smaller though nonrestrictive by Doppler recordings, a will now discuss the important echocardiographic features
close echocardiographic watch is justified. The progression of this entity.
of the hemodynamic narrowing of the bulboventricular
foramen is explained as follows: initially with acute volume
unloading of the ventricle after PA banding, there is an Tricuspid Atresia Type I
acute reduction of the ventricular volumes and reduction Tricuspid atresia is associated with normally related great
of the bulboventricular foramen area. Over long-term arteries in almost 75% of the cases. Rare instances are
follow-up, there is progressive ventricular hypertrophy, absence of interventricular communications and these
which too results in further restriction of bulboventricular
patients have pulmonary atresia and do not have an
foramen size. In neonates who are too young and small to
echocardiographically demonstrable RV at all (Type Ia).
tolerate an enlargement of bulboventricular foramen or
However, in many instances, the VSD is restrictive and
Damus–Kaye–Stansel procedure, alternative approaches
this restrictive VSD is the site of significant subpulmonary
followed include palliative arterial switch operation to
stenosis (Type Ib). Type Ia and Ib may require BT shunt
shift the restrictive rudimentary outflow chamber from
prior to the planned Glenn shunt depending upon the
subaortic location to subpulmonary location. Alternative
systemic saturations. In patients with normally related
method is the measurement of the VSD size and comparing
great arteries, a large VSD (Type Ic) is often associated
it with the aortic annulus size. If the 2D measurement of
with unrestricted pulmonary blood flow and these
the VSD is less than aorta, it requires enlargement.
patients present early with cyanosis and congestive
heart failure and need PA banding. Echocardiographic
Single Inlet Ventricle evaluation of tricuspid atresia for a presurgical evaluation
This subgroup needs to be echocardiographically is mostly similar to the issues discussed under double inlet
differentiated from morphological tricuspid atresia ventricles.
Table 72.17: Classification of Tricuspid Atresia

Type 1: Normally related great arteries (75% of cases)
Type 1a with pulmonary atresia
Type 1b with pulmonary hypoplasia, small VSD
Type 1c with no pulmonary hypoplasia, large VSD
Type 2: d-Transposition of great arteries (25% of cases)
Type 2a with pulmonary atresia
Type 2b with pulmonary or subpulmonary stenosis
Type 2c with no pulmonary hypoplasia and large VSD
Type 3: l-Malposition of great arteries (very rare)
Type 3a, pulmonary or subpulmonary stenosis
Type 3b, subaortic stenosis
(VSD: Ventricular septal defect)
Tricuspid Atresia Type II the duct, since the arch and ascending aorta get retrograde
flows from the patent ductus. Prompt recognition of
In patients with tricuspid atresia and d-transposition of such flow reversal in aortic arch and duct dependence
great arteries, the size of VSD decides on the develop- of systemic circulation will help in early institution of
ment of subaortic stenosis and hence it needs assessment prostaglandin E1 therapy and transfer to an advanced
similar to the bulboventricular foramen of univentricular cardiac surgical center.
heart. Another echocardiographically significant lesion Another important echocardiographic decision-mak-
frequently coexisting with tricuspid atresia and transposi- ing issue is identification of adequacy of interatrial septal
tion of great vessels is aortic arch anomalies including arch communication. Since ASD is the only outlet for pulmo-
and isthmic hypoplasia. In some patients with tricuspid nary venous blood, inadequate atrial communication will
atresia with transposition of great vessels, there will be left need urgent enlargement either in cardiac catheterization
juxtaposition of atrial appendages. This condition is rec- suites or operation rooms. In some cases of mitral atresia,
ognized echocardiographically by noting absence of right decompression of the LA through a small unroofing of the
atrial appendage normally demonstrated in subcostal coronary sinus may be present.
bicaval view as an anterior projection from the RA
immediately below the SVC–right atrial junction. In jux-
taposition of atrial appendages, the appendage is missing
Hypoplastic Left Heart Syndrome
in this location, but instead noted on left side of the great Initial echocardiographic assessment of the HLHS (before
arteries. Stage 1 palliation, i.e. before Norwood procedure): The
goal of the assessment is to provide an accurate diagnosis
MITRAL ATRESIA AND HYPOPLASTIC and complete hemodynamic information keeping in mind
that the parameters for future univentricular pathway
LEFT HEART SYNDROME need to be looked at.
(FIGS 72.204 AND 72.205) Objectives of echocardiography are to assess:
This condition is included in this discussion, since it • Anatomical and physiological components of HLHS:
also needs a univentricular type of repair. This syndrome LA, mitral valve, LV, LVOT, aortic valve, ascending
embraces a continuum of congenital cardiac anomalies aorta, aortic arch, and isthmus
characterized by underdevelopment of the aorta, aortic • Adequacy of interatrial communication
valve, LV, mitral valve, and LA to a varying extent. • Anatomy of the ductus and determine its adequacy
Echocardiographic diagnosis of this entity will help in and physiology
quick recognition of the duct dependency of the systemic • Anatomy of the RV, RV outflow, tricuspid valve, and
circulation. The aortic arch color Doppler flow imaging will pulmonary valve
show typical flow reversal in arch of aorta from the level of • RV function, AV regurgitation if any.
A B
C D
Figs 72.205A to D: Two-dimensional echocardiography with color flow mapping in a case of left ventricular (LV) hypoplasia. (A) Four-
chamber view with color flow mapping showing a restrictive interatrial communication; (B and C) Apical four-chamber view with color
flow compare showing a small LV cavity with thickened endocardium and flow acceleration across the mitral valve; (D) Suprasternal
view showing a hypoplastic ascending aorta and transverse arch. (As Ao: Ascending aorta; Ds Ao: Descending aorta; LA: Left atrium;
LV: Left ventricle; RA: Right atrium; TA: Transverse arch).
Associated Systemic Venous Anomalies as compared to the small LV. From the subcostal views,
a careful assessment of the adequacy of the interatrial
The evaluation is as for all CHD entities with sequential communication is done. The other feature that needs to
analysis. Certain characteristic features that may be looked be looked at includes pulmonary venous connections, size
at are enumerated below. and function of the ventricular chambers, and evaluation
of the AV valves.
Subcostal View Atrial septum: Frequently, there is a leftward deviation
As the transducer is tilted toward the cardiac base, the of the superior attachment of the atrial septum. The severity
coronary sinus, RA, and RV are visualized. A dilated of the restriction at the atrial septal communication can
coronary sinus should alert one for the presence of be assessed by using color or spectral Doppler. The mean
LSVC or anomalous pulmonary venous connection to gradient is obtained over three cardiac cycles.
the coronary sinus. The RA in typical scenario is dilated Pulmonary veins: Anomalous pulmonary venous
and hypertrophied from obligatory left-to-right shunting connection/drainage occur in 5 to 10% of the cases of HLHS.
through the patent foramen ovale. The RV also is dilated In cases of restricted interatrial septal communication,
a decompressing vein may be visualized from the LA measured in this view. Angling the transducer inferiorly
draining to variable locations. These may be stenosed and, allows assessment of the tricuspid valve and tricuspid
therefore, a full view of the course of the drainage of these regurgitation. Angling the transducer anteriorly shows the
venous channels is needed. This could be assisted by use pulmonary valve and the RVOT. This needs to be seen for
of PW Doppler interrogation and color flow mapping. the presence of any obstruction. Pulmonary regurgitation
Anterior angulation demonstrates the dilated RV needs to be evaluated.
and RVOT. This needs to be carefully evaluated as this Short-axis sweeps allow assessment of the mitral valve
will be the sole outflow tract from the cardiac chamber and subvalvular pathology. It also allows determining
in the near future. Associated pulmonary stenosis and the morphology of the aortic valve and the coronaries.
pulmonary valve regurgitation represent a poor subset. The RCA is usually prominent because of its supply to the
The hypoplastic (tiny) ascending aorta may at times be dominant RV. In the presence of LV to coronary fistulous
better visualized in the subcostal views. connections, the LCA may become prominent. High
PSAX view allows visualization of PA branches and the
Apical Views arterial ductus. This view will also show the length of the
decompressing venous channel by tilting the transducer
Apical four-chamber view provides comparison of the
posteriorly. Slight counterclockwise rotation may show
relative right and left sides of the heart. The RV is generally
the large ductus; this may be larger than the arch and the
large and hypertrophied, and the LV is small, muscle-
pulmonary arteries.
bound, and nonapex forming. The endocardial surface
of the LV is often echogenic because of endocardial
fibroelastosis. This in itself is a very important prognostic
Suprasternal Notch View
marker (particularly in borderline situations where one is This provides full assessment of the aortic arch. The
contemplating a two-ventricle repair). The morphology of sidedness of the arch should be determined; the aortic
the mitral and the tricuspid valve can also be evaluated arch has a more acute curve than normal in these patients.
in this view. The annulus of the mitral valve is typically The sizes of the ascending aorta, transverse arch, and
hypoplastic, severely stenosed, or may be atretic. This isthmus should be measured and the posterior shelf of
may be evaluated using color flow mapping and Doppler coarctation of the aorta should be evaluated. Doppler
assessment. In case of stenosis of the mitral valve without sampling of the distal segment of the aortic arch reveals
mitral regurgitation, one should be alerted to the likelihood retrograde flow in systole into the transverse arch and
of ventriculocoronary sinusoids. antegrade flow in diastole into the ductus arteriosus and
Tricuspid valve morphology and function needs to be pulmonary vascular bed.
assessed also. It is important to identify and quantify the
tricuspid regurgitation, an important prognostic feature. Echocardiographic Assessment
RV function is also assessed in this view. After the Stage 1 Palliation
Anterior tilt will show the aortic valve, ascending aorta,
and pulmonary trunk. Doppler assessment of the aortic The Norwood procedure consists of surgical
valve and color flow mapping will show the patency of the reconstruction and augmentation of the ascending aorta
aortic valve. The pulmonary valve should also be similarly and aortic arch with aortopulmonary amalgamation,
investigated. an atrial septectomy, and systemic to pulmonary shunt,
which can be either a Blalock–Taussig shunt or a RV to PA
conduit (Sano’s modification and PA banding). Interatrial
Parasternal Views septal assessment is important as restriction may occur
Parasternal views are important to assess ventricular mostly due to inadequate resection, an important cause
function and size and function of the AV valves. The of cyanosis. The right BT shunt may be visualized from
dilated RV is seen anteriorly and the small LV, posteriorly. the suprasternal short- or long-axis views with angulation
The ventricular septum is usually intact. The aortic valve of the transducer toward the right side. Postoperative
is either stenosed or atretic and LVOT may have subaortic complications of the shunt including pulmonary over
obstruction, which can also be seen in this view. The circulation, shunt stenosis, and hence PA distortion
morphology of the mitral valve should be studied and may occur and need evaluation. Sano’s shunt can be
the dimensions of the mitral and aortic valve annulus best visualized by subcostal and modified apical views.
Modified apical view is obtained by moving the transducer characterized by bilateral right atrial appendages and
anteriorly. Sliding the transducer up toward midsternum left isomerism with both appendages of left morphology.
will permit viewing the distal aspect of the conduit and Although there are several cardiac anomalies that are
PA branches. Narrowing of the shunt at any point along its common to both forms, certain unique features help to
course or at its insertion into the PA should be identified. differentiate the two forms. Right isomerism is associated
Color flow mapping and Doppler interrogation of the with absence of spleen in majority of cases and hence it is
conduit and proximal branch pulmonary arteries should also referred to as “asplenia syndrome.”328 Left isomerism
demonstrate a typical to and fro pattern of flow. is characterized by presence of multiple, albeit abnormal,
Evaluation of RV function and tricuspid regurgitation spleens and is hence called “polysplenia syndrome.”
should be performed from multiple imaging planes. Several extracardiac anomalies other than those that
Echocardiographic evaluation of the neoaorta and arch is involve the spleen are associated with these syndromes.
also important with the distal aortic arch deserving special Tables 72.18 and 72.19 compare cardiac and extracardiac
attention. The ongoing evaluation after the first stage anomalies found in these two syndromes.
Norwood is same as for Glenn and Fontan pathway.
Initial Echocardiogram (Table 72.20)
HETEROTAXY SYNDROME In view of the complex nature of associated cardiac
Introduction abnormalities, most of the patients will come to clinical
attention in the neonatal period or early infancy itself.
The term visceral heterotaxy originates from the Greek word The basic aim of the initial echocardiogram is to assess
“heteros – other than” and “taxis – arrangement.” It basically the entire spectrum of the cardiac anatomy with special
refers to any arrangement of body organs in patterns other emphasis on evaluation of pulmonary blood flow. A
than the usual (situs solitus) or its mirror image variant (situs segmental approach starting from the determination of
inversus).319–325 Very often, these conditions are associated the visceral and atrial situs, and then proceeding to each
with an abnormal number and arrangement of the spleen, segment in the heart and great arteries is recommended.329
and hence the term “splenic syndromes” has often been In most cases, initial palliation is possible on the basis of
given to these conditions. Cardiac malformations associated echocardiographic evaluation alone without resorting to
with these syndromes are often complex with abnormalities cardiac catheterization.
at various levels of the cardiac axis (visceroatrial situs, The steps involved in the initial echocardiographic
ventricular loop, and ventriculoarterial connections). The evaluation are as follows:
characteristic cardiac abnormality found in the heart is
referred to as “isomerism of atrial appendages,” whereby
Determination of Abdominal Situs and
both the atrial appendages have either right or left
morphology.326,327 Current echocardiographic techniques Cardiac Position
allow a complete structural evaluation of the entire cardiac This is achieved using the subcostal short-axis view. In
anomalies in these patients, so that cardiac catheterization situs solitus, the liver will be found to occupy the right side
is required only for hemodynamic evaluation prior to with the stomach on the left side of the spine. The aorta
consideration of single ventricle palliation in selected will occupy the left side of the spine and IVC on the right
cases. In this chapter, we discuss the steps involved in side. In situs inversus, this arrangement is reversed with
the systematic evaluation of the heart and great vessels in aorta occupying the right anterior aspect of the spine. The
patients with visceral heterotaxy. position of the heart can be determined by cranial tilt of
the transducer and determining which way the apex of
Anatomical Background: the heart points (left—levocardia; right—dextrocardia;
Characterization of Two Distinct middle—mesocardia). The position of the heart (levo or
dextrocardia) does not help in differentiation of right and
Heterotaxy Syndromes left isomerism.
Two distinct patterns emerge when we consider cardiac In patients with visceral heterotaxy, the position of
abnormalities in these complex syndromes—right the liver can be to the right, to the left, or transverse.
isomerism and left isomerism. Right isomerism is The stomach can be on either side or midline. These
Table 72.18: Cardiac and Extracardiac Anomalies in Isomerism Syndromes

Right Isomerism Left Isomerism
Dextrocardia 40% 40%
Right pulmonary isomerism 70% 10%
Left pulmonary isomerism Rare 60%
Bilateral superior vena cava (SVC) 50% 40%
Absent inferior vena cava (IVC) Rare 70%
Total anomalous pulmonary venous connection (TAPVC) 70% Rare
Partial anomalous pulmonary venous connection Rare 40%
Atrial septal defect (ASD)/common atrium 90% 80%
Atrioventricular (AV) septal defect 85% 40%
Single ventricle 50% 10%
Transposed great arteries 80% 30%
PS/pulmonary atresia 80% 30%
LVOTO Rare 40
Extracardiac anomalies CNS, gastrointestinal, Biliary atresia
skeletal and genitourinary Intestinal malrotation
Agenesis of gall bladder
Source: Gutgesell HP. Cardiac Malposition and Heterotaxy. In: Garson Jr, Bricker JT, Fischer DJ, Neish SR, editors. The Science and
Practice of Pediatric Cardiology. Baltimore, MD: Williams & Wilkins; 1998:1539–61.
relationships do not help differentiate between right and of atrium on both sides. The frequency of bilateral SVC is
left isomerism. However, the characteristic arrangement lower in left isomerism. SVC drainage to the atrium can be
of the aorta and IVC in relation to the spine will give a clue best demonstrated using the subcostal sagittal coronal or
to the diagnosis and help to differentiate between the two four-chamber and suprasternal views. LSVC drainage is
types. In right isomerism, the aorta and IVC will be found best noted by suprasternal long-axis and short-axis views
to occupy the same side of the spine (right or left), with the with leftward tilt of the transducer.332 In these views, the
IVC occupying a position more anterior to the spine than LSVC is seen descending in front and to left of the aortic
the aorta. In left isomerism, the IVC is often interrupted arch and pulmonary hilum toward the atrioventricular
with the abdominal portion of the IVC continuing as groove. In the suprasternal short-axis views, the presence
azygos or hemiazygos veins.330 This venous channel is of bilateral SVC can be demonstrated and the presence
distinguished by location on the same side of the spine as and size of the bridging vein can be assessed. In patients
the aorta, but in a posterior position. All these relations are with right isomerism, LSVC will be seen draining to the
best demonstrated using the subcostal sagittal scan and roof of the atrium between the left upper pulmonary
short-axis scan. vein and left-sided atrial appendage. In patients with left
isomerism, LSVC may drain to the coronary sinus. Color
Venoatrial Connections—Systemic Veins flow mapping should be used to determine the exact
point of insertion of the SVC into the atrium. PW Doppler
Abnormalities of the systemic veins are very common is useful in differentiating LSVC from similar appearing
in both syndromes. Knowledge of the systemic venous structures (left levocardinal vein) by demonstrating flow
anatomy is very important since a vast majority of these toward the heart. In cases with bilateral SVC, the actual
patients eventually require palliation via the univentricular sizes of both SVC and the presence and size of the bridging
pathway. The patterns and frequencies of these anomalies vein should be noted.
are summarized in Table 72.18.
Inferior vena cava: Most patients with right isomerism have
Superior vena cava: Right isomerism is characterized by no abnormalities of the IVC drainage with the IVC receiving
presence of bilateral SVC, which typically drains to the roof the hepatic veins and draining to the RA. However, a vast
majority of patients with left isomerism have abnormal aspect of the spine. In the subcostal sagittal view, this large
IVC drainage, with interruption in IVC being the most vessel can be traced to the posterior aspect of the aorta
common anomaly. The subcostal short-axis view shows draining to the posterior aspect of the right SVC.
the azygos vein as a large vessel on posterior and rightward In hemiazygos continuation of the IVC, the abdominal
portion of the right IVC is absent. Blood from the lower
Table 72.19: Venoatrial Connections in Isomerism Syndromes
body returns to the heart by way of a left-sided continuation
of a venous channel (“hemiazygos continuation”), which
Right Isomerism Left Isomerism
drains to LSVC or coronary sinus. The subcostal short-axis
Superior vena cava (SVC)
view of the abdomen shows a large venous channel on
Unilateral to right 29% 22% the left posterior aspect of the spine. Using the subcostal
Unilateral to left 20% 16% sagittal view with cranial angulation and suprasternal
Bilateral to roof 51% 38% view, the drainage of this vessel to the heart can be imaged.
Bilateral, one via — 24%
Hepatic veins: Evaluation of the hepatic vein drainage
coronary sinus
is vital in evaluation of patients with interrupted IVC,
Inferior vena cava (IVC)
especially before consideration of the bidirectional
To right-sided atrium 48% 12%
cavopulmonary shunt (BCPS; Kawashima Operation).
To left-sided atrium 52% 12%
Anomalies in the hepatic venous drainage have been
Interrupted on right — 34% implicated in the genesis of pulmonary arteriovenous
Interrupted on left — 42% fistulae after BCPS in these patients, leading to systemic
Hepatic veins desaturation.7 This will necessitate re-routing the hepatic
Confluence to IVC 76% 14% veins to the pulmonary circulation. In patients with left
Confluence to atrium — 43% isomerism, different patterns of hepatic venous drainage
Unilateral to atrium 6% 8% have been demonstrated (Table 72.19). The veins can
Bilateral to atrium 18% 35% drain unilaterally or bilaterally to the atrium or may form a
confluence and then drain to the atrium. The presence of
Pulmonary veins
bilateral drainage to both atria often makes a subsequent
To right-sided atrium 19% 26% Fontan conversion more difficult technically. In patients
To left-sided atrium 19% 14% with right isomerism, hepatic venous drainage is normal
Bilaterally to atriums — 60% with all veins forming a confluence and draining into the
suprahepatic portion of the IVC. The abnormalities of the
Centrally via confluence 3% —
hepatic venous drainage are best demonstrated using the
To extracardiac site 59% — subcostal short-axis view with cranial tilt and subcostal
Source: Uemura et al. Annals of Thoracic Surgery. 1995;60:561–9. sagittal view.333
Table 72.20: The Echocardiographic Checklist for the Univentricular Pathway Patient
Look at the electrocardiography (ECG) for any rhythm abnormality
Evaluation of the pulmonary artery (PA) pressures: these may be done from shunt lesions such as patent ductus arteriosus (PDA)
or pulmonary outflow gradients
Ventricular dysfunction
Systemic atrioventricular (AV) valve regurgitation
Aorta and subaortic obstruction if any
Presence of interatrial communication may be essential in certain circumstances
Pulmonary venous drainage
Systemic venous drainage and to look at systemic veins
Pulmonary artery anatomy: (a) size of the pulmonary arteries, (b) distortion of the pulmonary arteries
Abnormalities of coronary sinus: A characteristic feature Atria and Appendages

of right isomerism is the absence of the coronary sinus
with the cardiac veins draining directly to the atrium. The In some patients with heterotaxy, discordance between
absence of the coronary sinus can be demonstrated in the atrial and abdominal situs has been described. Drainage
apical four-chamber view and subcostal sagittal view. The of systemic veins, presence of tendinous insertion of the
LSVC, if present, will be directly draining to the roof of the Eustachian valve, and limbus of fossa ovalis on septal
atrium between the left upper pulmonary vein and left surface characterizes RA, while the flap valve of the
atrial appendage. Drainage of LSVC to coronary sinus can fossa ovalis on the other side is generally an indicator of
be seen in patients with left isomerism. LA. However, in patients with heterotaxy, the venoatrial
connections, vestibular morphology, and atrial septal
structure are all variable. Identification of the atrial situs is
Venoatrial Connections: Pulmonary Veins often best done by evaluating the morphology of the atrial
Abnormal pulmonary venous drainage is more common appendages. The characteristic features of the right atrial
in patients with right isomerism. A majority (>80%) of appendage are larger size, triangular appearance, broad
these cases have TAPVCs to right-sided atrium or systemic base, and presence of pectinate muscle extending till the
veins. TAPVC in the setting of right isomerism can occur crux of the heart. The left atrial appendage, in contrast, is
to supracardiac, intracardiac, or infracardiac locations. smaller, has a tubular appearance with narrow base, and
When pulmonary veins enter the atrium directly (without has pectinate muscle at the vestibule only. However,
confluence or common pulmonary vein), they tend to pectinate muscles cannot be profiled on echocardio-
drain to the smooth intercaval portion of the atria. Forty graphy. Using these features, the atrial situs can be deter-
percent of patients with left isomerism have PAPVC mined in the vast majority of patients with heterotaxy. The
with right veins entering the right-sided atrium and left term “situs ambiguous” should be reserved to the very few
veins to left-sided atrium. This may result from leftward patients where the atrial situs cannot be determined even
malalignment of atrial septum. Obstruction to pulmonary after evaluating all the above mentioned features.
venous flow is also more common in the setting of right The atrial appendages are best visualized using TEE.
isomerism. This is particularly important to identify before Using transthoracic echocardiography, the right atrial
consideration of placement of a systemic–PA shunt to appendage is well visualized in the PLAX view through
increase pulmonary blood flow. Failure to identify an the RV inflow tract and subcostal sagittal view. The left
obstructed TAPVC will lead to development of severe, atrial appendage is well seen in PSAX and subcostal four-
often fatal, pulmonary edema after placement of the chamber views.336,337
shunt. Pulmonary venous drainage can be imaged from
the subcostal coronal, apical four-chamber, PSAX, and Atrial Septum
suprasternal views. The presence of a confluence, size
Common atrium with both atria having right morphology
of individual veins, drainage of all veins to confluence,
is often associated with right isomerism. In patients
presence of additional directly draining veins, and
with left isomerism, 50% of cases have common atrium.
adequacy of communication of the confluence to atrium/
The morphology of the atrial septum does not help in
systemic veins should be identified. Color flow mapping
differentiating between right and left isomerism. The
and PW Doppler will help identify obstruction in the
ASD is best imaged using the subcostal coronal and
pulmonary venous drainage. Obstruction to pulmonary
sagittal views. The adequacy of the size of the interatrial
venous flow will produce characteristic high velocity (>2 communication is important before consideration of a
m/s), continuous flow signals on PW Doppler. Obstruction single ventricle repair.
to pulmonary venous drainage can occur at the site of
insertion of the veins to SVC, at the junction of vertical
vein to brachiocephalic vein, at the level of infracardiac
Atrioventricular Junction
drainage of the veins, or by external compression when the The atrioventricular connections in patients with
vertical vein runs a retrobronchial course (specific to right isomerism can be biventricular or univentricular. The
isomerism).334,335 ventricular topology can be either D-loop or L-loop,
with D-Loop patterns dominating. Left isomerism has of myocardium). The diastolic function of the ventricle
a higher prevalence of biventricular atrioventricular can be assessed by Doppler evaluation of the AV valve
connection compared with right isomerism (74% vs 46%). and pulmonary veins. The application of newer imaging
Univentricular connections are much more common in modalities like tissue Doppler imaging, harmonics, and
right isomerism. Irrespective of the nature of atrioventri- strain rate imaging are yet to be standardized for use in
cular connection, most patients with isomerism (93% in these patients.
right and 67% in left) have a common atrioventricular valve. In patients with biventricular connections (more
It is very important to assess the degree of regurgitation common in left isomerism), an attempt should be
of the common valve, its mechanisms, and possibility of made to assess for possibility of biventricular repair.
repair before consideration of single ventricle palliation, Factors that decide this include characteristics of the AV
since it is a major risk factor for surgery. The anatomy of valve (balanced vs. unbalanced, subvalvular apparatus
the valve is best assessed by subcostal coronal view with straddling) and characteristics of the VSD (location,
anteroposterior sweeps, subcostal en face view, and apical routability, presence of multiple VSDs, etc.). The
four-chamber views.338 relationship of the conducting system with respect to the
VSD also is an important consideration in view of risk of
Ventricles and Ventricular Septum heart block when biventricular repair is attempted.
Evaluation of the ventricles and ventricular septum is
A univentricular heart is more common in patients done using a combination of subcostal long- and short-
with right isomerism than left. Within the category of axis views, four-chamber view with cranial angulation,
isomeric hearts with single ventricle, morphological and parasternal long- and short-axis views.
RV type of single ventricle is more common. In patients
with univentricular heart and double inlet connection
via a common atrioventricular valve, the position of
Ventriculoarterial Connections
the ventricular septum relative to the common valve In patients with right isomerism, the common abnor-
will help to identify the ventricular morphology. If the malities of the ventriculoarterial connections are DORV
septum is found anterior to the valve, the ventricular with pulmonic stenosis/atresia and single outlet with no
morphology will be that of LV; if it is posterior, it suggests pulmonary outflow. Discordant ventriculoarterial conne-
RV. Failure to identify a ventricular septum indicates ctions may also be seen. The assessment of severity of
single ventricle of indeterminate morphology. In cases pulmonary stenosis is very important. The outflow tracts
of RV type of single ventricle, the rudimentary LV cavity can be imaged using subcostal coronal, four-chamber,
is located posterior or on the diaphragmatic surface of and PSAX views. Doppler evaluation of the gradient
the heart with no arterial outlets. If the single ventricle is across the pulmonary outflow (subvalvular and valve)
of the LV type, a rudimentary RV/outflow chamber will will provide an estimate of PA pressures. However, in
be present with at least one of the great arteries typically presence of pulmonary venous obstruction, Doppler
arising from that chamber. In patients with biventricular estimation of the gradient may underestimate the severity
connections and common AV valve (more common in left of the obstruction of outflow. In such cases, attention
isomerism), the ventricular morphology can be identified should focus on morphology of the outflow tract. In left
by the nature of trabeculations, papillary muscles, and isomerism, concordant ventriculoarterial connections are
site of septal attachment of inferior bridging leaflets. more common and obstruction to pulmonary outflow is
Associated anomalies of the ventricle include presence of less common.
noncompaction of the ventricular myocardium.
A thorough evaluation of the ventricular systolic Branch Pulmonary Arteries and Sources of
and diastolic function should be undertaken in the
initial evaluation as well as in evaluation prior to single
Pulmonary Blood Flow
ventricle repair. End-systolic and end-diastolic volumes Evaluation of branch pulmonary arteries constitutes one of
and ejection fraction are calculated. The ventricular the most essential aspects of evaluation prior to any form
mass can be calculated by subtracting the endocardial of surgical repair. This is best done from the PSAX view,
volume from the epicardial volume and multiplying the the ductal (high parasternal view), and suprasternal views.
resultant myocardial volume by 1.05 (specific gravity The presence of the confluence of pulmonary arteries is
first determined. The diameter of the branch PAs is then 2. Vermilion PR, Snider AR, Serwer AG, et al. Basic physical
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CHAPTER 73
Real Time 3D Echocardiography for
Quantification of Ventricular Volumes,
Mass and Function in Children with
Congenital and Acquired
Heart Diseases
Shuping Ge, Jie Sun, Lindsay Rogers, Rula Balluz
Snapshot
Left Ventricular Volumes, Ejection Fraction, and Mass
¾¾ Three-Dimensional Analysis of Regional Wall Motion,
¾¾
Right Ventricular Volumes, Ejection Fraction, and Mass
¾¾ Synchrony, and Strain
Single Ventricular Volumes, Ejection Fraction, and Mass
¾¾ Future Perspectives
¾¾
INTRODUCTION Quantitative measurement of left ventricular (LV)

volumes, mass, and function is one of the most common
The advent of real time three-dimensional echocar and important indications for echocardiography. These
diography (RT3DE) is a major milestone in the evolution measurements are among the most powerful tools for
of echocardiography. RT3DE has been used to generate diagnosis and prognosis of congenital and acquired heart
three-dimensional (3D) views of cardiac structures in an diseases and for assessment of medical, percutaneous,
intuitive and object format, obviating the need to mentally and surgical interventions. Awareness is also growing of
the importance of right ventricular (RV) volume, mass, and
create 3D images based on sweeps by two-dimensional
function in many cardiopulmonary diseases. Furthermore,
echocardiography (2DE). Furthermore, RT3DE provides
there are challenges and opportunities to measure the
an opportunity to quantitatively measure distances, areas,
volume, mass, and function of complex chambers such
volumes to quantify the cardiac structures, flows, and as the left atrium, right atrium, and the univentricular
function in 3D that may not possible by 2DE. A query of heart. As echocardiography continues to be the imaging
“3D echocardiography” yields over 4,500 publications modality of choice for these measurements, the strengths
in PubMed database. Therefore, the focus of this chapter and limitations of M-mode, two-dimensional (2D), and
is the use of RT3DE to quantitatively assess ventricular recently 3D echocardiographic methodologies for accurate
volumes, mass, and function in children with congenital and reproducible measurement of these indices have been
and acquired heart diseases. Other related topics can be extensively investigated for congenital and acquired heart
found in other chapters of this book. diseases.
Evidence suggests that 3DE provides improved

accuracy and reproducibility over 2D methods for measu
rement of LV volume and function calculation in adults1–3
and in children.4–10 Data have accumulated on the utility of
3DE for measuring chamber volumes and function for the
RV, and for the single ventricle,2,11–14 which may become
more widely used in clinical and research arenas in the
future. Finally, new advanced modes of analysis such as
3D strain and synchrony analysis by 3DE are promising
methodologies that warrant further investigation.
LEFT VENTRICULAR VOLUMES,

EJECTION FRACTION, AND MASS
The utilization of echocardiography to assess LV chamber Fig. 73.1: Semiautomated three-dimensional echocardiography
size and function dates to the advent of this technology. (3DE) algorithm for measurement of LVEDV, LVESV, and left ven-
tricular ejection fraction (LVEF). (LA: Left atrium; LV: Left ventricle;
Popp et al.15 reported on the change of cardiac dimensions
RA: Right atrium; RV: Right ventricle).
using M-mode echocardiography. Feigenbaum et al.16 Source: Adapted from Ref. 96.
used these changes to assess LV function and correlated it
to angiography. The correlation appeared good except in but it underestimates LV volumes with insignificant
cases with dilatation and regional wall abnormalities. The difference for the EF between the two modalities. Most
development of 2DE allowed the utilization of LV area and studies found the differences in volume calculations to be
the LV long axis to estimate volume. Wyatt et al.17,18 showed more significant with dilated and abnormal ventricles.30
that this technique was superior to M-mode, especially in Some experts propose including the endocardial surface
asymmetrical hearts. This technique is limited by the fact with the trabeculations to improve the correlation with
that 3D measurements are extrapolated from 2D-acquired CMR.31–33 Others suggest increasing the number of
data using geometrical assumptions. Also, on-axis cross- planes to better delineate the endocardial surface in an
sectional images may not be accurate because the whole attempt to eliminate geometric assumptions, especially
surface of the heart is not visualized. Finally, there may in abnormal ventricles.34,35 Others have had more success
be problems with foreshortening the long axis of the using direct volumetric measurement through the use of
ventricle. The logical next step is the development of 3D semiautomated border detection (Fig. 73.1).
constructions of the LV to assess volumes and function. RT3DE has also been applied in adults with congenital
Dekker et al.19 first attempted to create 3D images with heart disease for measurement of LV volumes. van den
a mechanical arm, which resulted in creating a 3D model Bosch et al.36 studied 32 patients ranging in age from
of the LV. Many investigators also published measurements 19–51 years with complex congenital heart disease,
of LV volumes using similar techniques with in vivo including tetralogy of Fallot, RV obstructive lesions,
validation using animal and human models.20–25 The and transposition of the great vessels after atrial switch
ability of 3D technology to overcome geometric assum surgery. Compared with CMR, RT3DE underestimated LV
ptions, avoid image positioning errors, and provide volume, but the difference was not statistically significant.
a surface reconstruction algorithm was the basis for These investigators found good correlations in LVEDV,
this improvement, as confirmed by many subsequent LVESV, and left ventricular ejection fraction (LVEF)
studies.26–29 between RT3DE by manual border detection and CMR,
The advent of RT3DE generated a renewed enthusiasm with correlation coefficients (r) = 0.98, 0.97, and 0.94,
for potential clinical use in the adult population. RT3DE respectively. Correlations in LVEDV, LVESV, and EF were
has been shown to have excellent correlations with modest between CMR and RT3DE using automatic border
cardiac magnetic resonance imaging (CMR) for assessing detection in this study of ventricles with altered geometries
chamber volumes and ejection fraction (EF) in adults, (r = 0.79, 0.83, and 0.54, respectively).
Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children 1723
Left Ventricular Mass provide a detailed basis for improving the accuracy of 3DE,
an indispensable step toward further clinical application.
RT3DE has also been used for the assessment of LV mass. Currently, for transthoracic and transesophageal
The technique requires identification of both endocardial assessment of LV volumes and EF, 3DE is recommended
and epicardial borders. This has made estimation of LV over the use of 2DE, as 3DE has been clearly demonstrated
mass more challenging in adult studies. A major challenge to provide more accurate and reproducible measurements.2
is the difficulty in visualizing the apex for mass calculation.
However, validation studies showed better correlation
Left Ventricle Analysis in Children
between RT3DE and CMR compared with estimating LV
mass estimation using M-mode or 2D methods, which Although M-mode and 2DE recordings are widely used
have been reproduced in many adult studies.37,38 to assess LV size, mass, and global systolic function in
children with congenital and acquired heart disease,
in many defects LV or ventricular septal geometric
Meta-Analysis
or functional alterations diminish the accuracy and
Shimada and Shiota39 conducted a meta-analysis to assess reproducibility of these measurements. Many validation
the sources of errors in evaluation of the LV by 3DE. Their studies have demonstrated that 3DE compares favorably
analysis included 3,055 subjects in 95 studies. The study with other independent reference standards such as
showed significant underestimation bias of both LVEDV CMR and is superior for assessing LV volumes, mass, and
and LVESV by RT3DE compared with CMR. The bias EF in children as well as adults (Table 73.1). Since our
for estimation of LVEF was not statistically significant. first validation of RT3DE by CMR,4 seven RT3DE studies
Sources of error included female sex and presence of have been published comparing measurements of LV
congenital heart disease, which were associated with volumes, mass, and EF with CMR measurements.4–10 These
more underestimation in the analysis. Semiautomatic reports showed excellent correlation, agreement, and
border detection and the use of matrix-array transducer reproducibility for volumes and mass between the two
were associated with less underestimation. Despite the modalities but only modest advantages in measurement of
differences between RT3DE volumetric estimation of the EF. We have also found that RT3DE using a semiautomated
LV and CMR, the literature supports the role of RT3DE as method is more accurate and reproducible than M-mode
both accurate and reproducible in assessing LV volume or Simpson’s 2D biplane method and is as efficient as 2DE
and LVEF, although it is not interchangeable with other in children.6 These findings suggest that the American
Society of Echocardiography’s recommendation that in
radiological modalities.
adults LV should be quantified using RT3DE may also be
Shimada and Shiota40 published another meta-analysis
applied to children.1,2
evaluating the use of RT3DE to assess LV mass. This analysis
Studies of LV mass assessment in children are very
assessed 25 studies including 671 comparisons. Studies
limited. However, the available studies report excellent
published in 2004 or earlier showed high heterogeneity
correlations between RT3DE and CMR, although RT3DE
[I(2) = 69%)] and significant underestimation of LV mass
overestimated LV mass in comparison to CMR in most
by 3DE (−5.7 g, 95% confidence interval −11.3 to −0.2, of these studies. Inter- and intraobserver variability in
P = 0.04). Studies published between 2005 and 2007 this assessment are excellent.4,6–8 These data suggest that
were still heterogeneous [(I(2) = 60%)] but showed less RT3DE quantification of LV mass is feasible for clinical
systematic bias (−0.5 g, 95% confidence interval −2.5 to 1.5, applications, although the normal ranges of LV volumes,
P = 0.63). In contrast, studies published in 2008 or later were mass, and EF in various age groups must still be defined.
highly homogeneous [(I(2) = 3%)] and showed excellent
accuracy (−0.1 g, 95% confidence interval −2.2 to 1.9,
RIGHT VENTRICULAR VOLUMES,
P = 0.90). Investigation of factors affecting the bias revealed
that evaluation of cardiac patients compared with healthy EJECTION FRACTION, AND MASS
volunteers led to greater bias (P < 0.05). In conclusion, Because of the complex geometry of the right ventricle,
this meta-analysis elucidates the underestimation of LV clinical assessment of its size, mass, and EF has largely
mass by 3DE, improvement of the technique over the past been qualitative. However, the importance of RV
decade, and factors affecting the degree of bias. These data chamber size and systolic function has increasingly been
Table 73.1: Real Time Three-Dimensional Echocardiography for Left Ventricle Volumes, Mass, and Function in Children
RT3DE Com-
Number of
Reference Year pared With Methods LV Indices Correlation
Patients
Other Method
LVEDV, LVESV,
Long-axis, eight
Bu et al.4 2005 19 CMR LV mass, LVEF, r = 0.86–0.97
planes
SV
LVEDV and
Iino et al.5 2007 25 LV angiography Semiautomated r = 0.979–0.996
LVES
LVEDV, LVESV,
Four-plane, eight-
Lu et al.6 2008 20 CMR LV mass, LVEF, r = 0.85–0.9
plane semiautomated
SV
LVEDV, LVESV,
Riehle et al.7 2008 12 CMR Semiautomated LV mass, LVEF, r = 0.93–0.99, 0.69 for EF
SV
Friedberg LVEDV, LVESV,
2010 35 CMR Disc summation r = 0.90–0.96
et al.8 LVEF
LVEDV, LVESV,
Semiautomated
Hascoët et al.9 2010 50 RT3DE LV mass, LVEF, r > 0.97 but 0.79 for EF
(Qlab vs. TomTec)
SV
2 Ultrasound systems/ LVEDV and
Laser et al.10 2010 49 CMR r = 0.91–0.95
semiautomated LVESV
(CMR: Cardiac magnetic resonance imaging; EF: Ejection fraction; LV: Left ventricle; LVEDV: Left ventricular end-diastolic volume
LVEF: Left ventricular ejection fraction; LVESV: Left ventricular end-systolic volume; RT3DE: Real time three-dimensional echocar-
diography; SV: Stroke volume).
recognized in both pediatric and adult heart disease. on in vitro and in vivo models.41–47 Multiple clinical studies
Many volume and pressure overloading conditions can in adults followed, comparing RT3DE volumes with
cause remodeling and dysfunction of the RV. Studies in CMR.48–53
children who have undergone repair of tetralogy of Fallot
have shown that longitudinal follow-up of right and left Right Ventricle Analysis in Children
ventricular remodeling and dysfunction are crucial to
guide timely intervention to replace the pulmonary valve
and in Patients with Congenital Heart
to avoid failure of the RV, arrhythmia, and sudden cardiac Disease
death. Although CMR has been established as a clinical Our group11 used a disc-summation method (Figs 73.2A
standard for quantitative analysis of the RV, quantitative and B) and studied 20 normal children between the ages
RT3DE may be useful in many instances, for example, of 6 and 18 years comparing RV volumes and function
as a potential screening tool and in those in whom CMR
obtained with RT3DE versus CMR. We found excellent
is contraindicated or not available. Because of the cost,
correlation between RT3DE and CMR, with correlation
portability, and availability of RT3DE and the growing
coefficients of 0.96, 0.98, and 0.89 for RVESV, RVEDV,
evidence of the utility of RV3DE, the application of RV3DE
for quantitative analysis of the RV may increase in the and RVEF, respectively. We also found good inter- and
future. intraobserver variability. There was small but significant
Unlike the LV, there is no established geometric model underestimation of RV volume, especially RVEDV, by
that can be used for quantitative analysis of RV volumes, RT3DE compared with CMR. The differences were not
mass, and function. Clinical evaluation of RV size and statistically significant for RVESV and RVEF. Niemann
function is largely qualitative for both adults and children. et al.12 used a Beutel semiautomated method
Quantitative analysis of the RV becomes a major potential (Fig. 73.2C) to study 17 adults and 16 children with
application for 3DE, especially since RT3DE hardware and congenital heart disease, including tetralogy of Fallot,
software have become available. Initial validation studies transposition of the great arteries, truncus arteriosus, atrial
of RV volumes and EF quantification by 3DE were based septal defect, ventricular septal defect (VSD), coarctation
A B
Figs 73.2A to C: (A) The disc summation method for measure-

ment of right ventricular (RV) volumes and ejection fraction (EF)
in children. Manual tracing of the RV endocardial contours was
performed on a stack of short-axis images. The four-chamber
views and coronal views are used as reference images; (B) Simp-
son’s principle is used to derive RVEDV, RVESV, and right ven-
tricular ejection fraction (RVEF); (C) The semiautomated method
for measurement of the RV indices. The method is based on the
Beutel model with manual tracing of the RV endocardial land
marks, including a set of coronal, sagittal, and four-chamber views.
(LV: Left ventricle; RV: Right ventricle).
C Source: Adapted from Ref. 96.
of the aorta, and atrioventricular septal defect. They used RVEDV, and EF, particularly in patients with larger volumes
a semiautomated border detection method in comparison (> 200 mL) and in younger patients (≤18 years). There was
with CMR. Excellent correlation was obtained, with corre no improvement for the analysis with time. The use of
lation coefficients of 0.99 for RVESV, 0.95 for RVEDV, and matrix-array transducer did not improve the correlation
0.97 for EF. either. There was no statistical difference with the use of
Therefore, as seen in adults with cardiovascular automated compared with manual border detection.
disease, including congenital heart disease,54–59 quanti
Although RT3DE is highly promising for the evaluation of
tative analysis of RV volumes and EF using RT3DE is also
RV indices, further investigation is warranted to address
feasible in children. The limitations of RT3DE for quanti
the limited feasibility and accuracy before widespread
tative analysis of the RV are related to image quality, border
clarity, and the difficulty imaging the entire RV, especially clinical application.2
in patients with an enlarged RV, such as those who have
undergone repair of tetralogy of Fallot. SINGLE VENTRICULAR VOLUMES,
EJECTION FRACTION, AND MASS
Meta-Analysis
One of the most complex types of congenital heart
Shimada et al.60 performed a meta-analysis of published disease in children is the functional single ventricle. These
studies comparing assessment of RV volumes and EF by patients often require two to three stages of palliation,
RT3DE versus CMR. The analysis included 23 studies and ultimately resulting in a Fontan type of palliation, and they
807 adults and children. They concluded that compared often have long-term structural and functional compli
with CMR, 3DE significantly underestimated RVESV, cations, arrhythmias, exercise intolerance, and limited
neurodevelopmental outcomes. RT3DE requires no geo global LV function more accurately and reproducibly
metric assumptions and is ideally suited for quantification but also to quantify the regional function of the 16 LV
of volumes and EF for this type of congenital heart disease. segments and to assess LV intraventricular synchrony.
Altmann et al.61 first used acoustic spatial locator 3D To analyze LV regional function and synchrony, the cyclic
reconstructive technology to assess the volume and EF changes of the volumes of each of the 16 LV segments
of single ventricles with LV morphology in comparison are plotted against time over the course of the cardiac
with 2DE and CMR. They studied 12 patients and showed cycle. The volume curves and plots allow measurement of
superior agreement with less data scatter in ESV, EDV, volume changes of each segment (i.e. segmental motion)
mass, and EF between 3DE (bias 3.4 ± 5.5 mL, 14.2 ± 8.3 and temporal differences of each segment to minimum
mL, 5.8 ± 8.4 g, and 4.4% ± 5.3%, respectively) and CMR volume (i.e. synchrony). Regional minimal volume (i.e.
than between 2DE and CMR (−2.9 ± 8.1 mL, 2.9 ± 10.4 mL, maximal contraction) normally occurs at the same time
−8.3 ± 12.0 g, and 8.5% ± 10.3%, respectively). The inter- in ventricular systole for all segments. The systolic dyssyn
and intraobserver variabilities were more favorable for chrony index (SDI) is calculated as the standard deviation
3DE (5.7–7.1%) compared with CMR than for 2D compared of time to regional minimal volume of the segments.
with CMR (10–24%). Altmann et al. concluded that 3DE Studies in the pediatric literature have validated
provides estimates of ventricular volumes, EF, and mass use of this new technology to assess LV synchrony in
that are comparable to CMR in this select group of patients volume and pressure loading conditions, the effect of
with single ventricles of LV morphology.
ventricular septal patch repair to LV synchrony, and
Soriano et al.62 applied RT3DE for the estimation of
outcome of resynchronization therapy in patients with
volumes, mass, and EF in patients with single ventricles
a single ventricle with LV morphology. Kobayashi et al.63
using a disc-summation technique. RT3DE was feasible
prospectively studied 27 children with end-stage renal
in 27 of 29 patients (93%). The RT3DE EDV correlated well
disease (13 on peritoneal dialysis and 14 on hemodialysis)
but was smaller than with CMR, and 3DE EF was smaller
and 29 normal controls. SDI was normalized to cardiac
than with CMR. There was no significant difference in
cycle duration (SDIp). SDIp (16 segments) and LV mass
measurements of ESV or mass. The 3DE interobserver
were significantly greater in the hemodialysis group. SDI
differences for mass and volumes were not significant
and SDIp (16 segments) improved after a hemodialysis
except for measurement of EF. Intraobserver differences
were not significant. They speculated that the modest session (P < 0.05); LV mass and LV mass index remained
correlation in EF between RT3DE and CMR was related unchanged. LV dyssynchrony was significantly greater
in part to the small range of EFs in this single-ventricle in patients with LV hypertrophy compared with those
population. This study suggested that RT3DE quanti without. Their study demonstrated an association between
fication of EDV, ESV, mass, and EF in patients with single LV volume load and LV hypertrophy with significant LV
ventricles is feasible, reproducible, and reasonably dyssynchrony in end-stage renal disease. SDI may provide
accurate, except in measurement of EF, and warrants a sensitive marker for mechanistic and early detection of
further validation. LV dysfunction.
We showed that a long-axis, eight-plane Simpson’s Veeram et al.64 studied patients with large VSDs who
technique is also feasible to assess volumes and EF for the underwent surgical patch closure during infancy to
functional single ventricle. Furthermore, our study showed investigate the long-term effects of the presence of akinetic
that EF values are an independent predictor of transplant- patch in the ventricular septum and postoperative right
free survival for this group. EF < 40% is associated with bundle branch block (RBBB) on LV mechanical synchrony
significantly lower freedom of transplant-free survival in and global systolic function. Their study showed that
this group of patients.14 pediatric patients 5–10 years after VSD patch closure have
normal LV function. The presence of the RBBB was associated
THREE-DIMENSIONAL ANALYSIS with mechanical dyssynchrony and tendency toward LV
dilatation in this group of patients. They demonstrated
OF REGIONAL WALL MOTION, that RT3DE is a sensitive technique for assessing LV
SYNCHRONY, AND STRAIN synchrony in postoperative VSD patients, especially
those with RBBB, who require long-term follow-up
Regional Wall Motion and Synchrony
for potential complications.
The capability of RT3DE to capture the entire LV in three Finally, Bacha et al.65 performed multisite pacing
dimensions offers the opportunity not only to assess studies in 26 patients undergoing stepwise single-ventricle
A B
Figs 73.3A and B: Three-dimensional speckle tracking echocardiography (3DSTE) offline analysis. The endocardial borders were
manually traced at left ventricle (LV) end-diastole and end-systole from the apical four-chamber, two-chamber, and LV long-axis views,
respectively. The LVEDV, LVESV, left ventricular ejection fraction (LVEF), systolic dyssynchrony index (SDI), LV three-dimensional (3D)
systolic peak global (A) and segmental strain (B) were automatically calculated by the software. The mean systolic 3D peak strain of the
16 segments represented LV global strain. The mean basal, mid, and apical segmental systolic 3D peak strain represented the LV seg-
mental peak systolic strain, respectively. The 3D strain indices derived included global systolic strain (GSS); global longitudinal systolic
strain (GLSS); systolic strain of basal, middle, and apical segments.
Source: Adapted from Ref. 96.
palliation. RT3DE was used in 10 subjects. They observed angle-dependent method.70 Recently, three-dimensional
significant response to multisite pacing, including speckle tracking echocardiography (2DSTE) has been
(a) improvement in QRS duration in 24 of 26 patients introduced as a new method to quantify myocardial strain.
(93.9 ± 17.5 ms vs 71.7 ± 10.8 ms; P < 0.001); (b) increase This technique measures myocardial deformation by
in systolic blood pressure in 25 of 26 patients (86.3 ± 20.0 means of frame-by-frame tracking and motion analysis
mm Hg vs 93.8 ± 20.2 mm Hg; P < 0.001); (c) increase in of speckles within B-mode images. Validation studies
cardiac index in 21 of 22 patients (3.2 ± 0.8 vs 3.7 ± 1.0 L with tagged CMR and sonomicrometry in adults70,71 and
× min−1 × m−2; P < 0.001); and (c) improvement in index children75,76 have provided evidence that 2DSTE is a reliable
of asynchrony in 8 of 10 patients (10.3 ± 4.8 vs 6.0 ± 1.4; method for determining ventricular myocardial function.
P < 0.04). The study suggests that RT3DE is potentially 2DSTE has many limitations, however. Firstly, myocardial
useful in assessing cardiac resynchronization therapy even deformation measurement by 2DSTE is affected by loss
in cases with complex geometries. of speckles due to motion outside the imaging plane.77
Secondly, 2DSTE has limited reproducibility, likely owing
Strain Analysis by Real time to the variabilities in the choice of image planes and lack
of standardization in image analysis.78 Finally, the analysis
Three-Dimensional Echocardiography is cumbersome and six planes are needed for complete
Conventional indices of regional and global ventricular analysis, which is a major limitation for automation and
function, defined by endocardial excursion, such as potential clinical use.
fractional shortening and EF, are considered to be load- More recently, the advent of three-dimensional speckle
dependent.66,67 Myocardial velocities by tissue Doppler tracking echocardiography (3DSTE) (Figs 73.3A and B)
imaging (TDI) do not rely on geometric assumptions but has shown the potential to overcome the limitations
are inherently unidimensional, angle-dependent, variable of 2DSTE imaging for the assessment of LV global
with aging, and influenced by anthropometrics and heart and regional systolic function. This method tracks the
rate.68,69 Myocardial strain has been shown to be more motion of speckles within the scan volume, allowing
robust for assessment of regional ventricular myocardial more complete and accurate assessment of myocardial
function using both echocardiography and CMR.70–74 TDI- deformation in 3D space79–82 by avoiding the loss of
based strain analysis has been extensively validated and speckles due to out-of-plane motion. 3DSTE has been
has been shown to be limited as a one-dimensional and validated for the quantification of LV volumes82 and LV wall
motion in ischemic heart disease83 in adults. Nonetheless, obesity was found for LV average circumferential strain
data are scarce and incomplete for children with or (b coefficient = 0.74; r2 = 0.55; P = 0.003). Their data
without congenital heart disease.75,76,84 Thus, feasibility, suggested that obesity-related cardiomyopathy is
reproducibility, maturational changes, and normal values associated not only with structural cardiac changes but also
in this population must be shown before these indices can with myocardial deformation changes in the childhood.
be used for assessing LV function for diagnosis, prognosis, Assessment of LV circumferential strain using 3D wall
and risk stratification of various congenital and acquired motion tracking is a marker with promising sensitivity for
heart diseases in the young before and after medical, identifying obesity-related cardiomyopathy.
percutaneous, and surgical intervention. The limitations of this technology include limited
We used RT3DE to study 256 consecutive healthy spatial and temporal resolution of the 3DE data sets and
subjects using full-volume 3D data acquisition with a 3D the variability in the measurements secondary to the
matrix-array transducer.85 Study subjects were divided hardware, software, and algorithms provided by various
into five age groups: group 1 (birth to age 1 year); group vendors. Further validation, improvement, and standar
2 (1–5 years); group 3 (6–9 years); group 4 (10–13 years); dization are warranted for clinical application.
and group 5 (14–18 years). The 3D LV global strain (GS),
global longitudinal peak systolic strain (GLS), global radial FUTURE PERSPECTIVES
peak systolic strain (GRS), and global circumferential Much evidence suggests that in the presence of adequate
peak systolic strain (GCS) values were determined using image quality, LV volumes, mass, and EF measurements
modality-independent software. A total of 228 cases by 3DE agree more closely with CMR measurements
(89%) were adequate for analysis and 28 cases (11%) were and have better reproducibility than does 2DE, making
excluded, including 10 cases excluded owing to the low 3DE the modality of choice for the everyday clinical
frame rates as determined by the system and 18 cases evaluation of LV volumes and EF. Quantitative analysis
excluded owing to poor 3D images. We found statistically of volumes, mass, and EF for the RV and for single
significant differences among the five age groups for GLS ventricles holds great promise and warrants future
and GCS, but no statistical difference between the age investigation. New modes of regional wall motion, strain,
groups for GRS and GS values. Multiple linear regression and LV dyssynchrony assessment using RT3DE are in
analyses showed that LV and RV dimension, mass, volume, the forefront of active research. Future advancements in
and EF were significantly associated with GLS, GS, GRS, hardware are expected to allow acquisition of wide-angle
and GCS. The interobserver variability of 3D systolic strain 3D data from the entire heart in a single cardiac cycle,
parameters ranged from 1.2% to 9.5%, and intraobserver with higher spatial and temporal resolution. We anticipate
variability ranged from 0.5% to 3.8%. Our study concluded that further development in automatic quantitative
that global 3D systolic strain measurement using the new analysis algorithms and software will make RT3DE an
3D RT STE is feasible and reproducible in children. GLS integral clinical methodology for diagnosis, prognosis,
and GCS are higher in early puberty and lower in late and assessment of medical, percutaneous, and surgical
puberty compared with infancy and early childhood. GRS intervention for congenital heart disease.87
and GS have no maturational changes. Future work will
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CHAPTER 74
Three-Dimensional Echocardiography
in Congenital Heart Disease
Steven Bleich, Gerald R Marx, Navin C Nanda, Fadi G Hage
Snapshot

Shunt Lesions/Septal Defects 
AorƟc Arch Anomalies

Common Atrium 
Atrial and Atrioventricular Valve AbnormaliƟes

Aortopulmonary Window 
Other AbnormaliƟes

Patent Ductus Arteriosus (PDA) 
Double Outlet Right Ventricle

Conotruncal Anomalies 
Sinus of Valsalva Aneurysm

Ouƞlow Tract ObstrucƟon
INTRODUCTION structural defect and nearby cardiac structures.6 Views

previously unobtainable by 2D echocardiography are
Congenital heart disease (CHD) includes an extensive captured readily by 3D imaging offering further insight
group of diagnoses affecting both pediatric and regarding morphology, location, specific dimensions, and
adult patients. The complexity of CHD has motivated dynamic changes of specific heart structures during the
cardiologists to better understand each individual defect cardiac cycle.4 3DE enhances cardiologists’ understanding
in order to maximize management of these patients of CHD by providing unique and valuable data that is
and improve treatment options. Two-dimensional (2D) useful in determining the appropriate treatment option for
echocardiography has been the gold standard imaging each specific defect.5
modality used because of its convenience, low cost, and lack
of harmful side effects as compared to magnetic resonance
imaging (MRI) and multidetector computed tomography
SHUNT LESIONS/SEPTAL DEFECTS
(CT).1,2 However, identifying and understanding CHD Longstanding congenital heart defects causing elevated
often requires a real life, three-dimensional (3D) image pulmonary artery pressure can cause significant strain on
of the defect.3–5 With 2D echocardiography, the reader the right ventricle, ultimately resulting in right ventricular
often expends effort to mentally reconstruct the heart and remodeling and dysfunction. The ability to measure right
great vessels in three dimensions, sometimes, preventing ventricular size and function can guide medical and
visualization of relevant details. The emergence of 3D surgical management before deterioration in right
echocardiography (3DE) provides a more comprehensive ventricular function. For example, in patients following
view of the cardiac anatomy which offers incremental surgical repair of Tetralogy of Fallot (ToF), follow-up
value in the diagnosis of CHD. imaging to detect ventricular dysfunction and remodeling
3DE has gained popularity in the field of CHD because can help to determine the appropriate time for placement
it can portray a dynamic and realistic picture of the of a competent pulmonary valve to avoid further
complications such as right ventricular failure, arrhythmia, Secundum ASD

and sudden cardiac death.7 Unfortunately, based on
Secundum ASDs and patent foramen ovale (PFO) occupy
the intricate shape of the right ventricle, measurement
the middle portion of the atrial septum. Although 2D
of its size and function pose a challenge to traditional
echocardiography has historically been the imaging
2D imaging.7 Cardiac MRI has previously been utilized
modality of choice in diagnosing these ASDs, images
to measure this data, but due to its cost, extensive time
obtained via this method can lack optimal viewing of the
necessary to obtain and interpret images, and restrictive
defect and therefore distort the measurements obtained.15
accessibility, its use is limited.7,8 3D-transthoracic
In the setting of multiple fenestrations such as the Swiss
echocardiography (TTE) has considerable advantage over cheese pattern or multiple hole pattern, the limitations of
2D TTE because of its ability to accurately assess volume 2D TTE are even more obvious. The key to any successful
and function of the right ventricle based on the unique echocardiographic image relies on a clear imaging
views it provides.9 Strong correlations between 3DE and window with the patient in a position that provides the
cardiac MRI for the quantitative analysis of the right best view for the reader. 3D TTE performed using the
ventricle have been reported, but limitations including apical, para-apical, right parasternal and subcostal views
underestimation of right ventricular volume and the provides adequate en face visualization of the defect in
visualization of the entire right ventricle in patients with most patients (Figs 74.2 and 74.3). The interatrial septum
an enlarged right ventricle in a single imaging window is often best observed in the right parasternal view with
must be recognized.7 With regard to 3DE, the American the patient in the right lateral decubitus position.15
Society of Echocardiography (ASE) suggests that capturing Information regarding exact dimensions and location of
higher quality images may facilitate increased utilization an ASD along with relative surrounding cardiac anatomy
of this modality.10 and rim size can be obtained more easily by 3D TTE than
Equally important in CHD is the assessment of single 2D transesophageal echocardiography (TEE).15,16 With
ventricular size and function. Studies comparing 3D the advent of real time 3D TTE, a PFO can be visualized
echocardiographic measurements of single ventricle as well as the motion of the valve covering it (Figs 74.4
volumes, mass, and ejection fraction compare favorably and 74.5).17 Recognizing the development of worsening
to MRI in young infants.11 The ability to measure left dyspnea and hypoxia with change in position from the
ventricular volumes in patients with borderline left hearts supine to sitting position suggests the possibility of
being considered for either single ventricle palliative platypnea-orthodeoxia syndrome. 3D TEE has been used
surgeries or biventricular repair is also significant. Often to document an open PFO with shunting of blood in the
the ventricles are spherical and thus 2D imaging relies on sitting position and closed PFO in the supine position
mathematical assumptions that often do not apply. Three- (Figs 74.6 and 74.7).18 One study done by Morgan et al.
compared the results obtained by 2D TEE to 3D TTE
dimensional imaging has been shown to again provide
with respect to maximum defect diameter, area and
measurements of left ventricular size and function in small
circumference of an ASD.19 Data obtained from these two
young infants as compared to MRI.12
modalities were not statistically different, however, 3D
TTE compared to 2D TEE provided clinically significantly
Atrial Septal Defects (ASDs) information. 3D was able to recognize appropriate
Atrial septal defects represent 7–10% of all congenital heart candidates for percutaneous closure of an ASD while
defects, often diagnosed incidentally in asymptomatic eliminating the need for more invasive imaging with TEE.
adults.13,14 An ASD can consist of one or multiple defects Repair of ASDs have traditionally been performed
and understanding the extent of the defects is key in with surgery, however, recent evidence recommends the
planning for the appropriate corrective procedure use of more percutaneous closure devices. The decision
(Figs 74.1A to D). This is especially germane when to proceed with surgery versus percutaneous approach
considering defects for catheterization closure. There are depends on the location of the ASD, its shape and size,
several different types of ASDs depending on their location and the surrounding tissue.4,15 In order to proceed with
in the atrial septum and they are discussed below. percutaneous repair, the cutoff with regard to rim size has
Chapter 74: Three-Dimensional Echocardiography in Congenital Heart Disease 1735
A B
C D
Figs 74.1A to D: Live three-dimensional right parasternal transthoracic echocardiographic examination of atrial septum and supe-
rior and inferior vena cavae. (A and B) Color Doppler examination in another patient showing four (numbered 1 through 4) separate
secundum defects at different levels of the atrial septum; (C and D) Two of these defects are viewed en face. In this 25-year-old female,
four atrial septal defects at different levels of the atrial septum could be demonstrated by sequential cropping of the three-dimensional
dataset. Only two of these defects could be visualized by two-dimensional transthoracic echocardiography. (LA: Left atrium; RA: Right
atrium). (Movie clip 74.1).
Source: Reproduced with permission from Patel V, Nanda NC, Upendram S, et al. Live three-dimensional right parasternal and supra-
clavicular transthoracic echocardiographic examination. Echocardiography. 2005;22:349–60.
been set at 5 mm. Obtaining a comprehensive view of the important when selecting the size of the occluder device
defect and nearby cardiac structures is feasible with 3D which can prevent postprocedure complications. Placing
TTE compared to 2D echocardiography, which helps in of an inappropriately sized occluder device can lead
determining the appropriate therapeutic intervention.20 to persistence of the shunt, invasion of nearby cardiac
In patients with secundum ASD, the most common type anatomy, breakdown of the device, device embolization
of ASD encountered in clinical practice, 3D TTE can and even perforation of the heart.23 Understanding the
view the defect and surrounding rims of tissue which is serious nature of these complications substantiates the
valuable when planning a transcatheter approach.15,21 The need to precisely document the defect, its size, and all
ability of 3D TTE to calculate accurate measurements of relevant cardiac anatomy. Furthermore, in the setting of an
the defect size has been validated by recording similar above mentioned complication, 3DE has been life saving
measurements during surgical and percutaneous in visualizing the abnormality and allowing immediate
repair.15,22 Knowing the exact dimensions of the defect is repair.24 Color Doppler 3D TTE can identify a small residual
A B
Figs 74.2A and B: Live three-dimensional transthoracic echocardiographic (3D TTE) assessment of atrial septal defect. Arrowhead
points to a large secundum atrial septal defect visualized from both right (RA, A) and left atrial (LA, B) aspects. Note the large rim of
tissue surrounding the defect. (AS: Atrial septum). Movie clip 74.2, Part 1 shows en face viewing of a small atrial septal defect from both
the left and right atrial aspects. There is an ample rim of tissue surrounding the defect. Movie clip 74.2, Part 2 from another adult patient
with a secundum atrial septal defect. Regular and QLab (Philips Medical Systems, Andover, MA) cropping of the 3D data set views the
large defect en face (two arrowheads). Reasonable amount of atrial septal tissue surrounds the defect. The defect measures 3.2 × 3.1
cm, area 9.2 cm2. Movie clip 74.2, Part 3 from another patient with a secundum atrial septal defect. The two-dimensional study shows a
large defect (arrowhead) in the apical 4-chamber and subcostal views. Regular and QLab croppings view the large defect en face (two
arrowheads/arrows). Although the defect is similar in size to the previous patient, there is hardly any rim of the tissue adjacent to the
aorta making it hazardous to close it in the catheterization laboratory. Movie clip 74.2, Parts 4 and 5. In this large patient, a secundum
defect in the atrial septum (AS) was suspected during subcostal examination but a definitive diagnosis could not be made. When the
images were acquired using a three-dimensional transducer and cropped, the defect (arrow) was clearly visualized with left and right
shunting. (LA: Left atrium; RA: Right atrium). [Movie clip 74.2, (Parts 1 to 5)].
Source: Reproduced with permission from Mehmood F, Vengala S, Nanda NC, et al. Usefulness of live three-dimensional transthoracic
echocardiography in the characterization of atrial septal defects in adults. Echocardiography J. 2004;21:707–13.
Fig. 74.3: Three-dimensional image of atrial septal defect: en

face view of the atrial septum, from the right atrium. Accurate
description of the surrounding rims and their measurements
provided by TomTec software, in the preoperative assessment of
this young patient. (AV: Atrioventricular; IVC: Inferior vena cava;
SVC: Superior vena cava; RV: Right ventricle; RA: Right atrium).
Source: Reproduced with permission from De Castro S, Caselli
S, Papetti F, et al. Feasibility and clinical impact of live three-
dimensional echocardiography in the management of congenital
heart disease. Echocardiography. 2006;23:553–61.
shunt after the placement of an ASD occluder device that Current ASE guidelines advocate for the utilization of
often occurs and usually resolves over time.25 3D TTE is 2D TEE during a percutaneous closure repair because of
useful in evaluating the morphology and effectiveness of precise measurements of the ASD’s maximum dimension
percutaneous closure devices used for closure of an ASD and bordering septal rims, however, its limitations must
and PFO (Figs 74.8 to 74.10). be understood.24 The success of 2D TEE relies on the
A B
Figs 74.4A and B: Live three-dimensional transthoracic echocardiography assessment of patent foramen ovale (PFO). (A and B)
Arrow shows the PFO while the arrowheads point to contrast signals moving through the defect into the left atrium (LA) following an
intravenous agitated saline injection. 3D TTE, three-dimensional transthoracic echocardiographic; (LV: Left ventricle).
Source: Reproduced with permission from Mehmood F, Vengala S, Nanda NC et al. Usefulness of live three dimensional transthoracic
echocardiography in the characterization of atrial septal defects in adults. Echocardiography J. 2004;21:707–13.
A B
C D
Figs 74.5A to D
Figs 74.5A to E: Real time/three-dimensional transthoracic

echocardiographic visualization of the valve of foramen ovale. Right
parasternal approach. (A) Arrowhead points to the atrial septum; (B
and C) En face view of the atrial septum from the left atrial (LA) side
shows the valve of foramen of ovale in the closed (B) and open (C)
positions. In the closed position it completely covers the foramen
ovale; (D) Arrowhead points to a mobile flap of tissue at the junction
of superior vena cava (SVC) and right atrium (RA) representing a
remnant of right-sided sinus venosus valve; (E) Arrowhead shows
the Eustachian valve. (IVC: Inferior vena cava). [Movie clip 74.5,
(Parts 1 to 8)].
Source: Reproduced with permission from Panwar SR, Perrien
JL, Nanda NC, Singh A, Rajdev S. Real time/three-dimensional
transthoracic echocardiographic visualization of the valve of
E foramen ovale. Echocardiography. 2007;24:1105–7.
Fig. 74.6: Transesophageal echocardiogram in the supine posi- Fig. 74.7: Transesophageal echocardiogram in the sitting posi-
tion. Echogram taken in the supine position showing the foramen tion. Echogram taken in the sitting position showing the foramen
ovale is closed (right figure), and no apparent right-to-left shunt by ovale is wide open (right figure), with a massive right-to-left shunt
Doppler color flow (left figure). (LA: Left atrium; RA: Right atrium). across the patent foramen ovale by Doppler color flow (left figure).
Source: Reproduced with permission from Sasaki T, Miyasaka Y, (LA: Left atrium; RA: Right atrium).
Suwa Y, et al. Real time three-dimensional transesophageal echo- Source: Reproduced with permission from Sasaki T, Miyasaka Y,
cardiographic images of platypnea-orthodeoxia due to patent fora- Suwa Y, et al. Real time three-dimensional transesophageal echo-
men ovale. Echocardiography. 2013;30:E116–17. cardiographic images of platypnea-orthodeoxia due to patent fora-
men ovale. Echocardiography. 2013;30:E116–17.
observer’s ability to obtain unlimited angles and views dimensions, and distance between the LA and the aorta in
of the ASD and mentally recreate these images into 3D. patients with secundum ASD determined to be appropriate
Undoubtedly, this task is difficult and may preclude a candidates for a percutaneous closure procedure.24 There
comprehensive evaluation of the defect before, during have been reports of encroachment of the LA occluder disc
and after the procedure. 3D TEE offers a distinct en face on the aorta observed by 3D TEE, putting patients at risk
view of the defect compared to 2D TEE, providing accurate of aortic erosion and even cardiac tamponade (Fig. 74.11).
measurements as well as confirmation of device placement Insufficient rim size and placement of a wrong sized
in its appropriate position. occluder device are likely contributing factors in causing
3D TEE is effective in measuring the defect size, rim aortic erosions. Bhaya et al. found a correlation between
size, left atrial (LA) and right atrial (RA) occluder disc the maximum dimension of the ASD, the length of the LA
A B C
D E F
Figs 74.8A to F: Live three-dimensional transthoracic echocardiographic assessment of transcatheter closure of atrial septal defect.
(A) Arrow points to the waist of the atrial septal defect (ASD) transcatheter closure device; (B and C) Shows the device viewed from the
top (B) and obliquely (C). Note that the left atrial disc (# 1) is larger than the right atrial disc (# 2); (D) Arrowhead points to the stainless
steel screw thread located in the center of the right atrial disc viewed enface; (E) Arrow points to the small residual shunt seen one day
after the device was positioned; (F) Amplatzer device used for transcatheter closure of ASD. Arrowhead points to the metallic cap and
the arrow points to the waist of the device. (LA: Left atrium; LV: Left ventricle; MC: Metallic cap; NW: Nitinol winding; RA: Right atrium;
RV: Right ventricle; ST: Screw thread). Movie clip 74.8 shows 3D TTE examination of the atrial septal defect closure device 6 weeks
after implantation. The device is well seated. (Movie clip 74.8).
Source: Reproduced with permission from Sinha A, Nanda NC, Misra V, et al. Live three-dimensional transthoracic echocardiographic
assessment of transcatheter closure of atrial septal defect and patent foramen ovale. Echocardiography. 2004;21:749–53.
disc, the waist size of the device, and the distance between case of a patient with a complex Swiss cheese type secundum
the LA disc and the aorta.24 3D TEE measurements of LA ASD resulting in device embolization to the left atrium and
and RA disc lengths have been nearly identical to the then right iliac artery (Figs 74.12A to G).26 Initial imaging
manufacturer’s assigned size, validating its calculations. with 2D TTE was inaccurate compared to follow-up imaging
3D TEE is capable of not only selecting proper patients for with 3D TTE in evaluating the complexity of the ASD based
the percutaneous procedure, but also identifying patients on the absence of en face views of the entire atrial septum.
at higher risk of complications prompting more regular When defects include multiple fenestrations, the risk
follow-up. of complications from a percutaneous repair increases
Device embolization is a serious complication of tremendously and alternative treatment options must be
percutaneous repair that can be minimized by accurate considered.26 3D TTE can help to select good candidates for
measurements of the ASD and placement of an appropriate percutaneous repair of ASD and to inform both patients and
sized occluder device. Wei et al. described an unfortunate physicians of the risk of possible complications.
Fig. 74.9: Live three-dimensional transthoracic echocardiographic

assessment of transcatheter closure of patent foramen ovale (PFO).
Represent the left atrial and right atrial discs of the transcatheter
device used to close the PFO. (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Sinha A, Nanda NC, Mis-
ra V, et al. Live three-dimensional transthoracic echocardiographic
assessment of transcatheter closure of atrial septal defect and pat-
ent foramen ovale. Echocardiography. 2004;21:749–53.
A B C
D E F
Figs 74.10A to G: Live three-dimensional transthoracic echocardiographic assessment of
transcatheter closure of atrial septal defect. In vitro studies. (A and B) Arrowhead shows the stainless
steel screw thread. # 1 and # 2 represent the left atrial and right atrial discs seen enface (A) and
from the side (B); (C) Arrowhead points to the metallic cap seen in the middle of the left atrial disc.
This is where the nitinol windings come together; (D to G) Real time, 2-dimensional transthoracic
echocardiography in embolization of atrial septal defect occlusion device; (D) Arrowhead points
to the device located at the bifurcation of the main pulmonary artery; (E) Color Doppler-guided
continuous-wave Doppler interrogation demonstrates absence of significant obstruction. Peak
velocity is only 1.47 m/s; (F and G) Live/real time, three-dimensional transthoracic echocardiography
in embolization of atrial septal defect occlusion device; (F) Arrowhead points to the device located
at the bifurcation of the main pulmonary artery; (G) Atrial septal defect viewed en face from the
right atrial side (dotted line). (AO: Aorta; LPA: Left pulmonary artery; RPA: Right pulmonary artery).
Source: Figs 10A to C Reproduced with permission from Sinha A, Nanda NC, Misra V, et al. Live
three-dimensional transthoracic echocardiographic assessment of transcatheter closure of atrial
septal defect and patent foramen ovale. Echocardiography. 2004;21:749–53. Source: Figs 10D to
G Reproduced with permission from Dod HS, Reddy VK, Bhardwaj R, et al. Embolization of atrial
septal occluder device to the pulmonary artery: A rare complication and usefulness of live/real time
G three-dimensional transthoracic echocardiography. Echocardiography. 2009;26:6.
Fig. 74.11: Multiplanar rendering mode: Upper panels. Top

arrowheads point to the left atrial disc, lower arrowheads to the
right atrial disc. D1 represents the measurement of the radius of
the left atrial disc from the middle of the central marker band to
the outer edge of the disc. D2 represents the measurement of the
radius of the right atrial disc from the middle of the end screw to
the outer edge of the disc. The arrow in the top right panel points to
the area of contact of the left atrial disc with the aorta (AO) Lower
panels. Arrowhead in the right lower panel points to the left atrial
disc covering the atrial septal defect. Movie clip 74.11 shows the
left atrial disc in contact with the aorta throughout the cardiac cycle.
Source: Reproduced with permission from Bhaya M, Mutluer
FO, Mahan III EF, et al. Incremental utility of live/real time three-
dimensional transesophageal echocardiography in percutaneous
closure of atrial septal defects. Echocardiography. 2013;30:
345–53.
A B
C D
Figs 74.12A to D
E F
Figs 74.12A to G: Live/real time, three-dimensional transeso-
phageal echocardiographic assessment of device embolization
during percutaneous atrial septal defect closure. (A) The arrowheads
point to multiple secundum atrial septal defects (ASD, “swiss cheese”
appearance) viewed en face from the left atrium (LA); (B) Color
Doppler assessment showing flow signals within the defects viewed en
face (left panel). QLAB examination (right panel) showing four defects
numbered 1, 2, 3, and 4; (C) QLAB examination demonstrating en
face view of one of the defects (1) using Color Doppler. In the upper
left panel the cropping plane is positioned exactly parallel to the defect
which resulted in en face viewing of the defect in the lower left panel.
Subsequently the area was measured by planimetry; (D) Demonstrates
the first ASD closure device (D1) in position (viewed from left atrium
and anatomically correct). Arrowhead shows a large residual defect
viewed en face. The arrow points to the device placement catheter;
(E) Shows the second ASD closure device (D2) in position, partially
G overlapping D1 (viewed from left atrium and anatomically correct).
Arrowhead shows the presence of one of the two significant residual
defects; (F) Shows embolization of one of the closure devices (D) to
Sinus Venosus ASD the LA; (G) Shows the device (D) in the proximal descending thoracic
aorta (DA) after percutaneous manipulation from the iliac artery. 1 and
Sinus venosus ASD (SVASD) represents ~4% to 11% of 2 denote the right and the left atrial sides of the device, which are
ASDs.24 Some experts believe that a sinus venosus defect viewed en face in the left lower and the right upper panels. (MV: Mitral
is not a true ASD, but rather the failure of interposition valve; RA: Right atrium).
of the wall between the superior vena cava and the right Source: Reproduced with permission from Wei J, Hsiung MC,
upper pulmonary veins of superior sinus venosus defects Tsai SK, et al. Atrial septal occluder device embolization to an
iliac artery: A case highlighting the utility of three-dimensional
or lack of the wall separating the right lower pulmonary transesophageal echocardiography during percutaneous closure.
veins from the back wall of the right atrium in patients Echocardiography. 2012;29:1128–31.
with inferior sinus venosus defects. Hence, the veins
connect normally but drain abnormally due to this lack of
interposing “walls” and therefore, a sinus venosus defect relation to the superior vena cava (SVC) and right superior
is often not characterized as simply a hole in the wall pulmonary vein offering significant advantage over 2D TEE
between the atria. (Figs 74.13A to C).27 Both modalities are successful in
The capabilities of 3DE have been shown to be sizing the defect comparatively to actual surgical measure-
effective in assessing a sinus venosus defect, 3D TEE ments, but 3D TEE was also capable of calculating the area
reconstruction images confidently visualize the defect in of the defect with the extra dimensions it obtained.
A B C
Figs 74.13A to C: Multiplane transesophageal 3D reconstruction of sinus venosus ASD. (A) The arrowhead points to the large defect in
the superior portion of the atrial septum. The arrow shows the right superior PV entering the SVC-atrial junction at the site of the defect.
(B and C) Orthogonal views demonstrating the size of the defect (ASD), which measured 3.69 cm2 in area. The maximal dimension of
the defect was 2.15 cm, which corresponded to the diameter of 2 cm measured at surgery. The top arrowhead in B points to the right
superior PV, and the bottom arrowhead points to the defect. (ASD: Atrial septal defect; SVC: Superior vena cava; LA: Left atrium; RA:
Right atrium).
Source: Reproduced with permission from Nanda NC, Ansingkar K, Espinal M, et al. Transesophageal three-dimensional echo assess-
ment of sinus venosus atrial septal defect. Echocardiography. 1999;16:835–7.
Unroofed Coronary Sinus Ventricular Septal Defects

Unroofed coronary sinus is an uncommon type of ASD Ventricular septal defects (VSD) represent a commonly
which involves a communication between the roof diagnosed congenital heart defect in the newborns.
of the coronary sinus and the left atrium and is often The defect is most often located within the muscular or
diagnosed in combination with a persistent left superior perimembranous portion of the interventricular septum.
vena cava.28 Unroofing of the coronary sinus can also The diagnosis of a VSD can be made by 2D echocardio-
occur intentionally with surgical repair when there is total graphy, however, the accuracy of its measurements are
anomalous return of the pulmonary veins to the coronary often inconsistent because of the typical oval shape of
sinus.29 The diagnosis of unroofed coronary sinus can be a VSD.30 As a result of its uneven contour, 2D views are
difficult to ascertain with 2D imaging, and when very large unable to accurately quantify the true dimensions of the
can be mistaken for a primum ASD. Importantly, when defect, often resulting in underestimation.4,30 The location,
an unroofed coronary sinus defect is associated with a dimensions and shape of the VSD are more accurately
left superior vena cava, right-to-left shunting can occur, assessed by live/real time 3D TTE (Figs 74.15A and B).22
leading to serious side effects such as a cerebral emboli or With a more complex VSD, the capabilities of 3DE are even
a brain abscess.28 more apparent when compared to 2D echocardiography.
An acquired unroofed coronary sinus was reported in 3D TTE presents an en face view of the defect to provide
an adult patient after there was breakdown of the surgical more reliable measurements of its area and circumference
patch that was placed over the coronary sinus ostium to to better assist the cardiologist in planning for treatment
repair total anomalous pulmonary venous return (TAPVR) (Figs 74.16A and B).31
during infancy (Figs 74.14A to E).29 With its technological Similar to ASDs, there has been some transition
advanced cropping capabilities and the views captured in the treatment of muscular, and to some extent
from unique angles, 3D TTE clearly visualized the perimembranous VSDs, from the traditional surgical
communication between the coronary sinus and the left approach to percutaneous repair because of reported
atrium confirming the diagnosing suggested by coronary decreased mortality rate and fewer complications com-
angiography. Furthermore, 3D color Doppler imaging pared to surgery.31 On the other hand, many medical
confirmed the left-to-right shunting of blood from left centers and expert pediatric cardiologists prefer not to
atrium through the defect in the roof of the coronary sinus perform percutaneous closure of membranous VSDs
into the right atrium. because of high incidence of heart block, damage to
A B
C D
Figs 74.14A to E: Real time/live three-dimensional transthoracic

echocardiographic findings in coronary sinus atrial septal defect.
(A and B) Arrowhead points to unroofed coronary sinus (CS)
which resulted in left atrial (LA) to right atrial (RA) shunting; (C)
Shows opening (arrow) of CS into RA; (D) Parasternal long-axis
view. Arrowhead points to a defect in the roof of CS resulting
in communication with LA; (E) Two-dimensional transthoracic
echocardiography in the same patient. Arrowhead points to a
questionable defect in lower portion of atrial septum. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle). (Movie clip 74.14).
Source: Reproduced with permission from Singh A, Nanda NC,
Romp RL, Kirklin JK. Assessment of surgically unroofed coronary
sinus by real time/live time three-dimensional transthoracic
E echocardiography. Echocardiography. 2007;24:74–6.
A B
Figs 74.15A and B: (A) The arrowhead points to a perimembraneous ventricular septal (VS) defect viewed en face; (B) Shows color
Doppler flow signals in the defect. (LA: Left atrium; LVOT: Left ventricular outflow tract; MV: Mitral valve; RV: Right ventricle). [Movie clip
74.15, (Parts 1 to 4)].
Source: Reproduced with permission from Mehmood F, Miller AP, Nanda NC, et al. Usefulness of live/three-dimensional transthoracic
echocardiography in the characterization of ventricular septal defects in adults. Echocardiography. 2006;23:421–7.
A B
Figs 74.16A and B: (A and B) Ventriuclar septal defect. The arrow in A points to a large defect in the trabecular ventricular system
visualized in the apical four-chamber view. The arrow in B shows the same defect viewed en face by cropping of the three-dimensional
data set. Note the generous margins of the defect. Color Doppler examination shows flow signals moving through the defect. (Movie
clip 74.16).
Source: Reproduced with permission from Nanda NC, Hsiung MC, Miller AP, et al. Live/real time 3D echocardiography. Chichester, West
Sussex: Wiley, Blackwell, 2010.
the aortic valve, and hemolysis. With the percutaneous interventionalist prior to the start of the procedure which
procedure, obtaining precise dimensions of the defect can be utilized in the preprocedural planning.30
and visualizing nearby cardiac structures becomes The use of 3D TEE for comprehensive assessment of
even more important. Compared to 2D TTE, 3D TTE VSDs has become more accepted because of the unique
provides a “surgeon’s view” of the cardiac anatomy to the angles and views obtained of the interventricular septum
and the accurate data with regard to size and shape of types A, B, and C are used to categorize complete ASVDs
the defect. 3D TTE can be used in clinical situations based on the superior bridging leaflet and its attachments
that previously relied on 3D TEE because of comparable to the crest of the ventricular septum and right ventricle.34
information provided by both and can be performed in less Partial ASVDs, on the other hand, have variable defects
invasive manner. Another advantage of 3D TTE compared affecting the atrial septum, ventricular septum or both
to 2D echocardiography is its ability to more accurately and is characterized by the presence of separate right and
depict the distance from the defect to the tricuspid valve, a left atrioventricular valves (Fig. 74.20A to E). Recognizing
very important measurement in preventing complications the narrow and elongated left ventricular outflow tract (the
during repair of the defect.30 Identification of the exact so-called “goose neck” deformity seen on the angiogram)
location of the atrioventricular valves and the extent of the and the lack of wedging of the aorta between the mitral
anatomical rim in relation to the VSD, plays a significant and tricuspid valves is diagnostic in patients with ASVD.
role in determining whether a patient would benefit These findings are presumed to be secondary to the
more from a surgical or a percutaneous intervention displacement of the left ventricular outflow tract and aortic
(Figs 74.17 and 74.18). Fortunately, 3D TTE is capable of valve in the anterior and superior direction.33,34
capturing such images clearly. The effectiveness of 3D TTE Investigation of 3D TTE in patients with ASVDs noted
in measuring maximum dimension, circumference, and obvious advantages over 2D TTE in measuring the size
area of the VSD was validated with comparable surgical and magnitude of the defect and valves, as well as nearby
and intraoperative findings by 3D TEE.30 By visualizing the cardiac structures.33 The ability of 3D TTE to provide an
VSD en face with 3D TTE, an added dimension of depth is en face view of a complete ASVD and furthermore a clear
incorporated into the surgical decision making process.32 delineation of the characteristic five leaflets in complete
defects, sets this modality apart from the capabilities
Atrioventricular Septal Defects of 2D TTE (Figs 74.21A to D).34 In addition, 3D TTE can
Atrioventricular septal defect (AVSD) is a type of CHD identify the superior bridging leaflet and its attachment
that occurs as a result of incomplete fusion of the superior which is responsible for categorizing ASVDs into modified
and inferior endocardial cushions. Absence of fusion Rastelli types.33,34 Singh et al. described a patient with a
of these structures during fetal development results presumed diagnosis of Rastelli type C ASVD based on
in abnormal development of both the atrioventricular imaging with 2D TTE, however, follow-up imaging with 3D
septum as well as the corresponding valves.33 There are TTE demonstrated that the defect was actually a Rastelli
three types of ASVDs: complete, partial, and intermediate. type A ASVD.33 Similarly, 2D TTE provided visualization
Complete ASVDs are distinguished by a large AVSD of a right and left atrioventricular valve annuli, suggesting
(primum ASD and inlet VSD) in combination with a single a partial ASVD, but an en face view of 3D TTE also
atrioventricular valve (Fig. 74.19A to G).33 Modified Rastelli identified features of a complete ASVD confirming the
Fig. 74.17: Shows the relationship and the distances of pulmonary

valve and tricuspid valve from the ventricular septal defect. (PV:
Pulmonary valve; TV: Tricuspid valve; RA: Right atrium; RV: Right
ventricle).
Source: Reproduced with permission from Chen FL, Hsiung
NC, Nanda NC, Hsieh KS, Chou MC. Real time 3-dimensional
echocardiography in assessing ventricular septal defects: An
echocardiographic-surgical correlative study. Echocardiography.
2006;23:562–8.
A B C D
E F G
Figs 74.18A to G: Perimembranous ventricular septal defect (inlet) (arrow). (A) Real time 3D echocardiography (RT3DE) volume-ren-
dered image of the right ventricle (RV) displaying the right aspect of the ventricular septal defect. The location of the defect in relation to
the tricuspid valve (TV) is shown; (B) Surgical view of VSD from the right aspect; (C) RT3DE volume-rendered image of the left ventricle
(LV) displaying the left aspect of the VSD. The location of the defect in relation to the left ventricular outflow tract (LVOT) is shown; (D)
2DE parasternal long-axis views showed the VSD in relation to aortic valve and right ventricle; (E to G) Live three-dimensional transtho-
racic echocardiography in a patient with tetralogy of Fallot; (E) Note the wide aortic root (AO) and narrow pulmonary artery (PA); (F) The
AO overrides the interventricular septum (IVS); (G) Four-chamber view. The ventricular septal defect (VSD) is located at the crux. (AO:
ZAorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle);
Source: Figures 18A to D reproduced with permission from Chen FL, Hsiung NC, Nanda NC, Hsieh KS, Chou MC. Real time 3-dimen-
sional echocardiography in assessing ventricular septal defects: An echocardiographic-surgical correlative study. Echocardiography.
2006;23:562–8.
Source: Figures 18E to G reproduced with permission from Wang et al. Echocardiography. 2003;20:593–604.
diagnosis of intermediate ASVD.33 3D echocardiography repair because of its ability to visualize the valve and its
can easily recognize the characteristic ASVD findings of surrounding anatomy, in addition to the color Doppler
an elongated and narrowed left ventricular outflow tract. capabilities. 3D TTE is also able to better evaluate valve
Additionally, 3DE can identify the so called cleft within the morphology and function. 3D TEE is effective in measuring
left atrioventricular valve and evaluate its length, width, the size, shape, and location of the ASVD which correlates
and rim size which is often a limitation of 2D TTE. well with the actual surgical measurements.37 3D TTE can
After repair of an ASVD, left atrioventricular valve confirm data already obtained by 2D TTE, but the above
(LAVV) regurgitation is a common complication affecting mentioned cases indicate the advanced capabilities of 3D
greater than 10% of patients. These findings are likely TTE in diagnosing complex CHD defects which 2D TTE
related to postsurgical changes leading to increased valve often misses.
diameter and consequent valve insufficiency.35,36 Knowing
about this potential complication substantiates the need
for a reliable imaging modality to diagnose and monitor
COMMON ATRIUM
progression of valvular disease.35 Live/real time 3D TTE is Common atrium is a type of ASVD that occurs as a
preferable to 2D TEE in evaluating the LAAV after ASVD result of absent atrial septal tissue and affixing of the
A B
C D
E F
Figs 74.19A to F
Figs 74.19A to G: Live/real time, three-dimensional transthoracic

echocardiography in complete atrioventricular septal defects. (A)
Arrow shows attachment of common atrioventricular valve to a
papillary muscle in the right ventricle (Rastelli type B). No attachments
were seen to the crest of the ventricular septum (arrowhead); (B) En
face view shows all five leaflets of common atrioventricular valve;
(C) En face view of the defect viewed from top and sides (arrows).
Arrowhead points to atrial septum; (D) Arrowhead points to elongated
and narrowed left ventricular outflow tract; (E) Shows absence
of wedging of aorta (AO) in relation to common atrioventricular
valve (CAV) annulus; (F) Arrowhead points to the vena contracta
of CAV regurgitation jet. Its area measured 0.1 cm2. Color Doppler-
guided continuous-wave Doppler interrogation of regurgitant
jet showed a velocity time integral (VTI) of 79 cm. Regurgitant
volume was calculated as 7.9 cm3; (G) Bicuspid aortic valve (AV).
(AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA:
Left atrium; LV: Left ventricle; MI: Mural inferior leaflet; ML: Mural
lateral leaflet; PA: Pulmonary artery; PV: Pulmonary valve; RA: Right
atrium; RV: Right ventricle; RAV: Right atrioventricular valve; RVO:
Right ventricular outflow tract; SB: Superior bridging leaflet). [Movie
clips 74.19A, B, C, E, (Parts 1 to 3)].
Source: Reproduced with permission from Singh A, Romp RL, Nanda
NC, et al. Usefulness of live/real time three-dimensional transthoracic
echocardiography in the assessment of atrioventricular septal defects.
G Echocardiography. 2006;23:598–606.
A B
C D
Figs 74.20A to D
Figs 74.20A to E: Live/real time, three-dimensional transtho-

racic echocardiography in partial atrioventricular septal defects.
(A) Arrowhead points to a prominent cleft in the anterior leaflet
of the left atrioventricular valve (LAV); (B) Arrows point to two
left ventricular papillary muscles located close to each other; (C)
Arrowhead points to a widened anteroseptal commissure (“cleft”)
of the right atrioventricular valve (RAV); (D) Arrowhead points to an
accessory LAV orifice; (E) Arrows point to the presence of only two
scallops in the posterior leaflet of LAV. The black arrowhead points
to the anterior leaflet of LAV. (AS: Anterosuperior leaflet; IB: Infe-
rior bridging leaflet; L: Liver; LA: Left atrium; LV: Left ventricle; MI:
Mural inferior leaflet; ML: Mural lateral leaflet; PA: Pulmonary
artery; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle;
RAV: Right atrioventricular valve; RVO: Right ventricular outflow
tract; SB: Superior bridging leaflet). Movie clip 74.20C.
Source: Reproduced with permission from Singh A, Romp RL,
Nanda NC, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in the assessment of atrioven-
E tricular septal defects. Echocardiography. 2006;23:598–606.
A B
C D
Figs 74.21A to D: Live/real time, three-dimensional transthoracic echocardiography (3DTTE) in atrioventricular septal defects (AVSDs).
(A) Complete AVSD. Arrowhead shows attachment of CAV to the crest of the ventricular septum (Rastelli type A). Arrow points to atrial
component of the defect; (B) Complete AVSD. Arrowhead points to an anomalous papillary muscle projecting into the left ventricular
outflow tract causing subaortic obstruction; (C and D) Intermediate AVSD; (C) En face view of CAV shows superior bridging (SB) leaf-
let crossing over into the RV; (D) Both the AO and the PA are seen arising from the RV consistent with double outlet right ventricle.
(AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA: Left atrium; LV: Left ventricle; MI: Mural inferior leaflet; ML: Mural
lateral leaflet; PA: Pulmonary artery; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle; RAV: Right atrioventricular valve; RVO:
Right ventricular outflow tract; SB: Superior bridging leaflet). (Movie clips 74.21B and C).
Source: Reproduced with permission from Singh A, Romp RL, Nanda NC, et al. Usefulness of live/real time three-dimensional transtho-
racic echocardiography in the assessment of atrioventricular septal defects. Echocardiography 2006;23:598–606.
atrioventricular valves to the interventricular septum.38 left atrium and ventricle secondary to chronic left-to-right
3D TEE has been used to diagnose a common atrium with shunting. 2D TTE has been the imaging modality of
a cor triatriatum membrane and an associated cleft mitral choice; however, there are obvious limitations that should
valve leaflet (typically seen in common atrium) (Figs be recognized. False positive results can occur in 2D TTE
74.22A to I). 2D TEE evaluated a prominence of the LA wall, from artifactual dropouts designating 3D TTE as a better
but was unable to identify it as a cor triatriatum membrane modality in diagnosing aortopulmonary window.40 The
and misdiagnosed these findings as a partial ASVD. ability of 3D TTE to combine different data sets from various
angles offers a better understanding of the defect and
AORTOPULMONARY WINDOW enhances the confidence of the diagnosis. The en face view
of the defect offered by 3D TTE also allows for more accurate
An aortopulmonary window is a rare congenital anomaly
measurement of its dimensions (Figs 74.23 and 74.24).40
involving a communication between the ascending
aorta and the pulmonary artery. Echocardiography is the
imaging modality of choice in diagnosing aortopulmonary
PATENT DUCTUS ARTERIOSUS (PDA)
window.39 The typical echocardiographic finding in The ductus arteriosus is a normal connection during fetal
patients with an aortopulmonary window is dilation of the life between the pulmonary artery and the descending
A B
C D
Figs 74.22A to D
E F
G H
Figs 74.22A to I: Three-dimensional transesophageal echo-
cardiographic examination in cor triatriatum with common atrium.
(A and B) The arrow points to the cor triatriatum membrane while
the arrowheads (black) outline a large nonobstructive opening
present within the membrane; (C) The common atrium (CA) with
no atrial septum and the cor triatriatum membrane are well seen;
(D to H) Show left and right upper pulmonary veins (LPV and RPV)
located superior to the cor triatriatum membrane (arrows). 1 and
2 represent jets of right and left atrioventricular valve (RAV and
LAV) regurgitation, respectively. Portions of both jets appear to
move into the common atrium through the large opening in the cor
triatriatum membrane. Short-axis view of left atrioventricular valve
showing a cleft (C) in the anterior leaflet. (AV: Aortic valve; LVO:
Left ventricular outflow; PL: Posterior left atrioventricular valve
leaflet. RV: Right ventricle; LV: Left ventricle).
Source: Reproduced with permission from Baweja G, Nanda NC,
Kirklin JK. Definitive diagnosis of cor triatriatum with common
atrium by three-dimensional transesophageal echocardiography
I in an adult. Echocardiography. 2004;21:303–6.
A B
C D
E F
Figs 74.23A to F: Live/real time, three-dimensional transthoracic echocardiographic assessment of aortopulmonary window.
(A) Aortic short-axis view showing no evidence of an aortopulmonary window at this level. Movie clip 74.23, Part 2. The arrow points to
the aortopulmonary window; (B) When the three-dimensional dataset was cropped posteroanteriorly, a large communication between
the aorta (AO) and pulmonary artery (PA) was revealed (arrowhead); (C to E) The aortopulmonary window (arrowhead) could be viewed
en face by cropping the 3D dataset from the side (C) and rotating it (D and E); (F) Color Doppler exam shows mild pulmonic regurgitation
(PR). The arrowhead points to the aortopulmonary window. (AV: Aortic valve; LA: Left atrium; LPA: Left pulmonary artery; RPA: Right
pulmonary artery). [Movie clip 74.23, (Parts 1 and 2)].
Source: Reproduced with permission from Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/real time three-dimension-
al transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:96–9.
A B
C D
Figs 74.24A to D: Live/real time, three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. (A and B)
Arrowhead points to the aortopulmonary window. Movie clip 74.24, Parts 1 to 2. The arrow points to the aortopulmonary window. The
asterisk in Movie clip 74.24, Part 3 denotes the descending thoracic aorta; (C) Arrow points to the interrupted aortic arch. Arrowhead
points to the aortopulmonary window; (D) Arrow points to a patent foramen ovale. (BCT: Brachiocephalic trunk; LCC: Left common
carotid artery; LSA: Left subclavian artery; MV: Mitral valve; RA: Right atrium; TV: Tricuspid valve). [Movie clip 74.24, (Parts 1 to 3)].
Source: Reproduced with permission from Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/real time three-dimension-
al transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:96–9.
thoracic aorta (Figs 74.25 to 74.28).41 Shortly after birth, pulmonary artery and descending thoracic aorta.42 3D TTE
the ductus arteriosus is expected to close, but in some can determine the type of PDA which is useful in selecting
patients it remains open and can persist into adulthood. optimal candidates for percutaneous intervention, the
Patent ductus arteriosus can be diagnosed by a variety of optimal anatomical approach for the procedure, and any
imaging modalities including 2D echocardiography (TTE, expected risks or complications.
TEE), CT, and MRI. A study by Sinha et al. in 2004 sought
to understand the usefulness of 3D TTE in the evaluation
of PDA.42 2D TTE can diagnose a PDA, however, the CONOTRUNCAL ANOMALIES
assessment of the defect is often limited without the use
of 3D TTE as well. 3D TTE can provide a realistic image
Transposition of the Great Arteries
of the PDA including its location, shape, length and width Transposition of the great arteries (TGA) is organized
as well as en face visualization of its connections with the into two distinct types: dextro (D-TGA) and levo (L-TGA;
A B
Figs 74.25A to C: Two-dimensional transthoracic echocardio-

graphy in an adult with patent ductus arteriosus. (A and B) Color
Doppler examination demonstrates flow signals (arrowhead)
moving between the main pulmonary artery (PA) and the descending
thoracic aorta (DA) indicative of patent ductus arteriosus; (C)
Color Doppler-guided continuous-wave Doppler examination
demonstrates flow signals moving from PA to DA in systole and
back into PA in diastole (arrowheads). The arrow points to the
continuous-wave Doppler cursor line. (AO: Aorta; DA: Descending
aorta; PA: Pulmonary artery; RPA: Right pulmonary artery).
Source: Reproduced with permission from Sinha A, Nanda
NC, Khanna D, et al. Live three-dimensional transthoracic
echocardiographic delineation of patent ductus arteriosus. Echo-
C cardiography. 2004;21:443–8.
A B
Figs 74.26A and B
Figs 74.26A to C: Live three-dimensional transthoracic echocar-

diography in an adult with patent ductus arteriosus. B mode images.
(A) The pyramidal section has been cropped to show the full extent
of the PDA (arrowhead) which connects the PA to DA. The arrow
points to the left atrial appendage; (B and C) The pyramidal section
has been cropped from the top to show the opening of the PDA
(arrowhead) into the pulmonary artery and its close location to the
origin of the left pulmonary artery (arrow). (LPA: Left pulmonary
artery; LV: Left ventricle; PV: Pulmonary valve).
Source: Reproduced with permission from Sinha A, Nanda NC,
Khanna D, et al. Live three-dimensional transthoracic echo-
cardiographic delineation of patent ductus arteriosus. Echo-
A B
C D
Figs 74.27A to D
E F
Figs 74.27A to G: Live three-dimensional transthoracic echocardio-
graphy in an adult with patent ductus arteriosus (PDA). Color Doppler
images. The pyramidal section has been cropped to visualize flow
signals in PA, PDA (arrowhead), and DA. (A) Shows the length of the
PDA (arrowhead) connecting the PA and DA. The arrow points to an
intercostal artery arising from DA; (B and C) Color Doppler images have
been isolated by completely suppressing B mode images. The isolated
color Doppler images could be rotated from 0° to 176°, thus enabling
comprehensive visualization of flows in PA, PDA (arrowhead), and DA in
three-dimensions. Images at 0° (top left), 45° (top right), 90° (lower left),
and 176° (lower right) rotation are shown; (D) Frontal sections showing
color Doppler signals moving from PA into DA in systole (left) and back
into PA in diastole (right); (E and F) Tilted (top half) and enface (lower half)
views of PDA (arrowheads) at aortic (E) and pulmonary (F) connections.
On the right, B mode signals have been suppressed resulting in only
flow visualization; (G) Schematic L, length of the ampulla, defined as the
distance between the mid-portion of narrowest diameter of PDA and the
mid-portion of the aortic end. This measured 0.94 cm in our patient. D,
PDA diameter at aortic insertion (ampulla diameter). This measured 1.31
cm in our patient. (DA: Descending thoracic aorta; LPA: Left pulmonary
artery; LV: Left ventricle; PV: Pulmonary valve; RV: Right ventricle).
(Movie clip 74.27).
Source: Reproduced with permission from Sinha A, Nanda NC, Khanna
D, et al. Live three-dimensional transthoracic echocardiographic
delineation of patent ductus arteriosus. Echocardiography. 2004;21:
G 443–8.
Figs 74.29 to 74.37). D-TGA is characterized by ventri- care for repair of TGA, however, long-term follow-up of
culoarterial discordance whereby the right ventricle these patients revealed significant morbidity associated
pumps blood to the aorta and the left ventricle pumps with postprocedural complications. These complications
blood to the pulmonary artery.43 A shunt lesion (ASD, include baffle leaks, tricuspid valve regurgitation,
VSD, or PDA) between the left and right side of the obstruction, arrhythmias, systolic dysfunction and even
heart must be present to ensure appropriate mixing of sudden death.45 The current recommended surgical
pulmonary and systemic return to enable perfusion of approach is an arterial switch operation, which involves
oxygenated blood to the body’s vital organs. In an atrial rearranging the aorta and pulmonary artery to the
switch surgery, deoxygenated blood is diverted from the appropriate anatomical ventricle.46
inferior and superior vena cavae to the left side of the heart L-TGA (or congenitally corrected TGA) is characterized
and from there into the pulmonary artery through the most often by situs solitus with atrioventricular and
creation of an intra-atrial baffle.44 Senning and Mustard ventriculoarterial discordance. In these patients, the
atrial switch procedures were historically the standard of right atrium connects to the left ventricle which pumps
Fig. 74.28: Thoracic magnetic resonance angiogram with Gado-

linium in the same patient as Figures 25 to 27. The arrowhead
points to patent ductus arteriosus (PDA) viewed in the left lateral
position. (DA: Descending thoracic aorta; PA: Pulmonary artery;
Source: Reproduced with permission from Sinha A, Nanda
NC, Khanna D, et al. Live three-dimensional transthoracic
echocardiographic delineation of patent ductus arteriosus.
A B
Figs 74.29A and B: Dextro-transposition of the great arteries. (A) The aorta (AO) arises from the right ventricle (RV); (B) The pulmonary
artery (PA) originates from the left ventricle (LV). The arrows point to left and right branches of the main PA. (Movie clips 74.29A and B).
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardio-
graphic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095–104.
deoxygenated blood to the pulmonary artery. The left 2D TTE is used to diagnose and monitor progression
atrium connects to the right ventricle which pumps of disease in L-TGA and to assess for complications
oxygenated blood to the aorta.44 Patients with L-TGA often after corrective surgery of D-TGA, but 3D TTE has been
remain undiagnosed into adulthood unless they have shown to have valuable advantages over 2D imaging.44 A
coexisting congenital defects which prompt corrective comprehensive view of the aortic and pulmonary valves
repair in childhood. Signs and symptoms of right with corresponding valve function is more easily obtained
ventricular heart failure can develop in adulthood due to by 3D TTE compared to 2D TTE. Specifically, the tricuspid
chronic pumping of the right ventricle against the higher valve is most often affected in TGA and 3D TTE with its
systemic pressure often prompting further workup and en face view and unique angles is capable of identifying
eventual surgical repair.43 each of three leaflets of the valve. The posterior or inferior
A B
Figs 74.30A and B: Levo-transposition (corrected transposition) of the great arteries. (A) The arrows point to two vena contractas of
tricuspid regurgitation (TR) jets; (B) En face view of the two TR vena contractas (arrows). The movie clips show cropping of the tricuspid
regurgitation jet (arrow) to view the vena contracta (arrowhead) en face in another patient with levo-transposition (corrected transposi-
tion) of the great vessels. (RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve). [Movie clip 74.30, (Parts 1 to 3)].
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardio-
graphic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095–104.
A B
Figs 74.31A to C: Dextro-transposition of the great arteries. In (A)

the aortic valve (AV) and aorta are located anterior and to the right
of the pulmonary valve (PV) and pulmonary artery. All four cardiac
valves are visualized; In (B) the AV and aorta are located directly
anterior to the PV and pulmonary artery. The arrow points to the
intra-atrial baffle; C shows normal relationship of the semilunar
valves and the great arteries in a patient without transposition of
the great arteries. The PV and the pulmonary artery are located
anterior and to the left of the aorta and the AV. All four cardiac
valves are visualized. (MV: Mitral valve; TV: Tricuspid valve).
(Movie clips 74.31A to C).
Source: Reproduced with permission from Enar S, Singh P,
Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries
C in the adult. Echocardiography. 2009;26:1095–104.
Fig. 74.32: Dextro-transposition of the great arteries. Multiple

anatomic defects are present in the tricuspid valve (TV) which is
viewed en face in the closed position in systole.
in the adult. Echocardiography. 2009;26:1095–104.
A B
Figs 74.33A to C: Dextro-transposition of the great arteries.

(A and B) The arrow points to a cleft in the anterior mitral valve (MV)
leaflet; (C) The arrow points to a narrow left ventricular outflow
tract. Systolic anterior movement of the anterior mitral leaflet (AML)
is also seen. The data set was cropped from the top. (RV: Right
ventricle; VS: Ventricular septum).
C in the adult. Echocardiography. 2009;26:1095–104.
A B
C D
Figs 74.34A to D: Dextro-transposition of the great arteries. (A and B) The intra-atrial baffle (B) appears as a shelf when viewed en
face by cropping from the bottom. A defect in the baffle is not well seen in the illustration but it is clearly visualized (arrowhead) in the
accompanying movie clips which view the baffle en face; (C) Shows the relationship of the baffle to the inferior vena cava (IVC); (D)
Color Doppler study shows systemic venous flow signals (arrow) moving through the left ventricle (LV) toward the pulmonary artery (PA).
(SVA: Systemic venous atrium). [Movie clip 74.34, (Parts 1 and 2)].
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095–104.
tricuspid leaflet is frequently visualized poorly by 2D TTE measurement of its size is important in determining
requiring assistance from 3D imaging. With regard to regurgitation severity. The product of the area of vena
function, 3D TTE reveals deficiencies in leaflet coaptation, contracta assessed by 3D TTE and the velocity time
prolapse of leaflet components, and provides specific integral of the regurgitation jet obtained by continuous-
details on the defect within the valve itself. Over time, the wave Doppler provides an accurate estimate of the volume
severity of valvular regurgitation should be monitored of regurgitation.44 In addition to the tricuspid valve, 3D
and progressively worsening function can be evaluated by TTE is unique in offering multiple views of the pulmonic
3D TTE by visualizing the shape and measuring the area valve and can detect systolic anterior motion of the
of the vena contracta, a function lacking in 2D TTE. The mitral valve and left ventricular outflow tract obstruction
vena contracta basically relates to the “hole” in a valve (affecting up to one-third of patients with TGA who have
through which regurgitation occurs and hence accurate undergone an atrial switch operation) with significant
Fig. 74.35: Dextro-transposition of the great arteries. The arrow

points to a shelf-like intra-atrial baffle located behind the pulmonary
artery (PA) in another patient. Note the anterior and leftward position
of the aortic valve and aorta (AO) in relation to the pulmonary valve
and pulmonary artery (PA). (TV: Tricuspid valve).
echocardiographic assessment of transposition of the great
arteries in the adult. Echocardiography. 2009;26:1095–104.
A B
Figs 74.36A to C: Dextro-transposition of the great arteries.

(A) Color Doppler examination. The arrow points to a defect
in the intra-atrial baffle (B); (B) En face view of the leak (arrow)
at the origin (vena contracta); (C) Arrow points to the defect in
the baffle seen after suppression of color Doppler flow signals.
(PVA: Pulmonary venous atrium; SVA: Systemic venous atrium).
(Movie clip 74.36).
echocardiographic assessment of transposition of the great
C arteries in the adult. Echocardiography. 2009;26:1095–104.
difficulty in performing such a comprehensive evaluation.36

3D TTE can supplement 2D TTE with precise measurement
of the size and shape of a VSD. Based on the usefulness
of 3D TTE in monitoring pulmonary regurgitation and its
severity, cardiologists can recognize high risk patients and
monitor progression of disease closely.
Coronary Artery Anomalies

Anomalous coronary artery (ACA) is not an uncommon
diagnosis and may be underestimated due to the fact that
only 20% of patients with this structural abnormality will
present with symptoms.50 Common presentations include
myocardial infarction, arrhythmias, angina, syncope, and
even sudden death.50,51 Complications from ACA are more
Fig. 74.37: Dextro-transposition of the great arteries. Arrow
points to a leak in the intra-atrial baffle (B) in another patient. (IVS:
likely to occur when coronary arteries are anatomically
interventricular septum). located between the aorta and the pulmonary arteries.
Source: Reproduced with permission from Enar S, Singh P, Doug- Obstruction of blood flow likely occurs secondary to
las C, et al. Live/real time three-dimensional transthoracic echo-
compression between the great vessels or acute angu-
cardiographic assessment of transposition of the great arteries in
the adult. Echocardiography. 2009;26:1095–104. lations in the coronary arteries.51
Coronary angiography remains the gold standard for
diagnosing anomalous coronary arteries, however, less
anatomical detail.44 Follow-up of patients who have had an invasive imaging with echocardiography can provide useful
atrial switch surgery relies heavily on the ability of 3D TTE information that angiography often misses.50 Multiplane
to evaluate for postprocedure complications such as baffle 2D TEE and 3D reconstruction can often provide similar
leaks (up to 40% of patients after Mustard procedure) or findings in ACA, but the 3D images offer supplemental
obstruction, functions that are often limited by 2D TTE.44,47 data used to increase the confidence of the diagnosis.51 3D
Ahmed et al. described an intra-atrial baffle obstruction TTE has previously been used to assess proximal coronary
years after surgical repair of a TGA diagnosed by 3D TTE arteries; however, Vengala et al. in 2003 demonstrated
based on its ability to view the defect en face in the short additional capabilities of 3D TTE in evaluation of coronary
axis. anatomy.52 ACAs first detected by 3D TTE were later
confirmed with angiography, validating its use. The en
Tetralogy of Fallot face view and unique maneuvering in multiple geometric
ToF is a complex cyanotic CHD defect that includes VSD, planes offered by live/real time 3D TTE depicts the origin
overriding aorta, pulmonary outflow tract obstruction and and course of the coronary artery. Coronary angiography
right ventricular hypertrophy. Surgical repair is necessary often has difficulty in visualizing the anomalous vessel
early in life and these patients require close follow- between the aorta and the pulmonary artery, which as
up to monitor for possible complications. Postsurgical described above carries a greater risk of complications.51
ToF patients can experience severe pulmonary valve Both 2D and 3DE can diagnose an anomalous origin
regurgitation or right ventricular outflow obstruction. of the left coronary artery from the pulmonary artery,
Very rarely, patients may have residual VSDs.48 3D TTE but 3D TTE can also visualize the opening of the ACA
has demonstrated its superiority compared to 2D imaging en face and its connections to the aorta and pulmonary
in assessing pulmonary regurgitation severity based on artery (Figs 74.38 and 74.39).53 This presentation requires
visualization of the vena contracta.49 Accurate assessment immediate surgical correction that previously consisted
of the size and shape of residual defects is crucial for of creating a tunnel between the aorta and the pulmonary
determining if repeat surgery is necessary and 2D TTE, artery reversing blood flow into the aorta. 3D TTE was
especially in the setting of other congenital anomalies, has capable of tracking the tunnel between the aorta and
A B
C D
Figs 74.38A to E: Two-dimensional transesophageal and trans-
thoracic echocardiography in an adult with anomalous origin of the left
coronary artery from the pulmonary artery. (A and B) Intraoperative
transesophageal study shows a dilated right coronary artery (RCA, A)
and the surgically created tunnel (T, B); (C to E) Transthoracic study
(performed 13 years after surgery). The arrow in C shows a small
defect in the tunnel (T) near the aorta through which shunting occurs
into the pulmonary artery (arrowhead in D). Color Doppler-guided
continuous-wave Doppler examination (E) shows shunting occurring
practically throughout the cardiac cycle (arrowheads). The arrow in
E points to the Doppler cursor line. (AO: Aorta; LA: Left atrium; LPA:
Left pulmonary artery; PR: Pulmonary regurgitation; PV: Pulmonary
valve; RA: Right atrium; RPA: Right pulmonary artery; RVOT: Right
ventricular outflow tract). Source: Reproduced with permission from
Ilgenli TF, Nanda NC, Sinha A, Khanna D. Live three-dimensional
transthoracic echocardiographic demonstration of anomalous origin
of left coronary artery from the pulmonary artery. Echocardiography.
E 2004;21:559–62.
A B
C D
E F
Figs 74.39A to F
Figs 74.39A to G: Live three-dimensional transthoracic echo-

cardiographic demonstration of anomalous origin of left coronary
artery from the pulmonary artery. (A) The arrowhead points to the
orifice of the anomalous coronary artery while the arrow shows
the defect in the tunnel; (B and C) Color Doppler examination.
In B, flow signals (arrowhead) are seen moving into the orifice
of the anomalous coronary artery from the tunnel (T). The arrow
points to the tunnel-pulmonary artery shunt. In C, the arrow points
to color Doppler shunt flow signals visualized in three dimensions;
(D) Coronary angiogram. Arrowhead points to fistula originating
from the left anterior descending coronary artery (LAD) and
draining into the pulmonary artery (PA) which is partially opacified.
(E to G) Live three-dimensional transthoracic echocardiography; E.
Arrowhead points to a small localized area of abnormal flow signals
in the pulmonary artery (PA) just distal to the pulmonary valve (PV)
in both diastole (F) and systole (G) consistent with entrance of
the fistula into the PA. The arrow in F points to mild pulmonary
regurgitation. (AV: aortic valve; CX: Circumflex coronary artery;
G LM: Left main coronary artery; RVO: Right ventricular outflow
tract). (PA: Main pulmonary artery; AO: Aorta; LA: Left atrium;
LPA: Left pulmonary artery; PR: Pulmonary regurgitation; PV:
Pulmonary valve; RA: Right atrium; RPA: Right pulmonary artery;
pulmonary artery and Doppler functions were used to RVOT: Right ventricular outflow tract). Source: Figure 74.39A to C
confirm the tunnel-pulmonary artery shunting.53 Based Reproduced with permission from Ilgenli TF, Nanda NC, Sinha A,
on the 3D views, the area of the defect was measured and Khanna D. Live three-dimensional transthoracic echocardiographic
demonstration of anomalous origin of left coronary artery from the
shunt volume into the pulmonary artery was calculated. pulmonary artery. Echocardiography. 2004;21:559–562. Source:
Nowadays, surgeons just reimplant the coronary artery Figure 74.39D Reproduced with permission from Mehta D, Nanda
between the pulmonary artery and the aorta. Diagnosis of NC, Vengala S, Mehmood F, Taylor J. Live three dimensional
transthoracic echocardiographic demonstration of coronary artery
the left main coronary artery fistula to the left ventricle has to pulmonary artery fistula. Am J Geriatric Cardiol. 2005;14:42–4.
also been made by 3D TTE (Figs 74.40A and B).54 Source: Figures 74.39E to G Reproduced with permission from
Mehta D, Nanda NC, Vengala S, Mehmood F, Taylor J. Live three
dimensional transthoracic echocardiographic demonstration of
OUTFLOW TRACT OBSTRUCTION coronary artery to pulmonary artery fistula. Am J Geriatric Cardiol.
2005;14:42–4.
Congenital Aortic Stenosis/Bicuspid
Aortic Valve TTE is unable to perform (Figs 74.41 to 74.43).56 Leaflet
The most common cause of aortic stenosis (AS) in adults perforation is an unfortunate complication that can result
is acquired, related to calcific degeneration of the valve, in significant AR which has been visualized by 3DE.
but congenital AS, most notably from bicuspid aortic valve Quadricuspid aortic valves, although rare, have been
(BAV), is a clinically relevant abnormality. When a patient diagnosed by 3D TTE. Burri et al. described a young
presents with symptoms of AS prior to the seventh decade female patient scheduled to undergo aortic valve surgery
of life, the diagnosis of BAV should be strongly considered. for AR who was misdiagnosed with BAV based on 2D
In addition to AS, aortic regurgitation (AR), infective echocardiographic findings (Figs 74.44A to C).57 On further
endocarditis, and aortic dissection have all been linked to investigation of the valve with preoperative 3D TTE and
BAV.55 intraoperative multiplane TEE, images were consistent
Compared to 2D TTE, 3D TTE offers better images of the with a quadricuspid aortic valve (Figs 74.45A and B).
aortic valve, especially the visualization of the individual Understanding the capabilities of 3D TTE and analyzing
leaflets.56 The identification of redundant aortic valve the valve carefully from each echocardiographic view can
leaflets are relevant in distinguishing the development of be instrumental in making a diagnosis that was previously
AS versus AR based on the BAV closure down the midline.1 missed. In this case, a quadricuspid aortic valve was
3D TTE is able to recognize redundancy of aortic valve visualized in the parasternal short-axis view, but not in the
leaflets and offer prognostic information, a feature 2D parasternal long-axis view.
A B
Figs 74.40A and B: Live/real time, three-dimensional transthoracic echocardiographic detection of left main coronary artery fistula into
the left ventricle. (A and B) The arrowhead in A points to the enlarged left main coronary artery (LM). Its entrance into the left ventricle
(LV) is denoted by an arrowhead in (B). (AO: Aorta). (Movie clips 74.40A and B).
A B
Figs 74.41A and B: Two-dimensional transthoracic echocardiography in an adult patient with bicuspid aortic valve and severe aortic
regurgitation. (A) The bicuspid aortic valve (AV) is shown in systole in the open position with no evidence of stenosis; (B) Color Doppler
examination shows an eccentric jet of aortic regurgitation originating posteriorly (horizontal arrow). The vertical arrow points to mild
pulmonic regurgitation. (PV: Pulmonic valve; RA: Right atrium; RV: Right ventricle). (Movie clips 41A and B).
Source: Reproduced with permission from Singh P, Dutta R, Nanda NC. Live/real time three-dimensional transthoracic echocardio-
graphic assessment of bicuspid aortic valve morphology. Echocardiography. 26.:4:478–80.
It is important to recognize that 2D echocardiography aortic valve stenosis and supravalvar stenosis in the same
with Doppler tends to overestimate the severity of stenotic patient. This feature cannot be done in 2D Doppler which
lesions due to the pressure recovery phenomenon and the will simply give the maximum gradient obtained from both
presence of localized high velocities (and gradients) which areas but cannot identify the severity of each individual
may not reflect the true gradient across the stenotic valve lesion. A major advantage of 3D over 2D echocardiography
or subvalvar membrane. Also, 3DE is particularly useful in is the ability to systematically crop the 3D data sets in a
characterizing adjacent stenotic lesions in tandem such as sequential manner such that an en face view of the valve
A B
Figs 74.42A and B: Live/real time, three-dimensional transthoracic echocardiography in the same patient. (A) Note the presence of
several folds in the bicuspid aortic valve in the closed position viewed from the ventricular side; (B) The arrow points to a well circum-
scribed perforation in the posterior cusp of the aortic valve. (LA: Left atrium; PV: Pulmonic valve; RA: Right atrium; RV: Right ventricle).
(Movie clips 74.42A and B).
Source: Reproduced with permission from Singh P, Dutta R, Nanda NC. Live/real time three-dimensional transthoracic echocardio-
graphic assessment of bicuspid aortic valve morphology. Echocardiography. 26;4:478–80.
A B
Figs 74.43A and B: Two-dimensional transthoracic echocardiography. (A and B) The aortic valve (AV) appears bicuspid. (LA: Left
atrium; RV: Right ventricle). (Movie clip 74.43).
Source: Reproduced with permission from Burri MV, Nanda NC, Singh A, Panwar SR. Live/real time three-dimensional transthoracic
echocardiographic identification of quadricuspid aortic valve. Echocardiography. 2007;24:653–5.
orifice is obtained at the level of the valve tip where the oblique planes. With 2D echocardiography, it is not always
stenosis is most significant. This can then be planimetered possible to be certain that one is visualizing the valve at
providing an accurate estimate of the stenosis severity. its tip and it is difficult to determine whether the plane is
3DE is also useful in making sure that the cutting plane parallel or oblique in relation to the valve orifice. This is
in the 3D data set is exactly parallel to the plane of the because, at any given time, 2D echocardiography provides
valve orifice preventing inaccuracies introduced by only a thin slice-like view of a cardiac structure such as the
A B
Figs 74.44A to C: Multiplane two-dimensional transesophageal

echocardiography. (A and B) Four aortic valve (AV) leaflets
(numbered in B) are well seen. The arrow in A points to diastolic
noncoaptation of AV leaflets which resulted in significant aortic
regurgitation. C. The arrow points to severe aortic regurgitation.
(AO: Aorta, RA: Right atrium). (Movie clip 74.44A and B).
Source: Reproduced with permission from Burri MV, Nanda NC,
Singh A, Panwar SR. Live/real time three-dimensional transtho-
racic echocardiographic identification of quadricuspid aortic valve.
A B
Figs 74.45A and B: Live/real time, three-dimensional transthoracic echocardiography. (A and B) Shows a quadricuspid aortic valve
with four numbered leaflets clearly visualized. (AO: Aorta, RA: Right atrium; TV: Tricuspid valve.). [Movie clip 74.45, (Parts 1 and 2)].
Source: Reproduced with permission from Burri MV, Nanda NC, Singh A, Panwar SR. Live/real time three-dimensional transthoracic
echocardiographic identification of quadricuspid aortic valve. Echocardiography. 2007;24:653–5.
aortic valve. This is unlike a 3DE data set that includes the Bandarupalli et al. the measurement of valve gradient and
whole valve which can then be studied at any time using orifice area by 2D TTE was indicative of severe obstructive
any desired plane and angulation. disease, but follow-up calculations by 3D TTE with the
subaortic membrane en face from the left ventricular
Subaortic Stenosis outflow tract were indicative of insignificant stenosis.60
Left heart catherization was performed to confirm the
Stenosis can occur not only at the level of the valve but echocardiographic findings, and in fact was consistent
above the valve (supravalvular) as well as below the valve with those obtained by 3D TTE (Fig. 74.48). 3D TTE is more
(subvalvular or subaortic). When subaortic stenosis is reliable because it evaluates the subaortic membrane
diagnosed it is important to distinguish the fixed from in its entirety and locates the defect in the membrane
the dynamic type. Fixed subaortic stenosis can be due to (Figs 74.49 to 74.51).59 The defect was viewed en face by
a focal, fibromuscular membrane or a diffuse tunnel-type rotation and cropping of data sets, a technology unique to
lesion. On the other hand, dynamic subaortic stenosis can 3DE. Furthermore, 3D TTE images can identify the “hole
develop as a result of systolic anterior motion of the mitral in a hole” which is diagnostic of subaortic stenosis.
valve, a common echocardiographic finding in patients
with hypertrophic cardiomyopathy.1 Interestingly, there is
a strong association between patients previously diagnosed
AORTIC ARCH ANOMALIES
with a VSD and subaortic stenosis which appears to be Coarctation of the Aorta
related to fibrous tissue formation from turbulent blood
flow at the prior surgical site.58 The diagnosis of coarctation of the aorta is often missed
With 3D TTE, the aortic valve, subaortic membrane, prenatally and immediately after birth manifesting as
and surrounding cardiac anatomy is clearly visualized shock, once closure of the ductus arteriosus occurs.61 Later
which is beneficial in measuring the severity of stenosis in life, patients with coarctation of the aorta present with
(Figs 74.46 and 74.47).1,59,60 2D TTE can be helpful significant differences in blood pressure readings between
in diagnosing subaortic stenosis based on Doppler the upper and lower extremities. If not repaired, patients
measurement of gradient across the membrane, however, are at risk of developing symptoms of congestive heart
these calculations are often inaccurate. In a study by failure, aortic rupture, and endocarditis.58 Percutaneous
A B
Figs 74.46A and B: Two-dimensional transthoracic echocardiogram in discrete subaortic membranous stenosis. (A) The arrowhead
points to the subaortic membrane; (B) Using color Doppler-guided continuous-wave Doppler, a peak gradient (PG) of 134 mm Hg
(arrow) was obtained across the left ventricular (LV) outflow tract. (AO: Aorta; LA: Left atrium; RV: Right ventricle; RVO: Right ventricular
outflow tract).
Source: Reproduced with permission from Agrawal GG, Nanda NC, Htay T, Dod HS. Live three-dimensional transthoracic echocardio-
graphic identification of discrete subaortic membranous stenosis. Echocardiography. 2003;20:617–9.
A B
Figs 74.47A to C: Live three-dimensional transthoracic echo-

cardiography in discrete subaortic membranous stenosis.
(A and B) The arrowhead points to a narrow opening in the
membrane imaged in short axis; (C) Membrane (arrowhead)
viewed from the top showing its attachment to the ventricular
septum (VS) and the anterior leaflet of the mitral valve. (AO: Aorta;
RA: Right atrium). The arrowhead in the Movie clip 74.47 points to
the obstructing membrane. (Movie clip 74.47).
Source: Reproduced with permission from Agrawal GG, Nanda
NC, Htay T, Dod HS. Live three-dimensional transthoracic echo-
cardiographic identification of discrete subaortic membranous
C stenosis. Echocardiography. 2003;20:617–9.
Fig. 74.48: Discrete subaortic membrane. Cardiac catheterization

pressure tracings showing no significant gradient across the LV
outflow tract.
Source: Reproduced with permission from Bandarupalli N,
Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis
of significant obstruction by Doppler in a patient with discrete
subaortic membrane: Correct diagnosis by 3D-transthoracic
echocardiography. Echocardiography. 2008;25:1004–6.
A B
Figs 74.49A and B: Discrete subaortic membrane. Two-dimensional transthoracic echocardiography. (A) Arrowhead points to the
membrane imaged in the parasternal long-axis view; (B) Shows peak and mean pressure gradients of 64 mm Hg and 31 mm Hg across
the left ventricular outflow tract by continuous-wave Doppler. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). (Movie
clip 74.49).
Source: Reproduced with permission from Bandarupalli N, Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of significant
obstruction by Doppler in a patient with discrete subaortic membrane: Correct diagnosis by 3D-transthoracic echocardiography. Echo-
cardiography. 2008;25:1004–6.
A B
Figs 74.50A and B: Discrete subaortic membrane. Live/real time, three-dimensional transthoracic echocardiography. (A and B) Sub-
aortic membrane (arrowhead) and orifice viewed en face. The orifice measured 2.29 cm2 by planimetry. (LA: Left atrium; PV: Pulmonic
valve; RA: Right atrium; RV: Right ventricle). (Movie clip 74.50).
Source: Reproduced with permission from Bandarupalli N, Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of significant
obstruction by Doppler in a patient with discrete subaortic membrane: Correct diagnosis by 3D-transthoracic echocardiography. Echo-
cardiography. 2008;25:1004–6.
balloon angioplasty with or without stent placement is approach. Evaluating a patient for coarctation of the aorta
becoming more popular as the treatment option for young includes 2D TTE with continuous-wave Doppler focusing
adult patients with coarctation of the aorta, while surgery on the identification of diminishing anterograde flow during
remains an option for those not candidates for percutaneous diastole to confirm hemodynamically significant stenosis.58
Fig. 74.51: Discrete subaortic membrane. Live/real time, three- Fig. 74.52: Real time, two-dimensional transesophageal echocar-
dimensional transthoracic echocardiography. Aortic valve orifice diogram in aortic arch vasum vasi to pulmonary artery fistula. The
viewed en face. It measured 2.94 cm2 by planimetry. (AO: Aorta; arrowhead points to flow signals moving from the wall of the aortic
LA: Left atrium; LV: Left ventricle; RV: Right ventricle). arch (AO) into the main PA. (Movie clip 74.52).
Source: Reproduced with permission from Bandarupalli N, Source: Reproduced with permission from Sadat K, Pradhan
Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of M, Nanda NC, Joshi D, Diddi HP. Two- and three-dimensional
significant obstruction by Doppler in a patient with discrete transthoracic echocardiography in the assessment of aortic
subaortic membrane: Correct diagnosis by 3D-transthoracic arch vasum vasi to pulmonary artery fistula. Echocardiography.
echocardiography. Echocardiography. 2008;25:1004–6. 2013;30:219–24.
2D TTE has traditionally been used to make the correct diagnosis. The diagnosis was confirmed based on
diagnosis of aortic arch abnormalities, but understanding calculations of flow acceleration within the aortic wall, the
its limitations allows for the use of more advanced pressure within the fistula itself, and the gradient between
technology.62 For example, postsurgical repair of coarcation the aorta and the pulmonary artery.64
of the aorta results in a tortuous aortic arch which makes
evaluation by 2D TTE difficult. 3D TTE can create an ATRIAL AND ATRIOVENTRICULAR
“echocardiographic angiogram” and rotate images in
various angles and planes providing the cardiologist with
VALVE ABNORMALITIES
a detailed understanding of the extravascular cardiac Cor Triatriatum Sinister
anatomy.63
Cor triatriatum sinister is a rare CHD characterized by
a fibromuscular membrane located in the left atrium
Aortic Arch to Pulmonary Artery Fistula
superior to the LA appendage.65 The location of the
Aortic lumen to pulmonary artery fistulas (APAF) is membrane distinguishes this entity from a mitral
extremely rare, and APAF forming from the vasum vasi supravalvular membrane which is located below the LA
in the posterior wall of the aortic arch is even more rare.64 appendage. The size of the defect in the membrane is
2D TEE demonstrated turbulent blood flow in the main indirectly proportional to the degree of obstruction of
pulmonary artery moving from a direction not consistent blood flow and subsequent hemodynamic significance.
with the lumen as the site of origin. Further evaluation 2D echocardiography has traditionally been the
with 3D imaging from various angles actually identified imaging modality of choice for the diagnosis of cor
the fistula beginning in the posterior wall of the aortic triatriatum sinister, however 3D imaging offers greater
arch likely from a vasum vasi (Figs 74.52 and 74.53). 3D precision in assessing the size and shape of the defect
TTE confidently assessed the fistula at its origin with its within the membrane.65 3D TTE visualizes the
en face view and aided 2D TTE in confidently making the membrane en face leaving the cardiologist with a better
A B
Figs 74.53A and B: Live/real time, three-dimensional transthoracic echocardiogram in aortic arch vasum vasi to pulmonary artery
fistula. (A) The arrowhead points to abnormal flow signals originating within the posterior wall of the aortic arch. A small area of flow
acceleration is also noted. Careful cropping revealed no connection of this abnormal flow signals with the aortic lumen; (B) En face view of
the fistula vena contracta. It is very small measuring 1.7 mm in diameter, area 3.5 mm2. (LA: Left atrium). (Movie clips 74.53A and B).
A B
Figs 74.54A and B: Live/real time, three-dimensional transthoracic echocardiography in cor triatriatum sinister. (A) Arrowhead points
to cor triatriatum membrane (M), which is located superior to left atrial appendage (LAA); (B) Arrowhead (arrow in movie) points to a
large opening in cor triatriatum membrane visualized en face. The dimensions were 3.06 × 1.03 cm and area 2.3 cm2. (LA: Left atrium;
LV: Left ventricle; LVO: Left ventricular outflow tract; RA: Right atrium; RV: Right ventricle). (Movie clip 74.54).
Source: Reproduced with permission from Patel V, Nanda NC, Arellano I, Yelamanchili P, Rajdev S, Baysan O. Cor triatriatum sinister:
Assessment by live/real time three-dimensional transthoracic echocardiography. Echocardiography. 2006;23:801–2.
understanding of the defect (Figs 74.54A and B). 3D TTE not comment on the clinical relevance of the defect. In the
can accurately diagnose cor triatriatum sinister based on late 1990s, Samal et al. described offline 3D reconstruction of
the echocardiographic characteristics described above 2D TTE images for enhanced visualization of cor triatriatum
and quantify the size of the defect which is valuable in sinister along with other atrial membrane defects.66 With the
understanding its clinical significance.65 Conversely, creation of 3D images, the exact size, shape, and location
2D TTE was able to make the diagnosis, but could not of the membrane defect was known to the cardiologist and
comment on the size and shape of the defect and thus could surgeon which was valuable in planning for surgical repair.
A B
Figs 74.55A to C: Live three-dimensional transthoracic echo-

cardiographic assessment of isolated cleft mitral valve. The
arrowhead points to the cleft in the anterior mitral valve leaflet
seen in the open (A and B) and closed (C) positions. (LV: Left
ventricle; PML: Posterior mitral leaflet; RV: Right ventricle). The
cleft is directed toward the left ventricular outflow tract unlike
the atrioventricular septal defect where the cleft points medially.
Movie clip 74.55, Parts 1 and 2 from another patient shows an
isolated cleft (arrow) in the anterior mitral leaflet in a 28-year-old
female. Color Doppler examination shows prominent regurgitation
(arrowhead) into the left atrium through the cleft. [Movie clip 74.55,
(Parts 1 and 2)].
Source: Reproduced with permission from Sinha A, Kasliwal
RR, Nanda NC, et al. Live three-dimensional transthoracic
echocardiographic assessment of isolated cleft mitral valve.
Isolated Mitral Valve Cleft Ebstein’s Anomaly

Isolated mitral valve cleft is a rare congenital defect that Ebstein’s anomaly is a rare congenital defect affecting
may cause mitral regurgitation. The direction the cleft less than 1% of patients with CHD that is characterized by
faces helps to distinguish an AVSD (toward the ventricular the attachment of tricuspid valve leaflets, often the septal
inlet septum) from isolated mitral valve cleft (toward the and posterior leaflets, to the walls of the right ventricle
left ventricular outflow tract), a feature easily performed and ventricular septum.69 The tethering of the septal
by 3D TTE.67,68 The diagnosis of isolated mitral valve leaflet to the ventricular septal wall causes an “apparent
cleft is often missed by 2D TTE making 3D TTE a more displacement” of these structures in the direction of the
enticing imaging modality. In patients with mitral valve right ventricular apex that is characteristically seen on
prolapse, the diagnosis of isolated mitral valve cleft by 2D TTE. Despite these findings, 2D TTE has difficulty
2D TTE is especially difficult because of the redundant identifying other important morphological features of
leaflets. Compared to 2D TTE, 3D TTE visualizes the Ebstein’s anomaly.
cleft in the anterior mitral valve leaflet from multiple Due to the limitations of the 2D TTE, 3D TTE can
viewpoints ensuring greater confidence in the diagnosis. be used in patients with Ebstein’s anomaly because it
In addition, it provides accurate dimensions of the defect can visualize each individual leaflet and the tethering
and the mitral rim and vena contracta measurements give to the right ventricular free wall or ventricular septum
reliable assessment of the severity of the resultant mitral (Fig. 74.57).70 Based on the comprehensive view of the
regurgitation (Figs 74.55 and 74.56).67 leaflets offered by 3D TTE, the size and location of the
A B
C D
Figs 74.56A to D: Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve. (A to C) The
arrowhead points to the cleft in the anterior mitral valve leaflet. Note thickened mitral leaflets with a narrow orifice indicative of associated
mitral stenosis; (D) Isolated cleft mitral valve in another child. This en-face transthoracic three-dimensional view shows the cleft (arrow)
dividing the superior and inferior components of the anterior mitral leaflet. Note the perception of the depth of the cleft with three-
dimensional imaging. (MV: Mitral valve; LV: Left ventricle; PML: Posterior mitral leaflet; RV: Right ventricle). (Movie clip 74.56D).
Source: Figures 74.56A to C reproduced with permission from Sinha A, Kasliwal RR, Nanda NC, et al. Live three-dimensional
transthoracic echocardiographic assessment of isolated cleft mitral valve. Echocardiography. 2004;21:657–61.
tethered and nontethered parts of the leaflets can be better OTHER ABNORMALITIES
evaluated, which is relevant when the patient is evaluated
for tricuspid valve repair. A surgeon’s understanding of the Chiari Network
amount of nontethered leaflet tissue is crucial in selecting
Chiari network is often clinically insignificant, but some
appropriate candidates for valve repair, a function that reports suggest increased risk of thrombus formation,
3DE has nearly mastered. The nontethered leaflets of the paradoxical embolization, or endocarditis.71 Chiari
tricuspid valve are known to extend outward creating a network is composed of a large fenestrated membrane
bubble-like appearance on 3D imaging that is diagnostic of in the right atrium with wide attachments at the crista
Ebstein’s anomaly (Figs 74.58 and 74.59).70 It is important terminalis and interatrial septum as a result of persistence
to recognize that not all tethered areas can be viewed of the right-sided sinus venosus valve. 3D TTE is valuable
en face by 3D TTE, one obvious limitation preventing a in distinguishing Chiari network from Eustachian and
complete understanding of the severity of this anomaly. Thebesian valves based on their smaller size and location,
A B
Figs 74.57A to C: Live/real time, three-dimensional transthoracic
echocardiography in Ebstein’s anomaly associated with trans-
position of the great vessels. (A) Four-chamber view shows apparent
displacement of the attachment of the septal leaflet of the tricuspid
valve (TV) toward the apex; (B) Tethering of the septal leaflet of the
TV results in a bubble-like appearance (yellow arrowhead) in the
middle portion of the ventricular septum as the nontethered portion
moves toward closure during systole. This transverse section
was taken at a level denoted by #1 in (A); (C) Transverse section
taken at a more inferior level (#2 in A) demonstrates bubble-like
appearance of both septal (yellow arrowhead) and posterior (black
arrowheads) TV leaflets produced by tethering. (a: Anterior TV
leaflet; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA:
Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Patel V, Nanda NC,
Rajdev S, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of Ebstein’s Anomaly. Echo-
A B
Figs 74.58A and B
C D
E F
G H
Figs 74.58C to H
Figs 74.58A to I: Live/real time, three-dimensional transthoracic

echocardiography in isolated Ebstein’s anomaly. (A) Transverse
section taken at the apex of TV shows a large area of noncoapta-
tion (N) as well as tethering and bubble-like appearance of ante-
rior (yellow arrows) and posterior (black arrowhead) TV leaflets;
(B to D) Transverse sections taken more basally demonstrate
multiple “bubbles” in the septal (yellow arrowheads) and posterior
(black arrowheads) TV leaflets. Inset in D shows all three leaf-
lets of the tricuspid valve in the open position; (E) Oblique sec-
tion shows multiple “bubbles” (black arrowheads) in the posterior
(p) TV leaflet produced by tethering to RV inferior wall. Inset in E
shows a long snake-like posterior (p) TV leaflet; (F) The oblique
section shown in E has been rotated to more optimally view
the attachment of posterior (p) TV leaflet to the RV inferior wall;
(G) The arrowhead in another patient with Ebstein’s anomaly
shows a bubble-like appearance resulting from tethering of the
septal leaflet (s) of the tricuspid valve to the ventricular septum; (H
I and I) The arrowhead points to a large defect in the anterior leaflet
of the tricuspid valve in a different patient with Ebstein’s anomaly.
Note also small, discrete nodular areas of thickening on the an-
and cor triatriatum dexter network based on its thickness terior tricuspid leaflet. Asterisks represent loss of tricuspid valve
with few or absent fenestrations (Figs 74.60 and 74.61). tissue which is considerable in this patient. The septal leaflet of the
tricuspid valve was tethered to the ventricular septum. (a: Anterior
Other clinical clues suggesting the diagnosis of cor tricuspid valve leaflet; AV: Aortic valve; LV: Left ventricle; RV: Right
triatriatum dexter are obstruction of blood flow and its ventricle; P: Posterior tricuspid valve leaflet). [Movie clips 74.58B,
frequent association with other congenital defects. 2D TTE 58D, 58D (inset), 58E (inset), and 58F, 58G, 58H].
Source: Figure 58A to F reproduced with permission from Patel
has diagnosed a defect as a Eustachian valve but follow-
V, Nanda NC, Rajdev S, et al. Live/real time three-dimensional
up imaging with 3D TTE confirmed the diagnosis of Chiari transthoracic echocardiographic assessment of Ebstein’s Anomaly.
network.71 Echocardiography. 2005;22:847–54.
Source: Figure 58G to I Reproduced with permission from Pothineni K,
et al. Live/real time three-dimensional transthoracic echocardiographic
DOUBLE OUTLET RIGHT VENTRICLE assessment of tricuspid valve pathology: incremental value over
the two-dimensional technique. Echocardiography. 2007;24:
Double outlet right ventricle is a congenital anomaly 541–52.
whereby the aorta and main pulmonary artery are
connected (entirely or partly) to the morphologic improves understanding of the defect and helps members
right ventricle. The presence of a VSD almost always of the medical team to plan for surgical repair.73 3D imaging
accompanies this diagnosis (Fig. 74.62). Some experts can predict if a repaired baffle between the left ventricle
consider double outlet right ventricle on a spectrum with and aorta will eventually cause tricuspid valve obstruction
other CHDs including ToF in the presence of a VSD and/or or right ventricular outflow obstruction. Furthermore,
pulmonary stenosis as well as TGA.72 The recommended 3D observation from the right ventricle elucidates the
surgical repair includes creation of a baffle that connects the proximity of the VSD to the aorta, pulmonary artery,
aorta to the left ventricle through the VSD. Other surgical and tricuspid valve preparing the surgeon for potential
options include arterial switch procedures. In planning for right ventricular outflow tract obstruction after a baffle
the proper repair procedure, a comprehensive evaluation procedure and the need for a right ventricle to pulmonary
of the location and size of the VSD and its proximity to the artery conduit.
semilunar valves as well as the relationship of the aorta
and main pulmonary artery to each other is essential.72,73
Surgeons have long relied on 2D echocardiography,
Left Ventricular-RA Communication
however, 3DE has become a feasible imaging option and Left ventricular-RA communication is often congenital
offers significant advantage in the visualization of cardiac in nature, however this defect can also be acquired
anatomy and in obtaining the necessary measurements secondary to endocarditis, mitral or aortic valve
related to double outlet right ventricle. Specifically, replacement, chest trauma, or myocardial infarction.74
3D imaging can crop images to better visualize the Hansalia et al. described a patient with acquired left
atrioventricular valves, the VSD, and the great vessels in ventricular-RA communication related to a previous
a single echocardiographic window. This information aortic valve replacement that was erroneously diagnosed
A B
C D
Figs 74.59A to E: Isolated Ebstein’s anomaly. (A to C) Shows

tethering of all three TV leaflets. The resulting “bubbles” are
denoted by arrowheads for the septal (yellow) and posterior (black)
TV leaflets and an arrow for the anterior (a) TV leaflet; (D) A three-
dimensional view from a child showing the large atrialized portion of
the right ventricle (RV); (E) Short-axis three-dimensional view from
the same child shows a large region of noncoaptation (arrow) of
the tricuspid valve when viewed en-face from RV apex. (A: Anterior
tricuspid valve leaflet; LA: Left atrium; LV: Left ventricle; RA: Right
atrium; S: Septal leaflet of tricuspid valve. AV: Aortic valve; LV: Left
ventricle; RV: Right ventricle; P: Posterior tricuspid valve leaflet).
(Movie clips 74.59D and E).
Source: Reproduced with permission from Patel V, Nanda NC,
Rajdev S, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of Ebstein’s Anomaly. Echo-
E cardiography. 2005;22:847–54.
Fig. 74.60: Real time, two-dimensional transesophageal echo-

cardiography. Arrowhead points to a linear structure in the right
atrium (RA) consistent with a Eustachian valve. Movie clip 74.60,
Part 2, arrow shows the tumor. [Movie clip 74.60, (Parts 1 and 2)].
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Singh A, Panwar SR, Gandhari S. Live/real time
three-dimensional transthoracic echocardiographic visualization of
Chiari Network. Echocardiography. 2007;24:995–7.
A B
Figs 74.61A to C: Live/real time, three-dimensional transthoracic

echocardiography. (A and B) Chiari network. Small arrowheads in
A point to some of the multiple openings in the large membrane,
outlined by red dots. Attachment of the Chiari membrane
(arrowhead) to the interatrial septum (*) is shown in (B). Movie
clip 74.61A arrowhead points to the Chiari membrane; (C) Renal
cell carcinoma. Arrow points to the tumor in the inferior vena cava
(IVC) at the right atrial (RA) junction. (L: Liver; LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; TV: Tricuspid valve). (Movie clips
74.61A and B).
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Singh A, Panwar SR, Gandhari S. Live/real time
three-dimensional transthoracic echocardiographic visualization of
C Chiari Network. Echocardiography. 2007;24:995–7.
as pulmonary hypertension by 2D TTE.74 The diagnosis artery trauma. While coronary angiography has been
was likely missed by 2D TTE because of the limited the recommended imaging modality in the diagnosis
views it offered, preventing complete visualization of the of coronary artery fistula, it is limited in its ability to
defect. The diagnosis in this case was also complicated by delineate intricate coronary anatomy and its proximity
simultaneous severe tricuspid regurgitation. Follow-up to other relevant structures. 2D TEE has been shown to
imaging with 3D and its volumetric data demonstrated the demonstrate a fistula between the right atrium and right
defect and shunting of blood from the left ventricle to the coronary artery with the use of color Doppler, however, it
has difficulty visualizing the fistula tract in its entirety and
right atrium.
therefore, often miscalculates its dimensions. On the other
hand, 3D TEE is capable of obtaining an en face view of
Right Coronary Artery Fistula the right coronary artery to right atrium fistula and has
been able to track the fistula along its course (Fig. 74.63).
A coronary artery fistula is a rare congenital abnormality Importantly, 3D TEE was able to clearly identify the origin
that empties blood into one of the cardiac chambers or and termination sites of the fistula and therefore make
into blood vessels through one or multiple fenestrations.75 accurate measurements, which can be used to plan for
These fistulas can also occur secondary to coronary treatment.75
Fig. 74.62: Double outlet right ventricle (DORV). This subcostal

three-dimensional 3 D acquisition in a child shows the relationship
of the aorta (AO, right) and pulmonary artery (PA, left). Both arise
from the right ventricle (RV) and the ventricular septal defect
(arrow) can be extended to the aorta. (LV: Left ventricle). (Movie
clip 74.62).
A B
Figs 74.63A and B
C D
E F
Figs 74.63A to F: Live/real time, three-dimensional transesophageal echocardiography of right coronary artery to right atrium fistula.
(A) Qlab study. Top arrows and the bottom right arrow point to the origin of the dilated RCA. The RCA opening viewed en face (arrow
in bottom left panel) measured 1.70 cm × 1.55 cm, area 1.96 cm2; (B) Vertical arrowhead shows the continuity of the descending and
the ascending (transverse arrowhead) limbs of the fistula. Arrow shows origin of the dilated RCA; (C) Qlab cropping also shows the
continuity (lower arrowhead) of the descending and ascending (upper right arrowhead) limbs of the fistula. Arrow in upper left and lower
right panels show the origin of dilated RCA; (D) The arrow shows flow signals moving from the fistula (arrowheads) into RA; (E and F)
Represent regular (E) and Qlab (F) en face views (arrow) of the opening of fistula into RA. This measured 1.83 cm × 1.43 cm, area 3.94
cm2. Arrowheads show segments of the fistula. (AO: Aorta; AV: Aortic valve; RA: Right atrium). (Movie clips 74.63B to 74.63F).
Source: Reproduced with permission from Mishra J, Puri HP, Hsiung MC, et al. Incremental value of live/real time three-dimensional
over two-dimensional transesophageal echocardiography in the evaluation of right coronary artery fistula. Echocardiography. 2011;28:
805–8.
SINUS OF VALSALVA ANEURYSM obstruction referred for surgical intervention.77 Using 3D

reconstruction capabilities, the ventricular septal aneurysm,
A sinus of Valsalva aneurysm is an unusual cardiac the right ventricular outflow tract, and the pulmonary valve
anomaly that can be congenital or acquired and often could be seen en face (Figs 74.66 and 74.67). With the short-
involves the right coronary sinus.76 Patients with sinus of axis view, the aneurysm could be visualized extending almost
Valsalva aneurysm are frequently asymptomatic, therefore, throughout the entire right ventricular outflow tract near
the diagnosis is either made incidentally on imaging or if the level of the pulmonary valve during systole consistent
the aneurysm ruptures. 2D TEE has value in identifying with severe obstruction, a feature 2D TEE was unable to
a sinus of Valsalva aneurysm, but has limitations in the perform. 2D TEE located the VSD, but calculations made
diagnosis of a rupture. Color Doppler can be helpful in by continuous-wave Doppler were indicative of less severe
this situation, however, in the setting of hypovolemic outflow obstruction likely related to the intricacy of lining
shock after a rupture, the findings are often limited. 3D up the Doppler wave in the appropriate direction along
TEE incorporates multiple geometric planes and various the right ventricular outflow tract.
angulations with cropping of images to localize a sinus of
Valsalva aneurysm and its exact site of rupture which may Hypoplastic Left Heart Syndrome
not be evident on 2D TEE (Figs 74.64 and 74.65). Due to
the severity of a ruptured aneurysm, urgent identification In this entity the left sided structures including the left
and intervention must take place providing 3D TTE with ventricle, the left atrium, mitral valve, aortic valve, and the
diagnostic utility. aorta are small and under developed to a varying degree. The
right heart is very prominent (Fig. 74.68). These newborns
need urgent surgery to maintain the systemic circulation.
Ventricular Septal Aneurysm Causing
Right Ventricular Outflow Obstruction CONCLUSION
Aneurysm of the ventricular septum can result in right The use of 3DE alone or along with 2D echocardiography
ventricular outflow tract obstruction and represents helps to increase the confidence in the diagnosis of
an unusual congenital cardiovascular abnormality. CHD.78,79 As technology continues to improve, 3DE has
Baweja et al. described a patient with a large VSD and the potential to expand and impact the management and
aneurysm with suspected right ventricular outflow tract treatment of this growing patient population.
A B
Figs 74.64A and B
C D
Figs 74.64A to D: Live/real time, three- dimensional transesophageal echocardiography in ruptured right sinus of Valsalva aneurysm.
(A and B) Arrow points to site of rupture of the aneurysm (AN); (C and D) Arrow points to the mouth of the aneurysm viewed en face.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). (Movie clips 74.64A to 74.64D).
Source: Reproduced with permission from Raslan S, Nanda NC, Lloyd L, Khairnar P, Reilly SD, Homan WL. Incremental value of live/
real time three-dimensional transesophageal echocardiography over the two-dimensional technique in the assessment of sinus of
Valsalva aneurysm rupture. Echocardiography. 2011;28:1041–5.
Fig. 74.65: Autopsy in ruptured right sinus of Valsalva aneurysm.

Arrow points to the rupture. (AO: Aorta, LV: Left ventricle).
Source: Reproduced with permission from Raslan S, Nanda NC,
Lloyd L, Khairnar P, Reilly SD, Homan WL. Incremental value of live/
real time three-dimensional transesophageal echocardiography
over the two-dimensional technique in the assessment of sinus of
valsalva aneurysm rupture. Echocardiography. 2011;28:1041–5.
A B
Figs 74.66A and B: Transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow
obstruction in an adult. (A) The arrow points to the ventricular septal aneurysm bulging into right ventricular outflow tract. The ventricular
septal defect is delineated by the arrowhead; (B) Color Doppler examination shows a narrow turbulent jet (black arrowheads) indicative
of significant obstruction produced by the aneurysm (arrow). (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery;
RA: Right atrium; TV: Tricuspid valve).
Source: Reproduced with permission from Baweja G, Nanda NC, Nekkanti R, Dod H, Ravi B, Fadel A. Three-dimensional transesopha-
geal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow obstruction in an adult. Echocar-
diography. 2004;21:95–7.
A B
Figs 74.67A and B
C D
Figs 74.67A to D: Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing
ventricular outflow obstruction in the same patient as in previous figure. (A) Short-axis (en-face) view just below the level of the
pulmonary valve (PV) showing the aneurysm (arrow) practically occupying the entire right ventricular outflow tract (RVO) indicative of
severe obstruction; (B) Long-axis view also shows the aneurysm (arrow) bulging into the RVO; (C and D) Short-axis view of the PV
(black arrow); (C) Normal opening of the PV during systole indicative of absence of any obstruction at this level; (D) The PV in closed
position in diastole. VS, ventricular septum. (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium;
TV: tricuspid valve).
Fig. 74.68: Right ventricle in hypoplastic left heart syndrome. This

is a subcostal acquisition of the entire right ventricle (RV) in an
infant with hypoplastic left heart syndrome (HLHS). (PA: Pulmonary
artery). (Movie clip 74.68).
ACKNOWLEDGMENT 3. Shirali GS. Three-dimensional echocardiography in con-

genital heart disease. Echocardiography. 2012;29(2): 242–8.
We thank Dr Maximiliano German Amado Escañuela for 4. De Castro S, Caselli S, Papetti F, et al. Feasibility and
his help. clinical impact of live three-dimensional echocardiography
in the management of congenital heart disease. Echocar-
diography. 2006;23(7):553–61.
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CHAPTER 75
Echocardiography in the Evaluation of
Adults with Congenital Heart Disease
Reema Chugh
Snapshot
Key Concepts of Echocardiography in Adults with 
Valvular Disease
Congenital Heart Disease 
Complex Congenital Heart Defects
Simple Congenital Heart Defects in Adults
Most of the fundamental ideas of science are essentially simple, and may,
as a rule, be expressed in a language comprehensible to everyone.
Albert Einstein
INTRODUCTION and function. Adults with devices that are not MRI
compatible may undergo CTA at the expense of exposure
Advancements in pediatric, medical care, surgical, and to radiation.
interventional techniques have improved survival into This chapter is specifically written for an adult
adulthood for over 85% of those born with congenital cardiologist and may appear very basic to a pediatric
heart defects (CHDs) in developed countries. Depending cardiologist. However, the knowledge of residua and
upon the type of defect, presenting symptoms, and the sequelae resulting from long-term survivorship of our
availability of medical resources, many are diagnosed in patients into adulthood, who are trending into geriatric
infancy and childhood, while in others, the diagnosis is not years may be beneficial to all, since both pediatric and
made until adulthood. Transthoracic echocardiography is adult cardiologists are vested in favorable outcomes
the primary diagnostic imaging test, while other modalities for all our patients. While two-dimensional (2D) echo-
such as cardiac catheterization, magnetic resonance cardiogram with Doppler and color flow imaging are an
imaging (MRI), or computed tomographic angiography essential part of routine diagnostic assessment, three-
(CTA) are used as confirmatory or complementary tests dimensional (3D) echocardiography adds more details.
for complete assessment and evaluation of extracardiac Transesophageal echocardiography (TEE) aids in viewing
structures in adults with congenital heart disease (ACHD). posterior structures, interatrial septum, pulmonary
While coronary angiography remains the gold standard for veins, pulmonary artery, and the aorta. Intraoperative
assessment of coronary artery disease, MRI is the reference TEE helps in directing and confirming the results of the
standard for quantification of the right ventricular volume surgery. A concise and adapted version of the American
Society of Echocardiography guidelines for performance interventional procedures, or surgeries in a timely

of transthoracic and transesophageal echocardiography manner. The American College of Cardiology (ACC)/
applicable to ACHD are listed in Tables 75.1 and 75.2, American Heart Association (AHA) guidelines for the
respectively.1,2 Exercise stress echocardiography assesses management of adults with congenital heart disease
functional capacity, systemic ventricular contractile
reserve, the impact of exercise on pulmonary hypertension, Table 75.2: Indications for Performing Transesophageal Echo-
cardiography (TEE) for Adults with Congenital Heart Diseases
severity of valve diseases, and arrhythmias.
(ACHD)
All adults with CHD need routine lifelong follow-
Diagnostic indications
up for early identification of problems. Many will need
reoperations and interventions due to residual shunts, • Identification of defects in adults with poor acoustic trans-
thoracic windows
prosthetic valve dysfunction, conduit stenosis, baffle
• Identification of defects that have limited visibility on TTE
leaks, or may require heart transplantation. Common
(such as a sinus venosus defect, fenestrated ASD, origin of
clinical issues faced by these individuals are impaired left the coronaries)
ventricular function resulting in heart failure, pulmonary
• Rule out cardioembolic source in an adult with stroke,
hypertension, progression of valvular disease (regurgi- including assessment of possible right-to-left shunt due to
tation and stenosis), bradyarrhythmias/tachyarrhythmias a PFO with an agitated saline contrast study; examination
(atrial and ventricular), endocarditis, aortic/pulmonary of the left atrial appendage for a thrombus, atherosclerotic
artery dilatation/aneurysms, shunt problems, and conduit plaques in the proximal aorta
failures. It is essential to make every effort to acquire prior • Examination of intra- or extracardiac baffles following the
medical records, especially the operative notes and results Fontan, Senning, or Mustard procedure
of previous imaging studies in order to understand each • Evaluation of vegetation, perforation, endarteritis, or sus-
pected abscess
individual’s unique anatomy and gauge follow-up.
In addition, women with CHD in their reproductive • Ruling out an intracardiac thrombus prior to cardioversion
for atrial flutter/fibrillation
years need echocardiography to aid in preconception
counseling, assessment of underlying defects to determine • Status of prosthetic valves
the maternal–fetal risk, guide follow-up during pregnancy, Guiding catheter-based interventions
labor, delivery, and postpartum. • Directing placement of ASD or VSD occlusion devices—
An echocardiographer also holds the responsibility of accurate assessment of size, shape, location of the defect,
and identifying associated defects
identifying lesions that may need medical management,
• Delineation of structures not clearly visible on transthoracic
echocardiography (such as pulmonary veins)
Table 75.1: Indications for Performing Transthoracic Echo-
cardiography (TTE) for Adults with Congenital Heart Diseases • Catheter tip placement for dilation and stent implantantion
(ACHD) for conduit/baffle stenoses in catheterization laboratory
Initial evaluation in an adult suspected of having CHD • Guidance during radiofrequency ablation procedures
Follow-up of ACHD when there is a change in clinical status Perioperative indications
or cardiac examination • Immediate preoperative definition of cardiac anatomy and
Re-evaluation to guide therapy in adults diagnosed with CHD function
Routine surveillance (<1 year) of adults with CHD following • Assessment of postoperative myocardial function
incomplete or palliative repair with residual structural or • Rule out postoperative surgical results, residual shunts, or
hemodynamic abnormality but without a change in clinical valvular regurgitation
status or cardiac examination • Assessment of the results of a minimally invasive surgical
Routine surveillance (<2 years) of adults with stable CHD incision or a video-assisted cardiac procedure
following complete repair, without a change in clinical status/ • Evaluation and monitoring of a postoperative patient with
examination, hemodynamic abnormality, or known residual an open sternum or poor acoustic windows
structural defects.
Source: Adapted from American Society of Echocardiography
Source: Adapted from 2011 American Society of Echocardiogra- 2005 guidelines for ASE Indications and Guidelines for Perfor-
phy guidelines for appropriate use criteria for echocardiography.1 mance of Transesophageal Echocardiography in the Patient with
(Free download of the detailed document is available at: Pediatric Acquired or Congenital Heart Disease2 (Free download
http://www.asecho.org/files/AUCEcho.pdf ) available: http://www.asefiles.org/pediatrictee.pdf)
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease 1793
determined Class I recommendations, for which there and management of structural heart diseases. They are also
are sufficient data to clearly support the benefits of not entrenched in the numerous classification systems
interventions/surgeries over the potential risks.3 On the or detailed embryological understanding of congenital
other hand, with the Class III recommendations, the risks heart defects. Often the terminology, vocabulary words,
outweigh the benefits and the treatment or procedure and terms are unfamiliar and baffling. This can make
should not be performed because it may even be harmful. echocardiography in ACHD for the adult cardiologist quite
The Class IIa and IIb recommendations lie in a “gray overwhelming and intimidating. Table 75.3 lists some of
zone” where limited benefits outweigh the risks, making the commonly used terms and Table 75.4 lists some names
the treatment/procedures reasonable on an individual of the common surgeries for CHD.
basis. In this chapter, the Class I and III recommendations
There is a shift from a wide spectrum of defects
identifiable by echocardiography are discussed, allowing
ranging from the “undiagnosed simple to complex” in
the echocardiographer to play an active role in appropriate
children versus “simple undiagnosed defects to previously
management of this population.
diagnosed/operated defects” with residua and sequelae
in adults. Traditionally, most centers perform adult
KEY CONCEPTS OF ECHOCARDIO- echocardiograms with the orientation of the images
GRAPHY IN ADULTS WITH “upside down” unlike the images seen in conventional
CONGENITAL HEART DISEASE pediatric echocardiography. A basic, practical, and yet
systematic approach is, therefore, necessary to allow an
A Simplified Segmental Approach adult cardiologist to assess this population with clarity,
Unlike echocardiography in infants and children, the accuracy, and confidence. As pointed out by Dr John
echocardiographic windows are far more limited in adults, Child, guru of echocardiography in adults with CHD,
primarily due to body habitus and rib spacing. In addition, “Always perform a complete examination!” This step-by-
adult cardiologists have a different approach to diagnosis step approach entails the following.
Table 75.3: Common Terminology in Adult Congenital Heart Disease (ACHD)

Atrioventricular (AV) concordance—right atrium empties into the right ventricle, left atrium empties into the left ventricle
Atrioventricular (AV) discordance—right atrium empties into the left ventricle, left atrium empties into the right ventricle
Dextrocardia—apex of the heart points to the right (old term dextroversion)—failure of pivoting of the cardiac apex to the left
Levocardia—apex of the heart points to the left
Mesocardia—apex of the heart points to the middle
Dextroposition—displacement of the heart in the right chest due to a space occupying structure
Eisenmenger physiology—development of irreversible pulmonary hypertension with equalization of pulmonary and systemic
pressures, with pulmonary vascular resistance exceeding systemic vascular resistance leading to flow reversal (right to left) through
an intracardiac shunt
Right isomerism—bilateral right handedness (two morphological right atria), asplenia, both the aorta (anterior) and inferior vena
cava (posterior) are on the same side of the spine
Left isomerism—bilateral left handedness (two morphological left atria), polysplenia, both the aorta (posterior) and inferior vena
cava (anterior) are on the same side of the spine
Situs inversus totalis—heart is located in the mirror-image position of normal
Ventriculoarterial concordance—the morphological right ventricle pumps into the pulmonary artery, while the morphological left
ventricle pumps into the aorta
Ventriculoarterial discordance—the morphological right ventricle pumps into the aorta, while the morphological left ventricle
pumps into the pulmonary artery (transposition of the great arteries)
Restrictive VSD—usually small in size with a left-to-right shunt without causing significant hemodynamic derangement
Nonrestrictive VSD—large VSD at risk for reversal of the shunt (right-to-left). Hemodynamically, the pulmonary arterial and aortic
pressures are equal so the magnitude and direction of the shunt is determined by the pulmonary vascular resistance (PVR).
Table 75.4: Common Surgical Terms in Adult Congenital Heart Disease (ACHD)
Classic Blalock–Taussig–Thomas shunt—subclavian to pulmonary artery anastomosis to increase pulmonary blood flow
Modified Blalock–Taussig–Thomas shunt—subclavian to pulmonary artery anastomosis, using a Gore-tex graft, to increase pulmo-
nary blood flow
Potts shunt—descending aorta to left pulmonary artery anastomosis to increase pulmonary blood flow
Glenn shunt—superior vena cava to right pulmonary artery anastomosis to provide low pressure pulmonary blood flow
Atrial switch repairs—in both types of atrial switch repairs, the deoxygenated blood from the vena cavae goes through a baffle into
the left ventricle, which pumps the blood into the pulmonary artery. The oxygenated blood returning from the lungs passes through
the pulmonary veins via another baffle into the right ventricle that pumps the blood through the aorta into the systemic circulation.
Thus, the morphological right ventricle becomes a systemic ventricle and the morphological left ventricle, a subpulmonic ventricle.
• Senning atrial switch repair for DTGA—baffles are created from the patient’s tissues (right atrial wall and part of the atrial septum)
• Mustard atrial switch repair for DTGA—baffles are created from the pericardium and synthetic material
Waterston shunt—ascending aorta to right pulmonary artery anastomosis to increase pulmonary blood flow
Rastelli procedure—right ventricle-to-pulmonary artery conduit to allow blood flow from the right ventricle to reach the branch
pulmonary arteries, usually in patients with right ventricular outflow tract obstruction
Rashkind procedure—catheter-based procedure for balloon atrial septostomy to allow intermixing of the deoxygenated right atrial
blood with the oxygenated left atrial blood in DTGA
Fontan (classic)—right atrial to pulmonary artery anastomosis in tricuspid atresia
Jatene—arterial switch for DTGA (anatomical correction with reimplantation of the coronaries)
Biventricular repair—operation to establish two functioning ventricles without the mixing of the venous and systemic circulations,
by closing the shunt defects and creating a connection between the right ventricle and the pulmonary circulation. Usually per-
formed for tetralogy of Fallot, pulmonary atresia with VSD, transposition of the great arteries, and double-outlet ventricles.
Position of the Apex

The position of the apex can be determined from the
standard subcostal views. In dextrocardia, the apex points
to the right, while in levocardia it points to the left. The
middle position is described as mesocardia. Congenitally
corrected transposition of the great arteries (CCTGAs)
should be considered in the presence of dextrocardia,
especially when the gastric bubble is on the left and the
cardiac apex is on the right.
Determination of Atrial Situs

In atrial situs solitus, the morphological right atrium is
located on the right side and the morphological left atrium
is located on the left side. The most specific identifying Fig. 75.1: Transthoracic echocardiogram (apical four-chamber
feature of the right atrium is the eustachian valve (Fig. view) showing an echodensity in the right atrium due to the rema-
nent of a eustachian valve. (EV: Eustachian valve; LA: Left atrium;
75.1). In the subcostal views or the apical four-chamber LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
views (with posteriorly tilted probe), it can often be traced
from the orifice of the inferior vena cava, across the floor, it can drain into either atria or into both as in the case of
to the lower portion of the atrial septum. The right atrium persistent left superior vena cava. The left atrium usually
can usually be identified by the drainage of the inferior has pulmonary veins draining into it, other than in the
vena cava except in situs ambiguous (isomerism). The case of an anomalous pulmonary venous drainage. In situs
superior vena caval drainage is not as predictable, since inversus, this relationship is reversed.
Determination of Atrioventricular
Relationship (the Location of Ventricles by
Bulboventricular Loop)
In the D-loop, the right ventricle is on the right while the
left ventricle is on the left. In the L-loop, there is ventricular
inversion with the morphological right ventricle on the
left and the morphological left ventricle on the right side.
Since the atrioventricular (AV) valves always follow their
respective ventricles, the tricuspid valve is attached to
the morphological right ventricle and the mitral valve is
attached to the morphological left ventricle. Accordingly,
the positions of the tricuspid and mitral valves are also
transposed in the L-loop. Other identifying features of the
AV valves are their positions and morphology. In the apical Fig. 75.2: Diagrammatic representation of the aortic arch (AoA)
in the suprasternal view, displaying the branches. (BC: Brachio-
four-chamber view, the position of the trileaflet tricuspid cephalic; C: Carotid; SC: Subclavian).
valve is closer to the apex as compared with the mitral
valve. In the parasternal short-axis view, the mitral valve is
shaped like a “fish mouth” and has chordal attachments to parasternal short-axis view. The aortic and pulmonary
the two papillary muscles. valves remain loyal to their respective great arteries.
The crescent-shaped right ventricle is heavily
trabeculated as compared with the ellipsoid left ventricle.
The left ventricle usually has a smooth endocardial border
Abdominal Situs
except in adults with left ventricular noncompaction or Abdominal situs is usually concordant with atrial situs.
in those with long-standing hypertension. In the right Hence, it is often used to determine the atrial situs,
ventricle, the presence of a moderator band (a prominent although the two follow each other around 70% of the
muscle bundle crossing from the septum to the free wall), time. In atrial situs solitus, this relationship can be revealed
and septal attachment of the tricuspid valve positioned in the subcostal views by tracing the path of the inferior
more apically that the mitral valve (since the AV valves are vena cava, which is nonpulsatile (blue on color Doppler)
always attached to their respective ventricles) provides and located on the right of the spine as it passes through
further confirmatory evidence. the liver. The descending thoracic aorta is pulsatile (red on
color Doppler) and courses on the left of the spine. Gastric
Ventriculoarterial (VA) Connections folds and stomach bubble are also seen on the left side.
However, in atrial situs inversus, all these positions are
In a normal/concordant connection, the right ventricle reversed with aorta to the right of the spine and the inferior
pumps into the pulmonary artery while the left ventricle vena cava to the left of the spine. The subcostal view also
pumps into the aorta, with the position of the aorta allows determination of direction along which the major
being posterior and to the right of the pulmonary valve. axis of the heart is aligned.
In transposition of the great arteries/discordant VA
connection, the morphological right ventricle pumps into
the aorta while the morphological left ventricle pumps into
Assessment of the Shunts
the pulmonary artery. The pulmonary artery is identified Adults with CHD may have congenital shunt defects or
by its right and left branches in the high parasternal short- surgically created shunts for palliative repairs. In addition,
axis view. The aorta is identified by its “candy cane” or patch leaks or suture dehiscence cause iatrogenic shunt
“hockey-stick” appearance in the suprasternal views. The lesions. While color Doppler is the initial method of
cranial branches (brachiocephalic, common carotid, and choice to detect a shunt, pulsed wave and continuous
subclavian) may also be delineated in this view (Fig. 75.2). wave Doppler allow evaluation of the gradient across the
The origin of the coronary arteries may be seen in the high shunt.
An agitated saline contrast study, popularly referred to combined effect results in a 30% to 50% increase in cardiac
as the “bubble study,” is useful for the right heart lesions. output. The pulmonary as well as the systemic vascular
On the other hand, contrast echocardiography using a resistances fall, leading to a mild drop in both the systolic
defined agent that lasts long enough to make its way to the and diastolic blood pressures.7
left heart allows further delineation of both right and left An echocardiogram during pregnancy also reflects
defects. An agitated saline contrast study can be performed these changes.8,9 During the second trimester, there is
by promptly injecting 10 mL of agitated saline contrast (8 increase in sizes of the left and right ventricles and the
mL of saline mixed with 1 mL of blood and 1 mL of air) left atrium. There is expansion of the left ventricular (LV)
through a peripheral intravenous catheter. Its accuracy in volume in systole and diastole. There is hypertrophy of
detecting a right-to-left shunt increases significantly by the left ventricular walls. By the third trimester, the heart
performing an adequate Valsalva maneuver. The images appears more globular. Biventricular reductions in global
are recorded for 5 to 12 beats to assess for an intracardiac and segmental longitudinal deformations have been noted
shunt (with prompt appearance of microbubbles) versus as compensatory changes to accommodate the increase in
a pulmonary arteriopulmonary shunt (with delayed cardiac dimensions during pregnancy. Ejection fraction
appearance of microbubbles).4,5 It should be avoided in does not change in women with anatomically normal
patients with a large intracardiac shunt because of the risk hearts. However, in women with CHD, the systemic
of cerebral microemboli. and pulmonic ventricular size and function should be
Contrast echocardiography using the perflutren followed up during pregnancy and in postpartum,
lipid microsphere injectable suspension should not be since a change is likely to occur in those with marginal
used in adults with suspected right-to-left, bidirectional, contractile reserves. In addition, evaluation of residual
or transient right-to-left cardiac shunts. Based on the or recurrent lesions, severity of pulmonary hypertension,
manufacturers approved protocol, it may be used in those and measurement of aortic root size should be performed.
without shunts to opacify the left ventricular chamber While stress echocardiography using treadmill
for better visualization of endocardial borders when exercise testing should be avoided during pregnancy,
echocardiograms are suboptimal. It has also been useful this test is very valuable before conception for assessing
in defining intracardiac masses, diagnosing left ventricular the baseline functional capacity, exercise duration,
noncompaction, left ventricular aneurysm/rupture, and and workload metabolic equivalents of task (METs).
aortic pathologies including dissection.6 Attention should be paid to exercise-induced symptoms,
In most adults with major shunt defects, like the atrial/ arrhythmias, or ischemia, since these are likely to recur
ventricular septal defects or patent ductus arteriosus, with the hemodynamic stress of pregnancy. Imaging
chamber enlargement serves as a valuable surrogate provides information about the contractile reserve with
for gauging the impact of a long-standing shunt on the response of systemic and pulmonic ventricle to exercise.
receiving chambers. The right heart is enlarged in atrial Changes in pre- and postexercise gradients across stenotic
septal defects, while the left heart is enlarged in ventricular valves, left or right ventricular outflow tract gradients, and
septal defects and patent ductus arteriosus due to increased estimated right ventricular systolic pressures (RVSP) are
pulmonary blood flow directed eventually to the left heart. also reflective of the potential changes during pregnancy,
A shunt volume calculation based on information derived labor, and delivery.
from a TTE and TEE plays a limited role for more accurate When strongly indicated, a submaximal treadmill
assessment of pulmonary to systemic flow (Qp/Qs) in stress test without exceeding 70% of the maximum age-
some adults. predicted heart rate on Bruce protocol may be performed
during pregnancy. Unless it is absolutely necessary, any
exposure to radiation should be avoided during pregnancy.
Echocardiography in Pregnancy The risk of radiation exposure to the fetus should be
The hemodynamic and physiological effects of pregnancy discussed with the mother. If possible, the procedures
in women with CHD pose an extra workload on the should be postponed, until late second or third trimesters
cardiovascular system. As the pregnancy progresses into of pregnancy. Abdominal shielding for protection of the
the second and third trimesters, there is a 30% to 40% fetus is always necessary.
increase in blood volume causing an 18% to 25% increase These women need individualized preconception
in stroke volume. The heart rate rises by 20% and the counseling. Maternal and fetal outcomes depend
upon the severity of underlying defects and maternal In our practice, we make all measurements after
functional status prior to pregnancy. Echocardiography freezing the most suitable systolic frame that allows:
allows preconception assessment and follow-up during (a) proper identification of the hinging points of the
pregnancy. Other complimentary imaging modalities may right and noncoronary cusps of the aortic valve; and (b)
need to be performed in select cases for identification and measurements recorded at reproducible anatomical
correction of hemodynamically significant underlying landmarks perpendicular to the axis of blood flow. These
defects before pregnancy. Women with severe pulmonary measurements are taken at the level of the aortic annulus,
hypertension, ascending aorta diameter > 4.5 cm, or midsinuses, sinotubular junction, and about 2 cm distal to
lesions that cannot be repaired before pregnancy should the sinotubular junction as shown in a parasternal long-
be counseled to avoid pregnancy due to high maternal axis view.14 Linear measurements are then recorded from
morbidity and mortality.10 inner edge to inner edge (Fig. 75.3). It should also be noted
that in radiographic imaging, aortic root measurements
Aortic Root in Adults with Congenital are made from outer edge to outer edge.
Heart Disease Aortic aneurysms have been known to cause dissection
in association with some of these defects. Operative repair
Structural abnormalities of the great arterial walls and
additive hemodynamic stress lead to dilatation of the is indicated in symptomatic patients with aortic diameter
aorta and the pulmonary arteries in many congenital over 4.4 to 5 cm and/or growth >0.5 cm/year, for ascending
heart defects.11 Progressive dilatation has been noted with aortic aneurysms associated with bicuspid aortic valve or
bicuspid aortic valve, coarctation of aorta, large ventricular other genetically mediated disorders that are at high risk
septal defect, tetralogy of Fallot/pulmonary atresia for dissection, according to the clinical practice guidelines
with ventricular septal defect, and in truncus arteriosus11 for thoracic aortic disease.15 The aortic root should be
(Table 75.5). assessed annually if the diameter ranges from 3.5 to 4.4 cm
Echocardiographic assessment of the aortic root and semiannually if it is 4.5 to 5.5 cm. A debate about more
dimension should be routinely performed. Roman et al specific guidelines for a variety of CHDs continues.
described a method of measuring the aortic root at end
diastole by the leading-edge convention in the parasternal
or occasionally, apical long-axis view that displayed the
maximum diameter parallel to the aortic annular plane.
The aortic annular diameter was measured between the
hinging points of the right and noncoronary cusps of the
aortic valve in systole.12 Normal limits in relation to age,
body size, and gender of 2D echocardiographic aortic root
dimensions have been described in patients aged 15 years
or older by Devereux et al.13 In absolute terms, aortic root
dilatation is defined as diameter at the level of a sinus of
Valsalva ≥ 4 cm.
Table 75.5: Congenital Heart Defects in Adults Commonly

Associated with a Dilated Aortic Root
Bicuspid aortic valve
Ventricular septal defect
Coarctation of aorta
Tetralogy of Fallot
Truncus arteriosus Fig. 75.3: Diagram showing the aortic root in the parasternal
long-axis view. Measurements are taken (leading edge to leading
Transposition of the great arteries edge) at the level of the (1) aortic annulus at the hinge points of
Single ventricle (univentricular heart) with pulmonary the leaflets, (2) midsinuses, (3) sinotubular junction, and (4) about
stenosis 2 cm distal to the sinotubular junction.
SIMPLE CONGENITAL HEART DEFECTS

IN ADULTS
Shunt Lesions
Patent Foramen Ovale
Ruling out a cardiac source of emboli in an adult with a
stroke or transient ischemic attack may be one of the
most common indications for performing a TEE in most
hospitals around the world. Although a TEE can visualize
various causes of cerebrovascular events such as systemic
ventricular/left atrial appendage thrombi, atheromatous
plaques in the ascending aorta/arch, the possibility of
a paradoxical embolus through a patent foramen ovale
(PFO) also needs to be ruled out. Fig. 75.4: Transesophageal echocardiogram showing an atrial
septal aneurysm (ASA), which can be described as a redundant,
Color Doppler with TTE in the subcostal view or on undulating, interatrial membrane in the region of the fossa ovalis.
multiplane TEE views may alert us of its existence in many (LA: Left atrium; RA: Right atrium).
cases. An agitated saline contrast study during TTE or
TEE requires an adequate Valsalva maneuver in an adult
to prove a right-to-left shunt. Transcranial Dopplers are
currently the reference standard for ruling out paradoxical with a potentially higher risk for cerebral embolic events
microemboli.16,17 Three-dimensional echocardiography is are eustachian valve anatomy or Chiari network favoring
more likely to demonstrate PFO as a slit-shaped tunnel- right-to-left shunting.24
like defect in the atrial septum by TTE in adults with good Device closure for PFO in adults with cryptogenic
acoustic windows or by TEE in all the adults. stroke remains controversial. Information from two
Persistent patency of the foramen ovale in adults prospective clinical trials, CLOSURE I (Evaluation of the
makes it the most common CHD, with an autopsy-derived STARFlex Septal Closure System in Patients with a Stroke
incidence of around 27% for a probe patent PFO.18 TEE with and/or Transient Ischemic Attack due to Presumed
saline contrast has detected the association between right- Paradoxical Embolism through a Patent Foramen Ovale)
to-left shunting at rest and high membrane mobility, in study25 and Randomized Evaluation of Recurrent Stroke
patients with cerebrovascular ischemic events who have a Comparing PFO Closure to Established Current Standard
PFO, and are at higher risk for recurrent brain embolism.19 of Care Treatment (RESPECT)26 show that between
The atrial septum (fossa ovalis membrane) may medical management and PFO device closure, there is
demonstrate thinning and marked redundancy. An atrial no significant benefit in the outcomes. These studies
septal aneurysm (ASA) is characterized by a redundant, evaluated the incidence of recurrent ischemic strokes
undulating, interatrial membrane in the region of the fossa in the primary intention-to-treat analysis associated
ovalis (Fig. 75.4). The diameter of the base can exceed with closure of a PFO in adults who had a cryptogenic
15 mm and the amplitude of the interatrial septum ischemic stroke. However, in a subset of this population,
excursion is above 10 to 15 mm. ASA may be associated device closure was superior to medical therapy alone (in
with one or multiple PFOs on an average in 70% of the the prespecified per-protocol and as-treated analyses),
cases.20 Besides PFO, the ASA may be associated with with a low rate of associated risks.26 Another prospective
mitral valve prolapse, dilated atria, and intracardiac study conducted by the PC trial investigators reports that
thrombi, which may explain the increased frequency closure of a PFO for secondary prevention of cryptogenic
of embolic stroke in this population.21 ASA and PFO embolism did not result in a significant reduction in the
synergistically potentiate the risk for cryptogenic stroke risk of recurrent embolic events or death as compared with
in adults <55 years of age.20,22,23 There is an increased medical therapy.27
likelihood of thrombus formation on the left atrial side Currently, the decisions to “close or not to close” the
of the ASA. Other echocardiographic findings associated PFO in young adults with cryptogenic stroke are being
made on humanitarian grounds on a case-by-case basis Right heart enlargement with diastolic flattening
after weighing individual risk benefit ratio and providing and paradoxical motion of the interventricular septum
an informed consent. indicate right ventricular volume overload in the presence
Intracardiac echocardiography (ICE) has played a of a significant left-to-right shunt. On transthoracic
major role in device closure.28 More recently, 3D TEE echocardiography, right atrial/ventricular enlargement,
has taken its place in many centers.29 However, there is a a D-shaped septum (on the short-axis view in diastole)
significant disagreement between TEE and rotational ICE with paradoxical motion of the interventricular septum,
in measuring fossa ovalis and selecting the device for PFO and varying degrees of pulmonary hypertension occur
closure, particularly in patients with ASA.30 due to long-standing left-to-right shunt. When pulmonary
stenosis is present, it may prevent the development of
Atrial Septal Defects secondary pulmonary hypertension.
In the absence of pulmonary stenosis, the estimated
Adults with undiagnosed atrial septal defects (ASD) right ventricular systolic pressure (RVSP) is a surrogate for
usually present with dyspnea, fatigue, and palpitations pulmonary artery pressure. As with acquired heart disease, it
due to atrial arrhythmias. The various types of atrial septal is assessed by applying the simplified Bernoulli equation:
defects are secundum, primum, sinus venous, and the rare RVSP = 4 (tricuspid regurgitation (TR) jet velocity)2 +
coronary sinus defect.31 right atrial pressure
Right heart enlargement and dilatation of the
Echocardiography pulmonary artery are signs of a significant shunt volume
Primarily, the subcostal, followed by the high parasternal and are sufficient to warrant closure of the atrial septal
and apical four-chamber views are the best views in defect. A Qp/Qs calculation is unnecessary in these cases.
assessment of the atrial septum. There is a likelihood Other concomitant defects that contribute to left heart
of a false-positive reading of an ASD due to midseptal volume overload are complete atrioventricular septal
dropout in the apical four-chamber view. Pulsed and defects associated with a cleft mitral valve and mitral
color flow Doppler define the direction of the shunt most regurgitation.
accurately at a lower Nyquist level. The direction of the
low-velocity shunt depends on ventricular compliance. Types of Atrial Septal Defect
Other low venous flows from the superior and the inferior
(Figs 75.5A to C)
vena cava or the coronary sinus can cause a false-positive
signal suggestive of an interatrial communication by color The secundum ASD is the most common type and
Doppler. A tricuspid regurgitant jet with the flow directed comprises nearly 75% of the ASDs. It is best seen in the
toward the interatrial septum can also be misleading. subcostal view. It can be seen in the apical four-chamber
A B C
Figs 75.5A to C: Diagram showing locations of the three common types of atrial septal defects (ASD): (A) Secundum ASD;
(B) Primum ASD; (C) Sinus venosus ASD. (LA: Left atrium; RA: Right atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena
cava).
view but there may be some false-positive dropouts of be associated with an atrioventricular septal defect, cleft
the midseptum. Besides right heart enlargement that mitral valve, partial anomalous pulmonary venous return,
may progress to right or biventricular heart failure, these and left superior vena cava. Pulmonary hypertension may
patients are at risk for paradoxical embolism, progressive be present due to increased pulmonary arterial flow. The
pulmonary hypertension, and have an increased incidence best views are the apical four-chamber and the subcostal
of atrial arrhythmias. views for detecting the absence of the interatrial septum,
The ostium primum ASD (20%) that rarely presents as defect in the membranous ventricular septum, right
an isolated finding is best seen in the apical four-chamber heart enlargement with 2D echocardiography and color
view as an echo dropout in the lower part of the interatrial Doppler. The parasternal short-axis views at the aortic level
septum. It is usually associated with a cleft anterior mitral show dilatation of the pulmonary artery due to pulmonary
valve, which is best seen in the parasternal short-axis view. hypertension.
Its association with atrioventricular septal defect is later
discussed in this chapter. Transesophageal Echocardiography
The sinus venous defect (5%) is usually associated A TEE is necessary in nearly all adults for localizing/sizing
with anomalous pulmonary venous drainage and often the secundum ASD, measuring septal rims (superior and
missed on a transthoracic echocardiogram. The entire inferior), entry of the superior vena cava, connection of all
atrial septum from the orifice of the superior vena cava to pulmonary veins, and for ruling out concomitant defects
the orifice of the inferior vena cava should be displayed to before planning device closure or surgery. Color Doppler
identify echo dropout. It is usually best seen in the subcostal can determine the major direction of the shunt (Fig. 75.6).
short-axis view, and occurs between the junction of the Measurements are made in orthogonal planes to allow
superior vena cava and the interatrial septum. It rarely selection for appropriate candidates for device closures.
presents in adults as a dropout between the inferior vena Three-dimensional TEE allows real time visualization of
cava and the interatrial septum. the defect. It also allows more defined measurements of the
A rare case of coronary sinus defect (1%) is suspected rims, size, and location with respect to adjacent structures,
especially in the presence left superior vena cava draining and assessment of concomitant defects. In the current
into the roof of the left atrium. There may be partial or era, it plays a major role in transcatheter implantation of
complete absence of the coronary sinus. It is best seen in the ASD closure device.32–36 TEE also helps in detecting
a modified apical four-chamber view. A large coronary procedural complications such as device embolization
sinus orifice shows evidence of left-to-right atrial shunting into the pulmonary artery or into the iliac artery.37,38 Early
due to defect in the roof of the coronary sinus (sinoseptal and late complications such as erosions caused by devices
defects). The right ventricular systolic pressure needs to can also be demonstrated by TEE.39
be determined, since pulmonary hypertension is likely
to occur in adults with significant shunts. If there is a
significant right-to-left shunt due to another defect, the
orifice of the coronary sinus may not be so enlarged and
go unrecognized. With the development of pulmonary
hypertension, the low velocity of the shunt flow across
the coronary sinoseptal defect may be confused with
other low-velocity flow states within the atria. Anomalous
pulmonary venous drainage is more commonly associated
with sinus venosus ASD but can also occur in this setting.
Tricuspid atresia has also been reported with a coronary
sinus defect.
Common atrium is a rare condition in which the
atrial septum is nearly or completely absent, causing an
intermixing of the oxygenated and deoxygenated blood. Fig. 75.6: Transesophageal echocardiogram with color Doppler
The right and left sides of the common atrium retain showing the left-to-right shunt due to a secundum atrial septal
morphological features of the right and left atria. It may defect (ASD). (AoV: Aortic valve; RA: Right atrium).
The presence of right heart enlargement and/or mild echocardiography.42,43 A synopsis of echocardiographic
pulmonary hypertension without a visible defect on TTE assessment of ASDs is reviewed in Table 75.6.
should be investigated by a TEE with the probe angled to
around 90° for delineating a sinus venosus defect, and with Contrast Echocardiography
posterior angulation of the probe to demonstrate a coronary
sinus defect. TEE plays a pivotal role in assessment of the An agitated saline contrast study is usually unnecessary
pulmonary veins.40,41 It is very useful in diagnoses of these in adults with ASDs but when performed, it may reveal
two types of defects that are often missed by transthoracic a bidirectional shunt with the major component of a
Table 75.6: Atrial Septal Defects

Types
• Secundum—most common type of ASD
• Primum ASD—often associated with an endocardial cushion defect/partial AV canal defect (inlet VSD) and cleft anterior mitral valve
• Sinus venous ASD—usually associated with partial anomalous pulmonary venous return
• Coronary sinus ASD—commonly seen with persistent left superior vena cava
• Common atrium
Associated defects
• Occurs mostly as an isolated anomaly
• Partial anomalous pulmonary venous return (sinus venosus defect)
May occur in association with the following defects:
• Mitral valve prolapse
• Ventricular septal defect
• Patent ductus arteriosus
• Pulmonary stenosis/right ventricular outflow tract obstruction
• Tetralogy of Fallot
• D-transposition of the great arteries
• Congenitally corrected transposition of the great arteries
• Truncus arteriosus
• Tricuspid atresia
• Ebstein’s anomaly
Echocardiographic assessment
• Identification of the type of defect
• Estimation of the size of the defect in orthogonal views to get the largest dimension with 2D echocardiography or by planimetry
with 3D echocardiography
• Color Doppler to assess to direction of the shunt (using low Nyquist limit)
• Pulsed wave Doppler—characteristic flow pattern begins in early systole, although most of the cardiac cycle with a broad peak in
late systole/early diastole, with peak velocity usually < 2 m/s (combination of pulmonary and systemic venous flow)
• Right heart size—right atrium and ventricle are enlarged when the shunt is significant with Qp/Qs over 1.5 (may avoid the need
to calculate Qp/Qs)
• Estimation of right ventricular systolic pressure/degree of pulmonary hypertension
Postoperative
• Rule out residual shunt
• Device closure
– Confirm alignment of the device along the interatrial septum
– Rule out impingent on surrounding structures—mitral valve, aortic valve, and pulmonary veins—or erosion of the device
left-to-right shunt. A dense bolus from an intravenous hypertension, right heart failure, and the likelihood of
agitated saline injection shows “negative” contrast jet atrial arrhythmias. Adults with large secundum ASD/septal
near the defect with passage of a few bubbles into the aneurysm, primum, sinus venosus, or coronary sinus
left atrium during transient periods of higher right atrial defects need surgery for closure of the defect and repair
pressure. Contrast echocardiography is contraindicated of concomitant defects. Although adults with small ASDs
in patients with Eisenmenger physiology, since a large (< 5 mm) are at low risk of having these complications,
bolus of microemboli entering the systemic circulation may closure may be considered to reduce the risk of paradoxical
potentially cause cerebral ischemia. embolism.
Exercise Testing The Postoperative Adult

Exercise testing is useful for objective assessment of Following repair of all ASDs or device closure of the
functional capacity and for revealing exercise-induced secundum ASD, echocardiography plays an important role
arrhythmias, and changes in oxygen saturation in adults
in ruling out residual shunts or pulmonary hypertension.
with pulmonary arterial hypertension. Maximal exercise
Intraoperative TEE checks the adequacy of the repair by
testing should be avoided and effort should be restricted to
confirming the absence of residual shunts in all types of
<70% of maximum age-predicted heart rate, in those with
ASDs after device or surgical repair. It is especially useful
severe pulmonary arterial hypertension.
after pericardial patch repair of the sinus venosus defect
in the superior vena cava, with baffle redirecting the
Cardiac Catheterization anomalous pulmonary veins to the left atrium. Narrowing
Diagnostic cardiac catheterization is not indicated in or stenosis of the superior vena cava or pulmonary veins
younger adults with uncomplicated ASD if they have will manifest as flow acceleration on color Doppler at the
had adequate noninvasive imaging. Shunt runs are of site of the obstruction.
value when the site of the defect is unclear, such as in Postoperatively, an urgent TTE is necessary in a
case of sinus venosus defect or a coronary sinus defect. surgical patient presenting with fever, pleuritic chest
Cardiac catheterization is essential for assessment of pains, and progressive dyspnea with or without abdominal
pulmonary vasoreactivity in adults with severe pulmonary symptoms to rule out pericardial effusion and possible
hypertension prior to ASD closure. In older adults, tamponade, since there is a likelihood of pericarditis for
coronary artery disease also needs to be ruled out before several weeks postoperatively.
surgical closure. Currently, cardiac catheterization is Device malalignment or impingement on surrounding
mostly performed in conjunction with device closure of an structures (pulmonary veins, aortic, and mitral valves)
ASD.
should be ruled out following device closure. Other
potential complications following ASD device closure are
Magnetic Resonance Imaging/Computed pericardial effusion, erosion of the atrial wall or aorta,
Tomographic Angiography device thrombosis, or endocarditis within first 6 months
of implantation. TTE should be performed on the day
MRI/CTA delineates the course of the anomalous
following device closure, at 1 month, 6 months, at 1 year,
pulmonary veins into the right atrium, persistent left
superior vena cava, and allows visualization of other and then annually for a few years post device closure.
extracardiac structures.
Ventricular Septal Defects
ASD Closure Although ventricular septal defects (VSDs) are the most
The Class I ACC/AHA recommendations for closure of an common defects in infancy, they are less prevalent in
atrial septal defect are visual or quantifiable evidence of adulthood due to spontaneous closure in early life. The
right heart overload (right heart enlargement or Qp/Qs clinical presentation of an isolated VSD through life will
over 1.5).3 Early closure of moderate to large defects lowers depend largely on defect size and pulmonary vascular
the risk of long-term complications including pulmonary resistance.31,44–47
Fig. 75.7: Transthoracic echocardiogram (apical four-chamber Fig. 75.8: Diagrammatic representation of the “venturi effect”
view) showing that the tricuspid septal leaflet prolapses in an causing prolapse of aortic valve (AoV) cusp in an attempt to close
attempt to close the ventricular septal defect (VSD) leading to the ventricular septal defect (VSD), leading to aortic regurgitation
the development of a membranous septal aneurysm. (ASD: Atrial (AR) as seen with a transesophageal echocardiogram.
septal defect; RA: Right atrium; RV: Right ventricle; TV: Tricuspid
valve).
Most individuals are diagnosed in childhood due to a also been numerically divided into four types, correlating
loud murmur. Device or surgical closure is not indicated with their position as noted below.48
in adults with small VSDs in the absence of pulmonary
hypertension. These adults are at a higher risk of acquiring Type 1: Supracristal (Also Known as Outlet,
endocarditis, where the VSD jet strikes the endocardial
surface close to the tricuspid valve causing tricuspid valve Subpulmonic, Infundibular) VSD
vegetations that may predispose to pulmonary embolism. The parasternal short-axis view is the best view to
The tricuspid septal tissue may prolapse in an attempt to document a left-to-right shunt in the 2 o’clock position at
close the VSD leading to the development of membranous the level of the aortic valve. It may present as a low-velocity
septal aneurysm (Fig. 75.7). Large membranous septal
diastolic shunt flow preceding a left-to-right shunt during
aneurysms can also cause obstruction to the right
systole, and can be mistaken as other defects such a sinus
ventricular outflow tract.
of Valsalva aneurysm or a coronary artery fistula, since
Similarly, aortic regurgitation can occur when the
they also similarly appear in the parasternal long-axis
aortic cusps (usually right or left) attempt to spontaneously
close a membranous VSD due to “venturi effect”—a view.
suction force that is created by a gradient across the VSD
that draws the aortic valve leaflet to prolapse and close the Type 2: Membranous (or Perimembranous)
VSD in a flap-like manner (Fig. 75.8). VSD
Moderate to large VSDs may present with impaired left
ventricular function and heart failure. Progressive tricuspid This is the most common type of VSD in adults and it is
regurgitation and increasing severity of pulmonary best seen in the parasternal long-axis and short-axis views
hypertension are also potential long-term issues. In with the help of color Doppler that reveals a turbulent
some unfortunate cases, a large VSD may predispose left-to-right shunt. Two-Dimensional echocardiographic
to irreversible pulmonary hypertension (Eisenmenger images may show a dropout in the membranous septum
physiology) that is no longer amenable to surgical closure. just below the aortic valve in the five-chamber view. Other
The VSDs are classified according to their position in suitable views for visualizing this defect are the apical and
the interventricular septum (Figs 75.9A to C). They have subcostal views.
A B
Figs 75.9A to C: Diagram showing locations of the four types

of ventricular septal defects (VSD). (AoV: Aortic valve; LA: Left
atrium; LV: Left ventricle; PA: Main pulmonary artery; RA: Right
C atrium; RV: Right ventricle).
Type 3: Inlet (or AV Canal Type) VSD with a rim of muscle around the defects. They are best
seen in the parasternal long-axis view or in the apical four-
This type of VSD is usually associated with a primum ASD chamber view with the help of color Doppler.
and involves the area around the crux of the heart. It may
present as an AV septal defect with associated AV valve
Echocardiography
abnormalities as seen in individuals with Down syndrome.
It is best seen in the apical four-chamber view. Transthoracic echocardiography with color Doppler
displays location, size, and number of the VSD. For
calculation of pressure gradient across the VSD with
Type 4: Muscular
continuous wave Doppler, the cursor should be aligned
These VSDs may be multiple or large enough to persist along the direction of the VSD for accurate assessment
into adulthood. They are located in the trabecular septum of the shunt. Heart chamber dimensions, presence of
right or left ventricular outflow obstruction, and left valve, because otherwise there will be device impingement
ventricular size and function should be estimated on serial on these structures.
echocardiograms. A significant volume of blood flow is Intraoperative TEE with color Doppler allows detection
directed from the left to the right shunt into the pulmonary of multiple VSDs, especially muscular or supracristal
artery leading to an increased left heart volume. Over preoperatively, since these may not be directly visible to
time, this causes left heart enlargement and decreases the surgeon.
left ventricular function. The aortic valve morphology After the operation, TEE confirms patch closure
and function should be evaluated to rule out prolapse and rules out residual defects. Patch closure and use of
and regurgitation. Estimation of right ventricular systolic intraoperative TEE have been shown to improve surgical
pressure (RVSP) is usually performed using the pulmonary outcomes.49
regurgitation jet, since contamination of the tricuspid jet A comprehensive assessment of concomitant defects
signal by a VSD jet, through the left-to-right shunt, may requiring surgery, such as aortic valve repair/replacement
overestimate RVSP. Associated defects that need to be for regurgitation, resection of subaortic membrane in
ruled out are listed in Table 75.7. case of left ventricular outflow tract obstruction, and
relief of right ventricular outflow obstruction by adequate
Magnetic Resonance Imaging/ resection and patch should be performed.
Computed Tomography
These imaging modalities are useful for assessment of The Postoperative Adult
apical and supracristal VSDs in unusual locations, and for Postoperatively, TTE is useful for assessment of left
evaluation of extracardiac structures such as the aorta. ventricular function, ruling out residual shunts or
pulmonary hypertension. Associated defects such as
Cardiac Catheterization aortic root dilatation and aortic regurgitation need long-
term follow-up.
It is usually performed in conjunction with device
closure of the defect. The major role of diagnostic Patent Ductus Arteriosus
cardiac catheterization is in assessment of pulmonary
artery pressures and pulmonary vasoreactivity prior to Survival into the ninth and tenth decades of life is possible
closure, when pulmonary hypertension is suspected on in an adult with an unoperated patent ductus arteriosus
echocardiography. In addition, coronary arteriography is (PDA). The PDA is a vascular structure that connects the
performed in older adults prior to surgical closure of the inferior curvature of the proximal descending aorta to
defect. the roof of the main pulmonary artery near the origin
of the left pulmonary artery in fetal life, and is designed
VSD Closure for spontaneous closure in early infancy. Persistent
ductus arteriosus may occur in adulthood when spon-
The Class I ACC/AHA recommendations for closure of a taneous, surgical, or device closure has failed to occur in
VSD are the evidence of left ventricular volume overload childhood.31
(Qp/Qs ≥ 2.0) or a history of infective endocarditis. VSD
closure is contraindicated in adults with severe irreversible
Echocardiography
pulmonary vascular disease.
At other times, VSDs are closed during surgery for Echocardiography is sensitive and very specific in
aortic regurgitation and other associated defects.49 diagnosing PDA. Tiny PDAs may sometimes be missed
Although there is no Class I indication for catheter- and there is low likelihood of a false-positive PDA.50,51
based device closure of a VSD in an adult, the Class IIa Two-dimensional TTE is able to demonstrate a
indications for device closure include high surgical risk persistent anatomical connection between the descending
with hemodynamically significant residual shunt or aorta and the pulmonary artery only in some adults. This is
history of infective endocarditis. In these cases, the VSD due to limited acoustic windows because of body habitus
should not be located proximal to the aorta or the tricuspid or secondary to lower ultrasonic frequencies hampering
Table 75.7: Ventricular Septal Defects

Types
• Perimembranous—most common
• Supracristal/infundibular/subpulmonic
• Muscular
• Atrioventricular (AV) septal defect [inlet/atrioventricular (AV) canal defect]
Associated defects
• Atrial septal defect
• Coarctation of aorta
• Aortic root dilatation
• Aortic regurgitation
• Tricuspid regurgitation
• Identification of the type of defect
• 2D and color Doppler evaluation of the size of defect in systole
• Color and continuous wave Doppler to determine the direction and gradient of the shunt
• Is it a restrictive or nonrestrictive VSD?
• Impact on chamber size
– Left atrial dilation, left ventricular dilatation (due to excess blood going through the lungs because of the left-to-right shunt)
– Right heart enlargement/right ventricular hypertrophy occurs in adults with Eisenmenger physiology
• Ventricular function—left ventricular systolic function
• Rule out associated defects
• Examination of the aortic valve (size in systole, degree of aortic regurgitation in diastole)—aortic regurgitation is usually associ-
ated with supracristal or perimembranous VSD
Postoperative
• Rule out residual ventricular septal defect
• Assessment of associated defects
• Ventricular function
with the visualization of the PDA, variations in position, to trace the path of the jet of color Doppler flow. Although
alignment, and shape and length of the ductus. In order the jet appears to be arising from the bifurcation of the
to see the entire ductus connect to the inferior curvature pulmonary artery, the actual origin of the shunt is in the
of the descending aorta, the probe should be rotated descending thoracic aorta.
clockwise. The best view is the high left parasternal short-axis
Color Doppler can diagnose whether the flow is due to view above the level of the aortic valve, or a modified
a PDA or not, by freezing or slowly moving frames in order suprasternal view that achieves a long-axis view of the
right ventricular outflow tract and the main pulmonary across the ductus causes pressure equalization between
artery. Color Doppler displays the bifurcation of the the aorta and the pulmonary artery first in diastole but
pulmonary artery and its branches, by angling the probe with increasing severity of pulmonary hypertension, the
leftward and superiorly in the high parasternal short-axis pressure difference between aorta and the pulmonary
view. The bifurcation of the main pulmonary artery may artery will decrease in systole. To improve the accuracy of
appear to “trifurcate” with the ductus arising along with estimated right ventricular systolic pressure calculated by
left and right pulmonary arteries (Fig. 75.10).52 using the modified Bernoulli’s equation (4V2), a bolus of
In a typical PDA with a left-to-right shunt, a color saline is injected via peripheral intravenous catheter.53 This
Doppler jet may be seen flowing through the left pulmonary enhances the profile of the maximum tricuspid regurgitant
artery into the main pulmonary artery with marked jet velocity obtained on pulsed Doppler. The left heart
spectral dispersion in systole and diastole (throughout the chambers size depends upon the size of the PDA. Adults
cardiac cycle in adults). The maximal velocity is recorded with a small PDA may have normal cardiac dimensions
by placing the Doppler sample volume just proximal to the and no echocardiographic evidence of pulmonary
bifurcation. The systolic and diastolic flow can be recorded hypertension, while those with moderate to large PDA may
from the high parasternal window, with an ultrasound have left heart enlargement with impaired left ventricular
beam aligned directly into the orifice of the PDA. Color function and varying degrees of pulmonary hypertension.
Doppler will display diastolic flow from PDA along the A dilated, aneurysmal, and calcified main pulmonary
lateral wall of the pulmonary artery, while pulsed wave artery may be seen in adults with a long-standing shunt
Doppler will demonstrate flow reversal along the anterior past the third decade of life. In a dilated pulmonary artery,
wall of the pulmonary artery. The “machinery” murmur low velocity retrograde flow can be demonstrated in late
auscultation correlates with the continuous variation of the systole. With rising pulmonary vascular resistance, there
flow between systole and diastole on echocardiography. is a bidirectional shunt with right-to-left flow seen in early
In the suprasternal view, pulsed wave Doppler may systole and a left-to-right flow seen in late systole/diastole.
demonstrate holodiastolic flow reversal in the descending This may further deteriorate to irreversible pulmonary
aorta due to antegrade flow into the ductus in diastole. Flow hypertension and Eisenmenger physiology. Although
most PDAs occur in isolation, associated defects such as
VSD, coarctation of aorta, and complex CHD should be
evaluated.
Coronary artery fistula, anomalous coronary artery,
aortopulmonary collateral, ruptured sinus of Valsalva,
and pulmonary regurgitation may appear like ductal flow,
since they produce turbulence in the main pulmonary
artery with color Doppler examination.
Three-dimensional transthoracic echocardiography
may be helpful in differentiating vascular structures
from artifacts and echo dropouts. For a comprehensive
assessment, one must acquire 3D color Doppler flow
signals from the PDA jet, main pulmonary artery, and the
descending thoracic aorta followed by rotation to view
the flow signals from all sides at any desired angulation.
By rotating these isolated color Doppler images from 0° to
Fig. 75.10: Diagrammatic representation of the high parasternal
180°, one can visualize flow in pulmonary arteries, PDA,
short-axis view on a transthoracic echocardiogram showing the bi-
furcation of the main pulmonary artery that may appear “trifurcate”
and the descending aorta in three dimensions. Color
with the ductus arising along with left and right pulmonary arteries. Doppler 3D transthoracic echocardiography can also
(Desc Ao: Descending aorta; LA: Left atrium; LPA: Left pulmonary demonstrate flow signals moving from the pulmonary
artery; MPA: Main pulmonary artery; PDA: Patent ductus arterio- artery into the descending thoracic aorta in systole and
sus; RA: Right atrium; RPA: Right pulmonary artery). back into the pulmonary artery in diastole.54–56
Transesophageal Echocardiography anatomical details, confirming the position of the device

after closure, and ruling out a residual shunt.56
In adults with limited acoustic windows, TEE is helpful in A synopsis of echocardiographic assessment of the
assessment of the PDA and associated defects. It may reveal PDA is reviewed in Table 75.8.
more details such as the anatomy of ductus arteriosus and
its relationship with the descending aorta and pulmonary
artery. Real time 3D TEE helps in imaging of the PDA,
Cardiac Catheterization
before and after Amplatzer device closure. However, the Cardiac catheterization is primarily performed at the time
cranial part of the aortic arch cannot be visualized because of device closure to determine the anatomy of the ductus,
of the near-field artifact.54 degree of shunt, pulmonary vascular resistance (PVR),
Live 3D TEE plays a major role in the perioperative pulmonary vasoreactivity, associated defects, and coronary
monitoring during transcatheter device closure by cutting artery disease in older adults. Catheter-based device closure
down the fluoroscopy time. It is useful in defining the is mainly guided by fluoroscopy and partially by TEE.
Table 75.8: Patent Ductus Arteriosus

Associated defects
• Usually occurs as an isolated anomaly
May be associated with the following defects:
• Valvular defects—aortic or mitral valves
• Complex CHDs—in association with complex defects such as congenitally corrected transposition of the great arteries or tetral-
ogy of Fallot.
• Identification of the defect—when visible, the bifurcation of the main pulmonary artery may appear “trifurcate” with the duc-
tus arising along with left and right pulmonary arteries—measure length and width when possible
• Color Doppler—jet seen through the left pulmonary artery into the main pulmonary artery with marked spectral dispersion in
systole and diastole (throughout the cardiac cycle in adults) in a typical PDA with a left-to-right shunt
• Assessment of pressure gradient across the PDA
• Measurement of left ventricular dimensions and quantitative assessment of left ventricular function—left heart enlargement is
common with moderate to large PDA
• Estimation of right ventricular systolic pressures/pulmonary hypertension (right ventricular hypertrophy occurs in adults with
Eisenmenger physiology)
• Rule out pulmonary artery dilatation, aneurysm, or calcification (often noted by the fourth decade of life)
• Assessment of the aortic arch—rule out flow acceleration/obstruction, diastolic flow reversal
Postoperative
• Left ventricular size and function, left atrial enlargement
• Rule out residual shunts
• Ensure proper device placement (a well-seated device can be visualized between the pulmonary artery bifurcation and the
inferior margin of the aorta. It should not protrude into the aortic lumen or the pulmonary artery)
Magnetic Resonance Imaging/Computed AV valve between the atria and ventricles. The cleft in the
left-sided AV valve leaflet results from incomplete fusion
Tomography of the superior and inferior bridging leaflets of the AV
These modalities are rarely required for imaging an valve.
isolated PDA for determining suitability for device closure.
Echocardiography
PDA Closure The AV septal defects are best seen in the apical-four
The ACC/AHA Class I recommendations3 for device or chamber views. The complete AV is classified into three
surgical closure of PDA in adults are: Rastelli types based on the extent of chordal attachments of
• Left heart enlargement (atrial and/or ventricular) due the superior bridging leaflet. This defect may be associated
to volume overload or in the presence of pulmonary with Down syndrome.31
hypertension due to a left-to-right shunt Two-dimensional echocardiography defines the
• History of endarteritis. size of the AV septal defect, AV valve attachments, and
Surgical or device closure of PDA is contraindicated in determines the presence of a common AV valve or two
adults with severe pulmonary hypertension due to reversal separate AV valves. A distinctive feature of AV septal defect
of the shunt (right to left). Calcification and tissue friability is the bridging leaflets of the AV valves that are viewed in
in adults can make surgical closure challenging and the same horizontal plane, on an apical four-chamber view
therefore, it is performed in limited cases. Surgery instead (Fig. 75.11). A cleft anterior mitral valve is identified in the
of device closure should be considered in adults who have parasternal short-axis view (Fig. 75.12).
calcified PDA, ductal aneurysm, history of endarteritis, Color Doppler is used to estimate the severity of the AV
and very large PDAs. valve regurgitation, and the level, size, direction of atrial
and ventricular shunts.
The Postoperative Adult
Transthoracic echocardiogram and color Doppler are used
in long-term follow-up after device closure or surgery.
Postoperatively, proper device placement is confirmed by
visualizing a well-seated device between the pulmonary
artery bifurcation and the inferior margin of the aorta,
without any protrusion into the pulmonary artery or
aortic lumen. In the immediate postoperative period,
residual shunts should also be ruled out. On subsequent
echocardiograms, smaller shunts are likely to resolve due
to endothelialization over time.
Atrioventricular Septal Defect

Atrioventricular septum is the partition between the left
ventricular outflow tract and facing right atrium in normal
hearts.31 Morphological variations in the anomalies of the
AV septum and the AV valves present as various types of
AV septal defects.57 In partial AV septal defects, there is
primum ASD, with two separate AV valves, a cleft mitral Fig. 75.11: Diagrammatic representation of the transthoracic api-
valve, and no VSD; in intermediate AV septal defect, in cal four-chamber view showing the distinctive feature of AV septal
addition to these findings, there is a small restrictive defect with the bridging leaflets of the AV valves viewed in the
membranous VSD; while in complete AV septal defect, there same horizontal plane (as shown with the dotted line). (LA: Left
is a large septal defect extending from the primum atrial to atrium; LV: Left ventricle; MV: Mitral valve; PV: Pulmonary vein;
RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).
the perimembranous ventricular septum with a common
coronary sinus into the right atrium in the rarest type of

ASD called the “unroofed coronary sinus defect.” It may
be associated with other defects such as a bicuspid aortic
valve, coarctation of the aorta, or cor triatriatum. In 20% of
those who have a persistent LVNC, the right superior vena
cava may be absent, leading to marked dilatation of the
coronary sinus because of increased blood flow.31 The right
superior vena cava may be absent in approximately 15% to
30% of the individuals born with a persistent LSVC. These
individuals have a high likelihood of having an enlarged
coronary sinus because of markedly increased blood flow
into the LSVC.60
The LSVC is often an incidental finding in asymptomatic
adults. It may be of clinical significance during pacemaker/
Fig. 75.12: Transthoracic echocardiogram showing a cleft ante-
defibrillator implantation, central venous catheter
rior mitral valve (MV) in the parasternal short-axis view. (AMVL:
Anterior mitral valve leaflet; PMVL: Posterior mitral valve leaflet). placement, and in patients undergoing cardiopulmonary
bypass surgery during retrograde cardioplegia, since the
Live/real time 3D TTE shows incremental value over solution can perfuse retrograde into the left persistent
2D TTE in the evaluation of an adult with a complete AV superior vena cava, thereby decreasing the effectiveness of
septal defect. Most importantly, it provides an en face view cardioplegia.
of all the five leaflets of the common AV valve. It allows
more detailed evaluation of shunts (especially from the Echocardiography
left ventricle to right atrium), quantitative assessment of
TTE with an agitated saline contrast study helps in
regurgitant lesions, and classification into one of the three
making the diagnosis. The agitated saline contrast
Rastelli types.58,59
injection (“bubble” study) in the left arm via a peripheral
intravenous catheter flows into the left brachiocephalic
The Postoperative Adult vein with appearance of microbubbles in the left atrium in
Postoperative long-term issues that are assessed by case of an unroofed coronary sinus. If the LSVC is draining
echocardiography include a search for a residual shunt into an intact enlarged coronary sinus, an injection
lesion (post-AV patch repair), degree of mitral (left AV into either the right or left arms (through a peripheral
valve) or tricuspid regurgitation, right ventricular systolic intravenous catheter) will demonstrate the microbubbles
pressure, degree of mitral stenosis following surgery of in the enlarged coronary sinus and then in the dilated
cleft mitral valve, or subaortic stenosis due to the long left right heart.61 If the right superior vena cava is absent, the
ventricular outflow tract that manifests as a “gooseneck
injection of agitated saline contrast into the right arm will
deformity” on cardiac catheterization. A synopsis of the
first appear in the enlarged coronary sinus followed by the
echocardiographic assessment of AV septal defects is
right atrium.62
reviewed in Table 75.9.
Persistent Left Superior Vena Cava Magnetic Resonance Imaging/Computed

Tomography
Left superior vena cava (LSVC) is the most common thoracic
venous abnormality.60 It is the embryological remnant of Often the LSVC and all the pulmonary veins cannot be
the persistent left anterior cardinal vein. Its prevalence easily identified by TTE or TEE. Like other extracardiac
varies from around 0.3% in the general population to 10% structures, the LSCV can clearly be identified on MRI or
in patients with CHD. The LSVC usually drains via the CTA with 3D reconstruction.60,61
Table 75.9: AV Septal Defect

Associated defects—Usually occurs in patients with Down syndrome
• Patent ductus arteriosus—most common
• AV valve regurgitation
• Coarctation of the aorta
• Left ventricular outflow tract obstruction
• Double orifice mitral valve
• Muscular ventricular septal defects
• Right ventricular outflow tract obstruction
• Pulmonary stenosis
• Left ventricle-to-right atrial shunt
• Gerbode defect—right ventricle-to-left atrial shunt
• Identification of the defect
– Primum atrial septal defect
– Inlet ventricular septal defect
– Cleft anterior mitral valve
– Tricuspid commissure—widened (anteroseptal)
• Estimation of right ventricular systolic pressures/pulmonary hypertension (right ventricular hypertrophy occurs in adults with
Eisenmenger physiology)
• Left ventricular size and function
• Right ventricular size and function
• Assessment of the size and the gradient across the atrial and ventricular shunt
• Degree of AV valve regurgitation
• Severity of the associated defects
Postoperative
• Biventricular size and function
• Degree of AV valve regurgitation—especially mitral regurgitation due to cleft mitral valve
The Postoperative Adult murmur to acute cardiogenic shock. Aneurysm formation

in the aortic sinus can occur at the junction of the aorta and
Adults with LSVC associated with coronary sinus defect the annular fibrosa, due to structural wall abnormalities. It
and partial anomalous pulmonary venous return
may extend to surrounding structures, causing obstruction
require patch closure of the coronary sinus type of ASD
or fistulae. An unruptured aneurysm may go undiagnosed
and an intracardiac baffle to redirect the anomalous
until it causes symptoms due to right ventricular outflow
pulmonary venous flow into the left atrium. Follow-up
obstruction.63 A small perforation may come to our attention
echocardiography focuses on ruling out residual shunts
on physical examination because of a continuous murmur
and persistent pulmonary hypertension from previously
on auscultation. An acute rupture may result in high output
long-standing left-to-right shunts.
decompensated heart failure. Coronary artery compression
may present as chest pain due to ischemia. Since the sinus
Sinus of Valsalva Aneurysm of Valsalva aneurysms originate more commonly from the
Sinus of Valsalva aneurysm may be congenital or acquired right coronary or the noncoronary cusp, they are more likely
with clinical presentation ranging from an asymptomatic to cause right coronary artery compression.
The differential diagnoses include an acquired chamber or vascular structure. The morphology can be
aneurysm from complicated infective endocarditis, a complex with single or multiple communications that may
coronary artery fistula, an aneurysm of the membranous form a maze of fine intramural channels communicating
ventricular septum, or a PDA. A normal coronary origin with each other. It most commonly affects the right side of
with normal coronary lumen favors the diagnosis of sinus the heart.65
of Valsalva aneurysm. Its location in aortic sinus above The clinical presentation depends upon the size of the
the plane of the origin of the coronaries distinguishes it shunt and the structures involved. Most coronary fistulae
from aneurysm of the membranous ventricular septum. drain into the right heart structures. Adults with a large
In adults with acquired aneurysm following complicated shunt may present with heart failure or angina due to
infective endocarditis, the fistula does not have an coronary steal phenomenon caused by diversion of blood
extended finger-like aneurysmal extension from the base flow away from the territory supplied by the coronary,
to the apex as seen in the sinus of Valsalva aneurysm. into the site of drainage (right heart, pulmonary artery, or
superior vena cava).
Echocardiography
Two-dimensional echocardiography shows an aneurys- Echocardiography
mal sac, which can present as an abnormal, circular, thin-
On a transthoracic echocardiogram, a markedly enlarged
walled structure protruding into a heart chamber. Ruptured
proximal part of the involved coronary artery alerts the
aneurysms will lead to chamber enlargement over time.
echocardiographer of its presence. The structure or the
Color Doppler with transthoracic echocardiogram will
show flow originating in the aorta and flowing into a cardiac chamber to which the coronary artery fistula
heart chamber (usually the right ventricle) in a ruptured drains is also enlarged in those with significant long-
aneurysm. Multiple views, starting with the parasternal standing shunts. The parasternal long-axis views with
long-axis and short-axis views at the level of the aortic color Doppler focusing on the aortic root may also bring
root, are required to locate the sinus from which the it to our attention. A high parasternal short-axis view
aneurysm originated. The transducer is then angulated to and its modifications help in identifying its origin and
obtain modified views and locate the aneurysm (usually site of drainage (Fig. 75.13). However, 2D transthoracic
above the aortic cusps). Contrast echocardiography allows echocardiography is limited in its ability to reliably trace
more precise definition of the aneurysmal sac. It aids in the course of the fistula as well as the site of drainage in
the diagnosis of a left-to-right shunt by demonstrating many adults.66
a negative contrast image in the right chambers with a
ruptured aneurysm and no negative contrast image with
an unruptured aneurysm.64 TEE delineates the anatomical
details such as the site of origin by displaying the aortic
root and sinuses more clearly. Three-dimensional
echocardiography adds incremental value by further
defining the spatial relationships between the aneurysm
and the cavity into which it drains.
Associated echocardiographic findings include aortic
regurgitation resulting from altered aortic root anatomy.
However, if there is severe aortic regurgitation, one
must rule out extensive damage to the aortic leaflets
by endocarditis or acute rupture of sinus of Valsalva
aneurysm. Chronic small fistulae with a left-to-right shunt
cause left heart enlargement or right atrial enlargement
when the fistula drains into the right atrium.
Coronary Artery Fistula Fig. 75.13: Transthoracic echocardiogram (high parasternal

short-axis view) showing the site of drainage of a coronary artery
Coronary artery fistula is defined as an abnormal fistula. (LA: Left atrium; PA: Pulmonary artery; RVOT: Right ven-
tricular outflow tract).
communication between a coronary artery and a cardiac
TEE offers better spatial resolution and delineates VALVULAR DISEASE

the course of coronary artery fistula from its origin to
the chambers into which it is draining in multiplane Tricuspid Valve
views.67
Three-dimensional TEE with its ability to provide
Ebstein’s Anomaly
en face views has been reported to reveal morphology First described by Wilhelm Ebstein in 1866, this rare
and dimensions of the coronary artery fistulae more cyanotic CHD occurs in <1% of CHD with varying degree
comprehensively.68,69 of apparent apical displacement of the point of attachment
of the basal insertions of the septal and posterior leaflets of
the tricuspid valve.71 Due to failure of delamination, there
Cardiac Catheterization is adherence of tricuspid valve leaflets to the underlying
Cardiac catheterization is best tool for diagnosing and myocardium. In addition, there is apical displacement
tracing the entire course of the coronary artery. Coronary of the functional tricuspid valve annulus with the septal
angiography also allows accurate assessment of obstructive leaflets more apically displaced than the posterior,
followed by the anterior leaflets.72
coronary atherosclerosis is adults who are at higher
The malformed tricuspid valve may be incompetent,
cardiovascular risk. While small coronary fistulae do not
stenotic, or rarely, imperforate. If the right ventricle is
require closure, the larger ones may require percutaneous
subdivided into the inlet, trabecular, and outlet portions,
closure or surgery to avoid the risk for developing heart
then the displacement of the tricuspid orifice to the
failure or angina. junction of the inlet and trabecular ventricular zones
describes the essence of this anomaly.73
Magnetic Resonance Imaging/Computed Due to “atrialization” of the right ventricle, by the
downward displacement of the functional tricuspid
Tomography annulus, the right atrium appears larger than its actual size.
MRI/CTA are the best noninvasive imaging modalities The right ventricle, therefore, appears to be smaller and
for defining the course of the coronary arteries and the may have impaired function over time. The left ventricular
enlargement of the chambers. The number of incidentally function may also decrease in adulthood. The downward
detected coronary artery fistulae in the era of multidetector displacement of the septal tricuspid valve leaflet is
CT scanning has increased.65 associated with discontinuity of the central fibrous body
and septal atrioventricular ring, thus creating a potential
substrate for accessory atrioventricular connections and
Surgery for Sinus of Valsalva Aneurysms and ventricular pre-excitation, making the patient at risk for
Coronary Artery Fistulae sudden death. Right heart catheterization, therefore,
carries a risk of inducing ventricular arrhythmias leading to
Surgical repair, ligation, bypass, or percutaneous closure sudden cardiac death. On angiography, morphofunctional
should be considered before the development of potential abnormalities of the left ventricle have been demonstrated
complications such as cardiac failure, infectious endocarditis, in many patients, which may be explained by increased
embolization, pulmonary hypertension, arrhythmia, or fibrosis in the left ventricular wall and the ventricular
ischemia related to myocardial hypoperfusion, aortic regurgi- septum as demonstrated by histological studies.74
tation, dissection, rupture, or death.70 The anterior tricuspid leaflet may be fenestrated
and redundant, giving it a “sail-like” appearance. The
The Postoperative Adult septal leaflet may be tethered. The severity of tricuspid
regurgitation, presence of a PFO, or ASD determines
Postoperatively, adults need follow-up echocardiography clinical outcomes. This defect is often associated with
to rule out residual patch leaks, compression of surrounding pulmonary stenosis/atresia and VSDs, congenitally
structures, and resolution of chamber enlargement and corrected transposition of the great arteries, and tetralogy
assessment of ventricular function. of Fallot.31
The term “Ebstenoid” or “Ebstein-like” may be used to anterior mitral valve leaflet to the point of attachment
describe the broad spectrum of variations in morphology of the septal tricuspid valve leaflet. This view also shows
of the “typical” Ebstein’s valve. Although it is usually severity of tricuspid regurgitation, its impact on the right
diagnosed in childhood, milder variations may be noted atrial enlargement, and biventricular ventricular function
on echocardiography in adulthood when the individual (Fig. 75.15).
presents with dyspnea, decreasing exercise tolerance, The parasternal short-axis view may also help define
right heart failure, and worsening atrial arrhythmias. the tricuspid valve anatomy. Tricuspid valve features
The common arrhythmias are atrial fibrillation/flutter or associated with decreased functional capacity are absence
supraventricular tachycardia due to Wolff–Parkinson– of the septal leaflet, pronounced tethering, restricted
White (WPW) syndrome in 15% to 20% adults. Cyanosis leaflet motion, and displacement of the anterior leaflet.76
occurs in the presence of an atrial shunt (PFO or ASD) Associated defects such as pulmonary stenosis and shunts
that may also increase the risk of paradoxical embolism (PFO/ASD/VSD) are also to be followed-up on serial
leading to transient ischemic attack or stroke. echocardiograms. TEE with agitated saline contrast is
used to indentify a patent foramen ovale in adults with
Echocardiography poor acoustic windows. A synopsis of echocardiographic
assessment is reviewed in Table 75.10.
In the current era, Ebstein’s anomaly is most commonly
diagnosed by echocardiography.72 On transthoracic
echocardiography, the Ebstein’s tricuspid valve is best
seen in the apical four-chamber view with its characteristic Stress echocardiography with treadmill testing offers an
“sail-like” elongated anterior leaflet and the “downward objective assessment of functional capacity, ventricular
displacement” of the septal leaflet caused by the tethering contractile reserve, impact of exercise on arrhythmias, and
to the septum, making it appear as though it is attached cyanosis in adults with Ebstein’s anomaly.
apically in relation to the point of attachment of the anterior
mitral leaflet (Fig. 75.14). The diagnostic feature of Ebstein’s Magnetic Resonance Imaging/Computed
anomaly is an apical displacement of both the septal
and the posterior tricuspid leaflets, exceeding 20 mm or
Tomography
8 mm/m2 in adults.75 In an apical four-chamber view, this MRI/CTA allows quantification of biventricular function
distance is measured from the point of attachment of the and assessment of tricuspid valve morphology in adults
Fig. 75.14: Transthoracic echocardiogram showing Ebstein’s Fig. 75.15: Transthoracic echocardiogram showing Ebstein’s
valve in an apical four-chamber view with its characteristic “sail- valve in the apical four-chamber view associated with tricuspid
like” elongated anterior leaflet and the “downward displacement” regurgitation. (LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
of the septal leaflet. (LA: Left atrium; LV: Left ventricle; MV: Mitral RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).
valve; RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).
Table 75.10: Ebstein’s Anomaly

Associated defects
• Patent foramen ovale
• Ventricle septal defect
• Patent ductus arteriosus (PDA)
• Pulmonary stenosis or atresia
• Congenitally corrected transposition of the great arteries (CCTGA)
• Left ventricular noncompaction (LVNC)—40%
• Tetralogy of Fallot
• Identify the large “sail-like” anterior tricuspid leaflet
• Degree of tricuspid valve displacement (septal leaflet attachment)
• Degree of tricuspid regurgitation
• Right atrial enlargement—usually marked
Postoperative
• Degree of tricuspid regurgitation
• Right atrial enlargement
with limited acoustic windows, who are unable to have Tricuspid valve repair or replacement with PFO/ASD
a TEE. MRI allows better visualization of the posterior closure is performed in:
tricuspid valve leaflet, tricuspid valve fenestrations, • A symptomatic adult with right heart failure, declining
quantification of right heart size, and right ventricular exercise capacity, or progressive right ventricular
ejection fraction, while echocardiography is more dilation/impaired right ventricular systolic function
accurate in assessment of valve morphology and detecting with worsening atrial and/or ventricular arrhythmias
small shunt lesions. Echocardiography and MRI provide • Worsening cyanosis with oxygen saturation < 90%
complementary data for appropriate risk stratification • Transient ischemic attack or stroke attributed to
prior to surgery.72 paradoxical embolism
• Increased cardiac silhouette on imaging.
At the time of surgery for the tricuspid valve and
associated defects, the Maze procedure is performed for
Coronary angiography is performed before surgical atrial arrhythmias, since catheter-based ablation is far
intervention in patients at risk for coronary artery disease more challenging in adults with Ebstein’s anomaly due
or for assessment of pulmonary vasoreactivity in adults to multiple accessory pathways. For those with previous
with severe pulmonary hypertension. tricuspid valve surgery, reoperation is indicated for
impaired valvular function.
Tricuspid Valve Surgery
The ACC/AHA Class I recommendations for surgical
treatment for adults with Ebstein’s anomaly are the The postoperative adult with Ebstein’s anomaly needs fol-
following:3 low-up for tricuspid regurgitation postrepair, assessment
of prosthetic valve function, biventricular function, and < 30 mm Hg), moderate (peak valvular gradient between
residual shunts. 30 and 50 mm Hg), and severe (peak valvular gradient >
50 mm Hg). Silvilairat et al. raised a possibility that the
Pulmonary Valve mean Doppler gradient may be the preferred method for
accurately estimating the severity of the pulmonary valve
Pulmonary Stenosis stenosis, since it correlates best with the peak-to-peak
While the majority of the individuals have narrowing of the gradient obtained by cardiac catheterization. According
pulmonary valve at the valvular level, stenosis may occur to their study, estimation of only the peak (maximum)
at the subvalvular or supravalvular levels. Among the three valvular gradient may lead to overestimation of the severity
morphological types, the commonest one is the dome- of pulmonary stenosis.78 Further studies are required to
shaped pulmonary valve—a mobile valve with narrow central figure out why exactly Doppler mean pressure gradients
opening and systolic doming seen on echocardiography. and catheterization peak-to-peak pressure gradients seem
It is usually associated with main pulmonary artery to show good correlation.79
dilatation that results from structural abnormalities.11 The Long-standing unoperated severe pulmonary stenosis
three rudimentary raphe fuse to cause valve stenosis that leads to right ventricular hypertrophy, and systolic
may increase in severity due to calcification. Over time, flattening of the interventricular septum due to increased
pulmonary regurgitation may develop and the jet usually pressure overload on the right heart. Spectral Doppler
directed toward the left pulmonary artery. Other associated examination may reveal a “dagger-shaped,” late-peaking
defects are ASD, VSD, and infundibular or right ventricular systolic signal characteristic of significant dynamic
hypertrophy leading to dynamic obstruction of the right
obstruction of the right ventricular outflow tract.
ventricular outflow tract.31
The right ventricular size, mass, and systolic function
Dysplastic pulmonary valve has significant myxo-
along with pulmonary regurgitation and pulmonary artery
matous thickening of the valve leaflets that reduces leaflet
dimensions should be assessed on echocardiography. The
mobility. Associated commonly with Noonan syndrome,
pulmonary artery may be considered as dilated when: (a)
it may also manifest with narrowing of the pulmonary
annulus and the right ventricular outflow tract. a ratio of maximum pulmonary artery diameter (usually
Unicuspid or bicuspid pulmonary valve may rarely be measured at the point of bifurcation into right and left
seen in adults with tetralogy of Fallot. The quadricuspid branches) to the aortic diameter (measured at the level of
pulmonary valve has been reported as a rare finding in the aortic valve or 2 cm beyond the aortic valve) is ≥1.4; and
literature, with a rudimentary accessory cusp interposed (b) a ratio of pulmonary ring to the aortic ring diameters
between three cusps of same size in most cases. It may is ≥1.5.80 Nearly half the pulmonary artery aneurysms
present with stenosis/regurgitation and pulmonary artery are associated with CHD. Besides pulmonary valve
dilatation/aneurysm. The most common CHDs associated stenosis, they may occur in individuals with pulmonary
with it are ASD, VSD, PDA, and bicuspid aortic valve.78 regurgitation, ASD, PDA, VSD, tetralogy of Fallot, and
Eisenmenger syndrome. The presence of a thrombus
Echocardiography should be ruled out.81
TEE is very important for examining the cusp anatomy
The era of echocardiographic detection of the pulmonary
of the pulmonary valve in the short-axis view. The best
valve was heralded by Dr Nanda who published his
view to visualize this valve is a modified view, with the
findings in 1972.77
TEE probe anteroflexed between 135° and 145°, so that
On 2D transthoracic echocardiography, the parasternal
short-axis view at the level of the aorta is the best view for it aligns the pulmonary valve into the short axis and the
assessment of the pulmonary valve morphology and aortic valve into long axis.78 In individuals with limited
assessment of regurgitation or stenosis. The parasternal transthoracic windows, TEE also helps in delineating the
long-axis view displays the right ventricular outflow right ventricular outflow tract and lesions in the branch
tract. The level and severity of the stenoses are important pulmonary arteries.
in determining the timing for intervention or surgery. A synopsis of echocardiographic assessment is
Standard grading ranges from mild (peak valvular gradient reviewed in Table 75.11.
Table 75.11: Pulmonary Stenosis

Associated defects
• Peripheral pulmonary stenosis
• Type of valve—dome-shaped, dysplastic or quadricuspid
• Valve annulus
• Thickness of the leaflets
• Gradient—degree of stenosis
• Concomitant pulmonary regurgitation
• Pulmonary artery dimensions—dilatation, aneurysm or narrowing
• Subvalvular area—infundibular obstruction/hypertrophy
Postoperative
• Valve gradient—stenosis
• Concomitant pulmonary regurgitation
• Pulmonary artery dimensions—dilatation, aneurysm or narrowing
Cardiac Catheterization Pulmonary Valve Intervention (Valvotomy)

Cardiac catheterization is rarely necessary for diagnosis and Surgery (Replacement)
and is usually performed when catheter-based intervention The Class I ACC/AHA recommendations for interventional
is indicated. For confirmation, the gradients should be and surgical treatment for adults with valvular pulmonary
obtained at the valvular, sub-, and supravalvular levels. stenosis are the following:3
The presence of obstruction in the infundibulum as well Catheter-based intervention with balloon valvotomy in:
as main, branch, or peripheral pulmonary arteries should • Asymptomatic adults with a domed pulmonary valve
be ruled out. It is important to note again that the peak- with a mean Doppler gradient > 40 mm Hg (peak
to-peak gradient by cardiac catheterization correlates instantaneous Doppler gradient > 60 mm Hg) if the
best with the mean Doppler rather than with the peak degree of pulmonic regurgitation is less than moderate
instantaneous Doppler gradient.78 Right ventriculogram is • Symptomatic adults with a domed pulmonary valve
performed for assessment of the right ventricular function with a mean Doppler gradient > 30 mm Hg (a peak
and degree of pulmonary regurgitation. instantaneous Doppler gradient > 50 mm Hg) if the
degree of pulmonic valve regurgitation is less than
Magnetic Resonance Imaging/Computed moderate.
Surgery is performed for the following indications:
Tomography • Adults with severe pulmonary stenosis and associated
MRI/CTA are indicated for accurate imaging of the main, defects such ASD, severe pulmonary regurgitation,
branch, and peripheral pulmonary arteries, quantification or stenosis at other levels (subvalvular/supravalvular
of right ventricular systolic function and degree of pul- pulmonary stenosis or hypoplastic pulmonary
monary regurgitation. annulus)
• Dysplastic thickened pulmonary valve membrane because of fibrosis or calcification. Others

• Concomitant surgery is indicated for associated lesions may develop atrial fibrillation or mitral regurgitation.
such as severe tricuspid regurgitation, a surgical Maze The differential diagnosis includes a supravalvular mitral
for atrial fibrillation, or when the main pulmonary ring or left atrial dissection. Hemodynamically, it may
artery is markedly dilated (usually over 4 cm) and is mimic pulmonary vein stenosis or left atrial mass (tumor,
causing symptoms by compressing on contiguous thrombus, or cyst).83–85
structures.
Of note, there are no clear cut guidelines for surgery Echocardiography
in an adult who has a dilated main pulmonary artery
The best view is the apical four-chamber view that shows
that is associated with a dome-shaped pulmonary
a thin undulating membrane moving toward the mitral
valve. There is less likelihood of rupture of these low-
valve in diastole and away from it in systole. The proximal
pressure aneurysms. Hence, pulmonary arterioplasty or
chamber is usually dilated due to a “back up” of flow
main pulmonary artery replacement with a tube graft/
caused by obstruction through the perforated membrane
valved tube graft is performed when there are significant
into the “low pressure” distal chamber. The left atrial
symptoms due to compression on contiguous structures
appendage is usually normal in size. Color flow Doppler
or when concomitant surgery is otherwise indicated.
shows flow acceleration at the sites of perforations in the
Bioprosthetic pulmonary valves are chosen because,
membrane. The right ventricular systolic pressure should
despite anticoagulation, the low pulmonary artery
be estimated from the tricuspid regurgitant jet to rule out
pressure and slow blood flow puts these individuals at a
pulmonary hypertension.
high risk of valve thrombosis with mechanical prosthetic
It is important to establish the position of the left atrial
valves.
appendage in the distal chamber in order to confirm cor
triatriatum and exclude a supravalvular mitral ring.86
The Postoperative Adult Transesophageal echocardiography is useful in
Besides the assessment of native/prosthetic valve confirming the diagnosis by clearly delineating the
function, the main pulmonary artery should be evaluated position of the left atrial appendage. It also allows complete
for dilatation. On long-term follow-up after surgery, assessment of any associated intracardiac defects such as
increasing severity of pulmonary regurgitation has been the patent foramen ovale.83 Contrast echocardiography is
reported. Following percutaneous balloon angioplasty, an important adjuvant technique where the perforation in
reinterventions may be required for recurrence of the membrane cannot be delineated by color Doppler. It
pulmonary stenosis, and mild pulmonary regurgitation is shows a differential opacification of the proximal versus
common.82 the distal chamber with delayed emptying of contrast into
the distal chamber through the communication between
the chambers.85 Three-dimensional reconstruction is
Mitral Valve
useful in diagnosing cor triatriatum by defining atrial
Cor Triatriatum membranes and the associated defects.87
Although these echocardiographic modalities may
Also known as cor triatriatum sinister, this is a very rare suffice, in some cases a left and right heart catheterization
cardiac defect that may go undiagnosed into adulthood with simultaneous pulmonary capillary wedge and left
especially when it presents as an isolated anomaly. It ventricular end-diastolic pressure measurements may be
is characterized by a perforated membrane (partial or required to reveal a significant mean gradient, prior to
complete) dividing the left atrium into two chambers— definitive surgical resection of the membrane.
the proximal (pulmonary venous chamber) receives the
pulmonary veins, and the distal “true” chamber comprises
the left atrium with the fossa ovalis and left atrial
Cleft Mitral Valve
appendage.31 Associated defects include patent foramen A cleft mitral valve can occur as an isolated defect or
ovale, secundum ASD, and rarely a common atrium. in association with other CHD. The cleft can be in the
Adults usually present with symptoms similar to mitral anterior leaflet usually in association with a primum ASD
stenosis due to obstruction to flow through the perforated or may occur in the posterior leaflet.88,89 Other defects
associated with a cleft mitral valve are atrioventricular coarctation of aorta, VSD, and subaortic stenosis. It may
septal defect, secundum ASD, VSD, coarctation of aorta, occur in association with more complex defects such as
double outlet right ventricle, and tricuspid atresia. The Shone’s syndrome, transposition of the great arteries, truncus
long-term sequelae are left atrial enlargement and left arteriosus, tricuspid atresia, Ebstein’s anomaly, and double
ventricular enlargement with impaired function due to outlet right ventricle.91
progressive mitral regurgitation. These adults are also at The clinical presentation of DOMV is variable and
risk for developing endocarditis, atrial arrhythmias, and may go undiagnosed until adulthood unless there
pulmonary hypertension. are significant mitral regurgitation, mitral stenosis, or
It is best seen in the parasternal short-axis view associated cardiac defects. Mitral regurgitation appears to
(Fig. 75.12). The direction of mitral regurgitation jet and occur more frequently than mitral stenosis.
its severity can be assessed based on the data compiled
from the parasternal long-axis, apical two-chamber, four- Echocardiography
chamber, and subcostal views. The 3D echocardiography
en face view shows the location of the cleft in relation to the It is usually best seen in the parasternal short-axis view.
scallops of the mitral leaflet most clearly.90 Other views are the subcostal short-axis view at the level
The indications for surgery are similar to those for of the mitral valve and the apical four-chamber view.
acquired mitral regurgitation. Surgical treatment involves Das et al. described the transthoracic echocardiographic
reconstruction with ring annuloplasty or mitral valve views that help in determining various morphology of
replacement. the DOMV; for the DOMV with complete bridging, the
best views are the parasternal or subcostal short-axis
with a sweeping of the transducer from base to apex; for
Double Orifice Mitral Valve DOMV with incomplete bridging, a single opening of the
In this rare congenital malformation, abnormal fusion of the mitral valve appears on basal cuts in the short-axis views
endocardial cushion leads to two equal or unequal mitral and then the double orifice becomes apparent only when
valve orifices. Double orifice mitral valve (DOMV) with the transducer is carefully swept toward the apex; for the
intact atrioventricular septum can present as reduplication hole-type DOMV, an apical four-chamber is the preferred
of the orifice caused by two separate valve orifices supported view. In their study, they reported that a smaller, accessory
by their own separate subvalvular apparatus. On the other orifice was located in the anterior leaflet of the mitral valve
hand, it can occur in association with an atrioventricular (AV) in all cases.92
septal defect with a fibrous bridge across the mitral orifice. The mitral valve may rarely be normal functioning
There may be abnormal papillary muscle rotation, fusion, and more often be stenotic or regurgitant. The mitral valve
or a single papillary muscle. Besides partial or complete area is calculated as the sum of the area of the two mitral
AVSD, other associated defects include atrial septal defect, orifices (Figs 75.16A and B). Isolated DOMV with normally
A B
Figs 75.16A and B: Transthoracic echocardiogram showing that the mitral valve area in an adult with a double orifice mitral valve (DOMV) is
calculated as the sum of the area of the two separate mitral orifices (A and B), in the parasternal short-axis views by planimetry.
functioning valves may go unrecognized until later in Transesophageal Echocardiography

life and then only discovered as an incidental finding.93
TEE offers a more detailed analysis of the defect and
DOMVs presenting in association with other defects, such
isolated defects in multiplane views. It often allows
as atrioventricular (AV) septal defect, VSD, ASD, Ebstein’s
differentiation between a true parachute and parachute-
anomaly, tetralogy of Fallot, left heart obstructive lesions,
like mitral valve. In a parachute-like mitral valve, most
and coarctation of aorta, are more likely to be diagnosed in
of the chordae tendinae are attached to a main papillary
childhood and undergo surgical repair or replacement of
muscle while the other papillary muscle is hypoplastic and
the mitral valve.
close to the main one.98 TEE should also be considered
when the severity of stenosis or regurgitation indicates
Parachute Mitral Valve surgery in accordance with the ACC/AHA guidelines for
The parachute mitral valve is a variant of abnormal valvular disease.99
attachment of the mitral valve chordae to a solitary or fused The ACC/AHA Class I recommendations for surgical
treatment for young adults with mitral valve disease are
papillary muscle. This can result in mitral stenosis due to
the following:99
a restrictive valve opening, as subvalvular obstruction
Congenital Mitral Stenosis:
due to fused chordae, or it can rarely present with mitral
• Symptomatic young adult patients with congenital
regurgitation because of prolapsed mitral valve. It can
mitral stenosis and NYHA functional Class III or IV,
also present as an isolated anomaly or part of the Shone’s
who have a mean mitral valve gradient > 10 mm Hg on
complex, which is characterized by a supravalvular
Doppler echocardiography.
membrane or ring, parachute mitral valve, subaortic Other Class I indications that apply to all mitral valve
stenosis, and coarctation of aorta.94 It may occur with disorders include:
other lesions such as an ASD or bicuspid aortic valve with • Symptomatic adults with NYHA functional Class
stenosis. Most cases present in infancy and are operated III–IV, who have moderate or severe mitral stenosis
early.95,96 In rare cases, parachute mitral valve with measured by other methods when percutaneous
Shone’s complex may go undiagnosed into adulthood.97 mitral balloon valvotomy is either not available;
The majority present with dyspnea, decreasing exercise contraindicated because of concomitant moderate
tolerance, or atrial fibrillation due to stenosis or to severe mitral regurgitation; persistent left atrial
regurgitation, while in others the finding is incidental. thrombus despite anticoagulation; or unfavorable
mitral valve morphology.
Echocardiography
The transthoracic parasternal long-axis and short-
Mitral Regurgitation
axis views (at the level of the papillary muscle), apical Mitral valve repair is preferred over MV replacement in
four-chamber and two-chamber views are valuable most cases:
in assessment of the morphology of the mitral valve, • Symptomatic adult with severe congenital mitral
subvalvular apparatus, papillary muscle, and associated regurgitation with NYHA functional Class III or IV
left heart lesions. The parasternal long-axis view may show symptoms
• Asymptomatic adult with severe congenital mitral
chordae tendinae converging to the single papillary muscle
regurgitation and left ventricular ejection fraction
either as short, thickened, underdeveloped, and adherent
≤ 60%
causing stenosis, versus elongated and floppy without
• Severe mitral regurgitation with NYHA functional
adequate coaptation of the mitral valve leaflets/prolapse
Class II, III, or IV symptoms in the absence of severely
resulting in mitral regurgitation. In the parasternal short- reduced left ventricular function (ejection fraction
axis view at the midpapillary level, a single papillary is < 30%) and/or end-systolic dimension > 55 mm
visualized posteromedially and the mitral orifice may be • Asymptomatic adult with chronic severe MR with
eccentric. In the parasternal short-axis view at the basal mildly to moderately reduced left ventricular function
level, parachute leaflets described as typical for this (ejection fraction between 30% and 60%, and/or end-
condition are often seen. systolic dimension ≥ 40 mm).
Aortic Valve Aortic root dilatation is commonly seen in adults with

bicuspid aortic valve and they are at least a ninefold greater
Bicuspid Aortic Valve risk for aortic dissection.106
Biscuspid aortic valve (BAV) is defined by the presence of
complete or partial fusion of two aortic leaflets, often mani- Echocardiography
festing as right and left leaflet fusion, otherwise as right In the transthoracic parasternal long-axis view, doming
and noncoronary cusp fusion, or as left and noncoronary of the bicuspid aortic valve leaflets in systole can be
cusp fusion.31 While right and left leaflet fusion is more appreciated in most young adults who are unlikely to
often associated with stenosis/regurgitation requiring have distorted dysplastic valve or restricted mobility due
early intervention, the right leaflet and noncoronary to calcification of the valve. The parasternal short-axis
cusp fusion is more likely to be associated with aortic view (at the aortic valve level), is the best view for defining
root dilatation.100–102 The cusp size may be unequal due to valve anatomy by showing fusion between two cusps (one
fusion of two cusps leading to one larger cusp. A central smaller and one larger) or a variation of it. The extent of
raphe may divide the larger of the two cusps at the site of commissural fusion determines degree of stenosis or
congenital fusion of two parts of the conjoined cusps. obstruction. In this view, the open bicuspid aortic valve
Although many adults are previously diagnosed with a appears to look like an elliptical “American football” or
bicuspid aortic valve because of a heart murmur, in others “fish-mouth” opening (Fig. 75.17).
the diagnosis is first made when they acquire endocarditis Two-dimensional echocardiography and continuous
and present with acute severe aortic regurgitation due wave Doppler are routinely used to determine the severity
to aortic leaflet destruction or perforation, leading to of valve stenosis using the same criteria as in the general
heart failure. Secondary infection of the mitral valve due population, defined by the ACC/AHA valvular heart disease
to contiguous spread of the infection is known to occur. guidelines.99 The peak aortic velocity should be assessed
Destruction of the infected valves causes regurgitation by continuous wave by Doppler echocardiography in
and the vegetations increase the risk of embolic stroke. multiple views and the highest values should be recorded
Sepsis is common in this scenario. Siniawski et al reported (usually best captured in the suprasternal views). The peak
high mortality (approximately 29%) in adults who have and mean gradients are derived accordingly. Since the
endocarditis with aortic ring abscess and secondary
infection of the mitral valve requiring double valve surgery.
According to their study, the most potent independent risk
factors for mortality were septic shock and severe aortic
root destruction.103
First-degree relatives of patients with bicuspid
aortic valve should be screened by echocardiography
for the presence of a bicuspid aortic valve, since familial
reoccurrence of bicuspid aortic valve is approximately 9%,
mostly likely due to an autosomal dominant pattern of
inheritance with reduced penetrance.104
Severe calcification or regurgitation can be present in
third or fourth decade, often accelerated by risk factors
such as hyperlipidemia. The calcification and fibrosis
primarily occurs at the raphe and base of the cusps.105
Aortic regurgitation may result from inability of the Fig. 75.17: Diagrammatic representation of the echocardiographic
leaflets to coapt due to aortic root dilatation and loss of the parasternal short-axis views showing morphological features of
the (1) closed normal trileaflet (Mercedes Benz sign) aortic valve
sinotubular junction, retraction of the leaflets caused by as seen on a transesophageal echocardiogram, and (2) an open
fibrosis, prolapse of aortic cusps, aortic valve attempting bicuspid (American foot ball), (3) open unicuspid (tear drop) (a)
to close a perimembranous VSD (“venturi effect”), or unicommissural, (b) acommissural (4) a closed quadricuspid (X-sign)
aortic valve as seen on transthoracic parasternal short-axis views.
following endocarditis due to extensive valve destruction.
peak instantaneous aortic valve gradient may overestimate Three-dimensional echocardiography is useful in
the severity of stenosis, the mean gradient is preferred. defining the morphology of the aortic valve. In adults
It correlates more closely with the peak-to-peak gradient with good quality transthoracic images, it may define
traditionally measured during cardiac catheterization. The thickening or redundancy of the aortic valve leaflets with
more universally accepted grading system is the following: multiple folds. It also helps in identifying vegetations and
mild—valve area > 1.5 cm2, mean gradient < 25 mm Hg, localizing perforations.110
or jet velocity < 3.0 m/s; moderate—valve area 1.0–1.5 cm2, Another variant of congenital aortic valve defects is the
mean gradient 25–40 mm Hg, or jet velocity 3.0–4.0 m/s; or unicuspid aortic valve, described as a single commissure
severe—valve area < 1.0 cm2, mean gradient > 40 mm Hg, or with two poorly developed cusps. The short-axis view of the
jet velocity > 4.0 m/s. aortic valve clinches the diagnosis on TTE or TEE. During
Aortic regurgitation is quantified as mild, moderate, diastole, the raphae may look like true commissures giving
or severe, according to the American Society of
it the “Mercedes Benz” sign appearance of a trileaflet
Echocardiography criteria using standard methods as
aortic valve. During systole, there is no cusp separation,
in acquired valve disease.107 Aortic regurgitation may be
and the unicuspid valve looks like a “teardrop” with an
evaluated by color Doppler in the parasternal long-axis,
short-axis, apical four-chamber, and five-chamber views. eccentric opening (Fig. 75.17). The more common type of
The rate of deceleration of the velocity signal of aortic unicuspid aortic valve is unicommissural and the other
regurgitation, the presence of retrograde flow (diastolic one, acommissural (with a lateral attachment to the aorta
flow reversal) in the proximal descending/abdominal at the level of the orifice). The origin of the coronaries
aorta on continuous wave Doppler echocardiography, should be normal due to normal development of the
and the ratio of proximal jet area to left ventricular outflow sinuses of Valsalva in these individuals.111,112
tract area in combination with the ratio of jet height to left In even rarer cases, there is development of an
ventricular outflow tract height allow assessment of the extra commissure during valvulogenesis, resulting
severity of aortic regurgitation. in a quadricuspid aortic valve. On transthoracic
The left ventricular size, function, and mass are echocardiography and more confidently with a TEE, it is
assessed on serial echocardiograms and are important best identified in the short-axis view at the level of the aortic
criteria in determining the need for surgery and long-term
valve during diastole, when it appears like an “X” sign (Fig.
clinical outcomes. Associated defects include coarctation
75.17). The 3D echocardiogram defines the anatomy of the
of the aorta, VSD, PDA, subaortic stenosis, and parachute
four cusps more clearly.113
mitral valve.31
Like the BAV, all other congenital malformations of the
Intrinsic structural abnormalities of the aortic wall
can lead to aortic root dilatation that can in turn progress aortic valve are prone to an increased rate of calcification and
to an aneurysm that carries risk of dissection five- to fibrosis leading to significant stenosis and/or regurgitation,
ninefold higher than in the general population.106 Other thereby requiring aortic valve replacement.114 They are also
contributory factors for increase in aortic diameters are associated with aortic root dilation in majority of the cases.
worsening aortic regurgitation, high body surface area, and The risk of aortic dissection is 18-fold more in individuals
advancing age.108,109 In the parasternal long-axis view, the with a unicuspid aortic valve.106
aortic root should be routinely measured in systole, from A synopsis of echocardiographic assessment of the
leading edge to leading edge, at the levels of the annulus, bicuspid aortic valve is reviewed in Table 75.12.
midsinus level, sinotubular junction, and the proximal
aorta.108 It is important to report these measurements in Cardiac Catheterization
all the cases, since progressive aortic dilatation may occur
in this population even in the absence of significant aortic Cardiac catheterization is recommended for assessment
stenosis and regurgitation. Depending upon the size of of coronary arteries before aortic valve surgery in
the aorta, imaging should be performed every other year adults who are at risk for coronary artery disease. It is
if the maximum dimensions are < 40 mm, and performed also recommended when a pulmonary autograft (Ross
annually if they are ≥ 40 mm. Unfortunately, replacement operation) is being considered, so that the origin of the
of the bicuspid aortic valve has not been shown to reduce coronary arteries can be defined in case CTA/MRI are not
the risk of further dilatation. being performed. It is of incremental value when there is
Table 75.12: Bicuspid Aortic Valve

Associated defects
• Aortic root dilatation/aneurysm or dissection
• Subaortic and supravalvular (aortic) stenosis
• Type of valve—fusion of raphe
• Valve annulus
• Thickness of the leaflets
• Gradient—degree of stenosis
• Concomitant aortic regurgitation
• Aortic root dimensions—dilatation or aneurysm
• Subaortic obstruction/hypertrophy—left ventricular outflow tract gradient
• Left ventricular size mass and function
Postoperative
• Assessment of valve gradient
• Grading aortic valve regurgitation/paravalvular leak
• Aortic root dimensions to rule out dilatation/aneurysm or dissection
a discrepancy between symptoms and echocardiographic Magnetic Resonance Imaging/Computed

findings.
Most adults will require aortic valve replacement.
Tomography
Catheter-based percutaneous balloon valvuloplasty is a The aortic root, arch, and the descending thoracic
possibility in a young adult with an isolated severe aortic aorta should be evaluated by either of these imaging
stenosis and noncalcified pliable valves, or it may offer modalities prior to aortic valve replacement or when
temporary relief in a markedly symptomatic pregnant echocardiography is suspicious for an enlarging aneurysm.
woman with severe stenosis refractory to medical
management during late second or third trimesters. Stress Testing
The ACC/AHA Class I indications for aortic valvotomy
in adolescents and young adults are the following:99 Although stress testing with exercise may be performed
• Symptoms such as angina, syncope, or dyspnea on cautiously to assess functional capacity and blood
exertion with a left ventricular peak-to-peak gradient pressure response in asymptomatic adults who do not
across the aortic valve of 50 mm Hg or higher in have critical stenosis, it is contraindicated in symptomatic
the absence of heavy calcification, on cardiac adults, in those with repolarization abnormalities on ECG,
catheterization or systolic dysfunction on echocardiography.
• Asymptomatic patient with a left ventricular peak-to- Dobutamine stress testing can be performed
peak gradient across the aortic valve > 60 mm Hg on judiciously in adults with low-gradient aortic stenosis in
cardiac catheterization the setting of low left ventricular ejection fraction, since
• Asymptomatic patient with ECG changes (ST or the low cardiac output may mask a higher grade of
T-wave changes over the left precordium) at rest or stenosis.
with exercise and left ventricular peak-to-aortic valve The ACC/AHA Class I indications for aortic valve
gradient > 50 mm Hg. replacement are the following:3
Aortic Stenosis: Although it usually presents as an isolated defect, the

• Severe aortic stenosis or chronic severe regurgitation subaortic fibrous ring may extend onto the anterior mitral
in an adult undergoing coronary artery bypass graft leaflet, accessory mitral tissue, or anomalous chords
surgery or any other cardiac surgery of the aorta or causing subaortic aortic stenosis. It is often associated
concomitant defect with a perimembranous VSD or an atrioventricular
• Severe aortic stenosis with left ventricular function ventricular septal defect. Also, it may become more visible
< 50% after ventricular septal patch closure.
• Symptomatic severe aortic regurgitation with left Long-term residua and sequelae include progressive
ventricular ejection fraction < 50% and left ventricular aortic valve damage and aortic regurgitation due to
dilatation increased turbulence in the subaortic left ventricular
• Aortic root surgery is indicated when the ascending outflow tract, in more than half the cases. Increasing
aorta diameter is ≥ 5.0 cm or when it is increasing by ventricular hypertrophy due to pressure overload
5 mm or more per year. eventually leads to impaired left ventricular function.
These adults are also at risk for infective endocarditis and
Aortic Regurgitation:
sudden cardiac death.115,116
• Symptomatic (angina, syncope, or dyspnea on
exertion) chronic severe aortic regurgitation
• Asymptomatic adult with chronic severe aortic Echocardiography
regurgitation with reduced systolic function (ejection Subaortic stenosis is best seen in the parasternal long-axis
fraction < 50% confirmed on two echocardiograms view that delineates the left ventricular out flow tract with
(1–3 months apart) the transducer positions perpendicular to the membrane.
• Asymptomatic chronic severe aortic regurgitation with The left ventricular outflow tract (LVOT) obstruction
progressive left ventricular enlargement (end-diastolic is examined carefully with color flow Doppler, since
dimension > 4 standard deviations above normal) the obstruction may present at multiple levels. Aortic
should receive aortic valve repair or replacement. regurgitation may occur due to long-standing subaortic
flow disturbance.
The Postoperative Adult In most adults, 2D transthoracic echocardiography
defines the anatomy of the subaortic stenosis, the
Echocardiography is useful for follow-up of prosthetic dimensions of the ascending aorta, adjacent mitral valve
valve function, left ventricular size and function, aortic root involvement, degree of left ventricular hypertrophy, and
dimensions, and for estimating right ventricular systolic function (both systolic and diastolic). Sometimes a thin,
pressures postoperatively. The aortic root dimensions need wispy, discrete fibrous subaortic ring may not be clearly
long-term follow-up, since significant enlargement of the visible on transthoracic echocardiography. Doppler helps
ascending aorta with aneurysm formation and dissection in estimating the degree of aortic valve regurgitation. The
continues even after valve replacement.104 When aortic root severity of the gradient across the left ventricular outflow
replacement with coronary reimplantation is performed, tract and the aortic valve can be determined by continuous
there is a risk of coronary ostial obstruction. Potential wave Doppler (Fig. 75.18).
prosthetic valve complications include periprosthetic The location of a VSD has an impact in the estimating
regurgitation with or without hemolysis, obstruction of the severity of subaortic stenosis. The degree of
related to pannus or thrombosis, and endocarditis. subaortic stenosis may be underestimated when the VSD
is proximal to subaortic obstruction and overestimated
Subaortic Stenosis when it is distal to it. As in the case of aortic stenosis,
reduced left ventricular systolic function will also lead
Subaortic stenosis is a congenital obstruction below the to underestimation of the gradient. Among the factors
aortic valve that may present as a discrete “shelf-like” affecting progression of the left ventricular outflow tract
fibrous ring or as a fibromuscular “tunnel-type” defect obstruction are the position of the membrane adjacent to
causing a “fixed” left ventricular outflow tract obstruction the aortic valve and whether it extends toward the mitral
below the aortic valve.31 valve.
End-hole or micromanometer-tipped catheters are

required for confirming the left ventricular outflow tract
gradient accurately. Timely surgery is important, since it
may be able to reduce the progression of left ventricular
hypertrophy and aortic regurgitation.
Surgical Resection of Subaortic Stenosis

The ACC/AHA Class I indications for surgical intervention
in adults with subaortic stenosis are the following:3
• Severe obstruction with peak instantaneous gradient
> 50 mm Hg or a mean gradient > 30 mm Hg
• Progressive aortic regurgitation with a left ventricular
end-systolic diameter of 50 mm or more or a left
Fig. 75.18: Continuous wave Doppler showing the severity of ventricular ejection fraction < 55%.
the gradient across the left ventricular outflow tract with Valsalva
maneuver in a patient with subaortic stenosis.
The left ventricular outflow tract should to be assessed
Transesophageal Echocardiography with 2D transthoracic echocardiography and Doppler in
the parasternal long-axis and apical four-chamber views,
TEE is useful preoperatively and intraoperatively, in adults
to rule out recurrence of subaortic stenosis or progression
with limited acoustic windows, to define the morphology
of aortic regurgitation. The postoperative residua and
of the subaortic stenosis, and assess associated defects.
Intraoperative TEE evaluates adequate resection of the sequelae in an adult with discrete “shelf-like” fibrous ring
subaortic membrane or ridge and rules out residual aortic are iatrogenic VSD, aortic and mitral regurgitation due to
regurgitation. It also confirms the absence of iatrogenic extensive resection of interventricular septum, and injury
damage to the anterior mitral valve leaflet or creation of a to the mitral valve. Pacemaker wires may be seen in the
VSD by aggressive resection of the membrane. Similarly, right heart chambers of those who suffered a conduction
3D echocardiography may further define left ventricular system injury during surgery.
outflow anatomy.
Supravalvular Aortic Stenosis
Exercise Stress Testing
In adults, supravalvular stenosis may present with
Stress echocardiography is used to determine any increase hypertension or symptoms suggestive of ischemia. In this
in gradient with exercise in symptomatic adults with a defect, there is a fixed obstruction extending from below
peak gradient < 50 mm Hg. the sinotubular junction distally into the aortic root. Even
though the obstruction usually occurs distal to the origin
Magnetic Resonance Imaging/Computed of the coronary arteries, there may be ischemia due to
Tomography limited diastolic flow in adults with high systolic pressures
that also cause left ventricular hypertrophy. In some
In adults with limited acoustic windows who are unable to
adults, there may be coronary ostial obstruction (partial
undergo a TEE, an MRI or CTA plays an important role in
or complete) or abnormalities of the coronaries such as
defining the anatomy and associated defects.
ectasia.117
In children with Williams syndrome, supravalvular
Cardiac Catheterization stenosis is the most common congenital heart defect.
Preoperatively, cardiac catheterization is performed in Associated defects include peripheral pulmonary artery
adults who are at high risk for coronary artery disease. stenosis and hypoplastic aorta.31
Echocardiography • Symptomatic supravalvular stenosis with Doppler

echocardiography demonstrating a peak (instan-
The left ventricular size, mass, systolic, and diastolic taneous) gradient > 70 mm Hg and a mean gradient
function should be assessed on serial echocardiograms in over 50 mm Hg
adults with supravalvular aortic stenosis. In most adults, the • Significant symptoms such as angina, dyspnea, or
parasternal long-axis view demonstrates the morphology syncope in the setting of left ventricular hypertrophy
of the aortic root and allows measurement of the diameter and/or impaired left ventricular function.
at the levels of the annulus, midsinus, sinotubular junction,
and the ascending aorta 2 cm distal to the sinotubular
junction. In others, the tubular portion of the aorta is not
well visualized and the best views are the suprasternal or Long-term follow-up is required to assess left ventricular
high right parasternal views. Increased continuous wave outflow tract obstruction, left ventricular mass/function,
Doppler gradient across an anatomically normal aortic and aortic and mitral regurgitation. There is a lifelong risk
valve should alert the echocardiographer about a possible of coronary involvement, left ventricular hypertrophy, and
obstruction. Pressure recovery phenomenon may be seen diastolic dysfunction due to hypertension. Adults with
in adults with long-segment or tubular stenosis. previous patch repair of the hypoplastic aorta are at risk of
Transesophageal echocardiography is useful in aneurysm formation at the site of patch repair.
obtaining this information in adults with limited acoustic
windows on transthoracic echocardiography. It also COMPLEX CONGENITAL HEART
defines the origins of the coronary arteries.
DEFECTS
Stress Echocardiography Complex defects are those that usually present as a group
of anomalies centered around a major condition.
In adults presenting with symptoms and signs suggestive
of ischemia, stress echocardiography can be performed
to assess for coronary involvement and evaluation of the
Coarctation of Aorta
gradient across the stenosis before and after exercise. Coarctation of aorta (COA) is discrete or segmental
narrowing of the aorta below the origin of the left
Magnetic Resonance Imaging/Computed subclavian artery, at the junction of the distal aortic arch,
and the descending aorta (in most cases) or its variation.31
Tomography It is appears more likely to be a diffuse arteriopathy with
Extracardiac structures—aorta, pulmonary artery, and associated structural abnormalities of the great arterial
its branches, coronaries as well as the left ventricular walls that are not just limited to focal stenosis. Hence,
outflow tract are best delineated by MRI/CTA. In Williams it is no longer considered as a “simple” defect.118 The
syndrome, the entire aorta and the renal arteries should be commonest associated defects are a bicuspid aortic valve
imaged to look for peripheral stenosis. followed by mitral valve disease. These individuals are also
prone to aortic root dilatation.
Cardiac Catheterization Prior to the era of surgeries or interventions, the
survival was only 50% by 32 years of age.119 While many
For definitive diagnosis of coronary involvement and for
individuals are operated in childhood in the present
accurately measuring the gradient across the supravalvular
era, some are still diagnosed in adulthood during an
stenosis, angiography is the preferred diagnostic imaging
evaluation for secondary hypertension.120 Despite relief of
modality.
stenosis by surgery or catheter-based intervention, adults
have long-term residua, sequelae, increased morbidity,
Surgery for Supravalvular Stenosis and reduced life spans. The most common issue is
The ACC/AHA Class I recommendations for surgical ambulatory hypertension and significant left ventricular
treatment for adults with supravalvular stenosis are the hypertrophy seen on an ECG or an echocardiogram.
following: Despite successful COA repair, recurrence at the site of
stenosis (recoarctation) or aneurysmal dilatation at the site Marx et al. had noted that the major pitfall of
of repair are not uncommon. If left uncorrected, these are echocardiography in COA was due to the significant
associated with high morbidity and mortality. Aneurysms collateral flow resulting in the failure to record an accurate
are most often seen at the site of coarctation repair but Doppler gradient.125 In the absence of collateral flow,
may involve the descending thoracic aorta and the distal Therrien et al. reported that echocardiography was a
aortic arch.121 Predictors of aneurysmal formation at the suitable screening test with a sensitivity of 87%.126 Limited
site of coarctation repair include the use of the patch visualization of the aortic arch has been another issue with
graft technique and late correction of coarctation. End- transthoracic echocardiography.
to-end repair of COA is associated with the lowest risk of An abnormal Doppler flow pattern may also be noted
aneurysmal formation at the site of repair. in the abdominal aorta, with decreased pulsatility and
Adults with a bicuspid aortic valve who have high absence of early diastolic flow reversal. The abdominal
preoperative pressure gradients are at higher risk of aortic Doppler profile will not show diastolic flow reversal
having an aneurysm of the ascending aorta, especially in such patients without a discrete obstructive lesion.
following late repair of COA. Aortic root aneurysms are Conversely, it is a useful technique for screening an obscure
also associated with morbidity and mortality due to a high obstruction of significance that will manifest by delayed
prevalence of severe aortic regurgitation, dissection, and systolic upstroke, diastolic flow reversal (antegrade flow),
rupture.122 Another site for aneurysm rupture is in the and turbulence during systole on color/spectral Doppler
circle of Willis. Adults with COA have been noted to have examination of the upper abdominal aorta. Abnormal
a higher prevalence of premature coronary artery disease. flow in collateral vessels can be detected by color flow and
A recent study points out that COA lesion alone does pulsed wave Doppler.
not predispose to premature coronary artery disease.123 Adults with coarctation often have a bicuspid aortic
Hypertension, vascular, and endothelial factors are more valve and aortic root dilatation. Therefore, it is important
likely the associated culprits. to measure the transverse dimensions at the level of the
aortic annulus, aortic sinuses, sinotubular junction, and
Echocardiography approximately 2 cm distally in the ascending aorta.
Due to long-standing hypertension and multiple
Two-dimensional echocardiography is highly specific factors, many adults are prone to hypertensive heart
in diagnosing aortic arch obstruction.124 The aortic disease and need assessment of left ventricular size,
arch should be first interrogated carefully by 2D mass (left ventricular hypertrophy), systolic, and diastolic
echocardiography with pulsed Doppler recording, under function. Other associated defects are ventricular septal
direct visualization. On transthoracic echocardiography, defects, subaortic stenosis, patent ductus arteriosus, and
the coarctation is best seen in the suprasternal or high right mitral valve disorder (supramitral ring).
parasternal views, also allowing visualization of the aortic A synopsis of the echocardiographic assessment of
arch and proximal descending aorta. Presence of color coarctation of aorta is reviewed in Table 75.13.
flow Doppler turbulence in the aortic arch brings to our
attention the maximum site of narrowing. Continuous
wave spectral Doppler interrogation is used to assess
the gradient. It shows characteristic flow profile with an Agrawal et al. showed the incremental value of TEE in
increased systolic flow velocity and continuous gradient delineating the aortic arch branches. Their protocol can
of forward diastolic flow. The more accurate formula be adapted for the multiplane TEE probe.127 The short-axis
for estimating the pressure gradient is 4(V2-V1)2 with view of the proximal descending aorta is first identified as
V1 being the velocity proximal to the coarctation and V2 a circular cavity in the transverse plane in the midthoracic
being the peak velocity at the site of maximal narrowing. level. Further withdrawal of the probe brings the aortic
This formula avoids inaccurate reports of high pressure arch into view with the loss of the circular configuration
gradients obtained by the simplified Bernoulli equation transitioning into a horizontal tube-like structure.
(4V2) in patients with long tubular narrowing of the aortic Switching from the transverse to the longitudinal plane (90°)
arch.125 A peak gradient of over 20 mm Hg indicates a demonstrates all three vessels (Fig. 75.1). The longitudinal
significant obstruction. However, the gradient may be cut of the aortic arch makes it appear circular or ovoid.
low or negligible if there are collaterals or ductal flow. Small movements of the probe (rotation, movement,
Table 75.13: Coarctation of the Aorta

Associated defects
• Bicuspid aortic valve
• Left heart obstructive lesions—subaortic stenosis, mitral stenosis
• Aortic root/arch dilatation or aneurysm
• Aortic arch anatomy—rule out aneurysm
• Aortic arch gradient
• Assessment of associated defects
• Left ventricular hypertrophy
• Left ventricular function
• Stress echocardiography for evaluation of ambulatory hypertension and coronary ischemia
Postoperative
• Rule out recoarctation or aneurysm at the site of the surgery (especially with patch repair)
• Left ventricular hypertrophy (hypertension usually persists despite surgical relief of the coarctation)
• Stress echocardiography for evaluation of ambulatory hypertension and coronary ischemia
and angulation) allow visualization of the aortic arch. of nonhypertensive patients with COA who are at increased
Clockwise rotation of the probe moves the vertical plane risk of developing of arterial hypertension.129
to the right bringing the innominate artery into view as
the most anterior vessel, while counterclockwise rotation Stress Echocardiography
moves it to the left, and reveals the left carotid and the left
subclavian arteries more posteriorly.127 The X-plane view Stress echocardiography with color and continuous wave
(Philips Echocardiography System) allows simultaneous Doppler from the suprasternal notch allows assessment
views of the transverse and longitudinal planes. of the coarctation gradient at rest and following exercise,
in addition to providing information obtained by the
treadmill stress testing. Coronary artery disease occurs
Treadmill Stress Testing prematurely in this population, especially in those with
Treadmill stress testing plays a very important role uncontrolled ambulatory hypertension.
in determining the maximum exercise systolic blood
pressure, which is a predictor for chronic hypertension Magnetic Resonance Imaging/Computed
in adults with COA.128 Antihypertensive therapy can then
be started in a timely manner. Adults with COA may have
Tomography
normal blood pressure during office visits and treadmill MRI is the diagnostic test of choice for complete
stress testing unmasks the ambulatory hypertension in evaluation of the coarctation of aorta due to limitations
response to exercise that they may be experiencing in their of echocardiography, especially TEE (because of the
daily lives. perpendicular plane of the Doppler in relation to the
It also allows assessment of functional capacity, obstructive lesion).126
exercise-induced arrhythmias, and ischemia. In patients undergoing surgery, preoperative MRI/
Depending upon its availability, cardiopulmonary MRA or CT angiography with 3D reconstruction allows
exercise testing (CPET) may hold a promise by using precise identification of the location, anatomy of the
exercise parameters that may assist in early identification coarctation, and the entire aorta along with the collaterals.
MRI is useful for defining the morphology, delineating following surgical repair in the absence of aneurysm/
aneurysms in postoperative cases, site of the stenosis, pseudoaneurysm formation, complex coarctation
extent and degree of narrowing, aortic arch anatomy, affecting the adjoining arch arterial branches, or need for
and aortopulmonary collaterals. Most importantly, MRI concomitant surgery (for aortic valve or aortic root).
provides hemodynamic data with calculation of the
pressure gradient across the stenosis. The Postoperative Adult
CTA is another preferred imaging modality for imaging
the area of coarctation and for obtaining extracardiac Recurrence of narrowing at the coarctation site can occur
information in an adult with a pacemaker or defibrillator. in adulthood. Aneurysm formation at the repair site
Within a short acquisition time, simultaneous visualization occurs in some adults after Dacron patch aortoplasty or
of the ascending aorta, aortic arch, descending aorta, aortic following resection of the coarctation. Some individuals
valve, course of the coronary arteries, coronary anomalies many have pseudoaneurysms while in others, there is a
and calcium risk score can be obtained.130 risk of dissection around the site of repair due to intrinsic
structural abnormalities of the aorta.
Magnetic resonance angiography (MRA) is performed
Doppler echocardiography, guided by transthoracic
every 5 years to search for aneurysms of the intracranial
2D images, is useful for follow-up after successful stenting
arteries, since intracranial aneurysms may occur even
of the coarctation site reflected by a significant decrease
in normotensive adults.131 MRA screening has reported
in peak systolic pressure gradient. Other promising
intracranial aneurysms in approximately 10% of the
parameters that may find more widespread use in the
patients with COA.132 Martin et al. reported a case of a young
future include diastolic velocity, diastolic velocity half-
woman with severe COA presenting and subarachnoid
time index, and diastolic pressure half-time index.134
hemorrhage.133
Evaluation of the repair site by MRI/CT should be
performed at intervals of 5 years or less, depending on the Tetralogy of Fallot
specific anatomical findings before and after repair. Four defects make the tetralogy of Fallot (TOF)—a VSD, an
aorta that overrides the large ventricular septal defect (by <
Cardiac Catheterization 50% of its diameter), subpulmonary infundibular stenosis,
and right ventricular hypertrophy. The presence of an ASD
Coronary artery disease should be ruled out in adults prior
would make it the “pentalogy of Fallot”!31
to surgery. Stent implantation is the preferred intervention
The clinical presentation is affected by the variable
compared to balloon angioplasty alone for coarctation of
degree of right ventricular outflow tract obstruction. The
aorta in adults, with persistent relief of stenosis and lower
most extreme form is pulmonary atresia with a VSD.
incidence of aneurysm formation. Associated defects are common and include abnormal
or absent pulmonary valve, pulmonary artery anomalies
Surgery or Intervention for (dilated or hypoplastic), aortic root dilation, aortic
Coarctation of Aorta regurgitation, right aortic arch (approximately 20–30%),
and abnormal origin of the coronary (left anterior
The ACC/AHA Class I recommendations for interventional descending coronary artery arising from the right coronary
and surgical treatment of coarctation of the aorta in adults artery and crossing the right ventricular outflow tract).
are the following:3 Most adults have had previous palliative shunts or
• Peak-to-peak coarctation gradient ≥ 20 mm Hg complete intracardiac repair but continue to present
• Peak-to-peak coarctation gradient < 20 mm Hg when with multiple residua and sequelae. The intracardiac
an imaging study shows direct evidence of significant repair comprises closure of the VSD with redirection of
coarctation with radiological evidence of significant the aorta toward the left ventricle, and resection of the
collateral flow. right ventricular outflow tract obstruction with relief of
In addition, the presence of a large aortic root, arch hypertrophied muscle that is contributing to infundibular
aneurysm, or severe degree of associated defects may also stenosis. Following an intracardiac repair of TOF, the most
prompt early intervention/surgery. common long-term cardiac issue is chronic pulmonary
Catheter-based intervention is the preferred valve regurgitation that requires reoperations (pulmonary
alternative to surgery for recurrent aortic coarctation valve replacement), approximately every 10 to 20 years.
Adults with pulmonary regurgitation (due to an absent Pulmonary regurgitation and right ventricular outflow
pulmonary valve or other pulmonary valve abnormalities) aneurysm/akinesia are independently associated with
who did not undergo pulmonary valve replacement right ventricular dilatation, leading to hypertrophy and
during their growing years, also present with progressive reduced function over time. It is, therefore, mandatory
pulmonary regurgitation. Those with absent pulmonary to restore pulmonary valve function and avoid RVOT
valve also have massive enlargement of the pulmonary aneurysm/akinesia in order to preserve biventricular
arteries with severe pulmonary regurgitation. Some other function.137
factors exacerbating pulmonary regurgitation in this Concomitant defects such as atrial or ventricular septal
population that can be assessed echocardiographically defect may present with residual patch leaks on Doppler
are the following: pulmonary annulus size, peripheral interrogation.
pulmonary artery stenosis, residual shunts (atrial and Although aortic root dilation is fairly common in adults
ventricular septal defects), pulmonary hypertension, and with tetralogy of Fallot, aortic dissection has only been
right ventricular diastolic dysfunction.
reported in rare instances in the published literature. In
Progressive pulmonary regurgitation leads to right
these reports, it invariably occurred with markedly severe
ventricular enlargement, impaired right ventricular
aortic root dilatation, with aortic dimensions varying
function (systolic and diastolic), and increasing tricuspid
between 7 and 9.3 cm.138–140 So far, the aortic arch sidedness
regurgitation and right atrial enlargement. The clinical
has not been proven to have an independent association
impact of these is variable and different patients will
with the degree of aortic root dilatation.
present with varying degrees of decreased exercise
tolerance, dyspnea on exertion, and predisposition to In addition to the hemodynamic effects, severe
atrial/ventricular arrhythmias. In a multicenter study, we pulmonary regurgitation exacerbates the scar tissue at the
reported that symptoms related to pulmonary regurgitation site of the RVOT patch aneurysm, making it a nidus for
may be well tolerated over a long period; progressively generation of monomorphic ventricular tachycardia.141
increasing from the beginning of the fourth decade with Pulmonary atresia with ventricular septal defect
more than half the adults becoming symptomatic in the represents the severest form of right ventricular outflow
following two decades.135 tract obstruction. Surgical repairs are accordingly more
Besides pulmonary regurgitation, other factors complex, involving unifocalization of the multiple
contributing to right ventricular enlargement and aortopulmonary collaterals into right and left conduit that
impaired function are the degree of tricuspid regurgitation, are then connected to the right ventricle to pulmonary
pulmonary hypertension, residual shunts, and distortion artery (RV to PA) conduit, to mimic the main pulmonary
of the shape of the right ventricle. The dilated right ventricle artery and its branches (Fig. 75.19).
may exhibit improved ejection fraction temporarily in
response to volume overload before spiraling down with Echocardiography
further decrease in myocardial contractility.
Left ventricular dilatation, decreased contractility, A comprehensive transthoracic echocardiographic evalu-
increased myocardial stiffness, and impaired function ation in an adult with tetralogy of Fallot postintracardiac
occurs over time following intracardiac repair of TOF.136 repair begins with the assessment of the right and left
The left ventricular size and function is impacted ventricular size and function. Left ventricular function
specifically by the severity of aortic regurgitation due to is estimated by standard methods. Besides the standard
inadequate coaptation of the aortic leaflets as a result of measures for assessing left ventricular function, a
aortic root dilatation. Due to interventricular dependence, Doppler-derived index of LV filling pressure (the ratio
the deteriorating right ventricular function adversely of early transmitral flow velocity to early diastolic mitral
impacts the left ventricular performance. annular velocity) is a powerful predictor of ventricular
For both the ventricles, the timing of palliative shunts arrhythmias.142
and definitive intracardiac repair affects myocardial Right ventricular enlargement and declining systolic/
contractility. Prolonged cyanosis, inadequate myocardial diastolic function are common especially in the setting
preservation during surgeries involving cardiopulmonary of progressive pulmonary regurgitation. The enlarging
bypass, and pressure and volume overload have deleterious right ventricle stretches the tricuspid annulus causing
effects on the myocardium predisposing it to fibrosis. progressive tricuspid regurgitation, followed by right atrial
independent parameter for right ventricular function is

myocardial acceleration during isovolumetric contraction,
a Doppler-based index, described by Frigiola et al.148 The
isovolumic acceleration index is calculated by dividing
the myocardial velocity during isovolumic contraction by
the time interval from the onset of the acceleration to the
time at peak velocity. It allows early detection of impaired
ventricular function due to the detrimental effect of
pulmonary regurgitation before the onset of symptoms.148
While qualitative assessment by experienced readers
continues to be a practical solution for serial follow-
ups, 3D quantification of right ventricular function with
speckle tracking is gaining popularity. In a study by Gopal
et al, 3D echocardiographic techniques have been shown
to correlate closely with MRI-derived right ventricular
end-diastolic volume and could reduce the need for MRI
in some patients.149
Right ventricular diastolic function: Isolated right ventri-
cular restriction late after tetralogy of Fallot repair is fairly
Fig. 75.19: Diagrammatic representation of the surgical repair
in pulmonary atresia with ventricular septal defect. Unifocalization
common. Methods for assessment of right ventricular
of the multiple aortopulmonary collateral arteries (MAPCAs) into diastolic function and restrictive physiology have been of
right and left conduits is performed, that are then connected to major research interest. The clinical applications of these
the main right ventricle to pulmonary artery (RV to PA) conduit, to time-consuming protocols have been limited so far due
mimic the main pulmonary artery and its branches. (LV: Left ventri- to difficulty in accurately using them on the distorted
cle; RV: Right ventricle; VSD: Ventricular septal defect). (Inspired
right ventricular anatomy. Gatzoulis et al. showed that
by an operation note sketch by Dr Hillel Laks).
antegrade diastolic flow in the main pulmonary artery,
coinciding with atrial systole (A-wave) throughout the
respiratory cycle was indicative of right ventricular
enlargement. The right ventricular systolic pressure is
restriction. On pulsed Doppler sampling (at the midpoint
estimated from the tricuspid regurgitant jet velocity. Some
between the pulmonary valve leaflets and bifurcation of
adults may have a residual right ventricular outflow tract
the main pulmonary artery), the duration of pulmonary
obstruction contributing to right ventricular hypertrophy,
regurgitation was noted to be shorter in those with right
while others may have an aneurysm of the right ventricular
ventricular restriction. This finding was thought to be
outflow tract.
reflective of reduced right ventricular diastolic compliance,
Right ventricular size and systolic function: The shape of with restrictive physiology at end-diastole, thereby making
the right ventricle, trabeculations, and distortion due to
the right ventricle a passive conduit between right atrium
previous surgeries involving the outflow tract have made
and pulmonary artery during atrial systole.150 However, it
accurate quantification very challenging. Transthoracic
can also be present in normal hearts during inspiration.
echocardiography is limited in visualizing the right
Therefore, it should be recorded for at least five consecutive
ventricular outflow tract for identifying aneurysm that
beats to be of diagnostic value in identifying reduced right
along with right ventricular dilatation have a major impact
ventricular diastolic compliance.
on the clinical status of those operated for tetralogy of
Fallot in the presence of pulmonary regurgitation. In Other features on pulsed Doppler that support restrictive
addition, changes in loading conditions may affect some of physiology include: Superior vena caval flow reversal
the measurements obtained by using echocardiographic with atrial systole (measured 1–2 cm proximal to the
parameters such as the Tei index,143,144 stress and strain rate right atrium), and shorter inspiratory and expiratory
imaging,145 and instantaneous rate of pressure increase transtricuspid E-wave deceleration times (measured at the
(dP/dt) by Doppler assessment.146,147 A relatively load- level of the tricuspid valve leaflets).
Pulmonary valve regurgitation/stenosis: While some adults • Potts shunt—descending aorta to left pulmonary artery
may have residual pulmonary stenosis that needs attention anastomosis to increase pulmonary blood flow
for its etiology, location, and severity, the most common • Waterston shunt—ascending aorta to right pulmonary
issue in this population is pulmonary regurgitation. artery anastomosis to increase pulmonary blood flow.
The best view for assessment of pulmonary regurgitation These palliative shunts were performed to enhance
is the parasternal short-axis view at the level of the great pulmonary flow in patients with severe right ventricular
arteries. This view can be modified to evaluate the proximal outflow tract obstruction prior to complete intracardiac
conduit, the main and branch pulmonary arteries. Color repair. Continuous wave Doppler shows nonlaminar
and pulsed Doppler are used to evaluate: (a) width of blood flow directed from the aorta to pulmonary artery
the regurgitant jet at the level of the pulmonary valve or both in systole and diastole. With the larger shunts,
proximal right ventricular outflow tract (RVOT) conduit/ pulmonary hypertension may develop over time. When
prosthetic valve; and (b) the degree of flow reversal in the
the pulmonary hypertension becomes severe, the diastolic
main and branch pulmonary arteries. For assessment of
flow ceases; and when pulmonary artery systolic pressure
the RVOT conduit, continuous wave Doppler is used to
exceeds systemic values, the systolic flow ceases.152 While
assess maximum velocity and mean gradient across the
the orifice of the BTT shunt between the subclavian and
conduit or a prosthetic valve in the parasternal long-axis
pulmonary artery shunt may be seen on a transthoracic
views, apical, or subcostal views. Pulmonary regurgitation
is graded as trace, mild, moderate, or severe as shown in echocardiogram, transesophageal echocardiography is
Table 75.14. required to see the side-to-side connections in Potts and
Although many other methods have been proposed in Waterston shunts.152
recent times for the assessment of pulmonary regurgitation, Aortic root dilation (with loss of the sinotubular
color Doppler continues to be the first one to bring it to our junction) is common in tetralogy of Fallot and may
attention. The regurgitant jet flow can be seen in the right contribute to progressive aortic regurgitation due to
ventricular outflow tract. Retrograde diastolic flow into the inadequate coaptation of the aortic leaflets. Aortic root
main pulmonary artery and its branches may be seen in dimensions should, therefore, be measured on serial
severe pulmonary regurgitation. Spectral Doppler sample echocardiograms as discussed in the section on aortic root
at the level of the pulmonary valve demonstrates normal in adults with congenital heart disease. Color Doppler
forward flow in systole and holodiastolic flow reversal due assists in ruling out residual shunts (ventricular or atrial
to severe pulmonary regurgitation. A pressure half-time septal defects).
of <100 milliseconds is also a good indicator of severe A synopsis of echocardiographic assessment of
pulmonary regurgitation.151 tetralogy of Fallot is reviewed in Table 75.15.
However, color Doppler may not be a reliable indicator
in low-pressure severe pulmonary regurgitation. In these Stress Echocardiography
cases, the pulsations in the right ventricular outflow tract/
main pulmonary artery that often extend into the branches Exercise testing is required for objective assessment
are the surrogate marker. of functional capacity and to unveil exercise-induced
The degree of pulmonary stenosis or residual obstruc- arrhythmias such as atrial fibrillation/flutter, premature
tion at the level of pulmonary valve, infundibulum, or ventricular complexes, and nonsustained ventricular
pulmonary artery branches should be estimated using the tachycardia in the postoperative adult with tetralogy of
standard continuous wave Doppler technique. Fallot. Monomorphic ventricular tachycardia most often
Echocardiographic assessment of palliative shunts: Although occurs in the presence of an old ventriculotomy scar. Poor
most of the adults have undergone intracardiac repair with heart rate response to exercise unmasks chronotropic
takedown of the shunt, a few may still have long-standing incompetence that may lead to pacemaker implantation.
palliative shunts. The most common shunts are: Stress echocardiography provides additional infor-
• Classic Blalock–Taussig–Thomas (BTT) shunt—sub- mation regarding the severity of pulmonary hypertension
clavian to pulmonary artery anastomosis to increase postexercise and the impact of pulmonary regurgitation
pulmonary blood flow on the right ventricular contractile reserve, by gauging
• Modified BTT shunt—subclavian to pulmonary its hyperdynamic response to exercise. Meijboom et al.
artery anastomosis, using a Gore-tex graft to increase showed that exercise capacity inversely correlates with right
pulmonary blood flow ventricular dilation in patients with repaired tetralogy.153
Table 75.14: Tetralogy of Fallot

Associated defects
• Right aortic arch—20% to 30%
• Hypoplastic pulmonary arteries
• Absent pulmonary valve
• Pulmonary atresia
• Pulmonary artery dilatation/aneurysm
• AV septal defects
• Muscular ventricular septal defects
• Anomalous origin of the coronary arteries (left anterior descending arising from right coronary cusp and courses across the
right ventricular outflow tract)
• Aortic root dilatation
• Subaortic stenosis
• Aortopulmonary window
• Aortopulmonary collaterals
• Absent left pulmonary artery
• Origin of a pulmonary artery from the aorta
• Ebstein’s anomaly of the tricuspid valve
• Anomalous coronaries
• Right and left ventricular size and function
• Right ventricular outflow tract (infundibular) obstruction
• Pulmonary regurgitation
• Pulmonary stenosis—at different levels, usually branch stenosis
• Aortic root dilation (loss of the curvature of the sinotubular junction)
• Shunt lesions—ventricular septal defects, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava
• Aortic arch anatomy (right aortic arch seen in 20–25%)
• Origins of the coronary arteries
Postoperative
• Residual VSD or patch leak
• Pulmonary regurgitation
• Right ventricular out flow tract size/aneurysm
• Aortic root size
Table 75.15: Echocardiographic Grading of Pulmonary Regurgitaiton

A. Based on the Color Doppler width of the jet on the ventricular aspect of pulmonary valve leaflets
Trace—a flash of color
Mild—jet width < 20% of the annular width of the valve/conduit
Moderate—jet width between 20% and 40% of the annular width of the valve/conduit
Severe—jet width > 40% of the valve/conduit annular width
B. Based on diastolic flow reversal
Trace—Diastolic color flow reversal limited to the beginning at valve/conduit
Mild—Diastolic color flow reversal limited to the proximal half of the main pulmonary artery
Moderate—Diastolic color flow reversal extending into distal half of the main pulmonary artery
Severe—Diastolic flow reversal extending into proximal branch pulmonary arteries
Source: Adapted from Brown DW et al. J Am Soc Echocardiogr 2012;25:383–92.
It also plays an adjunctive role in risk stratification of assesses coronary artery disease, pulmonary artery or
patients who are more likely to have sudden cardiac death branch stenosis, and pulmonary vascular resistance
by determining the impact of hemodynamic burden of in adults with pulmonary hypertension due to long-
pulmonary regurgitation and right ventricular impairment standing shunts. It is routinely performed in conjunction
on the electrophysiological substrates. with potential catheter-based interventions such as
In patients undergoing cardiopulmonary exercise percutaneous pulmonary valve implantation.
testing, a decline in functional aerobic capacity (maximum The Melody transcatheter pulmonary valve
VO2) to <70% of gender–age-predicted maximum value (Medtronic, Inc., Minneapolis, MN) is approved by the US
or a decline over 20% compared with serial testing is Food and Drug Administration for treatment of patients
considered significant. with regurgitant or stenosed right ventricular outflow
Adults with right ventricle to pulmonary artery (RV tract (RVOT) conduits. The indications for implantation
to PA) conduit should undergo evaluation for abnormal can vary from isolated pulmonary regurgitation to relief
ventricular function and conduit obstruction at peak of RVOT obstruction, from a bridging procedure to a more
exercise. Besides tetralogy of Fallot, others with RV to definitive therapy.155
PA conduit include patients with pulmonary atresia and Echocardiography complements MRI in determining
multiple aortopulmonary collaterals, truncus arteriosus, the suitability for percutaneous pulmonary valve
or d-transposition of great arteries. A RV-to-PA conduit implantation by assessing the following parameters: (a)
is considered to be stenotic when the mean continuous right ventricular outflow tract diameter between 16 and
wave Doppler gradient across the conduit is over 20 mm Hg 24 mm, (b) right ventricular outflow gradient of >30 mm
on echocardiography. Hasan et al. reported a protocol Hg, (c) the presence of discrete waist or calcification for
with mean instantaneous systolic gradient and maximum implantation, and (d) the absence of significant pulsatility.
instantaneous systolic gradient across the RV-to-PA MRI provides additional information about any limitations
conduit obtained at rest and immediately postexercise, in access to the right ventricle due to occlusion of the
using continuous wave Doppler through the conduit in the central vein.
parasternal short-axis view.154 Echocardiography also identifies procedural compli-
cations resulting from implantation of the percutaneous
pulmonary valve, including rupture of the RVOT conduit,
Cardiac Catheterization embolization or migration of the device, and perforation of
Angiography plays a limited role in the current era for a heart chamber that may manifest as a pericardial effusion.
the postoperative adult with tetralogy of Fallot. It is not a Other short- and long-term complications include stent
reliable test for assessment of pulmonary regurgitation, fracture resulting in recurrent obstruction, endocarditis,
since the angiographic severity may be influenced by valvular stenosis/regurgitation, paravalvular leak, and
catheter position across the pulmonary artery. It accurately valvular thrombosis. The most common complication
noted during follow-up of the Melody valves are stent 170 cc/m2; and/or when the right ventricular end-systolic
fractures with an incidence of approximately 20%.156 volume reaches 85 cc/m2.159,160
Other interventional procedures include ASD closures, Other criteria include widened QRS over 180
angioplasty/stent implantation of obstructed pulmonary milliseconds or an annual increase in QRS width over 3.5
arteries/branches, and deployment of coils in the collateral milliseconds in the setting of ventricular arrhythmias and
vessels or systemic–pulmonary artery shunts. worsening tricuspid regurgitation.
Concomitant surgical removal of the right ventri-
Magnetic Resonance Imaging/Computed culotomy scar, right ventricular outflow tract aneurysm,
pulmonary arterioplasty, aortic root, aortic valve repair/
Tomography replacement, and closure of residual shunts are performed
MRI is the current reference standard for quantification of to improve the hemodynamic status. Surgical relief of
right ventricular volume and the systolic function.157,158 It RVOT obstruction should be considered when RVSP is
provides accurate measurements of the aorta, pulmonary over two thirds of the systemic pressure.
artery, and its branches. Areas of myocardial fibrosis due Intraoperative transesophageal echocardiography
to previous surgeries can be identified by gadolinium- plays an important role, ensuring the adequacy of repair
enhanced MRI. in these patients.
For preoperative evaluation in adults who are unable
to have an MRI (due to devices such as pacemakers/
defibrillators), CTA is an alternative. In addition, it allows
us to identify anomalous coronaries and calcium score. Although patients may demonstrate some symptomatic
improvement with medical therapy, pulmonary valve
Surgery in a Postoperative Adult with replacement is the only treatment modality with proven
long-term benefit. Serial echocardiograms may show a
Tetralogy of Fallot reduction in right ventricular size, an improvement or at
The ACC/AHA Class I indication for surgery in a least stabilization of right ventricular function.
postoperative adult with tetralogy of Fallot is:3 In later years, following bioprosthetic pulmonary
• Pulmonary valve replacement for symptomatic severe valve replacement or placement of an extracardiac
pulmonary regurgitation with declining exercise conduit (from the right ventricle to pulmonary artery),
tolerance stenosis or regurgitation may develop and require further
Since bioprosthetic valves have a limited life expec- reoperations to maintain functional improvement.161 An
tancy, surgery should be performed only when it is clearly overall review of multiple studies shows that pulmonary
indicated. The porcine bioprosthetic valves have shown valve replacement in tetralogy of Fallot postintracardiac
better durability than the cryopreserved homografts. Many repair shows an improvement in clinical outcomes.
studies have proposed additional criteria in symptomatic Symptoms resolve and right ventricular function often
adults presenting with exertional dyspnea, heart failure, improves. Several retrospective reviews have reported
and ventricular arrhythmias. These include progressive reduction in right ventricular end-diastolic and systolic
right ventricular dilatation. volumes along with an increased right ventricular stroke
When the pulmonary valve replacement is performed in volume.162
a timely fashion, it may lead to improvement in symptoms, Meijboom et al. reported that surgery for pulmonary
reduction in right ventricular size, and recovery of systolic valve replacement should be offered to symptomatic
function. The timing of surgery in asymptomatic adults with patients for best outcomes in the majority of adults, who
pulmonary regurgitation remains controversial. It may be have undergone intracardiac repair in childhood and
considered when there is an objective evidence of a decline present with severe pulmonary regurgitation and right
in exercise capacity on stress testing and progressive right ventricular dilatation.163
ventricular enlargement confirmed on MRI. Based on the
data from two studies, in order to expect improvement
in right ventricular function following pulmonary valve
D-Transposition of the Great Arteries
replacement, the surgery should be considered when the In d-transposition of the great arteries (d-TGA), there is
right ventricular end-diastolic volume is between 150 and ventriculoarterial discordance with abnormal origins of
the aorta and pulmonary artery. This implies that the aorta ventricle, which is functioning as a systemic ventricle
arises from the systemic morphological right ventricle by pumping into the aorta. In the parasternal short-axis
and lies rightward and anterior to the pulmonary artery, views, the left ventricle appears compressed due to a
which in turn arises from the pulmonic morphological left D-shaped septum or bowing of the septum toward the
ventricle. morphological left ventricle. The aorta and the pulmonary
Common associated defects include a shunt lesion, a artery can be seen parallel to each other in the parasternal
VSD (45%), an ASD, or a PDA that allows intermixing of the long-axis view and in the same plane in the parasternal
deoxygenated and oxygenated blood to permit survival, short-axis view (Figs 75.20A and B).
since this circulation is a parallel circuit and will not be The systemic (morphological right) ventricular function
compatible with life without any communication. Most is impacted by the degree of systemic AV (tricuspid) valve
individuals are diagnosed early in life with this form of regurgitation in a way quite similar to the impact of the
cyanotic congenital heart disease and have undergone one mitral regurgitation on the left ventricle in normal hearts.
of the types of repairs. Other associated defects include left Progressive aortic regurgitation increases the workload on
ventricular outflow tract (LVOT) obstruction, coarctation the systemic (morphological right) ventricular function.
of the aorta, and anomalies of the coronary ostia due to Subpulmonic stenosis increases pressure load on the left
transposition.31 ventricle.
Patients with baffle obstructions or leaks may
Postatrial Switch Repair present with dyspnea or worsening atrial arrhythmias.
Flow acceleration, turbulent flow on color Doppler, and
Many older adults seen in our practice have had an atrial increased Doppler gradients indicate baffle narrowing
switch repair. In this type of surgery, the deoxygenated or obstruction. Baffle issues are more commonly seen
blood from the superior and inferior vena cava is directed following a Mustard rather than a Senning repair and
via a baffle into the left ventricle, and then pumped into usually involve the superior limb.166 Bottega et al. reported
the pulmonary artery. The left ventricle becomes the obstruction of the superior limb in 44% of the adults post-
subpulmonic ventricle. The oxygenated blood returns Mustard repair.167
through another baffle from the pulmonary veins into The velocities in the superior limb of the systemic
the right ventricle and gets pumped into the aorta. This venous baffle can be measured from a parasternal or
makes the morphological right ventricle the subaortic tilted apical four-chamber view. Baffle obstruction by
or the systemic ventricle. The two types of atrial switch Doppler echocardiography is considered when there is
operations are the Senning and the Mustard repairs. They a pulsed wave Doppler peak velocity of ≥ 1.5 m/s with a
differ primarily in terms of the material used to create the
baffles. In the Senning operation, the baffle is created from
the patient’s tissues (right atrial wall and part of the atrial
septum).164 The Mustard operation uses pericardium and
synthetic material to make the baffle.165 The interatrial
baffles can deteriorate over time leading to obstruction or
leaks. The scar tissue along atrial incisions and the foreign
material may become substrates for atrial arrhythmias.
Surgical damage of the sinus node and conduction system
makes pacemaker surgery more likely in adulthood. A B
Figs 75.20A and B: Diagrammatic representation of the
Echocardiography transthoracic echocardiogram in d-transposition of the great
arteries (DTGA). The aorta and the pulmonary artery (PA) are
Serial echocardiography is performed in adults who have seen in parallel to each other in the parasternal long-axis view (A)
undergone atrial switch repair to assess the morphological and in the same plane (rather than criss-cross) in the parasternal
right ventricular size and function. Most adults have a short-axis view (B). (LA: Left atrium; LV: Left ventricle; RV: Right
globular-shaped, hypertrophied morphological right ventricle).
characteristic loss of the biphasic Doppler profile.167,168 and superior vena caval patterns showing presystolic flow
Pulmonary venous baffles are easier to image by reversal with atrial contraction indirectly indicate patency
echocardiography and should be suspected when there is of the caval anatomosis. Residual ventricular septal
an increase in the left ventricular systolic pressure.169 defects or patch leaks should be examined with color
Baffle leaks usually result in right heart enlargement Doppler. A synopsis of echocardiographic assessment
as seen with long-standing atrial septal defects. In these of the postoperative adult with d-TGA is reviewed in
adults, the subpulmonary left ventricle can appear more Table 75.16.
D-shaped with a compressed appearance when there is Three-dimensional echocardiography may add
a hemodynamically relevant baffle leak, subpulmonary incremental value to visualization of the baffle and
obstruction, or pulmonary hypertension.169 Color Doppler tricuspid valve anatomy in the post-Mustards adults. It
may reveal the site of the shunt and an agitated saline may provide a more comprehensive assessment of baffle
contrast study may confirm it. obstruction by allowing it to be viewed in multiple planes
TEE may be required when there is limited visualization and projections.171 In addition to the en face viewing
by a transthoracic echocardiogram. TEE also allows direct of the intra-atrial baffle and measurements of vena
examination of caval anastomosis.170 Hepatic venous flow contracta from the baffle leaks, it provides complementary
Table 75.16: D-Transposition of the Great Arteries

Associated defects
• Shunt lesions—ventricular septal defect (usually perimembranous), atrial septal defect and patent ductus arteriosus
• Left ventricular outflow tract (LVOT) obstruction—pulmonary stenosis, usually valvular but may be subvalvular
• Coarctation of the aorta
• Coronary artery anomalies
• Right ventricular outflow tract (RVOT) obstruction (subaortic narrowing)
Most of the adults have had surgeries in their childhood
• Great arteries—aorta and pulmonary artery—are seen parallel to each other in the parasternal long-axis view.
• In the short-axis view they are seen next to each other with the aorta being anterior and rightward.
Postoperative
Atrial switch repair
• Assessment right (systemic) ventricular size systemic function
• Right ventricular hypertrophy
• Baffle leak
• Baffle obstruction
Arterial switch repair
• Assessment of left ventricular size and systolic function
• Coronary artery fibrosis may lead to ischemia/infarction—stress echocardiography is performed to assess wall motion abnor-
malities
• Neoaortic valve regurgitation
• Mild aortic root dilatation
• Pulmonary artery stenosis at the valvar supravalvar or peripheral level
Post-Rastelli repair
• Assessment of biventricular ventricular size and systolic function
• Conduit obstruction
anatomical details of the pulmonary valve and the left dysfunction. Baffle leak with left-to-right shunt
ventricular outflow tract.172 > 1.5:1, right-to-left shunt with arterial desaturation
at rest or with exercise, symptoms, and progressive
Stress Echocardiography ventricular enlargement that is not amenable to device
intervention
Exercise capacity may be limited postatrial switch
• Superior vena cava or inferior vena cava obstruction
repair due to stiffness and noncompliance of the baffles,
not amenable to percutaneous treatment
deterioration in systemic ventricular function, sick
sinus syndrome, or advanced conduction disease. Baffle • Pulmonary venous pathway obstruction not amenable
problems appear to have the most effect on exercise ability to percutaneous intervention
because of impaired ventricular filling and limited ability • Symptomatic severe subpulmonary stenosis.
to augment stroke volume during exercise.173 Hence,
functional capacity assessed objectively by exercise stress Postarterial Switch Repair
echocardiography should be followed by a search for causes
of hemodynamic impairment such as baffle obstruction, Dr A Jatene first described the arterial switch repair in
progressive tricuspid regurgitation, subpulmonic obs- which the great arteries are translocated to achieve their
truction, impaired systemic ventricular function, or anatomically correct positions and the coronary arteries
chronotropic incompetence. Routine repetition of exercise are reimplanted into the neoaorta.176 The most common
studies every 3 years is recommended even in the absence long-term issues postarterial switch repair in a young adult
of symptoms, since gradual declines in function often go with d-TGA are neoaortic root dilatation and neoaortic
unnoticed by patients.169 valve regurgitation, since the current aortic valve, which
was originally a pulmonary valve, is now placed in a
Cardiac Catheterization high pressure circulation. Supravalvular pulmonary
stenosis often occurs at the arterial anastomotic sites.
Systemic or pulmonary venous baffle obstructions are
diagnosed by angiography and can be often be relieved by Due to reimplantation of the coronary arteries into the
balloon-expanded stent implantation. Some of the baffle neoaorta, fibrosis and scarring at the anastomotic sites
leaks can be eliminated by implanting smaller devices. of the coronaries may cause ischemia in young adults.
Intracardiac echocardiography plays a role in guiding Coronary angiography or CTA is indicated when ischemia
percutaneous closure of atrial baffle defects.175 is likely. Adults with intramural or single coronary arteries
have a higher mortality than those with a typical coronary
Magnetic Resonance Imaging/Computed pattern. Among the long-term issues are supravalvular
stenosis and aortic aneurysm.
Tomography
Baffle stenosis or leak may be more clearly defined by MRI/ Echocardiography
CTA.167 Baffle patency should be evaluated by either of
these modalities before transvenous device implantation. Neoaortic dilatation and aortic regurgitation may develop
MRI is also the gold standard for accurate assessment of over time. Neoaortic constriction can occur at the site of
systemic right ventricular volumes and function, since anastomosis. Patients with previous pulmonary artery
most echocardiographic parameters have reduced banding (although it is now rarely performed) are more
sensitivity and marked intraobserver variability.174 likely to have neoaortic root dilatation but it may not
necessarily progress on late follow-up. Risk factors for
Reoperation for D-Transposition of the Great aortic regurgitation include older age at the time of
Arteries Postatrial Switch Repair presentation, presence of a VSD, and previous pulmonary
artery banding.177
The Class I ACC/AHA indications for surgery in d-TGA
postatrial switch repair (Mustard or Senning) are the
following:3
• Moderate to severe systemic (morphological tricuspid) Following atrial switch repair, stress echocardiography is
AV valve regurgitation without significant ventricular performed to screen for exercise-induced ischemia.
Cardiac Catheterization pressure, which is usually elevated in the setting of conduit

obstruction. Progressive tricuspid regurgitation with right
Coronary ostial fibrosis leading to coronary ischemia ventricular enlargement, hypertrophy, and failure are
prompts early catheterization in those adults who have bad prognostic signs indicating high risk of mortality. Left
undergone reimplantation of the coronaries into the base ventricular outflow tract (LVOT) obstruction can occur at
of the neoaorta. any level in the long intraventricular baffle through the
VSD to aorta. Structural abnormalities of the great arterial
Magnetic Resonance Imaging/Computed walls make these adults prone to aortic root dilatation and
Tomography aortic valve regurgitation.
These imaging modalities are helpful in assessment of

the neoaortic diameters, coronary artery ostial fibrosis
(that can lead to myocardial ischemia), and extracardiac Cardiac catheterization determines the conduit gradient
structures such as pulmonary artery stenosis. accurately. Catheter-based interventions such as intra-
conduit percutaneous pulmonary valve implantation, and
Reoperation for D-Transposition of the Great dilation with or without stent implantation for relief of
branch pulmonary artery stenosis can be performed when
Arteries Postarterial Switch Repair indicated.
The Class I indications for surgery in d-TGA postarterial
switch repair include: Magnetic Resonance Imaging/Computed
• Symptomatic severe right ventricular outflow tract
obstruction (conduit obstruction) not amenable to
Tomography
catheter-based intervention with a peak-to-peak Prior to surgery, MRI/CTA may be helpful in assessment
gradient > 50 mm Hg, or with concomitant severe of adults with significant conduit obstruction/leaks or
pulmonary regurgitation branch pulmonary stenosis.
• Symptomatic myocardial ischemia that cannot be
relieved by catheter-based intervention in an adult Reoperation for D-Transposition of the Great
with a coronary artery abnormality
• Severe symptomatic neoaortic valve regurgitation
Arteries Post-Rastelli Repair
• Severe neoaortic root dilatation ≥ 55 mm. The ACC/AHA Class I indications for reoperation after
Rastelli procedure in the following settings are:3
Post-Rastelli Repair Prior Conduit and/or Valve Replacement
• Symptomatic adult with conduit obstruction with a
The Rastelli procedure is performed in d-TGA that is
peak-to-peak gradient > 50 mm Hg
associated with a large VSD and significant pulmonary
• Subaortic (baffle) obstruction with a mean gradient
stenosis. In this surgical repair, a right ventricle to
> 50 mm Hg and left ventricular hypertrophy
pulmonary artery conduit allows blood flow from the right
• Severe symptomatic aortic regurgitation requiring
ventricle to reach the branch pulmonary arteries, usually in concomitant surgery.
patients with right ventricular outflow tract obstruction.178
Conduit Regurgitation
• Symptomatic/decreased exercise tolerance
Echocardiography • Severe right ventricular enlargement with severely
Echocardiography plays an important role in evaluation reduced right ventricular function
of long-term complications including right ventricular • Severe tricuspid regurgitation.
outflow tract (RVOT) obstruction, pulmonary conduit Residual Ventricular Septal Defect
obstruction with degeneration and calcification of the • Increased left ventricular size from volume overload
pulmonary valve, residual shunts, and degree of pulmonary • Reduced right ventricular function from pressure
and tricuspid regurgitation. The tricuspid regurgitant jet overload
velocity helps in estimation of the right ventricular systolic • Systolic pulmonary artery pressure > 50 mm Hg
• Right ventricular outflow tract obstruction with peak mesocardia (apex in midline) or dextrocardia (apex in the
instantaneous gradient > 50 mm Hg right side of the chest). The diagnosis of CCTGA should be
• Pulmonary artery pressure less than two thirds of highly considered in adults with dextrocardia.
systemic pressure and responsive to pulmonary Heart failure is common by the fourth and fifth
vasodilators. decades and has been reported in 51% of adults with
associated defects versus in 34% without any associated
The Postoperative Adult defects.180 The systemic right ventricular function is usually
impaired due to long-standing tricuspid (systemic AV
In addition to the residua and sequelae specific to the type valve) regurgitation and influences the timing of surgery
of surgery (atrial or arterial switch or Rastelli repairs) as in CCTGA with or without associated lesions.181 The
detailed above, the postoperative adult with d-TGA may most common associated defect is an abnormality of the
require the following surgeries: systemic AV valve that may occur in up to 90% of the cases
Pacemaker Surgery with Epicardial Leads (Ebstein-like, dysplastic).182,183 Other associated defects
• In adults with significant residual intracardiac shunts include a perimembranous VSD in approximately 70% and
or systemic venous obstruction, epicardial leads are subvalvular pulmonary stenosis in approximately 40%. In
the safest option. a multi-institutional study, Graham et al. reported aortic
Pulmonary Artery Stenosis or Branch Pulmonary Stenosis regurgitation in 25% to 36% of adults with CCTGA.180 The
• Surgery is performed when interventional procedures presence of a VSD and significant pulmonary stenosis may
are not feasible in adults with severe pulmonary artery contribute to cyanosis. A higher incidence of endocarditis
stenosis or branch pulmonary stenosis. (11%) has been reported in these adults.184
Most adults are likely to get a pacemaker implantation,
since conduction abnormalities are so common in this
Congenitally Corrected Transposition of
population with complete heart block occurring at the rate
the Great Arteries (CCTGA) of 2% per year.185
Although far less common than d-TGA, congenitally
corrected transposition of the great arteries (CCTGA) can Echocardiography
sometimes go undiagnosed into adulthood, especially
Unfortunately, this diagnosis may be missed on echo-
in the absence of any associated defects. Adults with cardiography because of failure to identify the abnormal
unoperated CCTGA are often misdiagnosed in adulthood position of the AV valves associated with ventricular
and referred late to ACHD specialists for appropriate inversion. In addition, the 1% of adults with CCTGA without
management, despite having symptomatic systemic AV associated defects are even more likely to go undiagnosed
valve regurgitation and impaired morphological right into adulthood. Identifying the relationship of the great
ventricular (systemic) function.179 arteries and defining the position of the morphological
In addition to the ventriculoarterial discordance (the tricuspid valve are essential for clinching the diagnosis on
great arteries–aorta and pulmonary arising from opposite echocardiography.
ventricles), there is atrioventricular discordance (the right As seen in d-TGA, the two great arteries are parallel to
atrium drains into left ventricle through the mitral valve, each other in the modified parasternal long-axis view. They
left atrium drains into right ventricle through the tricuspid are seen in the same cross-sectional plane in the parasternal
valve) due to ventricular inversion. The term “corrected” short-axis views. The position of aorta is anterior, leftward,
refers to the physiologically normal direction of blood flow and superior, while the pulmonary artery is rightward,
due to “double discordance,” with “two wrongs try to make posterior, and inferior. Identification of the ventricular
a right” resulting in the morphological right ventricle morphology and positions of the AV valve that are best seen
pumping into the aorta and the morphological left ventricle in the apical four-chamber view remains critical in making
into the pulmonary artery. It has been sometimes referred the diagnosis (Fig. 75.18). The AV valves always follow
to as “l-transposition,” because the morphological right their ventricles and, therefore, the tricuspid valve (referred
ventricle is to the left of the morphological left ventricle. to as the systemic AV valve) is on the left side with the
Levocardia with the apex of the heart in the left side of morphological right (systemic) ventricle posing as the left
the chest is most common, but some cases may have AV valve, while the mitral valve (referred to as the pulmonic
Fig. 75.21: Transthoracic echocardiogram in congenitally correct- Fig. 75.22: Transthoracic echocardiogram in congenitally correc-
ed transposition of the great arteries (CCTGA) showing ventricular ted transposition of the great arteries (CCTGAs) showing that the
inversion and tricuspid valve on the left side (apically placed in left ventricle pumps into the transposed pulmonary artery and
relation to the mitral valve). (LA: Left atrium; LV: Left ventricle; its branches (arrows) in a modified four-chamber view. (LV: Left
MB: Moderator band; MV: Mitral valve; RA: Right atrium; RV: Right ventricle; RV: Right ventricle).
ventricle; TV: Tricuspid valve).
Source: With permission from Vijayalakshmi IB, Rao PS, Chugh
R, editors. A Comprehensive Approach to Congenital Heart Dis-
eases. 1st ed. New Delhi: Jaypee Brothers Medical; 2013: 767.
AV valve) is on the right side with the morphological the systemic ventricular contractile reserve can be made
left (pulmonic) ventricle (Fig. 75.21). The systemic right by stress echocardiography that also allows assessment of
ventricle pumps into the aorta that is identified by its chronotropic insufficiency.
candy cane appearance. The left ventricle pumps into
the pulmonary artery, which is identified by its right and Magnetic Resonance Imaging
left branches in a modified apical four-chamber view
(Fig. 75.22). The complex shape of the morphological right The best method for quantification of systemic ventricular
ventricle makes quantification of systemic ventricular function is an MRI, which is currently the reference
function by echocardiography more challenging. standard. Since many adults with CCTGA have devices
Echocardiographic assessment of the systemic (pacemakers or defibrillators), CTA is the next best option
when MRI cannot be performed.
ventricular function and degree of systemic AV valve
(morphological tricuspid) regurgitation are extremely
important since long-term outcomes including heart Cardiac Catheterization
failure and mortality are impacted by the inter-relationship The role of cardiac catheterization is mainly for delineating
between the severity of regurgitation and the declining the coronary anomalies or atherosclerosis prior to surgery.
systemic ventricular function. The morphology of the It also allows evaluation of pulmonary vascular reactivity
systemic AV valve (morphological tricuspid), the dilatation in adults with significant pulmonary hypertension.
of its annulus (due to enlarging systemic ventricle), and the
presence of pacemaker wire may all contribute to failure of
Surgery for CCTGA
leaflet coaptation and further progression of regurgitation.
Long-standing tricuspid regurgitation is a major risk factor Referral to cardiac surgery should be sought for early
for deterioration of right ventricular function.181 systemic AV valve replacement in symptomatic adults or
before the systemic ventricular ejection fraction declines
below 45%.186 It is more likely to occur in adults with
Exercise Testing with Stress Echocardiography
tricuspid valve abnormalities with progressive tricuspid
While treadmill stress testing provides an objective regurgitation. Other echocardiographic signs that should
assessment of functional capacity, a good estimation of alert the clinician are an enlarging right ventricle, left atrial
enlargement, progressive pulmonary hypertension, and of the tricuspid valve. Following surgery, the prosthetic
the appearance of atrial arrhythmias. systemic AV valve (that replaced the tricuspid valve) should
The key ACC/AHA Class I indications for surgery in be monitored by echocardiography along with periodic
adults with CCTGA are the following:3 assessment of systemic ventricular function, pulmonary
• Unrepaired CCTGA or post-tricuspid valve repair with conduit function (post-Rastelli repair), degree of aortic
severe AV valve regurgitation regurgitation, progression of pulmonary hypertension,
• Left ventricle is functioning at systemic pressures and evaluation of residual septal defects.
postbiventricular repair After pacemaker implantation, pacing has been shown
• Progressive moderate to severe aortic regurgitation to cause a septal shift that may aggravate dilatation of the
leading to ventricular dilatation and impaired function systemic ventricle and precipitate worsening of systemic
• Conduit obstruction with AV valve regurgitation. Hence, these should be watched
– Markedly high right ventricular pressures and/or more closely after pacemaker implantation. A synopsis of
impaired morphological right ventricular function echocardiographic assessment of CCTGA is reviewed in
after anatomical repair Table 75.17.
– Markedly elevated left ventricular pressures in an
adult with a nonanatomical correction. Truncus Arteriosus
Truncus arteriosus is also known as truncus arteriosus
The Postoperative Adult communis and common aorticopulmonary trunk.
Tricuspid valve repair is less likely to be a long-term Approximately 80% of the infants survive at 1 year without
solution in many adults due to abnormal morphology surgical intervention.187,188 Since mortality without surgery
Table 75.17: Congenitally Corrected Transposition of the Great Arteries

Associated defects
• Ventricular septal defect—perimembranous (60–80%)
• Pulmonary, subpulmonary or supravalvular stenosis
• Tricuspid (left AV valve) anomaly—Ebstein’s anomaly, Ebstein-like or dysplastic morphological tricuspid valve
• Tricuspid (left AV valve) regurgitation
• Aortic or subaortic stenosis
• Conduction abnormalities—complete heart block
• Dextrocardia
• Coronary artery anomalies (inverted coronaries due to ventricular inversion)
• Systemic ventricular (morphological right ventricle) size and function
• Degree of systemic AV (tricuspid) valve regurgitation
• Identification of associated defects
Postoperative
• Systemic ventricular (morphological right ventricle) size and function
• Degree of systemic AV (tricuspid) valve regurgitation
• Rule out residual shunt lesions
• Conduit stenosis (in Rastelli repair)
• Thromboembolic risk
is very high in infancy, most adult survivors have had The origins and epicardial course of the coronaries are
previous repairs. This defect is characterized by a single variable and often anomalous. The left coronary usually
great artery with a single semilunar valve. The single great arises from the left posterior aspect of the truncus while
arterial vessel arises from the base of the heart giving the right coronary often arises from the right anterior
aspect of the truncus.31
rise to the coronary, pulmonary, and systemic arteries.
It is larger than the aorta with poorly developed sinuses Echocardiography
of Valsalva, and has a single semilunar valve that is also
On transthoracic echocardiography, the parasternal long-
known as the “truncal valve.” A large nonrestrictive,
axis view shows the truncal root, valve, and sometimes the
subarterial VSD is required for intermixing of blood
origins of the pulmonary arteries. The apical four-chamber
between the two ventricles. While the VSD is usually view is helpful in demonstrating a VSD and an overriding
described as membranous, it has also been thought to truncus. The suprasternal views allow identification of a right
result from absence of the distal pulmonary infundibulum aortic arch and coarctation of the aorta. Color Doppler is
(both septal and free wall).189 Since the VSD is roofed useful in identifying truncal valve stenosis or regurgitation,
by the truncal valve, there is poor truncal support, and presence of pulmonary stenosis at the origin of the branches,
truncal valve regurgitation occurs.31 The truncal valve may and for determining the direction of the shunt. An agitated
be trileaflet in the majority (nearly 70%), quadricuspid in saline contrast study is performed to rule out right-to-left
around 20% to 25%, and bicuspid in around 7% to 9%.189 shunts and to define the right ventricular outflow tract in
the parasternal short-axis views or the subcostal views.190,191
When the truncal valve is quadricuspid, it is a combination
Associated defects are coarctation of aorta, interrupted aortic
of the three leaflets of the aortic valve and pulmonary arch, right aortic arch, patent ductus arteriosus, ASD, and
leaflet tissue.189 The pulmonary arteries arise from the tricuspid stenosis. In the absence of significant pulmonary
posterolateral aspect of the common arterial trunk with stenosis, pulmonary hypertension develops. A synopsis
separate origins for the right and left branches. A right of echocardiographic assessment of truncus arteriosus is
aortic arch is present in 20% to 30% of the cases.31,189 reviewed in Table 75.18.
Table 75.18: Truncus Arteriosus

Associated defects
• Right aortic arch—30%
• Interrupted aortic arch
• Abnormal truncal valve
– Trileaflet (most common)
– Quadricuspid
– Bicuspid
• Truncal valve stenosis/regurgitation
• Left superior vena cava
• Secundum atrial septal defect
• Absence of branches of the pulmonary artery
• Anomalous origin of the coronary artery
Echocardiographic assessment—postoperative
• Aortic root size
• Aortic (truncal) valve regurgitation
• Assessment of any associated defects
• Conduit patency or regurgitation
Magnetic Resonance Imaging/Computed conduits to function for a longer duration. Another

major indication for reoperation that impacts long-
Tomography term outcomes/mortality is truncal valve regurgitation
Since extracardiac malformations occur in up to 40% of these requiring replacement or repair.195–197
individuals, MRI or CTA are very useful for assessment of The long-term residua and sequelae assessed
aortic arch anomalies and branches pulmonary stenosis. by echocardiography are progressive homograft or
conduit obstruction/regurgitation, truncal or prosthetic
Cardiac Catheterization valve regurgitation/stenosis, residual VSD, impaired
Besides evaluation of the coronaries, cardiac catheteri- biventricular function, truncal root dilatation, and
zation is performed in conjunction with stent placement pulmonary hypertension. These adults may have limi-
when there is conduit obstruction. tations in functional capacity, and are at risk of developing
heart failure, cardiac arrhythmias, and infective endo-
carditis.
Surgery for Truncus Arteriosus
Surgery is indicated for conduit failure not amenable to Double Outlet Right Ventricle
intervention, severe truncal regurgitation, and enlarging
truncal dimensions. A key feature in this heterogenous group of defects
described as the double outlet right ventricle (DORV) is that
the aorta and the pulmonary artery arise primarily (>50%)
from the right ventricle.198 Dr Joseph Perloff eloquently
Improvements in early diagnosis and primary neonatal states, “It has been argued that the malformation is
repair have allowed better survival.192 Palliation with virtually unclassifiable because of its excessively complex
pulmonary artery banding (which delays primary surgery and diverse anatomy.”31
to a later age) is no longer believed to offer any benefit and The clinical patterns in this spectrum of defects
is associated with high mortality rates. vary based on the size/location of the VSD, presence or
McGoon, Rastelli, and Ongley performed the first absence pulmonary stenosis, ventricular size, status of the
successful surgical repair of truncus arteriosus in 1967 atrioventricular valves (AV), and the degree of pulmonary
using an aortic homograft with an aortic valve to establish vascular resistance. Dr Perloff describes the two most
continuity from the right ventricle to the pulmonary common clinical types: (a) subaortic VSD without
artery.193 The surgical approach has been modified over pulmonary stenosis and (b) subpulmonary VSD without
the past four decades. In these operations, reconstruction pulmonary stenosis, with each one having either low or
of the right ventricular outflow tract is necessary to high pulmonary vascular resistance.31
establish a separate pulmonary circulation. However,
there continues to be a need for reoperations. In a recent Echocardiography
study, the actuarial survival at 30 years was reported as
approximately 83% with a reoperation rate of 76% among Most individuals with DORV are operated in childhood.
survivors, and truncal valve replacement was required in Echocardiography in an unoperated adult focuses on the
20% during long-term follow-up.194 relation of the aorta and pulmonary artery, the position
Most adults seen in our practice have undergone a and relation of the VSD, any obstruction (subpulmonic
homograft or xenograft repair. Homografts are preferred or subaortic), presence of pulmonary hypertension, and
because of lower rates of stenosis and longer durability identification of a constellation of associated defects.
with long-term freedom from reoperations. Smaller Irreversible pulmonary vascular disease with Eisenmenger
conduit size at the time of the initial surgery also leads physiology will preclude surgical repair in adulthood, as
to early reoperation because the child outgrows it during seen in adults with large subaortic or subpulmonic VSDs
developing years. Other reasons for graft failure are without pulmonary stenosis.199 The best views are the
conduit calcification, stenosis, and infective endocarditis. parasternal and subcostal views for identification of the
Catheter-based interventions for conduit valve obstruction origin of both great arteries from the morphological right
may reduce some of the reoperations by allowing the ventricle.
Surgery for Double Outlet Right Ventricle arteries, and the relationship between the chambers, great
vessels, and AV valves.202,203
Depending upon the type, morphology, and circulation,
in most cases a Rastelli repair is performed. A conduit
Univentricular Heart
from right ventricle (RV) to pulmonary artery (PA), and
another conduit from left ventricle to aorta restores The spectrum of univentricular hearts comprises multiple
the physiological circulation. Other types of repairs are defects that are not amenable to biventricular repair and
palliative single ventricle (Fontan) surgery, arterial switch rely on the dominant ventricle to support both the systemic
type repair (in subpulmonary VSD with transposition- and pulmonary circulations. CHDs that present with the
like physiology), or intracardiac repair as in tetralogy of “single ventricle physiology” are tricuspid atresia, mitral
Fallot (for subaortic VSD with pulmonary/subpulmonary atresia, hypoplastic right or left ventricle, and double inlet
left ventricle. The clinical manifestations and prognosis for
stenosis). Concomitant surgical procedures are performed
an adult with any form of a univentricular heart are related
for associated defects.200,201
to the pulmonary artery blood flow/pressure, which is
affected by the presence and degree of pulmonary stenosis,
The Postoperative Adult and the level of pulmonary vascular resistance. The
The postoperative adult with a Rastelli repair has an majority of the adult survivors have undergone palliative
external conduit directing blood from the right ventricle repairs: systemic to pulmonary artery shunt and/or Glenn
into the pulmonary artery and an internal connection procedure, or Fontan procedure.
between the left ventricle and aorta through the VSD.
Follow-up echocardiograms are performed to rule out Tricupsid Atresia and the Post-Fontan Adult
residual VSD, assess degree of AV valve regurgitation,
conduit obstruction (right ventricle to pulmonary artery The most common presentation seen in the adult con-
valve conduit), kinking of the left ventricle to aorta genital heart disease clinics is tricuspid atresia (imperforate
conduit, and status of associated defects (such as COA). tricuspid valve) with a large dominant morphological
The indications for conduit replacement post-Rastelli left ventricle and a hypoplastic right ventricle. The great
repair are discussed previously in the section on d-TGA. arteries may exhibit ventriculoarterial concordance or
discordance (complete transposition of the great arteries).
Both arteries may arise from the same chamber or the
Stress Echocardiography pulmonary artery may arise from the rudimentary right
Stress echocardiography with treadmill stress testing ventricle. There may be an atrial and/or ventricular septal
is valuable in assessing exercise intolerance related to defect. The majority of these people have undergone
residual hemodynamically significant lesions, exercise- Fontan surgery in childhood.
induced arrhythmias, and chronotropic incompetency. The classic Fontan is a right atrial-to-pulmonary
artery connection in a patient with tricuspid atresia.204
Cardiac Catheterization Over the years, there have been many variations of the
Fontan repair (as shown in Figs 75.23A to C) resulting in
In limited cases, cardiac catheterization is performed to complete or near-complete separation of the pulmonary
assess the pulmonary vascular resistance, and hemodynamic and systemic circuits (in those who are not candidates
status prior to repair and before transcatheter pulmonary for biventricular repair). In these patients, the pulmonary
valve implantation in the patients with RV to PA conduits. blood flow requires unobstructed pulmonary arteries, low
filling pressure of the single ventricle, normal ventricular
Magnetic Resonance Imaging/Computed function, low pulmonary vascular resistance, and absence
of aortic valve regurgitation. Modifications of the surgical
Tomography
techniques have been associated with more favorable
MRI/CTA is performed when transthoracic or trans- outcomes. There are several variables contributing to
esophageal echocardiography are inadequate for assess- overall early and late failure of the Fontan. Hypoplastic left
ment of the gradient across the conduits. These imaging heart syndrome with a right-sided tricuspid valve (as the
modalities allow delineation of the course of the coronaries, predominant atrioventricular valve) has been shown to
aortic root (dilatation/aneurysm), arch (COA), pulmonary increase the early risk more than threefold.205
A B
Figs 75.23A to C: The Fontan procedure and its common

modifications. (A) Classic right atrial to pulmonary artery (RA to
PA) connection, (B) lateral tunnel Fontan, (C) extra cardiac conduit
(ECC) Fontan. (HRV: Hypoplastic right ventricle; IVC: Inferior vena
cava; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA:
C Right atrium; SVC: Superior vena cava).
Echocardiography be measured. The best views are the apical four-chamber

or the subcostal views. For visualizing the atriopulmonary
Echocardiographic assessment of the post-Fontan adult connection (right atrium to pulmonary artery), one must
looks into the important determinants that have a long-
obtain modified short-axis suprasternal views with right
term effect on the systemic ventricular size and function.
angulation to expose the superior vena cava. Color Doppler
The tricuspid atresia is best seen in the apical four-
and pulsed Doppler interrogation of the flow are necessary
chamber view (Fig. 75.24). These include systemic AV
valve morphology, degree of valvular regurgitation, and to rule out an obstruction. In case of an obstruction, color
the presence of subaortic stenosis. Doppler will demonstrate flow turbulence, and pulsed
The cavoatrial, atrial septal, or ventricular septal wave Doppler will show high velocity flow over 1 m/s with
defects should be identified, since they contribute to the loss of respiratory variation (normal flow is low velocity
persistence of cyanosis. In some individuals, fenestrated with respiratory variation giving it a biphasic pattern).206
atrial septum or an adjustable ASD are a necessity. In these Spontaneous contrast seen in the Fontan circuit
cases, the gradient across the fenestration or shunt should represents slow blood flow in the pathway. Right atrial
by transthoracic echocardiography. Therefore, for conduit

stenosis (other than in right atrium to right ventricular
connection), TEE is the procedure of choice for examining
the entire Fontan circuit.209 It allows more detailed
examination for potential complications that involve
the right atrial portion of the Fontan such as cavoatrial
shunting, atrial septal shunting, and atrial to pulmonary
artery obstruction. TEE is also indicated to rule out a
thrombus in an adult with a thromboembolic event and
for assessment of anatomical abnormalities of the Fontan
pathway. TEE is more likely to detect atrial and pulmonary
thrombi that could not be seen by transthoracic imaging in
patients who have undergone the Fontan operation.209,210
Three-dimensional echocardiography may augment the
Fig. 75.24: Transthoracic echocardiogram showing hypoplastic detection or exclusion of thrombi in the Fontan conduit.211
right ventricle (RV), imperforate tricuspid valve in an adult with A synopsis of echocardiographic assessment of the
tricuspid atresia, as seen in an apical four-chamber view. (ASD: post-Fontan adult is reviewed in Table 75.19.
Atrial septal defect; LA: Left atrium; LV: Left ventricle; RA: Right
atrium).
Magnetic Resonance Imaging/Computed
Tomography
thrombus formation is common with a varying incidence
depending upon the type of surgical technique and the MRI is useful for investigating extracardiac structures such
number of postoperative years. It carries a significant risk as the patency of the conduits/cavopulmonary pathways,
of death, especially in the setting of pulmonary embolism and ventricular and valvular functions.212 MRI and CTA
that is capable of causing clinical instability. Tsang et al. provides information about the systemic and pulmonary
reported a right atrial thrombus in 12% of adults with the arterial/venous anatomy in addition to intracardiac
Fontan circulation, and an associated overall mortality complex defects, ventricular volumes, mass, ejection
rate of 18%.207 The diagnosis is made incidentally in some fraction, and regurgitant fractions.
adults who are asymptomatic. In others, the thrombus
may persist despite anticoagulation. There have been Cardiac Catheterization
various studies addressing antiplatelet therapy versus
The etiology of oxygen-unresponsive hypoxemia in adults
anticoagulation in this population. Even in patients
with Fontan is determined by cardiac catheterization that
with extracardiac conduit (ECC) Fontans, antiplatelet
therapy alone failed to prevent the rate of early or late can identify potential causes including persistent Fontan
thromboembolic events. Their bleeding risk was similar fenestration, systemic venous-to-pulmonary venous
to that when anticoagulation therapy was given alone or collaterals, and pulmonary arteriovenous malformations.
in association with antiplatelet drugs.208 Although the In heart failure patients, the cause of volume retention
debate continues, the current recommendation is that may be worsening ventricular function, increasing Fontan
adults with atrial arrhythmias or those with residual ASDs/ circuit pressure, and resistance that exaggerate the right-
fenestrations should be given anticoagulation therapy. to-left shunts. Catheter-based closure of residual shunts by
coils or ASD devices may relieve the symptoms.
Cardiac catheterization plays an important role in
Transesophageal Echocardiography assessment of protein-losing enteropathy (PLE) in the post-
The onset of heart failure symptoms with hepatic Fontan adult. Causes of increased resistance to effective
congestion and jugular venous distension (in those pulmonary flow, such as obstruction to pulmonary
without a Glenn shunt) or the development of new atrial flow at the pulmonary artery or venous levels, AV valve
intractable arrhythmias prompts a search for conduit stenosis or regurgitation may contribute to progressive
stenosis. Conduit obstruction may be partially assessable PLE. The aortic-pulmonary collaterals can be also be
Table 75.19: Univentricular Heart Post-Fontan Tricuspid Atresia

Associated defects
• Univentricular heart/single ventricle physiology with a hypoplastic right ventricle
• Transposition of the great arteries
Echocardiographic assessment—postoperative
• Systemic (usually left) ventricular dysfunction
• Right atrial enlargement
• Thrombus in the right atrium
• Atrioventricular (AV) valve regurgitation
• Conduit obstruction—obstruction of the Fontan connection—usually due to right pulmonary vein compression from an
enlarged right atrium or from conduit obstruction.
delineated by aortography. For these adults, creation or McAnallen, Albert Amoranto, and Janae Johnson) at
enlargement of an atrial septal communication (ASD or the Echocardiography Laboratory, Kaiser Foundation
fenestration) may decrease the central venous pressure. Hospital, Panorama City, California, for their innumerable
It is also a prerequisite to pulmonary vasodilator therapy clinical contributions and support; and to my dear
for pulmonary hypertension or heart transplantation daughter Tanisha for her unconditional love, patience, and
evaluation. understanding.
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transposition. J Thorac Cardiovasc Surg. 1995;109(4):642– arterial switch operation in patients with Taussig-Bing
52; discussion 652. anomaly. Ann Thorac Surg. 2011;92(2):673–9.
187. Marcelletti C, McGoon DC, Mair DD. The natural history 202. Ito D, Shiraishi J, Noritake K, et al. Multidetector computed
of truncus arteriosus. Circulation. 1976;54(1):108–11. tomography demonstrates double-inlet, double-outlet
188. Di Donato RM, Fyfe DA, Puga FJ, et al. Fifteen-year right ventricle. Intern Med. 2011;50(18):2053–4.
experience with surgical repair of truncus arteriosus. 203. Saleeb SF, Juraszek A, Geva T. Anatomic, imaging, and
J Thorac Cardiovasc Surg. 1985;89(3):414–22. clinical characteristics of double-inlet, double-outlet right
189. Van Praagh R, Van Praagh S. The anatomy of common ventricle. Am J Cardiol. 2010;105(4):542–9.
aorticopulmonary trunk (truncus arteriosus communis) 204. Fontan F, Baudet E. Surgical repair of tricuspid atresia.
and its embryologic implications. A study of 57 necropsy Thorax. 1971;26(3):240–8.
cases. Am J Cardiol. 1965;16(3):406–25. 205. Gentles TL, Mayer JE Jr, Gauvreau K, et al. Fontan
190. Vitarelli A, Gheorghiade M, Gentile R, et al. Echo- operation in five hundred consecutive patients: factors
cardiographic features of truncal abnormalities. Special influencing early and late outcome. J Thorac Cardiovasc
emphasis to the evaluation of pulmonary arteries. G Ital Surg. 1997;114(3):376–91.
Cardiol. 1984;14(4):245–52. 206. DiSessa TG, Child JS, Perloff JK, et al. Systemic venous and
191. Vargas Barron J, Sahn DJ, Attie F, et al. Two-dimensional pulmonary arterial flow patterns after Fontan’s procedure
echocardiographic study of right ventricular outflow and for tricuspid atresia or single ventricle. Circulation.
great artery anatomy in pulmonary atresia with ventricular 1984;70(5):898–902.
septal defects and in truncus arteriosus. Am Heart J. 207. Tsang W, Johansson B, Salehian O, et al. Intracardiac
1983;105(2):281–6. thrombus in adults with the Fontan circulation. Cardiol
192. Williams JM, de Leeuw M, Black MD, Freedom RM, Young. 2007;17(6):646–51.
Williams WG, McCrindle BW. Factors associated with 208. Marrone C, Galasso G, Piccolo R, et al. Antiplatelet versus
outcomes of persistent truncus arteriosus. J Am Coll anticoagulation therapy after extracardiac conduit Fontan:
Cardiol. 1999;34(2):545–53. a systematic review and meta-analysis. Pediatr Cardiol.
193. McGoon DC, Rastelli GC, Ongley PA. An operation for the 2011;32(1):32–9.
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echocardiography in congenital heart disease in the adult.
194. Vohra HA, Whistance RN, Chia AX, et al. Long-term
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follow-up after primary complete repair of common
210. Fyfe DA, Kline CH, Sade RM, et al. Transesophageal
arterial trunk with homograft: a 40-year experience. J
echocardiography detects thrombus formation not
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identified by transthoracic echocardiography after the
195. Russell HM, Pasquali SK, Jacobs JP, et al. Outcomes of Fontan operation. J Am Coll Cardiol. 1991;18(7):1733–7.
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discussion 169. 212. Markl M, Geiger J, Kilner PJ, et al. Time-resolved three-
196. Rajasinghe HA, McElhinney DB, Reddy VM, et al. Long- dimensional magnetic resonance velocity mapping of
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a twenty-year experience. J Thorac Cardiovasc Surg. Fontan circulation. Eur J Cardiothorac Surg. 2011;39(2):
1997;113(5):869–78; discussion 878–9. 206–12.
CHAPTER 76
Echocardiographic Evaluation for
Acquired Heart Diseases in Childhood
Jie Sun, Rula Balluz, Lindsay Rogers, Shuping Ge
Snapshot
Infective Endocarditis
¾¾ Rheumatic Heart Disease
¾¾
Modified Duke Criteria for the Diagnosis of Infective
¾¾ Jones Criteria, Updated 1992
¾¾
Endocarditis Kawasaki Disease
¾¾
¾¾Echocardiographic Findings Coronary Ectasia and Aneurysms by Echocardiography
¾¾
¾¾Complications of Infective Endocarditis
INTRODUCTION IE can happen on normal heart or abnormal heart.

The patients with certain conditions are at increased risk
As with congenital heart disease, acquired heart diseases are
to develop IE, such as congenital heart disease, acquired
also common in children. The spectrum includes infective
heart disease, indwelling catheters, intracardiac devices,
endocarditis, rheumatic heart disease (RHD), Kawasaki
prostheses, immunodeficiency, and intravenous drug
disease, and other cardiovascular involvement related
to other systems or organs, such as hypertension, sickle abuse. The true incidence of IE in pediatric population
cell disease, chronic renal disease, and cancer survivors. or adults is unknown. IE accounts for about 1 in 1,280
Echocardiographic diagnosis and assessment have become pediatric admissions per year.1 The vegetation can occur
a routine practice to evaluate these patients initially and on valve leaflets, the walls, chordae, paraprosthetic tissue,
possibly longitudinally to facilitate diagnosis, prognosis, and shunts, or conduits. There is an increased proportion
guidance for treatment. In this chapter, we will discuss three in repaired congenital heart disease due to increased
of the most common acquired cardiovascular diseases in population in this group. The clinical diagnosis of IE is
children, namely, infective endocarditis, RHD, and Kawasaki based upon a combination of clinical features, positive
disease, and further in-depth discussion can be found in blood culture, and evidence of vegetation by imaging or
comprehensive texts and other sources. pathology.
Turbulent blood flow produced by congenital or
INFECTIVE ENDOCARDITIS acquired heart disease, such as flow from high- to
Infective endocarditis (IE) is an uncommon but life- low-pressure chambers or across a narrowed orifice,
threatening infection. Despite advances in diagnosis, traumatizes the endothelium. This creates a predisposition
antimicrobial therapy, surgical techniques, and manage for deposition of platelets and fibrin on the surface of the
ment of complications, patients with IE still have high endothelium, which results in nonbacterial thrombotic
morbidity and mortality rates related to this condition. endocarditis. Invasion of the bloodstream with a microbial
Chapter 76: Echocardiographic Evaluation for Acquired Heart Diseases in Childhood 1857
species that has the pathogenic potential to colonize this • Community-acquired enterococci, in the absence of a
site can then result in IE. Bacteria can be entrapped and primary focus
colonize to initiate focus of infection; platelets and fibrin • Persistently positive blood culture, defined as
are deposited forming vegetation. It almost always involves recovery of a micro-organism consistent with infective
a valve and can cause significant valve insufficiency or endocarditis from blood cultures drawn more than
perforation. Its complications include congestive heart 12 hours apart or all of three or a majority of four or
failure, embolization, abscess, heart block, mycotic more separate blood cultures, with first and last drawn
aneurysm, pericarditis, or myocarditis. at least 1 hour apart
Subacute presentation of IE is the more common • Single positive blood culture for Coxiella burnetti or
type. Its presentation can be insidious, nontoxic, and phase I antibody titer > 1:800.
with immune phenomena. The most common pathogen
is viridans strep; other pathogens such as fungal, HACEK Evidence of Endocardial Involvement
group, and coagulase-negative Staphylococcus aureus
can also be the cause of subacute cases. The extracardiac Positive Echocardiogram for Infective Endocarditis:
manifestations can be splenomegaly, hematuria, immune • Transesophageal echocardiography (TEE) recom
phenomena including Roth spot, splinter hemorrhages, mended as first test in the following patients: (a)
Janeway lesion, and Osler node. The extracardiac prosthetic valve endocarditis; or (b) those with at
manifestation in children with subacute IE is less common least “possible” IE by clinical criteria; or (c) those
than in adults. The patients with acute presentation of IE with suspected complicated IE such as paravalvular
can present with high fever and appear very toxic. The abscess. Transthoracic echocardiography (TTE)
acute IE mostly happens in postoperative patients or recommended as first test in all other patients.
patients with an indwelling catheter. The most common • Definition of positive findings: Oscillating intracardiac
cause of acute IE is Staphylococcus aureus. mass, on valve or supporting structures, or in the
The clinical diagnosis of IE is established based on the path of regurgitant jets, or on implanted material, in
modified Duke criteria.2 Two major or one major and three the absence of an alternative anatomical explanation
minor criteria or five minors are required to establish a or myocardial abscess or new partial dehiscence of
diagnosis of IE. prosthetic valve.
The pathological criteria for IE include micro-organism New valvular regurgitation: Increase or change in pre-
demonstrated by culture or histology of a vegetation, or existing murmur not sufficient.
in a vegetation that has embolized, or in an intracardiac Minor Criteria:
abscess or pathological lesions—vegetation or intracardiac • Predisposing heart condition or intravenous drug use
abscess—confirmed by histology showing active IE. • Fever ≥ 38.0°C (100.4°F)
• Vascular phenomena: Major arterial emboli, septic
pulmonary infarcts, mycotic aneurysm, intracranial
MODIFIED DUKE CRITERIA FOR
hemorrhage, conjunctival hemorrhage, and Janeway
THE DIAGNOSIS OF INFECTIVE lesions
ENDOCARDITIS • Immunological phenomena: Glomerulonephritis,
Osler’s nodes, Roth spots, and rheumatoid factor
Major Criteria • Positive blood culture not meeting major criterion as
noted previously (excluding single positive cultures
Positive Blood Culture for Infective for coagulase-negative Staphylococci and organisms
Endocarditis that do not cause endocarditis) or serological evidence
of active infection with organism consistent with
• Typical micro-organism for infective endocarditis from
infective endocarditis.
two separate blood cultures: Viridans streptococci,
Staphylococcus aureus, Streptococcus bovis, HACEK
group (Hemophilus spp. Actinobacillus actinomycete ECHOCARDIOGRAPHIC FINDINGS
mcomitans, Cardiobacterium hominis, Eikenella spp., Echocardiography should be performed in any patient sus
and Kingella kingae) or pected of having IE to allow earlier diagnosis, treatment,
and prevent complications. The echocardiographic appear (Fig. 76.1). When the aortic valve is affected, perforation
ance of vegetations, noninfectious thrombi, and other through the annulus into the myocardium or into either
intracardiac masses may be indistinguishable. The incidental atria is possible. Newly acquired AV block together with
echocardiographic finding of a mass in a patient who has clinical suspicion of IE may be a strong indicator for the
no associated clinical suspicion of IE likely warrants further presence of para-aortic ring abscess. Heart conditions
investigation and follow-up but should not be considered a most associated with IE were unrepaired ventricular septal
vegetation without supporting clinical features. defect, mitral regurgitation, and bicuspid aortic valve.6 In
The accuracy of TTE for detecting vegetations in IE has patients who undergo palliative surgery, infected aorta to
been evaluated in a variety of studies. Studies show that pulmonary artery shunts predominate. In those who have
TTE had a mean sensitivity of 79% for the detection of corrective surgery, the most common sites for developing
vegetations.3 There is generally good agreement between IE include right ventricle to pulmonary artery valved
the location and identity of large vegetations on TTE and conduits, prosthetic valves, and ventricular septal defect
pathological findings from autopsy or surgery.4 The role of patch closure. Patients with indwelling catheters such as
the more invasive and expensive TEE in the diagnosis of central lines can also develop line infection or associated
IE stems from its high sensitivity in detecting and defining vegetation (Fig. 76.2).
valve vegetations. The sensitivity of TEE is substantially
higher (above 90%) than the values achieved with the Vegetation Size and Embolic Risk
transthoracic approach.5
In general, a larger vegetation size appears to be predictive
of embolic risk. In a study of 105 patients with IE, patients
Vegetation Location with a vegetation diameter above 10 mm had a significantly
The morphology of IE vegetations is dependent on the higher incidence of embolic events than did those with
location of the endothelial lesion and always follows the smaller vegetations (47 vs 19%, P < 0.01).7 The association
“pathological” bloodstream. When IE occurs in association was particularly strong in patients with mitral valve
with ventricular septal defect, the vegetation is typically endocarditis. Vegetation size did not appear to predict
visualized on the right ventricular aspect of the septum other complications such as severe heart failure and death
and/or on the site where the high-velocity jet strikes the and was not related to the location of endocarditis or type
right ventricular free wall. In the case of patent ductus of organism.
arteriosus, the vegetation may float through the pulmonary A series of 211 patients with 28 embolic events found
artery. In patients with regurgitation of atrioventricular that vegetation size was associated with embolic risk for
valves, the vegetation is located on the atrial side staphylococcus and mitral valve vegetations, but was not
Fig. 76.1: Mitral valve vegetation. A vegetation involves the posterior Fig. 76.2: Inferior vena cava (IVC) vegetation. IVC vegetation
leaflet of mitral valve (white arrow). A perforation with color flow sig- (black arrow) in a patient who had an indwelling central line caus-
nals moving through it is noted (red arrow). Mitral valve insufficiency ing direct trauma of endothelium.
is present (yellow arrow). This patient had cerebral embolism.
associated with embolic risk for streptococcus or aortic Infective Endocarditis Prophylaxis
vegetations.8
Vegetation mobility confers incremental risk beyond
Recommended for Invasive Dental
vegetation size. In a series of 178 patients with definite Procedure10
IE who underwent TEE, the incidence of embolism, as • Prosthetic cardiac valve or prosthetic material used
diagnosed by cerebral and thoracoabdominal CT scans, for cardiac valve repair
was higher when vegetation size was ≥ 15 mm and when • Previous IE
the vegetation was moderately or severely mobile (62 vs • Unrepaired cyanotic congenital heart defect (CHD),
20% for low mobility).9 Embolic events were particularly including palliative shunts and conduits
frequent when both severely mobile and very large • Completely repaired congenital heart defect with
vegetations were present. Embolic risk generally falls with prosthetic material or device, whether placed by
time after institution of appropriate antibiotic therapy. surgery or by catheter intervention, during the first
6 months after the procedure†
• Repaired CHD with residual defects at the site or
COMPLICATIONS OF INFECTIVE
adjacent to the site of a prosthetic patch or prosthetic
ENDOCARDITIS device (which inhibit endothelialization)
In addition to its role in diagnosing IE, echocardiography is • Cardiac transplantation recipients who develop
important for recognizing the intracardiac complications cardiac valvulopathy
Except for the conditions listed above, antibiotic
associated with IE including regurgitant valve lesions,
prophylaxis is no longer recommended for any other form
chordal rupture, valve perforation, prosthetic dehiscence,
of CHD.
and paravalvular leak. Vegetations may also extend to
the outside of valve into surrounding structures to cause
abscess, fistula, or pseudoaneurysm formation. RHEUMATIC HEART DISEASE
Rheumatic fever is an acute inflammatory illness that
Negative Result occurs following an upper respiratory infection with
group A b-hemolytic streptococci (GAS). Rheumatic fever
The implications of an initial TEE examination that fails particularly affects children 5–15 years old. It is the most
to show vegetations in a patient with suspected IE should common form of acquired heart disease in children and
be carefully considered. Although TEE cannot definitively young adults throughout the world. It is a rare disease
rule out IE, its high diagnostic sensitivity results in a in the United States. However, several outbreaks were
low probability of the disease when negative results are reported in certain geographical areas.11 The treatment
obtained in a patient with an intermediate likelihood of of strep pharyngitis shifts to nonrheumatogenic strains
the disease. However, repeat examination is important, of GAS. Its presentation is less classic. The significance
particularly in patients at high risk for IE. Although the rate of rheumatic fever is chronic rheumatic valvar disease
of false-negative TEE examinations in patients with IE is including mitral regurgitation, aortic regurgitation, mitral
low, negative results confer an obligation of careful follow- stenosis, or aortic stenosis. It usually takes over decades
up with exercise of sound clinical judgment. from valvar regurgitation to valvar stenosis.
Infective Endocarditis Prophylaxis JONES CRITERIA, UPDATED 199212

In patients with underlying cardiac conditions associated Major Criteria
with the highest risk of adverse outcome from IE, IE • Carditis
prophylaxis for dental procedure is reasonable. In patients • Polyarthritis
with conditions associated with the highest risk of adverse • Chorea
outcome from endocarditis, giving prophylaxis for dental • Erythema marginatum
procedures is reasonable. • Subcutaneous nodules
†
Prophylaxis is reasonable because endothelialization of prosthetic material occurs within 6 months after the procedure.
Minor Criteria Aortic regurgitation occurs in approximately 20–25%

of patients with acute rheumatic carditis, usually in
• Arthralgia combination with mitral regurgitation. Isolated aortic
• Fever regurgitation occurs in approximately 5% of patients with
• Elevated acute phase reactants
acute rheumatic carditis14 (Fig. 76.4).
• Erythrocyte sedimentation rate
• C-reactive protein
• Prolonged PR interval Chronic Rheumatic Heart Disease
Plus supporting evidence of an antecedent group A Chronic mitral regurgitation is the most common form of
streptococcal infection: RHD in children and adults.
• Positive throat culture or rapid strep test
Mitral valve stenosis usually occurs in the third to
• Elevated or rising streptococcal antibody titers
fifth decade of life.15 The natural history of rheumatic
mitral valve disease can begin with regurgitation, to
Acute Valvulitis complete resolution of regurgitation without evidence
The mitral valve is the predominant valve affected during of heart disease, and then on to later development of
acute carditis.13 Characteristic changes include annular clinically significant mitral stenosis, and/or regurgitation
dilation and chordal elongation, leading to prolapse when patients reach adulthood.16 Characteristic changes
of the anterior leaflet. As a result, the leaflets no longer occur to the mitral valve apparatus in rheumatic fever,
coapt appropriately and there is a regurgitant orifice. including thickening and the scarring of the valve leaflets,
In rheumatic carditis, the regurgitant jet is typically commissures, and chordae tendineae. Fusion of the mitral
directed toward the posterolateral wall of the left atrium, cusps and chordae tendineae leads to thickening and
causing thickening and calcification of the endocardium
shortening of these structures, resulting in a mitral orifice
(Fig. 76.3). It is important to differentiate rheumatic mitral
that is restricted in size and shaped like a funnel. Women
prolapse from mitral prolapse seen in Barlow’s syndrome.
are more likely than men to develop rheumatic mitral
In rheumatic fever mitral carditis, only the coapting
portion of the anterior leaflet prolapses and there is no stenosis.17
billowing of the medial portion. In contrast, billowing of On echocardiography, patients with rheumatic mitral
the posterior or both leaflets of the mitral valve occurs stenosis have thickened echodense leaflets, commissural
in Barlow’s syndrome (mitral valve prolapse). Another and/or chordal fusion, and abnormal diastolic leaflet
differentiating feature is that chordal rupture frequently excursion resulting in a bent knee or hockey stick
occurs with Barlow’s syndrome, but rarely in patients with appearance of the anterior leaflet. Mitral stenosis and
rheumatic mitral valve disease. regurgitation may coexist.
Fig. 76.3: Mitral valve insufficiency. Mitral valve insufficiency Fig. 76.4: Aortic insufficiency. Aortic insufficiency (white arrow) in
(white arrow) in a patient with acute rheumatic heart disease. a patient with acute rheumatic heart disease.
Like mitral valve stenosis, aortic valve stenosis is a CORONARY ECTASIA AND ANEU-
form of chronic RHD and occurs about 20–40 years after
the initial acute illness. On echocardiography, imaging
RYSMS BY ECHOCARDIOGRAPHY
of chronic rheumatic aortic valve stenosis demonstrates The major sequelae of Kawasaki disease are related to
thickened leaflets with variable degrees of commissural the coronary arterial system. Cardiac imaging is critical
fusion and leaflet retraction. Dooming of the leaflets, for the evaluation of all patients with suspected Kawasaki
increased echogenicity, and restricted motion present as disease. It requires serial echocardiograms. The initial
aortic valve stenosis progresses. echocardiogram should be performed as soon as the
diagnosis is suspected, but initiation of treatment should
KAWASAKI DISEASE not be delayed by the timing of the study. This initial
Kawasaki disease is an acute, self-limited vasculitis that study establishes a baseline for longitudinal follow-up
occurs predominantly in infants and young children. It of coronary artery morphology, cardiac function, and
was first described in Japan in l967 by Tomisaku Kawasaki. the evolution and resolution of pericardial effusion
The disease is now known to occur in both endemic and when present. Two-dimensional (2D) imaging should be
community-wide epidemic forms in the Americas, Europe, performed with the highest frequency transducer possible.
and Asia in all races. Kawasaki disease is characterized These probes allow for higher-resolution and detailed
by fever, bilateral nonexudative conjunctivitis, erythema evaluation of the coronary arteries. The focus should be
of the lips and oral mucosa, changes in the extremities, on imaging the left main coronary artery (LMCA), left
rash, and cervical lymphadenopathy. Coronary artery anterior descending coronary artery (LAD), left circumflex
aneurysms or ectasia develop in approximately 15–25% coronary artery (LCX), and right coronary artery (RCA).
of untreated children with the disease and may lead to Common sites of coronary aneurysms include the proximal
myocardial infarction, sudden death, or ischemic heart LAD and proximal RCA, followed by the LMCA, then
disease.18–20 LCX, and finally the distal RCA and the junction between
In the United States, Kawasaki disease is the leading the RCA and posterior descending coronary artery22
cause of acquired heart disease in children. Treatment of (Figs 76.5 to 76.7). In addition to measuring coronary artery
Kawasaki disease in the acute phase is directed at reducing dimensions, imaging the coronary arteries also may reveal
inflammation in the coronary artery wall and preventing
the lack of normal tapering and perivascular echogenicity
coronary thrombosis. The long-term therapy in individuals
or “brightness”.23
who develop coronary aneurysms is aimed at preventing
myocardial ischemia or infarction.
The etiology of Kawasaki disease remains unknown,
although clinical and epidemiological features strongly
suggest an infectious cause. It also is possible that Kawasaki
disease results from an immunological response that is
triggered by any of several different microbial agents.
Kawasaki disease is a generalized systemic vasculitis
involving blood vessels throughout the body. Aneurysms
may occur in other extraparenchymal muscular arteries.
The early stages in the formation and development of
arteritis in Kawasaki disease have been well studied
morphologically in relatively large muscular arteries.21
Cardiovascular manifestations can be prominent in the
acute phase of Kawasaki disease and are the leading cause
of long-term morbidity and mortality in these patients.
During this acute phase, the pericardium, myocardium, Fig. 76.5: Left main coronary artery (LMCA; white arrow) and left
endocardium, valves, and coronary arteries may all be anterior descending artery (LAD; red arrow) ectasia in a patient
involved. with Kawasaki disease without aneurysm.
Table 76.1: Echocardiographic Views of Coronary Arteries in Patients with Kawasaki Disease
Left main coronary artery: Precordial short-axis at level of aortic valve; precordial long-axis of left ventricle (superior tangential);
subcostal left ventricular long-axis
Left anterior descending coronary artery: Precordial short-axis at level of aortic valve; precordial superior tangential long-axis of
left ventricle; precordial short-axis of left ventricle
Left circumflex: Precordial short-axis at level of aortic valve; apical four-chamber
Right coronary artery, proximal segment: Precordial short-axis at level of aortic valve; precordial long-axis (inferior tangential) of
left ventricle; subcostal coronal projection of right ventricular outflow tract; subcostal short-axis at level of atrioventricular groove
Right coronary artery, middle segment: Precordial long-axis of left ventricle (inferior tangential); apical four-chamber; subcostal
left ventricular long axis; subcostal short-axis at level of atrioventricular groove
Right coronary artery, distal segment: Apical four-chamber (inferior); subcostal atrial long-axis (inferior)
Posterior descending coronary artery: Apical four-chamber (inferior); subcostal atrial long-axis (inferior); precordial long-axis
(inferior tangential) imaging posterior interventricular groove
Fig. 76.6: Left main coronary artery (LMCA; white arrow) and left Fig. 76.7: Right coronary artery aneurysm (white arrow) in a
anterior descending artery (LAD; yellow arrow) ectasia and early patient with history of Kawasaki disease.
aneurysm near the bifurcation (red arrow) in a patient with Kawa-
saki disease.
The most commonly used view is the parasternal short- axial and lateral diameters are nearly equal or as fusiform
axis at the level of the aortic root. However, a compre if symmetric dilatation with gradual proximal and distal
hensive study of both the left and right coronary arteries tapering is seen. In the United States, aneurysms were
and their branches should be performed from parasternal, classified as small (< 5 mm internal diameter), medium
four-chamber, and subcostal views to ascertain full (5–8 mm internal diameter), or giant (> 8 mm internal
evaluation of the coronary system (Table 76.1). diameter). The Japanese Ministry of Health criteria classify
The coronary arteries should be measured by 2D coronary arteries as abnormal if the internal lumen
echocardiography from inner edge to inner edge excluding diameter is > 3 mm in children < 5 years old or > 4 mm in
points of branching. Particular attention should be paid children ≥ 5 years old; if the internal diameter of a segment
to describe coronary abnormalities, including ectasia, measures ≥ 1.5-times that of an adjacent segment; or if the
aneurysm, or intraluminal thrombi. When a coronary coronary lumen is clearly irregular.22
artery is larger than normal (dilated) without a segmental In addition, assessment of left ventricle (LV) function
aneurysm, the vessel is considered ectatic. The use of z should be a part of the echocardiographic evaluation
scores are available for the LMCA, proximal LAD, and of all patients with suspected Kawasaki disease. LV end-
proximal RCA. Aneurysms are classified as saccular if diastolic and end-systolic dimensions and a shortening
fraction should be measured from standard M-mode 5. Shively BK, Gurule FT, Roldan CA, et al. Diagnostic
tracings. Apical imaging allows the estimation of LV value of transesophageal compared with transthoracic
end-diastolic and end-systolic volumes and an ejection echocardiography in infective endocarditis. J Am Coll
Cardiol. 1991;18(2):391–7.
fraction. Evaluating regional wall motion may be useful,
6. Di Filippo S, Delahaye F, Semiond B, et al. Current patterns
especially in children with coronary artery abnormalities of infective endocarditis in congenital heart disease. Heart.
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should be performed to assess the presence and degree of in infective endocarditis: reassessment of prognostic
valvular regurgitation (in particular for mitral and aortic implications of vegetation size determined by the
transthoracic and the transesophageal approach. J Am Coll
valves). Finally, the presence of pericardial effusion should Cardiol. 1989;14(3):631–8.
be evaluated as a part of routine evaluation. 8. Vilacosta I, Graupner C, San Román JA, et al. Risk of
For follow-up studies in patients with Kawasaki disease, embolization after institution of antibiotic therapy for
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It is important to recognize the limitations of echo
12. Dajani AS, Ayoub E, Burman FZ, et al. Secial Writing Group
cardiography in the evaluation and follow-up of patients of the Committee on Rheumatic fever, Endicarditis, and
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valve stenosis. Eur Heart J. 1991;12 Suppl B:55–60.
16. Virmani R, Farb A, Burke AP, et al. Pathology of acute
REFERENCES rheumatic carditis. In: Narula J, Virmani R, Reddy KS,
Tandon R, editors. Rheumatic Fever. Washington, DC:
1. Van Hare GF, Ben-Shachar G, Liebman J, et al. Infective
American Registry of Pathology Press;1999:221.
endocarditis in infants and children during the past
17. Stollerman GH. Rheumatic fever in the 21st century. Clin
10 years: a decade of change. Am Heart J. 1984;107(6):
Infect Dis. 2001;33(6):806–14.
1235–40. 18. Kato H, Sugimura T, Akagi T, et al. Long-term consequences
2. Li JS, Sexton DJ, Mick N, et al. Proposed modifications to of Kawasaki disease. A 10- to 21-year follow-up study of
the Duke criteria for the diagnosis of infective endocarditis. 594 patients. Circulation. 1996;94:1379–85.
Clin Infect Dis. 2000;30(4):633–8. 19. Dajani AS, Taubert KA, Gerber MA, et al. Diagnosis and
3. O’Brien JT, Geiser EA. Infective endocarditis and echo therapy of Kawasaki disease in children. Circulation.
cardiography. Am Heart J. 1984;108(2):386–94. 1993;87:1776–80.
4. Gilbert BW, Haney RS, Crawford F, et al. Two-dimensional 20. Taubert KA, Rowley AH, Shulman ST. Nationwide survey
echocardiographic assessment of vegetative endocarditis. of Kawasaki disease and acute rheumatic fever. J Pediatr.
Circulation. 1977;55(2):346–53. 1991;119:279–82.
21. Naoe S, Takahashi K, Masuda H, et al. Kawasaki disease. Kawasaki disease: a statement for health professionals
With particular emphasis on arterial lesions. Acta Pathol from the Committee on Rheumatic Fever, Endocarditis
Jpn. 1991;41:785–97. and Kawasaki Disease, Council on Cardiovascular Disease
22. Newburger JW, Takahashi M, Gerber MA, et al.; Committee in the Young, American Heart Association. Circulation.
on Rheumatic Fever, Endocarditis and Kawasaki Disease; 2004;110(17):2747–71.
Council on Cardiovascular Disease in the Young; American 23. Newburger JW, Taubert KA, Shulman ST, et al. Summary
Heart Association; American Academy of Pediatrics. and abstracts of the Seventh International Kawasaki
Diagnosis, treatment, and long-term management of Disease Symposium: December 4–7, 2001.
SECTION 7
Miscellaneous and Other

Noninvasive Techniques
Chapters
Chapter 77 Echocardiography in Systemic Diseases Chapter 82 Lung Ultrasound in Cardiology
Chapter 78 Echocardiography in Women Chapter 83 The Future of Echocardiography and Ultrasound
Chapter 79 Echocardiography in the Elderly Chapter 84 A Primer on Cardiac MRI for the
Chapter 80 How to do Echo for the Electrophysiologist Echocardiographer
Chapter 81 Echocardiography in Life-Threatening Conditions Chapter 85 Cardiac CT Imaging
1867
CHAPTER 77
Echocardiography in
Systemic Diseases
Mahdi Veillet-Chowdhury, Smadar Kort
Snapshot

Systemic Lupus Erythematosus 
Carcinoid

Rheumatoid ArthriƟs 
Chagas Disease

Hypereosinophilic Syndrome 
Sarcoidosis

Systemic Sclerosis 
Thyroid Disorders

Renal Disease 
NutriƟonal Deficiency

Amyloidosis
INTRODUCTION atherosclerosis, valvular disease, pericardial disease, and

myocardial disease.1,2 Echocardiography plays a vital role
The presence of cardiac involvement in patients who suffer in the detection of involvement of cardiac structures,
various systemic diseases is relatively common and is often such as detection of pericardial effusions or diastolic
associated with worse prognosis; therefore, early diagnosis dysfunction. In fact, the presence of pericarditis is one
is critical. Echocardiography is a simple, noninvasive of the classifying criteria established by the American
imaging modality that is valuable in the evaluation of College of Rheumatology for diagnosing SLE. Studies
patients with suspected cardiac manifestations of certain have shown the prevalence of pericardial effusions in
disease processes, from autoimmune syndromes to patients with SLE ranging from 20% to 30% in some
inflammatory conditions to various infections. In this populations.3,4 The chronic inflammatory state can
chapter, we will discuss the role of echocardiography in lead to early atherosclerosis, vasculitis, and ultimately
the assessment of these patients and illustrate how newer myocardial involvement, leading to left ventricular
features of echocardiography such as tissue Doppler (LV) diastolic dysfunction. A study of 85 SLE patients
imaging, three-dimensional (3D) echocardiography, and described abnormally prolonged isovolumetric relaxation
speckle-tracking can be used to better assess specific times (IVRTs) and significantly greater thickness of the
pathologies. interventricular septum and posterior wall, indicating
worsened diastolic function.5
SYSTEMIC LUPUS ERYTHEMATOSUS Another frequent finding in patients with SLE is the
detection of Libman–Sacks endocarditis. Libman–Sacks
Systemic lupus erythematosus (SLE) is an autoimmune endocarditis is a nonbacterial verrucous valvular lesion
disorder involving antinuclear autoantibodies that causes that often involves the mitral valve (MV) (Fig. 77.1).6 It is
a systemic inflammatory state in patients. Cardiovascular believed that the deposition of fibrin-platelet thrombi on
involvement is common, which includes accelerated the affected valve leads to the vegetations. As patients
1868 Section 7: Miscellaneous and Other Noninvasive Techniques
the MPI, the transmitral flow propagation velocity was

shown to identify diastolic dysfunction in patients with
long-standing history of RA.15 In addition, patients with
RA have prolonged left and right ventricular deceleration
times (DTs) and IVRTs compared to control subjects.16
A study involving 60 patients with long-standing RA
demonstrated that the ratio of the early diastolic velocity
of the mitral annulus (E') to the late diastolic velocity of the
mitral annulus (A'), as well peak A’ and the ratio between
the early diastolic filling (E) to E' (E/E') parameters were
significantly impaired. Tissue Doppler imaging (TDI)
can add incremental value to conventional Doppler
echocardiography in RA patients, especially with RV
dysfunction, as RV diastolic impairment can be a predictor
of subclinical myocardial and pulmonary disease. A study
Fig. 77.1: Libman–Sacks endocarditis in a 58-year-old woman
evaluating RV diastolic function in 35 RA patients revealed
with systemic lupus erythematosus (SLE). The transesophageal
echocardiogram (TEE) demonstrates a verrucous lesion on the that the prevalence of RV diastolic abnormalities defined
anterior mitral valve leaflet (arrow). (LA: Left atrium; LV: Left as a ratio of (E) to atrial filling (A) of <1, was higher in RA
ventricle). patients compared to control subjects.17
The most common cardiac structure involved in RA
are usually asymptomatic, Libman–Sacks endocarditis is is the pericardium, with evidence of pericarditis reported
often identified at autopsy, resulting in underestimation to be 30–50% in these patients.18 Although <10% of the
of the true prevalence of this entity by initial studies.7 patients with pericarditis are symptomatic, constrictive
However, with increased awareness of this lesion and pericarditis or rapidly developing exudative effusions
the development of more advanced echocardiographic portend a poor prognosis.18,19
modalities, a prevalence of up to 43% has been reported.6,8 Valvular disease is also common, with a reported
The vegetations seen on transthoracic echocardiography prevalence of approximately 39% in patients with RA.20
(TTE) and transesophageal echocardiogram (TEE) are The inflammatory state leads to the formation of nodules
usually irregular in shape, firmly attached to the surface and fibrosis throughout the entire valvular apparatus,
at the valve ring and commissures, and vary in size and most frequently involving the mitral and aortic valves
echodensity.9–11 (Movie clip 77.1; Figs 77.2A to C).20–22 Mitral regurgitation
is the most common valvular abnormality, while aortic
RHEUMATOID ARTHRITIS regurgitation and stenosis can also occur.23
Rheumatoid arthritis (RA) is a systemic autoimmune
disease of unknown etiology that can affect several organs, HYPEREOSINOPHILIC SYNDROME
including the heart. Cardiac manifestations include Hypereosinophilic syndrome (HES), or Loeffler’s syndrome,
coronary artery disease, conduction disease, valvular heart is characterized by a persistently elevated eosinophil
disease, pericardial disease, and myocardial disease.12 count (≥1.5 × 109/L) for at least 6 months as well as
Both systolic and diastolic LV dysfunction can occur eosinophil-related end organ damage with no identifiable
due to a combination of accelerated cardiovascular disease, cause. HES tends to occur more commonly in males
chronic inflammation, and use of cardiotoxic medications between the third and sixth decades of life. Cardiac
causing nodules and fibrosis in the myocardium.13 Since involvement has been reported in >50% of patients and
the myocardial performance index (MPI) is independent of suggests a poor prognosis.24 Cardiac damage classically
heart rate, preload, and afterload, it has been used to assess occurs in two stages. In the first stage, acute myonecrosis
global LV dysfunction in RA patients. A study involving occurs due to eosinophilic infiltration of the myocardium
40 patients with active RA revealed that the MPI of the LV and subsequent release of toxic proteins. The later
was significantly higher than those of control patients, stage is described by the formation of mural thrombi,
indicative of abnormal global LV function, while the right endomyocardial fibrosis, valvular disease, and restrictive
ventricular (RV) MPI was preserved.14 In combination with cardiomyopathy.25,26
Chapter 77: Echocardiography in Systemic Diseases 1869
A B
Figs 77.2A to C: (A) A three-dimensional (3D) echocardiogram of a

56-year-old gentleman with rheumatoid arthritis (RA). A parasternal
long-axis view of a thickened and fibrotic mitral valve (MV) leaf-
lets and chordae (arrow); (B) A 3D parasternal short-axis view of
the thickened and fibrotic MV (arrow); (C) A two-dimensional (2D)
parasternal short-axis view of a thickened aortic valve (AV) with
a small nodular density on the left coronary cusp (curved arrow).
C (LV: Left ventricle).
On echocardiography, there can be evidence of of cardiac involvement, such as decreases in transmitral

endomyocardial thickening with plaques larger than E-wave DT and an E/A ratio consistent with restrictive
2 mm. In addition, valvular regurgitation due to adhesion physiology.
of the valvular apparatus can become clinically signi-
ficant. Progressive scarring causes restriction of the SYSTEMIC SCLEROSIS
chordae tendinae, which often affects the posterior
mitral leaflet.27,28 In addition, there can be evidence of Systemic sclerosis (SSc) is a connective tissue disorder
fibrothrombotic growth on both the ventricular apices due characterized by vascular lesions and widespread fibrosis
to blood stasis and denuded myocardium, occasionally of the skin and other organs, frequently the heart, with
causing obliteration of the cavities (Figs 77.3 and 77.4). one study showing the prevalence of cardiac involvement
Studies have also reported a predilection for pericardial to be as high as 32% in patients with diffuse SSc.29 A
involvement, with one study demonstrating pericardial range of cardiac manifestations can be seen, including
effusions in almost one-third of subjects with HES.25 conduction system disease, coronary artery disease,
As mentioned earlier, later stages of HES can cause pericardial involvement, RV dysfunction due to pulmonary
restrictive cardiomyopathy. As the endomyocardial hypertension, and both LV systolic and diastolic
thickening and fibrosis leads to diastolic dysfunction, dysfunction. As studies have shown that patients with
Doppler echocardiography and TDI measurements of cardiac involvement confer a poor prognosis, screening
diastolic parameters can help in evaluating the full scope for subclinical disease with echocardiography is vital.30
Fig. 77.3: A 74-year-old gentleman with hypereosinophilic syn-

drome (HES). A two-dimensional (2D) apical four-chamber view
demonstrating thickening and infiltration of the right ventricle
(RV; arrow). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
A B
C D
Figs 77.4A to D: (A) A 67-year-old gentleman with asthma and hypereosinophilic syndrome (HES) with an eosinophil count of
5.6 × 109/L. A two-dimensional (2D) parasternal long-axis view demonstrating thickening and infiltration of the left ventricle (LV); (B) A
parasternal short-axis view demonstrating thickening and infiltration of the LV (arrow); (C) An apical four-chamber view demonstrating
thickening and infiltration of the LV (arrow); (D) An apical two-chamber view demonstrating thickening and infiltration of the LV. (LA: Left
atrium; LV: Left ventricle; RV: Right ventricle).
Myocardial fibrosis is a hallmark cardiac manifestation conditions such as hypertension and diabetes leads to
of SSc and can lead to increased ventricular mass, ventri- myocardial fibrosis and the development of left ventricular
cular wall motion abnormalities, and impaired diastolic hypertrophy (LVH). However, due to the high tendency
relaxation.31,32 TDI can be used to measure systolic for pressure and volume overload to occur, the cardiac
longitudinal velocity (S') and mitral annular velocity (E') structure can display varying types of hypertrophy.44,45
to identify patients with systolic and diastolic dysfunction, This cardiac alteration leads to dysfunction in both
respectively.33 A study with 100 consecutive patients diastolic and systolic properties. Even though LVH is fairly
with SSc showed that using TDI to evaluate mitral and common in this population, the accurate assessment of
tricuspid annular velocities, the presence of subtle RV and LV mass index is especially difficult due to variations in
LV dysfunction was able unmasked.34 Speckle-tracking patients’ volume status.44,46 Similarly, diastolic assessment
echocardiography can also provide insight toward in these patients could be challenging. Elevated LV filling
subclinical LV and RV systolic dysfunction. Another study pressures signify worsening relaxation and compliance of
showed that in SSc subjects with a normal radionuclide the LV; however, in the CKD patient, the wide variations in
ejection fraction, speckle imaging was able to detect volume status makes mitral inflow velocity measurements
lower systolic and diastolic strain rates.35 Speckle-tracking challenging.47 Therefore, other assessments can further
imaging of the RV revealed that while TDI indices were aid in evaluation of diastolic dysfunction. In hemodialysis
similar in subjects with SSc compared with controls, the (HD) patients, left atrial (LA) enlargement, assessed by
SSc subjects were found to have reduced strain.36 calculating the LA volume index, has been found to be a
Due to pulmonary involvement, the presence of predictor of mortality.48,49 Furthermore, the ratio of early
secondary RV dysfunction needs to be fully excluded. mitral flow velocity to early mitral annulus velocity (E/E')
has been proven to be a reliable measure of LV filling
Therefore, validated measurements of RV function should
pressures in end-stage renal disease (ESRD) patients.50
be incorporated into the routine assessment in SSc, and
Uremic cardiomyopathy is characterized by LV enlar-
should include echocardiographic parameters such as
gement, hypertrophy, and systolic dysfunction. Frac-
tricuspid annular plane systolic excursion, RV free wall
tional shortening, a measurement of global LV systolic
TDI-derived S', and pulmonary artery pressures.37,38
function in the absence of regional abnormalities, could
Pericardial disease in SSc can range from asymptomatic
overestimate contractility in patients with concentric
pericardial effusions to constrictive pericarditis. Although
LVH and, therefore, should not be used in these patients.
up to 78% of patients with SSc have been reported to have
In dialysis patients, tissue velocity and strain imaging
some pericardial involvement, the prevalence of clinically
can detect changes in LV function and are less affected
symptomatic disease is low at 5–16%.39–41 Pericardial by the volume status of the patient.51 The utility of stress
effusions in the setting of pulmonary hypertension is a echocardiography for excluding coronary atherosclerotic
prognostic indicator in patients with SSc, as the presence disease in these patients is uncertain, given the relatively
of pericardial effusions together with increased right lower specificity of this modality in the presence of LVH, a
atrial (RA) size and abnormal interventricular septal common finding in this patient population.52
displacement during diastole predicts poor outcomes.42 Another common finding in HD patients is myocardial
Valvular disease has also been described, with nodular stunning due to acute myocardial ischemia during dialysis
thickening of the MV reported in up to 38% of patients sessions, which over time increases the risk of heart failure
with SSc.43 and arrhythmias.53 A study examining 70 HD patients
found a significant reduction in systolic function in 64% of
RENAL DISEASE subjects.54
Given the abnormalities in calcium and phosphorus
Cardiovascular mortality is significantly increased in metabolism and inflammation associated with renal
patients with chronic kidney disease (CKD), leading to disease, valvular and vascular calcifications are common
earlier atherosclerosis, valvular and pericardial disease, in patients with CKD, particularly mitral annular
arrhythmias, and heart failure. Use of echocardiography calcifications (MACs), which has been reported to occur
plays a crucial role in the evaluation of these patients as in > 40% of ESRD patients (Movie clip 77.2; Figs 77.5A
the structural and functional abnormalities can alter the and B).55–57 Of great importance are the findings of mobile
management and prognosis of this patient population. components associated with MAC, as these lesions can
Over time, a combination of CKD and other medical become sources of emboli.58–60
A B
Figs 77.5A and B: (A) A 59-year-old woman with endstage renal disease (ESRD). A transesophageal echocardiogram (TEE) demon-
strating a large echodensity (arrows) on the atrial surface of the posterior mitral valve (MV) annulus and leaflet consistent with mobile
mitral annular calcification (MAC); (B) A TEE zoomed in view demonstrating a large echodensity (arrows) on the atrial surface of the
posterior MV annulus and leaflet consistent with mobile mitral annular calcification (MAC). (LA: Left atrium; LV: Left ventricle; RA: Right
Uremic pericarditis is used to describe ESRD patients vessels, they can involve the intramural vasculature and
who develop clinical manifestations of pericarditis. Studies lead to microvascular ischemia. In addition, although
have reported a wide range of prevalences, but generally relatively rare, amyloid can accumulate in the pericardium
occur in < 20% of patients with ESRD.61–63 Similar to the and lead to constrictive physiology.72
development of pericarditis, pericardial effusions can also As there is no single test with a great sensitivity to
develop, with studies showing prevalences of 11–27% in diagnose cardiac amyloid, when evaluating a patient with
ESRD patients.62,64–68 suspected cardiac amyloid it is important to correlate the
clinical picture with the diagnostic findings. Echocardio-
AMYLOIDOSIS graphy can be a valuable tool to support establishing the
diagnosis. Although not specific, one of the characteristic
Amyloidosis is a systemic disorder that is caused by two-dimensional (2D) echocardiographic features is a
extracellular deposits of insoluble aggregated proteins granular appearance of the myocardium (Movie clip 77.3;
with a β-pleated sheath configuration. The incidence Figs 77.6 to 77.8). The most common finding is thickening
of light chain (AL) amyloidosis or primary amyloidosis of the LV wall in the absence of other contributing factors
is more than 10 per million person-years in the US such as hypertension or aortic stenosis.73 A study showed
population and has a predilection for men in the sixth that the presence of low voltage on the electrocardiogram
decade of life.69 Amyloid can affect numerous organ (ECG) associated with an interventricular septal thick-
systems but infiltration of the heart confers an extremely ness of >1.98 cm has a sensitivity of 72% and specificity of
poor prognosis, as amyloid patients with congestive heart 91% for the diagnosis of cardiac amyloidosis.74 It has been
failure (CHF) have a median survival of about 6 months.70 shown that this finding has a strong inverse relationship
In cardiac amyloidosis, amyloid deposits infiltrate the with survival in patients with heart failure due to amyloid.75
myocardium with progression to myocyte necrosis and A recent study examining seven consecutive patients with
local interstitial fibrosis. endomyocardial biopsy-proven cardiac amyloid found
As the heart is thickened, diastolic dysfunction pro- that an echocardiographic pattern of preserved motion
gresses, eventually leading to restrictive cardiomyopathy.71 of the LV apex and hypokinesis of the basal to mid
Due to the reduced compliance of the LV, the chamber segments was consistently identified in all patients.76
diameters remain normal, but the free wall and septum Other striking features include biatrial enlargement and
thicken.72 While amyloid deposits are rare in the epicardial thickened valves.
Fig. 77.6: An 89-year-old woman with systemic amyloidosis. Three-

dimensional (3D) apical four-chamber view showing right ventricle
(RV) infiltration, can be used to differentiate thickening of the free
wall from a moderator band (asterisk). (LA: Left atrium; LV: Left
A B
Figs 77.7A to C: (A) An 85-year-old gentleman with systemic

amyloidosis. A two-dimensional (2D) apical four-chamber view
demonstrating a starry-sky speckled appearance and thickening
of the left ventricular (LV) myocardium; (B) A pulsed wave Doppler
echocardiography of the mitral valve (MV) demonstrating a trans-
mitral E-velocity (arrow): A-velocity (curved arrow) ratio > 2:1 and
a short deceleration time, consistent with restrictive physiology;
(C) A tissue-Doppler imaging (TDI) echocardiography of the lateral
mitral annulus demonstrating a decreased E'-velocity (asterisk),
consistent with abnormal diastolic function. (LA: Left atrium; LV:
C Left ventricle; RA: Right atrium; RV: Right ventricle).
Early amyloid deposition does impair isovolumetric eventually leading to restrictive cardiomyopathy with
relaxation leading to mild diastolic dysfunction with E/A ratio > 2:1. TDI can further assist in the diagnosis
reversal of the transmitral E/A velocity ratio initially, but as it can show decreased mitral annular velocities and
A B
Figs 77.8A to C: (A) An 80-year-old gentleman with systemic

amyloidosis and congestive heart failure. A two-dimensional (2D)
apical four-chamber view demonstrating thickening of the left ven-
tricular (LV) myocardium; (B) A pulsed wave Doppler echocardiog-
raphy of the mitral valve (MV) demonstrating a transmitral E-velocity
(arrow): A-velocity (curved arrow) ratio > 2:1, consistent with
restrictive physiology; (C) A tissue Doppler imaging (TDI) echocar-
diography of the lateral mitral annulus demonstrating a decreased
E'-velocity (asterisk), consistent with abnormal diastolic function.
C (LV: Left ventricle).
elevated filling pressures. Investigators have shown that global myocardial deformation was significantly lower in
the myocardial velocity profiles of the LV septum and patients with cardiac amyloid compared to those with LV
posterior wall show a distinctive serrated pattern not hypertrophy due to other causes. Other helpful techniques
present in patients with hypertension or hypertrophic include the use of contrast echocardiography to evaluate
cardiomyopathy.77 for coronary flow reserve and 3D echocardiography to
Another useful technique that can provide prognostic assess for the presence of intraventricular dyssynchrony.
information is tissue Doppler-based strain and strain rate Although these findings are not specific, their presence
imaging (SRI), which can allow accurate assessment of could help support the diagnosis of this rare condition.82,83
regional myocardial deformation with high spatial and
temporal resolution.78 Studies have shown that Doppler- CARCINOID
derived longitudinal systolic strain averaged for the
Carcinoid tumors are rare neuroendocrine malignancies
16 segments of the LV can differentiate biopsy-proven derived primarily from enterochromaffin cells, with the
cardiac amyloid patients from healthy control patients.79,80 primary site usually being in the gastrointestinal tract. The
A related newer technique is speckle and derived SRI, usual presentation occurs in the fifth to seventh decade of
which was shown to differentiate cardiac amyloidosis life with the classic symptom triad of flushing, secretory
and hypertrophic cardiomyopathy from other causes diarrhea, and bronchospasm.84 Carcinoid heart disease
of increased LV wall thickness.81 The authors found that has been reported to be present in up to 50–60% of patients
with carcinoid syndrome, and should therefore be TDI also can play an important role in evaluating
excluded in these patients.85 Cardiac involvement por- the progression of carcinoid heart disease. A study of 41
tends a poor prognosis, as one study has shown reduced patients with carcinoid syndrome demonstrated that E/E'
mean survival in patients with carcinoid heart disease ratio ≤ 8 was an independent marker of death.96 Myocardial
(life expectancy of 1.6 years compared to 4.6 years in those strain echocardiography can also help in the evaluation
without cardiac manifestations).86 Cardiac involvement of this population. Aside from valvular disease, carcinoid
occurs through paraneoplastic effects when tumor growth can also lead to the development of mural endocardial
within the liver allows the secretory products, such as fibrosis of the ventricles. A study involving 89 patients
serotonin, to reach the right side of the heart without having with carcinoid disease revealed reduced RV function
gone through inactivation in the hepatic or pulmonary using strain compared with healthy control subjects.
circulatory bed. However, left-sided heart disease can also This reduced RV function was independent of valvular
occur, although the incidence is much less due to the low involvement, further supporting the role of tissue Doppler
levels of serotonin present after passing through the lung.86 when assessing patients with carcinoid.97
This disease process manifests in plaque-like endocardial
deposits of fibrous tissue on the ventricular and arterial CHAGAS DISEASE
aspects of the tricuspid and pulmonic valves (PVs),
Chagas disease is a tropical disease endemic in Mexico,
respectively.87 The clinical symptoms usually encompass
Central, and South America caused by the parasite
that of right-sided heart failure, although conduction
Trypanosoma cruzi. There is usually an acute or early
disease, coronary vasospasm, constrictive pericarditis,
phase and a chronic or late phase. Although infrequent,
and restrictive cardiomyopathy have all been reported.88–91
fulminant myocarditis can occur in 1–5% of patients in the
The presence of right-sided heart failure is an independent acute phase.98 More commonly, about 30% of seropositive
predictor of mortality in this population.92 individuals develop cardiomyopathy several years after the
Echocardiography is a key tool in aiding the diag- acute phase.99 This usually occurs through a combination
nosis and management of carcinoid heart disease. of cardiac dysautonomia, microvascular disease, parasite,
Typical features include thickening of the valve leaflets and immune-mediated myocardial injury. A recent
and cusps, causing them to become retracted, fixed, systematic review demonstrated that positive serology for
and noncoapting, resulting in tricuspid and pulmonic Chagas disease is associated with a higher risk of death in
stenosis and regurgitation (Movie clip 77.4; Figs 77.9 and patients with heart failure.100
77.10).86 Most commonly, thickening involves the septal Echocardiography can provide valuable information
and anterior leaflets of the tricuspid valve (TV), as well regarding the extent of myocardial damage and, therefore,
as the chordae and papillary muscles.93 This results in help determine the prognosis of patients with Chagas heart
echocardiographic signs of severe tricuspid regurgitation, disease. Apart from acute myocarditis, the early phase
such as enlarged vena contracta width and regurgitant of Chagas disease can lead to the rapid development of
volume, dilated inferior vena cava (IVC), and systolic flow pericardial effusions, with one study noting the presence
reversal in the hepatic veins. Typically, continuous wave of pericardial effusions in 42% of subjects.101 Most of the
Doppler echocardiography shows a dagger-shaped profile, structural heart changes take place slowly over years
with an early peak pressure and rapid decline.86 If the PV after the initial infection goes unnoticed (Movie clip 77.5;
is involved, the predominant location of the plaques are Figs 77.11A to F). Studies have shown that > 50% of
at the pulmonic root, causing constriction of the root and patients with Chagas disease develop apical aneurysms,
orifice.94 However, concomitant pulmonic regurgitation and become at higher risk of forming a mural thrombus.102
is not uncommon as demonstrated by prior studies.86 If a Both TTE and TEE were shown to recognize potential
patent foramen ovale (PFO) is present, the increased RA cardiac source of emboli with high accuracy.103
pressure due to tricuspid and pulmonic valvular disease More advanced cardiac disease include global cardiac
can result in a significant right-to-left shunt, promoting dilatation and diffuse hypokinesis; however, early in the
the likelihood of developing left heart involvement with disease process, segmental wall motion abnormalities
carcinoid, and therefore the presence of a PFO should can also be identified on 2D echocardiograms, with the
routinely be excluded.95 apical and posteroinferior walls being the most commonly
A B
C D
Figs 77.9A to D: A 53-year-old gentleman with metastatic carcinoid disease to bones and liver. A two-dimensional (2D) echocardiography
showing a thickened, retracted, and noncoapting tricuspid valve (arrow); (B) A color flow Doppler echocardiography showing severe
tricuspid regurgitation with a large regurgitant jet; (C) A continuous wave (CW) Doppler echocardiography showing an increased
E-velocity across the tricuspid valve (TV), consistent with severe tricuspid stenosis (curved arrow). However, the mean pressure gradient
of 5 mm Hg across the valve excludes the presence of significant stenosis as the etiology; (D) A three-dimensional (3D) short-axis view
demonstrating a thickened, fixed, noncoapting tricuspid valve (arrow). (RA: Right atrium; RV: Right ventricle).
affected.104–106 Dobutamine stress echocardiography has moderately specific for detection of any stage of diastolic
a role when evaluating these patients, as a blunted heart dysfunction.105 In addition, in symptomatic patients, the
and contractile response during dobutamine infusion can MPI is markedly higher for both the LV and RV, consistent
be found, even in subjects without baseline regional wall with combined severe systolic and diastolic myocardial
motion abnormalities.107 Interestingly, in this study, few dysfunction.108
patients demonstrated a biphasic response to dobutamine,
with improvement in contractility at a low dose, and SARCOIDOSIS
deterioration in contractile function at a higher dose.
Over time, patients infected with Chagas are at high Sarcoidosis is a multisystem disorder that is characterized
risk of developing both systolic and diastolic dysfunction. by the presence of noncaseating granulomas that can
A study of 169 patients with Chagas cardiomyopathy affect numerous organs in the body. The etiology remains
reported that a reduced TDI septal E'-wave of 11 cm/s unknown, although environmental, occupational, infe-
and a septal E/E' ratio > 7.2 were both highly sensitive and ctious, and genetic causes have all been proposed. While
A B
Figs 77.10A and B: (A) A 53-year-old gentleman with carcinoid heart disease. A two-dimensional (2D) short-axis view showing a thick-
ened and fixed pulmonic valve (PV; arrow); (B) A continuous wave (CW) Doppler echocardiography showing mild pulmonic stenosis with
a mean transvalvular pressure gradient of 20 mm Hg (curved arrow).
A B
C D
Figs 77.11A to D
E F
Figs 77.11A to F: A 28-year-old gentleman emigrated from El Salvador and presented with Chagas heart disease. A two-dimensional
(2D) parasternal long-axis view showing a dilated left ventricle (LV; asterisk); (B) A 2D echocardiogram short-axis view showing a dilated
LV (asterisk); (C) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow); (D) A zoomed-in 2D apical four-
chamber view showing mitral regurgitation (MR); (E) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow);
(F) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow) (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
it can affect people across all ethnic groups and ages, evaluated using echocardiography.112 The LV free wall
studies have shown that there is a predilection among and interventricular septum are the most commonly
African Americans and Northern Europeans.109 While affected sites.111 LV dilatation and wall thinning can
the disease can remain asymptomatic for many years, occur leading to aneurysm formation.113 Granuloma
the most common presentation involves the pulmonary penetration into the myocardium can cause myocardial
system, causing cough and dyspnea. However, cardiac fibrosis and lead to systolic and diastolic dysfunction with
involvement is not uncommon, with a prevalence in wall motion abnormalities, usually at the mid and basal
the United States reported to be about 25% in patients levels.114 In addition, both thickening and thinning of
with systemic sarcoidosis, and should, therefore, be the interventricular septum with dyskinetic segments as
excluded in these patients.110 All cardiac structures can be well as papillary muscle dysfunction leading to valvular
involved—infiltration of the granulomas in the conduction
insufficiency have also been reported.114,115 It is important
system, myocardial involvement of all chambers, and
to identify these echocardiographic abnormalities as
the development of pericardial effusions have all been
studies have shown that the severity of LV dysfunction
reported.111
and end-diastolic diameter are independent predictors of
Although considered the gold standard for estab-
mortality in this patient population.116
lishing the diagnoses, endomyocardial biopsy often is
unable to demonstrate pathological evidence of cardiac Other echocardiographic abnormalities have been
sarcoidosis.112 Therefore, noninvasive modalities, such as noted in patients with sarcoidosis. Pericardial effusions
echocardiography, are a valuable tool in the diagnoses have been reported in up to 19% of patients with cardiac
and management of sarcoid heart disease, as it can sarcoidosis.117 In addition, constrictive pericarditis has
demonstrate a range of structural abnormalities, and can been reported, although this is infrequent.118 One study
be repeated as needed (Movie clips 77.6 to 77.8; Figs 77.12 looking at 69 patients with chronic sarcoidosis found
and 77.13). that patients with sarcoid had lower midwall fractional
Given that pulmonary disease is the most common shortening compared with controls.119 Furthermore,
manifestation of sarcoidosis, right-sided heart failure echocardiography can be used to detect cyclic variation of
due to pulmonary hypertension is commonly observed; integrated backscatter to estimate the myocardial acoustic
therefore, evidence of elevated RV pressures should be properties to recognize cardiac sarcoidosis.120
Fig. 77.12: A 41-year-old gentleman with sarcoidosis and Fig. 77.13: A 74-year-old woman with sarcoidosis. A two-
severe segmental left ventricular (LV) systolic dysfunction, who had dimensional (2D) apical four-chamber view demonstrates
normal coronary arteries on cardiac catheterization. A two-di- right ventricular (RV) hypertrophy, mildly dilated RV, and right
mensional (2D) parasternal short-axis view showing a dilated LV. atrium (RA). LA: Left atrium; LV: Left ventricle; RA: Right atrium;
(LV: Left ventricle). RV: Right ventricle.
THYROID DISORDERS SRI reveals a significant decrease in diastolic function. In

patients with Graves’ disease, valvular abnormalities have
Hyperthyroidism is characterized by elevated peripheral been reported, such as tricuspid regurgitation and MV
free thyroid hormone levels combined with decreased prolapse.128
thyroid stimulating hormone (TSH) levels. Thyrotoxicosis In contrast to hyperthyroidism, hypothyroid is chara-
can lead to significant changes in the cardiac structure
cterized by decreased lower peripheral thyroid hormone
and function, causing hypertension, heart failure, and
levels and elevated TSH levels. Physiologically, there is
arrhythmias. Echocardiography can play an important
evidence of decreased cardiac output and contractility.
role in the evaluation of patients with thyroid disorders,
As compared to hyperthyroidism, there is a predil-
as even subclinical hyperthyroidism can lead to cardiac
ection for arrhythmias due to prolonged QT interval and
abnormalities. Elevated thyroid hormones leads to a high
the prevalence of heart failure is much less. Again,
cardiac output by up to 50–300% as well as increased
echocardiographic data plays an important role in the
preload, leading to an increase in LV mass and LA
evaluation of these patients. Numerous studies have
size.121–124 Due to the increased cardiac contractility and
heart rate, shortened interventricular conduction time shown abnormalities in systolic function indices, such as
and pre-ejection period have been demonstrated.123 SRI reduced ejection fraction and fractional shortening, and
has shown enhancement of systolic stain rate in patients increased pre-ejection period (Movie clip 77.6; Figs 77.14A
with subclinical hyperthyroidism due to the greater and B).129–132 In patients with subclinical hypothyroidism,
early systolic phase deformation.125 However, stress TDI was shown to be able to demonstrate systolic
echocardiography has demonstrated a blunted increase dysfunction during exercise.133,134 LV diastolic dysfunction
in LV ejection fraction and cardiac output.126 In regards can easily be diagnosed on echocardiograms by demon-
to RV function, TDI has demonstrated enhanced systolic strating the presence of significant impairment of LV
function in subjects with overt hyperthyroidism.127 filling, prolonged IVRT, and prolongation of the MPI.135–138
Mild diastolic dysfunction has also been demonstrated Although cardiac tamponade physiology is rare, peri-
by Doppler echocardiography, with reduced peak E-wave cardial effusions can also be found frequently, with studies
velocity and a significantly higher peak A-wave velocity.125 showing a prevalence of up to 25% in patients with overt
TDI reveals impairment of the mitral annular velocity and hypothyroidism.139
A B
Figs 77.14A and B: (A) A 59-year-old gentleman with hypothyroidism. A two-dimensional (2D) parasternal long-axis view demonstrat-
ing a dilated left ventricle (LV); (B) An apical four-chamber view demonstrating dilated atria and LV. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle).
A B
Figs 77.15A and B: (A) A 46-year-old gentleman with a history of alcohol abuse and thiamine deficiency. A two-dimensional (2D) apical
two-chamber view showing a dilated left ventricle (LV) with reduced systolic function; (B) A 2D apical two-chamber view showing small
LV diameter and significantly improved systolic function after thiamine replacement. (LA: Left atrium; LV: Left ventricle).
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evaluation of the regional diastolic function in chagas’
968–74.
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124. Smit JW, Eustatia-Rutten CF, Corssmit EP, et al. Reversible
107. Acquatella H, Pérez JE, Condado JA, et al. Limited
diastolic dysfunction after long-term exogenous subclinical
myocardial contractile reserve and chronotropic incom-
hyperthyroidism: a randomized, placebo-controlled study.
petence in patients with chronic Chagas’ disease:
J Clin Endocrinol Metab. 2005;90(11):6041–7.
assessment by dobutamine stress echocardiography. J Am
125. Di Bello V, Aghini-Lombardi F, Monzani F, et al. Early
Coll Cardiol. 1999;33(2):522–9.
abnormalities of left ventricular myocardial characteristics
108. Yacoub S, Birks EJ, Slavik Z, et al. Early detection of
associated with subclinical hyperthyroidism. J Endocrinol
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126. Kahaly GJ, Wagner S, Nieswandt J, et al. Stress echocar-
2003;97(5):528–34. diography in hyperthyroidism. J Clin Endocrinol Metab.
109. Rossman MD, Kreider ME. Lesson learned from ACCESS 1999;84(7):2308–13.
(A Case Controlled Etiologic Study of Sarcoidosis). Proc 127. Arinc H, Gunduz H, Tamer A, et al. Evaluation of right
Am Thorac Soc. 2007;4(5):453–6. ventricular function in patients with thyroid dysfunction.
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systemic sarcoidosis. Circulation. 1978;58(6):1204–11. and tricuspid regurgitation in patients with Graves’ disease
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CHAPTER 78
Echocardiography in Women
Jennifer Kiessling, Navin C Nanda, Tugba Kemaloglu Öz, Aylin Sungur, Kunal Bhagatwala, Nidhi M Karia
Snapshot

Differences in Echocardiographic Measurements and 
Takotsubo Cardiomyopathy
Technical Considera ons 

Structural Heart Disease: MVP, Mitral Stenosis, and 
Echocardiography in Pregnancy, Peripartum
Mitral Annular Calcifica on Cardiomyopathy, Fetal Echocardiography

Ischemic Heart Disease/Stress
Echocardiography/Polycys c Ovarian Syndrome
INTRODUCTION (Table 78.1). The aortic root size, LV end systolic, and
LV end diastolic dimensions are larger in men than in
In terms of cardiac disease, it is often thought that men
women.1–5 There are also differences in Doppler velocities
are more affected, while women are somehow protected.
and mitral annular velocities. In women, the diastolic
This has proven to be untrue. This chapter will illustrate
early mitral annular velocities are higher using pulsed
the use of the echocardiogram in women. Furthermore,
wave tissue Doppler imaging (TDI) compared to men. For
it will highlight some differences in the standardization example, in healthy women, the normal early diastolic
of echocardiographic measurements as well as some early mitral annular velocities were 11.8 ± 3.2 cm/s in
technical challenges in women. Echocardiography is often women and 10.8 ± 3.0 cm/s in men.5 When comparing
the primary diagnostic tool to diagnose patients with the E-wave deceleration time in men versus women, it is
structural heart disease. Structural heart diseases such shorter in women. Furthermore, it has been reported that
as atrial septal defects, mitral valve prolapse (MVP), and the left ventricular end-diastolic diameter (LVEDD) and
pulmonary arterial hypertension are more commonly seen left ventricular end-systolic diameter (LVESD) volumes
in women. Also, the utilization of stress echocardiography decrease with age in both men and women, but the
in women to diagnose underlying coronary artery disease changes are more pronounced in women. Also, the muscle
(CAD) is discussed and compared to other current mass and systolic function, assessed by left ventricular
imaging modalities. The usage of the echocardiogram in ejection fraction (LVEF), increase with age, but more so in
pregnant women is discussed in detail. Finally, the role of women than men.
fetal echocardiography is highly useful to diagnose and In women who are obese or have large breasts, this
manage fetal cardiac anomalies. poses a challenge to the sonographer. Furthermore, if it is
technically difficult to obtain the ultrasound images due
DIFFERENCES IN ECHOCARDIO- to the patient’s body size and/or pendulous breasts, then
GRAPHIC MEASUREMENTS AND it proves to be even more difficult to interpret the images.
Breast implants can also cause significant technical
TECHNICAL CONSIDERATIONS challenges. In 1992, silicone implants were removed from
Reference values for chamber sizes, vessel diameters, and the market, however; there were some silicone implants
left ventricle (LV) mass differ between men and women being implanted and studied for investigative purposes.
Chapter 78: Echocardiography in Women 1887
Table 78.1: Reference Limits and Partition Values of Left Ventricular Mass and Geometry
Women Men
Reference Mildly Moderately Severely Reference Mildly Moderately Severely
Range Abnormal Abnormal Abnormal Range Abnormal Abnormal Abnormal
Linear Method
LV mass, g 67–162 163–186 187–210 ≥ 211 88–224 225–258 259–292 ≥ 293
2
LV mass/BSA, g/m 43–95 96–108 109–121 ≥ 122 49–115 116–131 132–148 ≥ 149
LV mass/height, g/m 41–99 100–115 116–128 ≥ 129 52–126 127–144 145–162 ≥ 163
LV mass/height2,7, g/m2,7 18–44 45–51 52–58 ≥ 59 20–48 49–55 56–63 ≥ 64
Relative wall thickness, cm 0.22–0.42 0.43–0.47 0.48–0.52 ≥ 0.53 0.24–0.42 0.43–0.46 0.47–0.51 ≥ 0.52
Septal thickness, cm 0.6–0.9 1.0–1.2 1.3–1.5 ≥ 1.6 0.6–1.0 1.1–1.3 1.4–1.6 ≥ 1.7
Posterior wall thickness, cm 0.6–0.9 1.0–1.2 1.3–1.5 ≥ 1.6 0.6–1.0 1.1–1.3 1.4–1.6 ≥ 1.7
2D Method
LV mass, g 66–150 151–171 172–182 > 193 96–200 201–227 228–254 > 255
2
LV mass/BSA, g/m 44–88 89–100 101–112 ≥ 113 50–102 103–116 117–130 ≥ 131
Source: Reproduced from Roberto M. Lang, Michelle Bierig, Richard B. Devereux, et al. Recommendations for Chamber Quantification:
A Report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification
Writing Group, Developed in Conjunction with the European Association of Echocardiography, a Branch of the European Society of
Cardiology. J Am Soc Echocardiogr. 2005;18:1440–63.
(BSA: Body surface area; LV: Left ventricular; 2D: 2-dimensional).
Bold italic values: Recommended and best validated.
A B
Figs 78.1A and B: Breast implant. Two-dimensional transthoracic echocardiography. (A) Arrows point to images produced by a saline
breast implant; (B) Apical four-chamber view. Arrow points to a breast implant and the arrowhead to a pacing wire in the region of the
tricuspid valve, which shows fatty infiltration (F). A prominent moderator band (M) is seen in the right ventricle. (LA: Left atrium; LV: Left
ventricle; RA: Right atrium). (Movie clips 78.1A and B).
Consequently, the majority of implants in the late 1990s reported to have a more cohesive gel than the previous
and early 2000s are saline. Then, in 2006, the Food and silicone implants.6 As the number of women having
Drug Administration (FDA) approved the first silicone silicone breast implants is expected to increase, there will
filled implant for the use of breast augmentation. Even continue to be limitations in diagnostic cardiac testing
more recently, there have been approvals by the FDA for such as echocardiography. Saline and silicone breast
a new silicone gel-filled implant. This newer implant is implants interfere with the penetration of ultrasound
beams and produce artifacts6 (Figs 78.1A and B). Despite between 5% and 15%. Using the more modern criteria,
attempts from the sonographer to increase the gain or the prevalence of MVP in the general population is
change the ultrasound wave’s frequency, the images are estimated at 2–5%.7 Comparing the two genders, women
still of poor quality and cluttered with artifacts. These tend to have less posterior prolapse, less flail, but more
make interpretation of echo findings more difficult. The leaflet thickening.8 Also, compared to men, women
artifacts which are produced from the breast implants are tend to have less frequent severe regurgitation. M-mode
similar in some ways to the artifacts caused by air in the echocardiography allows for the diagnosis of MVP with
lung. To obtain interpretable echocardiographic views, the obvious leaflet thickening and posterior bowing of the
echocardiographer often needs to find alternative windows mitral valve apparatus during systole.7–9 On the two-
such as the subcostal approach which avoids passage of dimensional (2D) echocardiogram MVP is diagnosed
the ultrasound beam through the implants.6 Sometimes, by noting localized displacement of one or both leaflets
depending on the size of the breast implant, a sonographer into the left atrium with or without mitral regurgitation.
can modify the standard echocardiographic views in order Chordal rupture may complicate MVP (Figs 78.2 and 78.3).
to prevent the ultrasound beam from crossing the silicone Women with MVP tend to have both cardiac as well as
implant.6 However, even with attempts to reposition the noncardiac abnormalities. Examples of this include skeletal
probe in order to obtain better quality echocardiographic abnormalities such as pectus excavatum and scoliosis.9
images, the images still remain suboptimal. Furthermore, Other associations with MVP include atypical chest pain,
there is significant limitation in the ability to interpret the easy fatiguability, abnormal electrocardiographic (EKG)
study. response to exercise (ST-T changes), and a variety of
atrial and ventricular arrhythmias. The collection of these
STRUCTURAL HEART DISEASE: MVP, symptoms in the setting of MVP is often termed the MVP
syndrome (MVPS) or dysautonomia. It has been reported
MITRAL STENOSIS, AND MITRAL
that the clinical features are due to autonomic dysfunction.
ANNULAR CALCIFICATION Both the sympathetic and parasympathetic nervous
systems are likely involved, but not well understood
Mitral Valve Prolapse
Mitral valve prolapse (MVP) and regurgitation is seen
more commonly in women. Some studies report that MVP
Mitral Valve Stenosis in Women
affects ~6% of women. MVP was overdiagnosed during the Epidemiologic studies conducted in Western countries
1970s and 1980s due to lack of strict echocardiographic have demonstrated a higher prevalence of mitral stenosis
criteria. Earlier studies estimated the prevalence of MVP in women. The largest study conducted to date included
A B
Figs 78.2A and B: Mitral valve prolapse in a 42-year-old patient. (A) Two-dimensional transthoracic echocardiography. Arrowhead
points to prolapse of both mitral leaflets. (B) M-mode study was useful in demonstrating prolapse in mid to late systole (arrow).
(AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RV: Right ventricle; SVC: Superior vena cava). (Movie clip 78.2).
A B
Figs 78.3A and B: Ruptured chordae tendinae and mitral valve prolapse in a 71-year-old female. Live/real time three-dimensional
transesophageal echocardiography. (A) The arrowhead shows a ruptured chord of a severely prolapsing A2 segment of the anterior
mitral valve leaflet (AML); (B) Color Doppler imaging. Numbers 1 and 2 point to two jets of severe MR. The arrowhead points to the rup-
tured chord. (LA: Left atrium; MV: Mitral valve; MR: Mitral regurgitation; PML: Posterior mitral valve leaflet). (Movie clips 78.3A and B).
Source: Reproduced with permission from Nanda et al. Comparison of real time two-dimensional with live/real time three-dimensional
transesophageal echocardiography in the evaluation of mitral valve prolapse and chordae rupture. Echocardiography. 2008;25:1131–7.
echocardiograms performed on 12,926 women and 11,339 a marker of obstructive CAD, especially in the setting of
males to evaluate for mitral stenosis.10 The prevalence for anginal symptoms.12 Furthermore, in women, the absence
women is estimated at 1.8%. It is not clearly understood of mitral annular calcification was a marker for the absence
why the association with the female sex exists.10 Further- of obstructive CAD. However, this did not hold true for
more, it has been shown that women, in addition to men.
being more prone to the development of rheumatic heart
disease/mitral stenosis, are at higher risk of death in the ISCHEMIC HEART DISEASE/STRESS
setting of rheumatic heart disease.11 Figures 78.4 to 78.10 ECHOCARDIOGRAPHY/POLYCYSTIC
demonstrate various aspects of echocardiographic find-
ings in rheumatic mitral valve disease. The color Doppler
OVARIAN SYNDROME
examination in Figures 78.4A to F nicely illustrates a tur- Ischemic heart disease remains the leading killer in the
bulent jet in the LV which originates from the mitral valve United States. Although there have been advances in
as well as the prominent flow acceleration, characteristic treating ischemic heart disease (IHD), there still remains
of mitral stenosis. The continuous wave Doppler further a significant growth in prevalence of IHD. It has been
illustrates the severity of mitral stenosis by demonstrating shown in prior studies that 38% of deaths in women were
a significant mean gradient. related to CAD. This will only likely continue to increase
as the population continues to age.13 Furthermore, more
Mitral Valve Calcification women than men, annually, have died from CAD, refuting
the old belief that heart disease was a “man’s disease.” It
Although mitral annular calcification is a chronic degene- has been shown that women who are critically at risk for
rative process and progresses with increasing age, it is CAD are often missed by the traditional approaches to
more commonly seen in women, especially those over disease management, partly because of atypical clinical
the age of 70 (Figs 78.11A to C). Consequences of mitral presentation and low suspicion of index on the part of the
annular calcification may include mitral stenosis, mitral examining physician.
regurgitation, infective endocarditis, atrial arrhythmias, Women represent a unique and challenging patient
and heart block. It has been shown that the presence of population to the clinician. Women tend to have greater
mitral annular calcification on echocardiography may be symptom burden and a lower prevalence of obstructive
A B
C D
E F
Figs 78.4A to F: Rheumatic mitral stenosis. Two-dimensional transthoracic echocardiography in a 45-year-old female with
a history of rheumatic fever several years ago. (A) Arrow points to thickened mitral valve leaflets with a typical hockey-stick
appearance visualized in the parasternal long axis view; (B) Short-axis view of the mitral valve (MV) at the leaflet tips. MV area
measured 1.10 cm2 suggestive of fairly severe stenosis; (C) Color Doppler examination shows a turbulent jet (arrowhead) in the
left ventricle (LV) originating from the MV. Note the prominent flow acceleration (arrow); (D) Color Doppler guided continuous-
wave Doppler show peak and mean gradients of 34 and 19 mm Hg, respectively; (E) Pressure half time assessment also
showed severe MV stenosis with a valve area of 0.87 cm2; (F) Live/real time three-dimensional study confirmed the presence of
severe stenosis with no calcification of commissures. There was only mild mitral regurgitation. The patient is on the wait list for
percutaneous mitral valvuloplasty (Movie clips 78.4A, C, and F). (AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
A B
C D
Figs 78.5A to E: Rheumatic mitral stenosis in 34-year-old female

patient with history of paroxysmal nocturnal dyspnea. Two-dimen-
sional transthoracic echocardiography. (A and B) Parasternal long
axis (A) and apical four-chamber (B) views show thickened mitral
valve leaflets with doming in diastole indicative of mitral stenosis.
The subvalvular apparatus is also thickened; (C) Color Doppler
examination shows mild mitral regurgitation and moderate aortic
regurgitation; (D) Live/real time three-dimensional transesophageal
echocardiography demonstrates a very small mitral orifice (arrow)
indicative of severe stenosis; (E) Severe mitral regurgitation
developed following percutaneous mitral valvuloplasty (Movie
clips 78.5A to E). (PA: Pulmonary artery; RA: Right atrium). Other
E abbreviations as in previous figure.
CAD on coronary angiography, compared to men. Inter- In women, the prevalence of obstructive CAD is low
estingly, women often times have a more adverse outcome before menopause, the average age of menopause being
despite having lower angiographic disease burden. ~51 years.13 After the age of menopause, the prevalence
of CAD in women increases to become almost equal with have lower total cholesterol measurements until about the
men at the age of 70 years (Figs 78.12 and 78.13). While age of 50 years. Above the age of 50 years, women tend to
women may have overall lower rates of hypertension and have greater values. The risk factors for CAD seen in post
smoking, both elderly hypertensive women and young menopausal women consist of obesity, hypertension, and
female smokers are very prominent at risk subgroups. dyslipidemia. The clustering of the risk factors commonly
Population studies have also demonstrated that women termed the metabolic syndrome consists of the following
A B
Figs 78.6A and B: Female patient with rheumatic involvement of mitral and tricuspid valves. Transthoracic three-dimensional echocar-
diography. (A) Apical four-chamber view showing thickened mitral (MV) and tricuspid (TV) valves. (B) Cropping of the three-dimensional
data set and en face viewing shows a very small mitral orifice in diastole indicative of severe stenosis. Note absence of calcification in
the commissures. In comparison, the TV shows a much larger opening consistent with absence of significant stenosis. The movie clip
shows fairly preserved motion of anterior (A) and posterior (P) leaflets but marked restriction of the septal (S) leaflet of the tricuspid
valve. The septal leaflet is identified by its close proximity to the ventricular septum. The anterior and posterior leaflets are recognized
by their anterior and posterior locations in relation to the left ventricular outflow (LVO) tract. The three-dimensional technique is consid-
ered the gold standard for assessing the mitral orifice area because, unlike two-dimensional echocardiography, the cropping plane can
be positioned exactly parallel to the flow limiting orifice tip. Also, three-dimensional echocardiography easily assesses all three leaflets
of the TV en face which is difficult with two-dimensional imaging (Movie clip 78.6). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
A B
Figs 78.7A and B
C D
Figs 78.7A to D: Mitral stenosis and regurgitation in a female patient. Transthoracic three-dimensional echocardiography. (A) Apical
four-chamber view shows restricted mobility of both mitral valve (MV) leaflets which are thickened; (B) Cropping of the three-dimensional
data set was performed to view the mitral orifice (arrow) at its tip. Planimetry was consistent with significant stenosis. Note presence of
calcification in the body of the posterior leaflet but the anterior leaflet and both commissures are free of calcium; (C) Mosaic color signals
in the left atrium with a prominent flow acceleration point to severe mitral regurgitation; (D) Meticulous cropping of the three-dimensional
data set was done to view en face the vena contracta of the mitral regurgitation jet. This was performed practically at the level of the
mitral leaflets between the flow acceleration and the regurgitant jet. It measured more than 0.6 cm2 indicative of severe regurgitation
(Movie clip 78.7). Other abbreviations as in previous figures.
A B
Figs 78.8A and B: (A) Rheumatic mitral stenosis and regurgitation in a female patient. Transesophageal two-dimensional echocardio-
graphy. Four-chamber view shows thickening of both mitral leaflets with restricted motion. Note thickening of chordal apparatus also;
(B) Color Doppler examination demonstrates significant mitral regurgitation (MR) (Movie clips 78.8A and B). Other abbreviations as in
previous figures.
conditions: insulin resistance, dyslipidemia [elevated shifting energy substrates toward myocardial and peri-
triglycerides, low high-density lipoprotein (HDL)], hyper- pheral glucose metabolism. Therefore, the traditional
tension, and abdominal obesity. The chest pain symptoms stress testing which is based on demand ischemia may fail
which women experience may be explained by the to detect obstructive CAD in subsets of women without a
metabolic alterations which occur. This may result in significant coronary stenosis.13
A B
Figs 78.9A and B: Rheumatic mitral stenosis and regurgitation in a female patient. Transesophageal three-dimensional echocar-
diography. (A) En face view of the mitral valve (MV) shows mild narrowing of the thickened MV consistent with mild to moderate
stenosis; (B) Systolic view shows noncoaptation of the mitral leaflets indicative of significant mitral regurgitation. (AV: Aortic valve).
(Movie clip 78.9).
A B
Figs 78.10A and B: Rheumatic mitral stenosis in another female patient. Transesophageal three-dimensional echocardiography.
(A) The left atrium is viewed from the top and shows a large clot attached to the supero-posterior wall. The mitral valve (MV) is heavily
calcified; (B) Three-dimensional two-chamber view demonstrates clots (arrowheads) in the body and appendage of the left atrium
(Movie clips 78.10A and B). Abbreviations as in previous figures.
Using Stress Echocardiography in is made by stress echocardiography. Treadmill exercise

echocardiography is most commonly used because it
Women results in greater patient oxygen consumption and there-
In some patients with obstructive CAD, resting echo- fore a higher sensitivity in detecting exercise induced wall
cardiograms may demonstrate tell-tale wall motion abnor- motion abnormalities and diagnosing CAD as compared
malities or an akinetic or dyskinetic area with fibrotic scar to pharmacologic agents such as dobutamine. The usage
formation may reveal the presence of an old myocardial of pharmacologic agents (dobutamine or dipyridamole)
infarction which may have been silent or undiagnosed. to perform stress echocardiography helps to overcome
However, in most patients the diagnosis of obstructive CAD the challenges of women who are incapable of maximal
A B
Figs 78.11A to C: Mitral annulus calcification in an 80-year-old

female. Two-dimensional transthoracic echocardiography. (A to C)
Arrowhead points to a heavy mitral annular calcification while the
arrows show aortic annular calcification. The aortic valve leaflets
are also calcified with restrictive opening and continuous-wave
Doppler demonstrated high velocity consistent with significant
stenosis (Movie clips 78.11A and B). (AO: Aorta; LA: Left atrium;
LV: Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right
C ventricle).
exercise. The advantages of stress echocardiography echocardiography is very useful in diagnosing 2 and 3
include the lower cost, absent radiation exposure, and the vessel disease with accuracy exceeding 80% to 85% but,
ability to image both ventricular function as well as cardiac like radionuclide testing, is less useful in diagnosing
structures. However, the utility of echocardiography can 1 vessel disease especially involving the circumflex artery.
be limited if factors such as obesity exist which will limit Contrast echocardiography serves as a valuable adjunct
acoustic windows. Furthermore, it is known that as women to stress echocardiography in patients with suboptimal
progress through menopause, they seem to have a greater acoustic windows. Intravenous injections of commercially
loss in physical functioning when compared with men. available contrast agents produce full opacification of
Thus, if there is reduced exercise tolerance, the utility of both right and left ventricular cavities resulting in comp-
stress echocardiography is markedly reduced. Acquisition lete delineation of the endocardial borders facilitating
of peak stress echocardiography images is limited by accurate assessment of wall motion abnormalities. Echo
the experience of the sonographer and the clinician. contrast agents consist of microbubbles of a nontoxic
Even with the limitations, exercise echocardiography is gas smaller or same size as the red blood cells and they
highly accurate at detecting CAD in women. Both the travel with them into the coronary circulation and hence
sensitivity and specificity of stress echocardiography are they opacify the ventricular myocardium also. This
comparable to radionuclide techniques with the added property has been used to evaluate myocardial perfusion
advantage that one does not have to cope with breast abnormalities developing during stress echocardiography
shadows which may interfere with accurate interpretation by demonstrating areas of reduced or absent opacification
when reading radionuclide studies in women. Stress providing corroborative evidence of the presence of
Figs 78.12A and B: (A) Aortic atherosclerosis in a 72-year-old

female with coronary artery disease, systemic hypertension and type
2 diabetes mellitus presenting with chest pain. Two-dimensional
transthoracic echocardiography. Suprasternal examination. Arrow-
head points to a mobile plaque in the proximal descending
thoracic aorta (DA); (B) Aortic atherosclerosis in the same patient
as above. Two-dimensional transesophageal echocardiography
performed to rule out aortic dissection. Arrowhead in the left panel
points to a large mobile plaque in the anterior portion of the DA,
arrowhead in the right panel demonstrates a large ulcer in the
plaque posteriorly. Ischemic heart disease in a 75-year-old female.
Two-dimensional transthoracic echocardiography. The parasternal
long-axis view shows dyskinesis of the proximal left ventricular
posterior wall (PW). (Movie clips 78.12A to C). (ACH: Aortic arch;
IA: Innominate artery; LA: Left atrium; LCC: Left common carotid
artery; PA: Pulmonary artery; AO: Aorta; LA: Left atrium; RV: Right
A ventricle; SVC: Superior vena cava; VS: Ventricular septum).
obstructive CAD. More recently, live/real time three- be recropped at will any time by the same or different
dimensional (3D) echocardiography is increasingly used cardiologist to double check the findings. A significant
together with contrast echocardiography in an attempt to disadvantage relates to the quality of 3D images which is
further enhance the accuracy of stress echocardiography lower than 2D images and hence the technique is useful
in assessing CAD. With 3D echocardiography, it is only in patients with good acoustic windows and in those
possible to capture the whole LV in the pyramidal shaped in whom the image quality has been enhanced by contrast
3D data set which can then be cropped in a systematic echocardiography.
and sequential manner to examine all ventricular walls Although not commonly used, there are reported
and segments for stress induced motion abnormalities. cases where transesophageal echocardiogram has actually
This obviates the limitations of the 2D technique which revealed the diagnosis of CAD. It should be standard
produces only thin slice-like sections of the LV at any protocol to interrogate the left and right coronary arteries
given time precluding comprehensive assessment of when they are visualized during a routine transesopha-
all segments. Apical foreshortening, common with 2D geal echocardiogram exam. Figures 78.13A to M nicely
echocardiography, is avoided or reduced with the 3D demonstrate images of CAD. Notice the utilization of
approach which also has been shown to have much less pulsed Doppler interrogation which reveals high diastolic
intra- and interobserver variability in the assessment of velocities, consistent with severe stenosis. The coronary
left ventricular function. Another advantage is the 3D arteriograms included confirm the severity of the stenosis
data can be stored in the equipment or offline and can suspected by TEE.
A B
C D
E F
Figs 78.13A to F
G H
I J
K L
Figs 78.13G to L
Figs 78.13A to M: Coronary stenosis in a 61-year-old female. Trans-

esophageal echocardiographic examination. (A) Top arrow points
to dilatation of the proximal left anterior descending coronary artery
(LAD), whereas the bottom arrow demonstrates turbulent flow in
mid-LAD. Pulsed Doppler interrogation of this area revealed a high
diastolic velocity of 1.3 m/s (arrowhead in the inset), consistent
with significant stenosis; (B) Top arrow points to turbulent flow in
mid-LAD, whereas the bottom arrow shows turbulent flow in the
more distal portion of LAD; (C) Arrow demonstrates narrowing in
the first diagonal branch (arrowhead) of LAD. Continuous-wave
Doppler interrogation reveals a very high diastolic velocity of 3.0
m/s (arrowhead in the inset) indicative of very severe stenosis; (D)
Arrows show large segments of proximal, mid and distal segments
of LAD visualized in this view; (E) Arrow demonstrates the presence
of turbulent flow in the proximal right coronary artery; (F) Pulsed
Doppler interrogation (arrowhead) of right coronary artery (RCA)
M demonstrates a high velocity of 1.3 m/s (arrowhead in the inset)
indicative of significant stenosis; (G) Arrow points to the presence of turbulent flow in the left circumflex coronary artery; (H) Arrow points
to the origin of the left circumflex coronary, which appears normal. However, reversed flow (blue) with turbulent flow signals (arrowhead)
is noted in the mid and distal portions of the circumflex vessel, consistent with filling from collaterals and significant stenosis. Doppler
interrogation of this area shows a high diastolic velocity of 1.0 m/s (arrowheads in the inset); (I) Arrowhead demonstrates turbulent flow
signals in the intramyocardial coronary arteries consistent with stenosis; (J) The interventricular vein (V) is imaged next to the dilated
LAD (arrow). Note that the flow in the interventricular vein is in the opposite direction of LAD flow; (K) Coronary angiogram. The top
arrowhead demonstrates 90% stenosis at the origin of the diagonal branch. The proximal LAD is dilated, and beyond the dilatation,
the mid-LAD shows 50% stenosis (just beyond the bottom arrowhead). Note multiple areas of significant stenosis in the more distal
segments of LAD; (L) Arrow points to total occlusion of proximal circumflex coronary artery. Retrograde filling of more distal portions of
the circumflex vessel from collaterals was noted; (M) Coronary angiogram. The arrow points to significant stenosis in the proximal right
coronary artery. Note significant stenosis in the mid and distal portions. (AO: Aorta; LA: Left atrium; LM: Left main coronary artery; LV:
Left ventricle; MV: Mitral valve; PA: Pulmonary artery; RVO: Right ventricular outflow tract). Reproduced with permission from Nanda
et al. Transesophageal echocardiographic diagnosis of coronary stenosis in a stroke patient. Echocardiography. 1999;16:589–92.
In Movie clip 78.13, 1, Left anterior descending coronary artery; 2, Diagonal branch; 3, Proximal right coronary artery; 4, circumflex
artery; 5, Intramyocardial coronary arteries (cannot be visualized by angiogram). V, coronary vein. Upper arrow shows proximal LAD.
Lower arrow shows distal LAD. Arrowhead points to post-stenotic dilatation of the diagonal branch.
Polycystic Ovarian Syndrome/Syndrome men (9:1 female/male ratio). The echocardiographic

features include transient dyskinesia/akinesia usually
X in Women localized to the apex of the LV (see Movie clips 78.26A and
Women with polycystic ovarian syndrome (PCOS) have 78.26B in chapter on Nonobstructive Cardiomyopathy).
been shown to be at higher risk for CAD compared to Associated involvement of the left atrium has also been
women without PCOS. Women with PCOS have risk factors reported.11 In some patients only the middle portion of
which mirror a risk factor profile for that of a man. These the ventricle is involved. The clinical presentation is very
risk factors include: anovulation, hyperandrogenism, and similar to that of acute coronary syndrome. The clinical
insulin resistance. PCOS could be thought of as the best history often reveals that the person has been under
example of syndrome X. If the cluster of risk factors known extreme emotional or physical stress which precipitates
as the metabolic syndrome/syndrome X are indeed risk the abrupt onset of symptoms, including chest pain and
factors of atherosclerosis and CAD, then women with shortness of breath. Examples of emotional stressors
PCOS should have more atherosclerosis than women
include grief, fear, anger, relationship conflicts, and
without PCOS, especially at younger ages.14 Therefore,
financial problems. Examples of physical stressors include
these women with PCOS would need to be followed as the
acute asthma exacerbation, surgery, chemotherapy, or
risk of developing obstructive CAD would be higher.
stroke. This form of cardiomyopathy has a predilection
for women, specifically those over the age of 50.15,16 Only
TAKOTSUBO CARDIOMYOPATHY 10% of cases have been reported to occur in men. Usually,
Takotsubo cardiomyopathy is a very common clinical patients present to the emergency department with
entity which occurs far more often in women than in concerns of having a myocardial infarction. Initially, both
Takotsubo and acute coronary syndromes share a similar Vector velocity imaging (VVI) has been used to dem-
presentation, with abnormalities in the electrocardiogram onstrate that in patients with Takotsubo cardiomyopathy,
(ECG) as well as biomarkers. In fact, this syndrome has there is both LV systolic and diastolic longitudinal dys-
been reported to represent 1.5% to 2.2% of acute coronary function, not just systolic radial dysfunction.11 Traditional
syndrome that presents with ST elevation or Q-waves on 2D echocardiography is typically used to assess radial dys-
EKG.16,17 As a result, most patients are taken to the cardiac function. VVI uses a tracking algorithm which incorpo-
catheterization laboratory and are found to have normal rates both velocities of set points (i.e. mitral annulus and
coronary arteries in addition to the unique shape of the LV tissue-cavity border) as well as speckle tracking. The result
which is seen during the LV gram. This unusual shape of is segmental quantitative velocity, strain, and strain rate.
the LV, which resembles a Japanese octopus pot, is what VVI has demonstrated that there are definitive reductions
has given Takotsubo its name. in both longitudinal systolic and diastolic dysfunction in
The classic case of stress induced cardiomyopathy Takotsubo cardiomyopathy.11 It has also been shown that
is characterized by the presence of apical ballooning there is improvement in the longitudinal function with
involving all left ventricular walls with a hyperdynamic time. Furthermore, there has been evidence to suggest that
base. These abnormalities are not limited to single the left atrium is also affected in Takotsubo cardiomyopa-
coronary artery territory. There are theories to explain thy.11 Specifically, the left atrium’s systolic strain and strain
why the apex is affected and the base remains unaffected. rate and the diastolic velocity and strain rate are reduced.
This could be due to the fact that the apex is considered However, there is some data to suggest that there is impro-
to be more responsive to adrenergic stimulation.11,15–19 This vement in the involved walls of the left atrium.11
assumes that the catecholamine surge is the mechanism
of Takotsubo.15 Echocardiography can prove to be very CONGENITAL HEART DISEASE
useful in the early diagnosis of this disease, and could
actually prevent patients from undergoing unnecessary Atrial Septal Defects
cardiac catheterizations given the detection of wall Congenital heart disease seems to have an association with
motion abnormalities not confined to typical coronary gender. Defects which involve the inflow tract such as the
artery distribution in association with recent emotional atrial septal defect and Ebstein’s (see Figures 57, 58, and
or physical stressors. Echocardiography is also useful 59 in chapter on Three-Dimensional Echocardiography
in assessing complications of Takotsubo such as clot in Congenital Heart Disease) are seen most commonly in
formation in the LV apex and in the follow-up of these females (Figs 78.14 and 78.15). Of patients with secundum
patients. Some of these patients show full recovery with atrial septal defects (ASDs), 65–75% are female. In
complete normalization of wall motion abnormalities contrast, there is equal gender distribution for both sinus
while in others residual wall motion abnormalities persist venosus and ostium primum ASDs.20–24 Outflow tract
for a long time. abnormalities such as aortic stenosis and transposition
A B
Figs 78.14A and B
Figs 78.14A to C: Secundum atrial septal defect in a 53-year-old

female. Two-dimensional transthoracic echocardiography. (A and B).
Apical four-chamber views show an atrial septal defect (arrows) with
flow signals moving from the left atrium (LA) into the right atrium
(RA). The right ventricle (RV) is enlarged; (C) is a suprasternal
view in the same patient showing continuity of flow signals (arrow)
between the pulmonary artery (PA) and the descending thoracic
aorta (DA) indicative of an associated patent ductus arteriosus.
C (ACH: Aortic arch, LV: Left ventricle). Movie clips 78.14A to C.
Fig. 78.15: Secundum atrial septal defect in a 37-year-old female. Two-dimensional transesophageal echocardiography. Arrow points
to flow signals moving from the left atrium into the right atrium through a secundum defect. Abbreviations as in previous figure. Movie
clip 78.15 is from another female patient demonstrating en face visualization of a secundum atrial septal defect (upper arrow) using
live/real three-dimensional tranesophageal echocardiography. Note a large rim in relation to the aorta (AO), tricuspid valve (lower left
arrow), and mitral valve (MV). Lower right arrow points to the pulmonary valve. (PA: Pulmonary artery). Other abbreviations as in previ-
ous figure.
of the great arteries are more commonly seen in men. greater than 25 mm Hg, in conjunction with a normal
Atrial and ventricular septal defects also have a significant cardiac output and normal pulmonary capillary wedge
association with pulmonary hypertension. There has been pressure (Figs 78.16 to 78.18).25 The association with the
some thought that the secundum ASD begins the cascade female sex is quite interesting. In fact, women have a 35%
of vascular injury ultimately resulting in the pulmonary higher risk of developing pulmonary hypertension.25,26
vascular remodeling and finally the development of Idiopathic pulmonary arterial hypertension is thought to
pulmonary arterial hypertension. This could explain the be rare, with an incidence of 2 to 5/million/year. However,
gender disparities in pulmonary arterial hypertension.23 it is much more common in women compared to men with
a reported ratio of 2.5:1.25,26 Various genetic mutations and
Pulmonary Hypertension polymorphisms have been reported to be associated with
Pulmonary arterial hypertension is typically defined as idiopathic pulmonary arterial hypertension, and none
a measured mean pulmonary artery pressure (mPAP) appear to be sex-linked.
A B
C D
Figs 78.16A to D: Severe systemic level pulmonary artery pressure in a 37-year-old female with primary pulmonary hypertension.
Two-dimensional transthoracic echocardiography. Apical views. (A) Note the marked enlargement of both the right ventricle (RV) and
the right atrium (RA). The atrial septum bulges prominently into the left atrium (LA). Movie clip 78.8A shows diastolic bulging of the ven-
tricular septum into the left ventricle (LV) indicative of right-sided volume overload; (B) Color Doppler examination shows the presence
of severe tricuspid regurgitation (TR); (C) Continuous-wave Doppler examination reveals a very high pulmonary artery systolic pressure
of 118 mm Hg (arrow); (D) Bubble study shows delayed appearance (after 4 beats) of the micro bubbles (arrowhead) in the left heart
consistent with intrapulmonary shunting. This is related to dilatation of the pulmonary arterioles. (LV: Left ventricle; TV: Tricuspid valve).
(Movie clips 78.16A to C).
In the included figures, specifically Figure 78.16, there intrapulmonary shunting. Intrapulmonary shunting is
is an excellent example of severe pulmonary hypertension seen when there is a delayed appearance of microbubbles
in a young woman. The classic features of pulmonary in the left side of the heart.
hypertension include the marked enlargement of both
the right ventricle and right atrium. Furthermore, there ECHOCARDIOGRAPHY IN PREGNANCY,
is diastolic bulging of the interventricular septum from PERIPARTUM CARDIOMYOPATHY,
right to left indicating the significant right-sided volume
overload. Color Doppler highlights the severe tricuspid FETAL ECHOCARDIOGRAPHY
regurgitation seen in cases of pulmonary hypertension. Many physiological changes develop in many organ
A bubble study should always be performed in a case of systems during the course of pregnancy. Cardiac output
pulmonary hypertension as it will reveal the presence of increases by 30% to 40% during the first trimester of
A B
Figs 78.17A and B: Severe pulmonary hypertension with right-sided involvement. Two-dimensional transthoracic echocardiography.
(A and B) The tricuspid valve (TV) annulus is dilated with systolic noncoaptation (arrow) of the leaflets leading to severe tricuspid regur-
gitation (arrowhead). The pulmonary annulus is also dilated resulting in severe pulmonic valve regurgitation (PR). (CS: Coronary sinus;
DA: Descending aorta; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle). (Movie clips 78.17A and B).
A B
Figs 78.18A to C: Pulmonary artery aneurysm in a 21-year-old

female patient with familial primary pulmonary hypertension. Two-
dimensional transthoracic echocardiography. (A) The pulmonary
artery (PA) is markedly enlarged at 4.89 cm; (B) The right ventricle
(RV) is larger than the left ventricle (LV) in the apical four-chamber
view. Note displacement of the ventricular septum into the LV
during systole; (C) Bubble study was negative for intracardiac
or intrapulmonary shunting. (LA: Left atrium; MV: Mitral valve;
PV: Pulmonary valve; RA: Right atrium; TV: Tricuspid valve).
C (Movie clips 78.18A to C).
pregnancy. These changes can be seen on Doppler echo- Peripartum Cardiomyopathy

cardiography with increased blood flow velocities and
more prominent color Doppler flow signals in the cardiac In some women, the extra workload of the heart results in
cavities. Increased color Doppler flow signals in the region ventricular failure in the peripartum period (Figs 78.19A
of the atrial septum may mimic an atrial septal defect to G). Peripartum cardiomyopathy is characterized by
especially in the presence of septal dropouts common in systolic dysfunction, ventricular dilatation, and secondary
the apical four-chamber view. In this instance, continuity mitral regurgitation, all well assessed by echocardiography.
of color flow signals between the two atria in the region Thrombus formation especially in the left ventricular apex
of the septal dropout may result in an erroneous diagnosis may be noted. This is a worrisome complication because
of a secundum atrial septal defect. At 8 to 11 weeks, of the potential for embolization and anticoagulants are
an average cardiac output of 6.7 L/min will increase to essential. Anticoagulants like warfarin which facilitate
8.7 L/min at 36 to 39 weeks. This increase is mostly due to the natural clot lytic mechanisms present in the blood
an increase in stroke volume, but also a more rapid stream work by first initiating a small area of liquefaction
heart rate. Also, there is a reduction in systemic vascular within the middle portion of a thrombus and this gradually
resistance. It is these changes which characterize the spreads all the way to the periphery eventually resulting
hyperdynamic circulation seen in pregnancy. On the in complete resolution of the thrombus. Thus, on serial
echocardiogram, hyperkinetic ventricular wall motion is follow-up of 2D transthoracic echocardiograms the size
evident during this period of pregnancy. of a thrombus may appear unchanged and anticoagulant
A B
C D
Figs 78.19A to D
E G
Figs 78.19A to G: Peripartum cardiomyopathy. Live/real time three-dimensional transthoracic echocardiography in a 29-year-old
female. (A) Arrow points to a large clot in the left ventricular (LV) apex. Both ventricles showed poor function; (B to D) A transverse plane
(TP) section through the clot (B) shows a large area of echolucency (arrow) consistent with clot lysis (C and D). This indicated that the
clot had practically completely dissolved and only a thin rim remained; (E) Schematic showing coagulation cascade and thrombus lysis.
Plasminogen-activating factor (PAF) is secreted by endothelialized mesenchymal cells lining the microscopic crypts which develops
within the thrombus. PAF activates plasminogen to plasmin which digests fibrin leading to thrombus lysis. (F and G) A TP section taken
at the attachment point of the clot shows it to be highly echogenic consistent with collagen. This patient had been on anticoagulant
therapy for a long time but on the two-dimensional study the clot did not appear to regress. However, the three-dimensional technique
was useful in assuring us the effectiveness of anticoagulant therapy with almost complete clot resolution. The remainder of the clot
regressed completely with no clinical or laboratory evidence of embolization (Movie clips 78.19). (vWF: von Willebrand factor; TF: Tissue
factor; WBC: White blood cell; RBC: Red blood cell; CF: Clotting factors). Other abbreviations as in previous figures.
therapy ineffective although the thrombus in fact may be mbus with a small tenuous cross-sectional attachment
undergoing progressive liquefaction and resolution from site may be more prone to embolization than one with
within. In these cases, it is important to perform live/real a much larger attachment area. Also, a bright echogenic
time 3D echocardiography which can provide multiple attachment area suggests increased collagen content and
short-axis sections of the thrombus and comprehensively fibrosis providing a more firm and stable attachment of the
assess the presence and extent of clot lysis. The technique clot to the ventricular wall.
can also assess the cross-sectional size and nature of the Women with peripartum cardiomyopathy will usually
area of the attachment of the thrombus to the ventricular present shortly after childbirth, but there are some women
wall and this may provide information regarding its who will have the clinical presentation consistent with
potential for embolization. For example, a mobile thro- peripartum cardiomyopathy late in the third trimester.
Fetal Echocardiography fetal cardiac anatomy and physiology (Figs 78.20 to 78.22).
M-mode studies of the mitral valve and Doppler tracings
In high risk pregnancies, fetal echocardiography plays a of mitral and tricuspid inflow have been found useful in
very important role in evaluating normal and abnormal evaluating fetal arrhythmias.27 Using both 2D and 3D fetal
A B
C D
E F
Figs 78.20A to F
Figs 78.20A to G: Normal fetus. Live/real time three-dimensional

echocardiographic study. (A) 31-week-old fetus. Arrowhead
points to the foramen ovale. Arrowhead in (B) denotes the ductus
arteriosus connecting aorta (AO) to pulmonary artery (PA); (C to F)
19-week-old fetus. Arrowhead points to foramen ovale visualized
in a four-chamber view (C) and from top (D). Color Doppler
image shows physiological right to left shunting (arrowhead, E).
Arrowhead in F points to a normal tricuspid valve. Both atrial
septum (AS) and ventricular septum (VS) are viewed en face in
this image; (G) 22-week-old fetus. Color Doppler image shows
ascending aorta (AA), aortic arch (ACH) and descending thoracic
aorta (DA). (IVC: Inferior vena cava; LA: Left atrium; LV: Left
Source: Reproduced with permission from Maulik et al. Live
Three-dimensional echocardiography of the human fetus. Echo-
G cardiography. 2003; 20:715–21.
A B
C D
Figs 78.21A to D
E F
G H
I J
Figs 78.21E to J
K L
M N
Figs 78.21A to N: Complete atrioventricular septal defect in 36-week-old fetus. Live/real time three-dimensional echocardiographic
study. (A to D) Four-chamber views cropped to show the common atrioventricular valve (V) and the defect (asterisks). Arrowhead in C
points to the atrial septum. (E to F) The pyramidal section has been cropped from the top and rotated toward the examiner to display all
five leaflets of V: posterior (P), left lateral (L1), left anterior (A1), right anterior (A2), and right lateral (L2). Small portions of the ventricular
septum (S) and atrial septum (AS) have been retained to show their relationship to V. V is open in E and closed in F. (G) Arrowhead
demonstrates multiple chordal attachments of V to S; (H to J) En face viewing of the defect (asterisk) from above (H), from the inferior
aspect (I) and from the right side (J); (K to M) Five-chamber view shows the aorta (AO) arising from LV. In M, the pyramidal section has
been cropped to show regurgitation (R) from the right-sided component of V. (N) Arrowhead shows the ductus arteriosus. (AV: Aortic
valve; S: Ventricular septum). (Movie clip 78.21).
Source: Reproduced with permission from Maulik et al. Live three-dimensional echocardiography of the human fetus. Echocardiography
2003;20:715–21.
echocardiography allows diagnosis of many congenital attachments to papillary muscles in the opposite ventricle
cardiac lesions. This information is crucial to the are not amenable to surgical repair and hence these
obstetrician and the pediatric cardiologist who can discuss babies carry a dismal prognosis. Also, cyanotic babies with
the prognosis with the parents and also aids them in the dextro transposition of the great vessels need intervention
management of these conditions in the neonatal period. For immediately after birth. In some specialized centers in
example, in complete atrioventricular septal defects, valve the country, percutaneous intrauterine interventions
have been successfully performed to correct aortic and

pulmonary valve stenosis.27–30 This is done in an attempt
to prevent the development of hypoplastic left and right
heart syndromes in the newborn. 3D echocardiography
actually provides more information in assessing the
severity. Both valves are enclosed in the 3D data sets, and
they can be cropped to view the maximum flow limiting
orifice area. It is the direct en face visualization coupled
with the quantification of the stenotic semilunar valve
orifice which makes 3D echocardiography more reliable
than 2D.27–30 Furthermore, there is added information
obtained from 3D echocardiography due to the ability to
view cardiac chambers, valve leaflets, and great vessels in
Fig. 78.22: Thickened tricuspid valve in a 20-week-old fetus. three dimensions. This added information can prove to
Live/real time three-dimensional echocardiographic study. The be useful in guiding catheter based interventions in the
arrowhead points to a markedly thickened tricuspid valve. (LA:
Left atrium; RA: Right atrium). intrauterine setting after birth.
Source: Reproduced with permission from Maulik et al. Live Other pathologic conditions seen in women are
three-dimensional echocardiography of the human fetus. Echo- found in Figures 78.23 to 78.37 with corresponding movie
cardiography. 2003; 20:715–21.
clips.
A B
C D
Figs 78.23A to D: Systemic hypertension. The patient is a 37-year-old female with very severe hypertension. Two-dimensional tran-
sthoracic echocardiography. Parasternal long-axis (A), short-axis (B), and apical four-chamber (C) views. Note the marked concentric
hypertrophy of both the ventricular septum (VS) and the posterior wall (PW). The left atrial appendage (LAA) is clearly delineated and
is free of any clot. (D) M-mode study shows left ventricular hypertrophy. (AO: Aorta; LA: Left atrium; MV: Mitral valve; RA: Right atrium;
RV: Right ventricle). (Movie clips 78.23A to C).
Fig. 78.24: Left ventricular hypertrophy in a 30-year-old patient Fig. 78.25: Mitral valve vegetations in a 43-year-old female with in-
with chronic renal disease. Two-dimensional transthoracic echo- fective endocarditis. Live/real time three-dimensional transesoph-
cardiography. Both the ventricular septum (VS) and the posterior ageal echocardiography. The black arrow (horizontal arrowhead
wall (PW) are hypertrophied and contain echo densities related in the video clip) points to a mitral valve vegetation involving the
to fibrosis. (AO: Aorta; LA: Left atrium; RV: Right ventricle). A1 segment and commissure. Note the presence of central per-
(Movie clip 78.24). foration. Two other vegetations are also seen involving A2 and A3
segments of the anterior mitral leaflet. (AO: Aorta; PV: Pulmonary
valve; TV: Tricuspid valve). (Movie clip 78.25).
Source: Reproduced with permission from Hansalia et al. The
value of live/real time three-dimensional transesophageal echo-
cardiography in the assessment of valvular vegetations. Echocar-
diography. 2009;26:1264–73.
A B
Figs 78.26A and B: Mitral valve vegetation in a 32-year-old female with infective endocarditis. Live/real time three-dimensional
transesophageal echocardiography. (A) The arrowhead points to a large mitral valve vegetation with a central perforation; (B) The
lower arrowhead points to an annular abscess and the upper arrowhead points to the mitral valve. (AO: Aorta; PV: Pulmonary valve).
(Movie clips 78.26A and 78.28B).
Source: Reproduced with permission from Hansalia et al. The value of live/real time three-dimensional transesophageal echocardio-
graphy in the assessment of valvular vegetations. Echocardiography. 2009;26:1264–73.
A B
Figs 78.27A and B: Right-sided heart failure resulting from severe tricuspid regurgitation due to infective endocarditis. Two-dimensional
transthoracic echocardiography. (A) Arrowheads denote vegetations on the tricuspid valve leaflets; (B) Color Doppler examination
shows severe tricuspid regurgitation (TR). (AO: Aorta; CS: Coronary sinus; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
(Movie clips 78.27A part 1, 78.27A part 2 and 78.27B).
A B
Figs 78.28A and B: Systemic lupus erythematosus in a young female patient. Two-dimensional transthoracic echocardiogram. (A and B).
Parasternal long-axis view shows the presence of pericardial effusion (PE, A) which resolved during follow-up (B). There are no valvular
abnormalities. (AO: Aorta; CS: Coronary sinus; DA: Descending aorta; LA: Left atrium; LV: Left ventricle; PW: Posterior wall; RV: Right
ventricle; SVC: Superior vena cava; VS: Ventricular septum). (Movie clips 78.28A and B).
A B
Figs 78.29A and B: Metastatic left pleural effusion in a 53-year-old female with chronic myeloid leukemia. Two-dimensional transtho-
racic echocardiography. (A) In the parasternal long-axis view, the pleural effusion (PLE) is diagnosed by noting an echo free-space post-
eriorly extending beyond the descending thoracic aorta (DA); (B) PLE is readily diagnosed by placing the ultrasound transducer in the
posterior intercostal spaces with the patient sitting up. (LA: Left atrium; LB: Left back; LU: Lung; LV: Left ventricle; RV: Right ventricle).
(Movie clips 78.29A and B).
A B
Figs 78.30A and B: Bicuspid aortic valve in a young female. (A and B) Both two-dimensional (A: Left panel, systole; right panel,
diastole) and three-dimensional (B) transthoracic echocardiography show the presence of a bicuspid aortic valve with horizontal cusp
orientation. (AO: Aorta; LA: Left atrium; PV: Pulmonary valve; RA: Right atrium, RV: Right ventricle). (Movie clips 78.30A and B).
A B
Figs 78.31A and B: Bicuspid aortic valve. Two-dimensional transesophageal echocardiogram showing a bicuspid aortic valve imaged
in long (A) and short (B) axis views. The arrow in A points to valve redundancy and prolapse into the left ventricular (LV) outflow tract.
In B, the cusps are vertically oriented and appear to be equal in size. (AO: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle;
TV: Tricuspid valve). (Movie clips 78.31A and B).
A B
C D
Figs 78.32A to D
E F
Figs 78.32A to G: Quadricuspid aortic valve in a 54-year-old

female. (A and B) Two-dimensional transthoracic echocardiogra-
phy. The aortic valve (AV) appears bicuspid. (C to E) Multiplane two-
dimensional transesophageal echocardiography. (C and D) AV leaf-
lets (numbered in D) are well seen. The arrow in C points to dias-
tolic noncoaptation of AV leaflets which resulted in significant aortic
regurgitation. (E) The arrow points to severe aortic regurgitation.
(F and G) Live/real time three-dimensional transthoracic echo-
cardiography show a quadricuspid AV with four numbered leaflets
clearly visualized. (AO: Aorta; LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve). (Movie
clips 78.32A to D, 78.32F part 1 and part 2).
Source: Reproduced with permission from Burri et al. Live/real time
three-dimensional transthoracic echocardiographic identification of
G quadricuspid aortic valve. Echocardiography. 2007;24:653–5.
Fig. 78.33: Muscular outflow ventricular septal defects. Two-

dimensional transthoracic echocardiography. Short-axis view
at the level of the LV outflow tract demonstrates two adjacent
ventricular septal defects (1 and 2). Note the two defects are
not related to the tricuspid or pulmonary valve and hence are
not in the perimembranous or supracristal location. (AO: Aorta;
PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle; TV:
Tricuspid valve). (Movie clip 78.33).
A B
C D
Figs 78.34A to D: Levo (corrected) transposition of the great vessels with a ventricular septal defect and pulmonary artery banding in
a 25-year-old female. Parasternal long-axis views (A and B) show a large ventricular septal defect with flow signals moving from the
pulmonary ventricle (morphological LV) into the aorta (AO). The arrow points to a band in the pulmonary artery (PA) surgically placed
in an attempt to protect the patient from pulmonary hypertension. Two-dimensional (C) and three-dimensional (D) transthoracic short-
axis views show the aortic valve and the AO located directly anterior to pulmonary valve (PV) and PA indicative of transposition of the
great vessels. (CS: Coronary sinus; LV: Left ventricle; MV: Mitral valve; RA: Right atrium). (Movie clips 78.34A to D).
A B
Figs 78.35A and B
Figs 78.35A to C: Embolization of a left atrial appendage clot

into the systemic circulation in a 67-year-old female undergoing
coronary artery bypass surgery. Two-dimensional transesophageal
echocardiography. This was initially performed to rule out the pres-
ence of mitral regurgitation because an apical systolic murmur was
heard pre-operatively. No significant mitral regurgitation was noted
but a clot was demonstrated in the left atrial appendage (LAA) (A).
The patient had two brief episodes of atrial fibrillation in the past
which probably accounted for the formation of the clot. Before the
surgeon could make any decision, the clot was noted to embolize
from the appendage into the systemic circulation (B and C). In the
immediate post bypass period, the left femoral pulsation was found
to be absent pointing to the location of the clot which was success-
fully removed. The patient had an uneventful recovery without any
stroke or other complication. (AO: Aorta; LA: Left atrium; LV: Left
C ventricle). (Movie clip 78.35).
A B
C D
Figs 78.36A to D
E F
Figs 78.36A to F: Combined valvar and supravalvar aortic stenosis. Live/real time three-dimensional transthoracic echocardiography.
(A) Horizontal arrowhead points to supravalvar aortic stenosis produced by calcification at the sinotubular junction. The vertical arrow-
head shows heavy mitral annular calcification; (B) Supravalvar stenotic orifice viewed in short axis (arrowhead); (C) Short-axis view at
the level of the aortic valve (AV) leaflets demonstrating mild valvar stenosis. Live/real time three-dimensional transthoracic echocardiog-
raphy in a patient with calcification at the aortic sinotubular junction but no stenosis; (D) The arrowheads point to prominent calcifications
at the sinotubular junction viewed in long axis; (E) Short-axis view at the sinotubular junction shows a large orifice (arrowhead) that
measured 2.5 cm2; (F) Short-axis view at the level of the AV (left) and immediately above it (right). The AV orifice measured 1.7 cm2 by
planimetry, consistent with mild aortic stenosis. The arrowhead in the right panel points to sinotubular calcification protruding into the
aortic lumen imaged just beyond the AV leaflets. (AO: Ascending aorta; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA:
Right atrium; RV: Right ventricle; RVO: Right ventricular outflow tract; TV: Tricuspid valve). (Movie clip 78.36).
Source: Reproduced with permission from Rajdev et al. Live/real time three-dimensional transthoracic echocardiographic assessment
of combined valvar and supravalvar aortic stenosis. Am J Geriatr Cardiol. 2006;15:188–90.
A B
Figs 78.37A and B
C D
Figs 78.37A to D: Paravalvular mitral regurgitation in a 69-year-old female. Paravalvular mitral regurgitation (arrowhead) is noted by
transthoracic (A) and transesophageal (B) echocardiography. (C and D) Represent live/real time three-dimensional images showing two
plugs (arrowheads) used to successfully close the leak percutaneously in the cardiac catheterization laboratory. Small arrows in D point
to intact sutures. (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; MR: Mitral regurgitation; MVR: Mitral valve replacement; RA: Right
atrium; RV: Right ventricle). (Movie clips 78.37A to D).
CONCLUSION 3. Lauer MS, Larson MG, Levy DL. Sex-specific reference

M-mode values in adults: population-derived values with
Echocardiography proves to be a very useful diagnostic consideration of the impact of height. J Am Coll Cardiol.
1995;26:1039–46.
tool for women, but it is not without its challenges. There
4. Sadaniantz A, Hadi BJ, Saint Laurent L. Gender Differences
are structural heart diseases that occur more commonly in in Mitral Inflow Parameters of Doppler Echocardiography.
women, and it is important to remember that women can Echocardiography. 1997;14(5):435–40.
often have atypical presentations, which sometimes delays 5. Vasan RS, Larson MG, Benjamin EJ, et al. Echocardiographic
the accurate diagnosis. Often, the diagnosis is delayed in reference values for aortic root size: the Framingham Heart
Study. J Am Soc Echocardiogr. 1995;8(6):793–800.
women because it is thought that the likelihood of a certain
6. Movahed MR. Impairment of echocardiographic acoustic
disease process is rather low. Hopefully, this discussion window caused by breast implants. Eur J Echocardiogr.
has helped to illustrate and highlight the structural heart 2008;9(2):296–7.
disease processes that are actually seen more frequently in 7. Avierinos JF, Inamo J, Grigioni F, et al. Sex differences in
women. morphology and outcomes of mitral valve prolapse. Ann
Intern Med. 2008;149(11):787–95.
8. St John Sutton M, Weyman AE. Mitral valve prolapse preva-
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1921
CHAPTER 79
Echocardiography in the Elderly
Gopal Ghimire, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia
Snapshot

AorƟc Atherosclerosis and PenetraƟng AorƟc Ulcer 
LeŌ Ventricular Mass, Dimensions and FuncƟon

AorƟc Valve Sclerosis 
Echocardiography in Stroke PaƟents: Assessment of

AorƟc Stenosis Coronary Stenosis

AorƟc Aneurysm 
Mitral Annular CalcificaƟon

AorƟc DissecƟon 
ProstheƟc Valves
INTRODUCTION was 7.6%. The prevalence of aortic plaques increased

progressively from 8.4% in the ascending aorta to 31% in
Aging induces significant alteration in the structure and aortic arch and 44.9% in the descending aorta. Similarly,
function of the cardiovascular (CV) system. Although these the prevalence of the complex plaques in the respective
adaptive changes are physiological and asymptomatic, locations was 0.2%, 2.2%, and 6.0%. The atherosclerotic
they are at times difficult to distinguish from preclinical plaque on the aortic arch is now considered as the third
disease. Furthermore, presence of coexisting CV diseases leading cause of embolic stroke.1 The prevalence of ≥4 mm
can modulate these adaptive responses and complicate aortic plaque in stroke patients (14–21%) is on the same
the clinical picture. In this chapter, we aim to discuss the order of magnitude as that of the other two important
CV peculiarities in elderly patients. causes of embolic stroke—carotid artery disease
(10–13%) and atrial fibrillation (18–30%).3,4 Although the
AORTIC ATHEROSCLEROSIS AND aortic plaque can be construed as a surrogate marker for
PENETRATING AORTIC ULCER generalized atherosclerosis and stroke risk, the French
Aortic Plaque in Stroke (FAPS) study established causality
(FIGS 79.1A TO C) between the stroke and complex aortic arch atheroma as
Atherosclerosis is a systemic disease with a strong the odds ratio (OR) for stroke in patients with plaques in
predilection to involve the aorta especially in the elderly. the descending aorta (unlikely to embolize upstream to
A study revealed that the average age of those with the cerebral vessels) was only 1.5 for the plaques ≥ 4 mm
large/complex plaques (measuring ≥ 4 mm in size) in when compared with an OR of 13.8 for those in the aortic
the aorta on transesophageal echocardiography (TEE) arch.4 Furthermore, this study also established a gradient
was 70 years.1 In the Stroke Prevention: Assessment of between increasing plaque thickness and incremental
Risk in a Community (SPARC) study,2 of 588 patients stroke risk with OR significantly greater in those with a
(average age, 66.9 years) undergoing TEE, the overall plaque thickness ≥ 4 mm: the OR for stroke in patients
prevalence of aortic plaque in any location was 43.7%, with submillimeter plaques was 1.0 (no increased risk), in
of which complex plaque (defined as ≥ 4 mm or mobile) contrast to 3.9 for 1–3.9 mm plaques, and 13.8 for plaques
A B
Figs 79.1A to C: Aortic atherosclerosis in three elderly patients.

(A and B) Two-dimensional (2D) transesophageal echocardiog-
raphy (A) Upper arrow points to a mobile atherosclerotic plaque
and the lower to a fixed one in the descending thoracic aorta (AO).
Arrowhead shows prominent ulceration in the plaque; (B) Arrow,
in another patient, shows a mobile plaque in the ascending aorta
(AA); (C) 2D transthoracic echocardiography. Arrow, in a differ-
ent patient, shows a fixed plaque in the proximal abdominal aorta
(AB) imaged using the subcostal approach (Movie clips 79.1A to C).
C (L: Liver).
≥ 4 mm. Although transthoracic echocardiography (TTE) has predilection for the thoracic and abdominal aorta
can visualize the aortic root and proximal ascending aorta, more than in the arch or ascending aorta. PAU typically
the aortic arch, and the descending thoracic aorta from mimics presentation of the classic aortic dissection with
various windows, the resolution of the TTE is not optimal acute chest or back pain, and indeed PAUs constitute
enough for accurate evaluation of the atherosclerotic up to 2–8% of acute aortic syndrome.7 However, 25% of
plaque. In contrast, the TEE probe is closer to the aorta patients with PAU are asymptomatic and the lesions were
and can be used at a higher frequency, thus allowing for identified during axial imaging for other indications.8 The
higher resolution than on TTE. An accurate and detailed patients are typically older and hypertensive individuals
evaluation of the aorta, including the origin of the great with multiple coronary risk factors and coexisting vascular
vessels, is possible,5 and there is excellent interobserver disease. Imaging techniques for PAU include aortography,
and intraobserver variability.6 computed tomography (CT), magnetic resonance imaging
Very uncommonly, the atherosclerotic lesion pene- (MRI), and TEE with TEE showing aortic atherosclerotic
trates the internal elastic lamina into the media, leading plaque with focal ulceration of the intima. The management
to formation of a penetrating aortic ulcer (PAU). The of the patient with PAU must be individualized depending
natural history of this entity may involve progression to a on the location and disease progression. The general
variable degree of intramural hematoma (IMH) formation, consensus is that a large or unstable PAU or a PAU with
pseudoaneurysm formation, aortic rupture, or late aneu- progression should have surgical intervention. The pre-
rysm.7 Although PAUs can occur throughout the aorta, it dictors of disease progression are refractory or recurrent
Chapter 79: Echocardiography in the Elderly 1923
Fig. 79.2: Aortic valve sclerosis. Three-dimensional transesopha- Fig. 79.3: Aortic valve sclerosis. Three-dimensional transesopha-
geal echocardiography. Short axis cropping at the tip of the aortic geal echocardiographic reconstruction. Shows a thickened tricus-
valve (AV) obtained from a parasternal long-axis data set shows pid aortic valve (AV) with no significant pressure gradient. (LA: Left
a large aortic orifice (arrowhead in the Movie clip 79.2) with no atrium; RA: Right atrium; RV: Right ventricle) (Movie clip 79.3).
evidence of stenosis. The AV leaflets are mildly thickened consist-
ent with sclerosis. (LA: Left atrium; TV: Tricuspid valve). (Movie
clip 79.2).
Source: Reproduced with permission from Nanda NC, Hsiung MC,
Miller AP, Hage FG. Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.
pain, ulcer craters > 20 mm in diameter or 10 mm in

depth, interval worsening of aortic hematoma, periao-
rtic hematoma, expanding pseudoaneurysm, increasing
pleural effusion, and rupture. Due to segmental nature
of this entity, endovascular intervention is an attractive
alternative, especially since these patients tend to be
elderly and have multiple comorbidities.9–13
AORTIC VALVE SCLEROSIS

(FIGS 79.2 TO 79.4)
Fig. 79.4: Aortic valve sclerosis. Three-dimensional transeso-
phageal echocardiographic reconstruction demonstrates a mildly
Murmur thickened, obliquely oriented bicuspid aortic valve (AV) with raphe
A systolic murmur in the aortic area is present in and no significant gradient consistent with AV sclerosis. (LA: Left
atrium; RA: Right atrium; RV: Right ventricle). (Movie clip 79.4).
approximately 50% of elderly population.14,15 The reliability
of clinical examination alone in the assessment of aortic
sclerosis or stenosis (AS) in the elderly is poor.16 In one that echocardiography should not be done for patients who
study, 17% of patients who were deemed to have AS only have a Grade II or softer midsystolic murmur identified as
on physical exam were found to have mitral regurgitation innocent or functional by an experienced observer.18
(MR) as the only significant finding.17 Most of the murmurs
in the elderly subjects are related to sclerotic aortic valve Prevalence, Pathophysiology, and
(AV) leaflets, flow into tortuous, noncompliant great
vessels, or a combination of these. Such murmurs must
Echocardiographic Findings
be distinguished from murmurs caused by mild to severe AV sclerosis is focal thickening of the AV leaflets with com-
valvular AS, or atrioventricular valve regurgitation, which missural sparing, normal leaflet mobility, and no evidence
are prevalent in this age group. The ACC/AHA recommends of left ventricular outflow tract (LVOT) obstruction with
transaortic gradient of <2.5 m/s. The degenerative process AORTIC STENOSIS

may not be confined to the leaflets alone; it can also ex-
tend to the aortic root. Furthermore, 50% of patients with Prevalence and Pathophysiology
AV sclerosis also have associated mitral valve sclerosis.19
AS in adults is caused by a calcific process involving a
Approximately, one in four subjects more than 65 years
normal trileaflet or congenital bicuspid aortic valve (BAV).
may have AV sclerosis and the prevalence increases with
This calcific process can progress from the base of the
advancing age.20 However, AV sclerosis is not an inevi-
cusps to the leaflets, eventually causing a reduction in
table consequence of aging as 25–45% of octogenarians
leaflet motion and effective valve area without leading to
have no evidence of AV calcification.21,22 The relationship
commissural fusion.29 The AV is more likely to be tricuspid
between AV sclerosis and age is nonlinear with a sharp
in patients in their eighth, ninth, and tenth decades of life,
increase in prevalence at age about 65 years in men and at
and bicuspid in younger patients.30–32 However, a study
age about 75 years in women.
revealed that three out of nine nonagenarians undergoing
Despite normal hemodynamics, AV sclerosis is not
surgical AV replacement had bicuspid valves.33 In the
benign; it is independently associated with poor CV
Cardiovascular Health Study, AS was present in 2% of
outcome, for example, with a relative risk of death of
the entire study cohort (adults ≥ 65 years), 2.6% of those
1.66 [95% confidence interval (CI) 1.23–2.23]23 or of a
75 years or older, and 4% of those 85 years or older.
new coronary event of 1.76 (95% CI 1.52–2.03).24 In LIFE
Changing demographic pattern in the western society and
study (Losartan Intervention For End point reduction in
changing epidemiology of rheumatic heart disease have
hypertension), composite CV outcome occurred in 15%
rendered calcific AS as one of the most prevalent valvular
of hypertensive patients with AV sclerosis, compared with
diseases. AS currently is considered the most frequent
8% patients with normal AV.25 Patients admitted with chest
indication for valve replacement surgery, and the second
pain and AV sclerosis had a higher incidence of CV events
most common indication for cardiac surgery in older
(16.8% vs 7.1%) and worse event-free survival than did
adults, which is surpassed only by coronary artery bypass
those without AV sclerosis.26 In the CV health study, the
grafting (CABG).34
rate of death from any cause or death from CV cause of
patients with AV sclerosis was twice that of patients with
a normal AV.23 Two-dimensional (2D) echocardiography Two-Dimensional Echocardiography
is the best noninvasive modality to diagnose AV sclerosis. (Figs 79.5 to 79.7; Also see Fig. 78.11
The mobility of AV leaflets and the severity and extent
in Chapter 78)
of calcification on the cusps and in the aortic annulus
along with peak and mean pressure gradients across the Echocardiography is an integral part of the evaluation of
AV should be measured. The absence of high gradients AS in order to confirm the diagnosis, and assess its severity
distinguishes it from significant AS. Three-dimensional and evaluate its upstream consequences on the left
(3D) echocardiography can supplement the 2D technique ventricle (LV). Clinical signs and symptoms are of limited
by providing a more comprehensive evaluation of AV use in distinguishing critical from noncritical AS due to
anatomy, leaflet motion, and exact sites and degree of unsatisfactory sensitivity and specificity in the aged.35
calcification. Although cardiac catheterization is regarded as the gold
AV sclerosis may progress to AS. Of 2,131 patients with standard for evaluation of valvular dysfunction, it is invasive
AV sclerosis, 15.9% developed AS over a mean follow-up and is associated with a higher risk of complications in the
of 7.4 years with 5.4% developing clinically significant elderly. In a single center study, 22 of 101 patients with
(moderate to severe) AS.27 In another study of 400 patients valvular AS who underwent retrograde catheterization
with AV sclerosis followed for a mean duration of 44 ± 30 of the AV had focal diffusion-imaging abnormalities in
months, 32.75% developed some degree of AS with 2.5% cranial MRI, a pattern consistent with acute cerebral
developing severe AS defined as a peak jet velocity ≥ 4 m/s, embolic events after the procedure; three of these patients
5.25% of patients progressed to moderate AS, which was (3%) had clinically apparent neurological deficits.36 Thus,
defined as a peak jet velocity between ≥3.1 and ≤3.9 m/s, cardiac catheterization in the elderly portends substantial
and 25% developed mild AS, which was defined as a peak risk of clinically apparent cerebral embolism and frequent
jet velocity between >2 and ≤3 m/s.28 silent ischemic brain lesions. Cardiac catheterization is
A B
C D
Figs 79.5A to D: A 79-year-old man with exertional angina and syncope. Two-dimensional transthoracic echocardiography. On the
parasternal long-axis view (A) a calcified aortic valve with restricted opening is shown at the arrow. The maximal velocity across the
valve on continuous wave (CW) Doppler is 4.1 m/s (B) with a velocity time integral (VTI) of 84 cm, which correspond to a peak gradient
of 67 mm Hg and mean gradient of 35 mm Hg. The PW across the left ventricular outflow tract (LVOT) measured a velocity of 88.4 cm/s
across the LVOT (C); (D) The aortic valve area and the dimensionless index are calculated, both corresponding to severe aortic stenosis.
Notice that small errors in the measurement of the LVOT diameter will result in large errors in the calculated aortic valve area, while
the dimensionless index is unaffected by this measurement. Adapted from Adegunsoye A, et al. Echocardiography. 2011;28:117–29.
currently recommended for assessment of severity of free edge remains mobile despite immobility of the base,
AS only in symptomatic patients when noninvasive tests and fusion of the commissures does not occur primarily. If
are inconclusive or when there is a discrepancy between a discrete orifice can be demonstrated, then the valve area
noninvasive tests and clinical findings regarding severity can be measured with planimetry, but direct planimetric
of AS.18 measurements of the aortic orifice are of insufficient
During echocardiography, it is of utmost importance sensitivity and specificity as a result of the uncertainty that
to check for the consistency of different echocardiographic the plane of imaging is at the leaflet tips (where maximum
findings and with clinical assessment. In patients with stenosis commonly occurs) and is exactly parallel to the
severe AS, two-dimensional transthoracic echocardio- orifice.
graphy (2D TTE) shows augmented reflectance of the The determinants of pressure gradient across a stenotic
valvular cusps along with significant thickening and valve are the valve orifice area and the transvalvular flow.37
deformity. The valve leaflets are domed, less mobile, and Thus, in the presence of depressed cardiac output, relatively
with restricted leaflet excursion. In intermediate cases, the low pressure gradients may be obtained in patients with
facilitated by using color Doppler as a guide that enables

easy visual identification of the aortic jet.38 The peak and
mean velocities across the AV can be translated into
peak and mean pressure gradients by using the modified
Bernoulli’s equation.39 An important caveat is that even
with color Doppler guidance, optimization in the cursor
position is paramount to obtain the maximum velocity.
This is because the AS jet has an outer region of relatively
low velocity flow and a central core of higher velocity that
can be appreciated only when viewed in three dimensions
(Fig. 79.6).40 Malalignment of the beam with the flow
across the valve will lead to underestimation of the velocity
and, therefore, of the severity of AS. In order to minimize
this source of error, the velocities have to be measured
using several echocardiographic windows, the apical and
Fig. 79.6: Schematic. Because a stenotic jet consists of a small
central region or “core” of high velocity and a larger outer region
right parasternal views, and the highest value assumed to
of lower velocity, the continuous wave Doppler cursor must be correspond to the proper alignment.41 Since transvalvular
positioned in the jet core in addition to being aligned parallel to velocity is squared in the equation, a small change in
the jet direction to measure the maximum jet velocity. The three- transvalvular velocity can result in a significant change
dimensional structure of the jet may cause the continuous wave in the transvalvular gradient. Estimation of the aortic
cursor to appear to be correctly positioned in the jet while, in reality,
the cursor may not be properly placed in the core. Therefore,
valve area (AVA) can also be performed with the use of
after the initial alignment of the continuous wave cursor in the the continuity equation, which depends on the principle
visualized jet, minimal transducer angulations are still required of the law of continuity of flow.42 This has been shown to
to interrogate the jet core, which may be in the azimuthal plane. be a reliable index of AS severity that correlates highly
Failure to interrogate the core results in an underestimation of the
with cardiac catheterization measurements and surgical
peak transvalvular velocity and thus the severity of the stenotic
lesion. In this illustration, the aortic stenosis (AS) jet is shown to findings.43 This calculation entails measuring the LVOT
consist of a central core, which has the highest velocity, and an diameter, the time velocity integral at the LVOT using
outer region of lower velocity flow surrounds this central core. The pulsed wave Doppler, and the time velocity integral at the
highest velocity is thus obtained if the continuous wave Doppler AV (using continuous wave Doppler, Fig. 79.2). LVOT cross-
cursor is aligned parallel to the core of the jet (cursor 2), while
sectional area is computed from the diameter assuming
lower velocities are recorded if the cursor is positioned outside the
core (cursors 1, 3, 4, and 5). that the outflow tract has a circular configuration. The
Source: Reproduced with permission from Nanda NC. Textbook of outflow tract diameter is measured immediately proximal
Color Doppler Echocardiography. Philadelphia, PA: Lea and Fe- to the attachment of the aortic leaflets during systole from
biger, Inc; 1989:178–90.40 the standard parasternal long-axis plane, and the largest
inner diameter is selected.44 The peak LVOT velocity is
severe AS. Conversely, during exercise or other high-flow estimated with the pulsed Doppler technique in the apical
states, significant pressure gradients can be measured transducer position, with the sample volume placed
in minimally stenotic valves. Therefore, comprehensive 1.0–1.5 cm proximal to the AV to avoid the area of AS jet
assessment of AS requires measurement of transvalvular flow acceleration. The continuity equation is independent
flow, mean transvalvular pressure gradient using Doppler of transaortic flow and the presence of aortic or mitral
interrogation, and calculation of the effective valve orifice insufficiency, and the equation has been validated in the
area. presence of severe aortic incompetence.45 The continuity
Doppler echocardiography utilizes ultrasound reflec- equation assumes that the LVOT diameter and the flow
ting off moving red blood cells to measure the velocity across the LVOT are measured at the same level, which
of blood flow and allows the noninvasive assessment of is technically difficult since they are assessed in different
normal and abnormal blood flow patterns. Continuous echocardiographic views. Also, since continuous wave
wave Doppler echocardiography measures the highest Doppler is used to measure the highest velocity across a
velocities along the plane of interrogation, but for straight path, it also assumes that there is no other form
reliable velocity measurements, the cursor must be of LVOT obstruction other than AS such as supravalvular
aligned parallel to the flow jet. The accuracy of this task is or subaortic stenosis. Nevertheless, the most frequent
A B
C D
E F
Figs 79.7A to F: Mitral and aortic annular calcification in a 99-year-old patient. Two-dimensional transthoracic echocardiography.
(A and B) Parasternal long-axis view (A) and parasternal short-axis view (B) at the level of the aortic valve show heavy calcification
involving the aortic valve, and aortic and mitral annuli (1), mitral valve (2), and mitral subvalvular apparatus (3, 4); (C) Parasternal
short-axis view at the level of the mitral valve shows calcification involving the posterior mitral leaflet and mitral annulus (2); (D)
Apical four-chamber view showing calcification involving the ventricular septal muscle (arrow). Arrowhead points to a pacemaker in
the right atrium (RA); (E) Apical two-chamber view showing calcification involving the mitral valve and annulus (2). R represents a
reverberatory artifact deep in the left atrium from the calcified mitral valve. (F) Color Doppler-guided continuous wave Doppler inter-
rogation of the aortic valve demonstrates peak and mean gradients of 30 and 18 mm Hg, respectively (arrow), consistent with mild
aortic stenosis. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle) (Movie clips 79.7A to E).
Table 79.1: Limitations of Two-Dimensional Transthoracic/Color Doppler Echocardiography

The continuity equation provides an indirect rather than direct estimation of the AVA
True LVOT velocity can be difficult to determine from the area of increased flow acceleration
Accurately measuring LVOT diameter may be difficult (e.g. in patients with aortic or mitral annular calcification)
Doppler cursor may not be in the jet core even with color Doppler guidance, resulting in AS severity underestimation
Localized high gradients may be present in the region of the aortic valve; these do not reflect the true gradient across the AV,
resulting in overestimation of AS severity
AS severity cannot be correctly assessed in the presence of coexisting subaortic or supravalvular stenosis
Source: Adapted from Vengala et al.52

(AS: Aortic stenosis; AV: Aortic valve; AVA: Aortic valve area; LVOT: Left ventricular outflow tract).
source of error in measuring the AV area is due to the presumably related to calcific areas in the AV, mistaken
inaccuracy of LVOT diameter measurement. This is identification of a jet (the jet of MR can be mistaken for
especially difficult in older adults with accumulation of AS or the smaller velocity aortic jet may be contaminated
calcium at the annulus. Furthermore, since the LVOT is with the much higher velocity signals from the associated
often elliptical rather than circular in nature if measured MR jet), concomitant stenotic lesions present in tandem
proximal to the LV–aortic junction, 3D modalities of (such as supravalvular stenosis or discrete subaortic
imaging may be more accurate in measuring the LVOT stenosis), and nonrepresentative jet selection (e.g. a post
than 2D TTE.46 The age-related alteration in the LVOT extrasystolic beat). Table 79.1 depicts the limitations of
geometry with reduction in the septoaortic angle with the transthoracic/color Doppler echocardiography for
advancing age might render the outflow tract narrow in evaluation of AS.
the parasternal long-axis view.47 Since the LVOT diameter Multiplane transesophageal echocardiography (2D TEE)
is squared in the calculation of AVA, even minor errors in permits detailed evaluation of the morphology of the
measurement will amplify the inaccuracy of estimation stenosed AV that may provide useful information about
of AVA. This is particularly pertinent during evaluation the etiology of AS (degenerative, rheumatic, or bicuspid)
of the progression of severity of stenosis in an individual independent of the transthoracic acoustic window. By
patient, since large factitious variations in the calculated gradually advancing the transesophageal probe to detect
valve area can be due to erroneous measurements of the the flow-limiting tip of the valve that is stenotic allows
LVOT diameter. This limitation can be obviated by usage direct planimetric quantification of the anatomical AVA.
of the dimensionless index, which is simply the ratio of The accuracy of this semi-invasive method may be limited
the velocity across the LVOT to that across the AV and by difficulties in obtaining the optimal imaging plane
completely eliminates the area of the LVOT from the orientation at the level of the tip of the stenosed AV cusps.
equation.42 A ratio of 0.9–1.0 is considered as normal and Prior studies found clinically important overestimation
a ratio of <0.25 is regarded as severe stenosis. Another of effective AVA by TEE planimetry compared with flow-
potential source of error that is particularly relevant in derived methods with a variable reproducibility and
older population is the overestimation of the severity of AS accuracy.53–56 In geriatric population, precise planimetric
due to the phenomenon of pressure recovery, which leads quantification of AVA by 2D TEE poses unique challenges
to the overestimation of AS severity when measured by due to the altered aortoseptal angle with an obliquely
Doppler due to the conversion of kinetic energy upstream placed aorta, prior valve replacement, or severely calcified
to potential energy downstream across the stenotic valve. valvular cusps, which cause acoustic shadowing and
This effect is further exaggerated in individuals with interfere with visualization. In clinical echocardiographic
domed and tubular stenoses, a narrow ascending aorta practice, AS is considered severe when the AVA is < 1.0 cm2,
(<3 cm), or a narrow LVOT (≤2 cm).48–51 Other situations indexed AVA57 is < 0.6 cm2/m2, and the mean transvalvular
in which Doppler echocardiography can potentially gradient is > 40 mm Hg.18,57 Severe AS is unlikely if stroke
overestimate the severity of AS includes confounding volume (that translates into transvalvular flow) is normal
of the true AS jet with localized high velocity gradients and there is a mean pressure gradient of < 40 mm Hg.
Low Flow, Low Gradient Aortic Stenosis stress echo (DSE) up to a maximum dosage of 20 μg/kg/min
is useful to discriminate severe from pseudosevere AS and
with Low Ejection Fraction (Fig. 79.8) evaluate the LV contractile reserve.59,61 This test should
In the presence of low transvalvular flow, lower pressure be performed with great caution in the elderly because
gradients may be encountered even in presence of severe of the increased potential for ventricular arrhythmia
AS; since the gradients are a squared function of flow, especially in the presence of coexisting coronary artery
even a modest decrease in flow may lead to a significant disease (CAD). Typically, pseudosevere AS in the elderly
reduction in gradient. A low-flow, low-gradient (LF-LG) shows a significant increase in AV opening and AVA with
state is encountered in 5 to 10% of patients with severe relatively little increase in gradient, whereas true severe
AS.58 Typically, these patients have severely depressed AS is characterized by no significant increase in AVA
LV systolic function59–62 and portend a dismal prognosis even though there is significant change in LV ejection
irrespective of modality of treatment with survival rates of fraction and stroke volume as reflected by an increase in
50% at 3-year follow-up if treated medically, and 6 to 33% the LVOT velocity time integral (V TI). No change in AVA
if treated surgically.58,61–63 with <20% change in stroke volume or LVOT V TI points
LF-LG severe AS is defined as EOA 1.0 cm2 or 0.6 cm2/m2 to poor LV contractile reserve. Patients with low-flow
when indexed for BSA, mean transvalvular gradient reserve constitute approximately 30–40% of patients with
< 40 mm Hg, and left ventricular ejection fraction (LVEF) low LVEF, LF-LG AS, and its presence portends a higher
< 40% in the presence of a low-flow state that is defined operative mortality (22–33%) than those with normal
as a cardiac index < 3.0 L/min/m2 and a stroke volume flow reserve (5–8%).59–62 However, the postoperative LVEF
index < 35 mL/m2.57,64,65 Stroke volume can be measured by recovery and the late survival rate in patients who survived
Doppler echocardiography,66–68 biplane Simpson method surgery were equivalent to those with normal flow reserve70
(2D TTE), or cardiac catheterization.69 However, the mere and much better than in those with no flow reserve treated
presence mean gradient of <40 mm Hg and small AVA and medically.66 In summary, the assessment of LV flow
low LVEF does not definitely confirm LG LF severe AS, reserve by DSE is useful to estimate the operative risk but
since mild-to-moderately diseased valves may not open the absence of flow reserve is not predictive of recovery of
fully due to low-flow state, resulting in a “functionally small LV function, improvement in symptomatic status, and late
valve area” (pseudosevere AS). In these cases, dobutamine survival after surgery.61,62,70 The absence of LV flow reserve,
therefore, should not preclude consideration of AVR in
these patients.61,66 However, since the operative risk for
surgical AVR is generally very high in the absence of flow
reserve, transcatheter aortic valve replacement (TAVR)
may be a valuable alternative in these patients.71
Paradoxical Low Flow, Low Gradient

Aortic Stenosis with Preserved Ejection
Fraction
As many as one third of patients with severe AS on the basis
of AVA calculation have unequivocally low transvalvular
gradients (mean gradient < 40 mm Hg) despite a preserved
LV ejection fraction of >50%.72 These patients tend to
be in the older age group, have small, concentrically
hypertrophied LV with a lower LV diastolic volume index
Fig. 79.8: Dobutamine stress echocardiography to evaluate the
(52 ± 12 mL/m2), a higher level of LV global afterload
left ventricular flow reserve and to distinguish the true severe
aortic stenosis from pseudosevere aortic stenosis. (EOA: Effective reflected by a higher valvuloarterial impedance, and a
orifice area (in square centimeters); EOAProj, projected EOA at lower overall 3-year survival a poor outcome if managed
normal flow rate (in square centimeters); P: Mean transvalvular medically.72–74 The diagnosis is suggested on echo/Doppler
gradient (in mm Hg); SV: Stroke volume).
studies by noting paradoxically low flows with a calculated
stroke volume index of 35 mL/m2 or less in the presence transformed the complex technique of 3D imaging into an
of normal LV ejection fraction as well as decreased LV efficient, cost-effective, and clinically viable procedure.78,79
midwall shortening. Currently, paradoxically low-flow, Rotating the 3D data set and cropping from the aorta to
low-gradient severe AS is considered a distinct clinical the LVOT and stopping at the tips of the trileaflet or BAV
entity. The distinctive features of this entity are robust allows for the direct planimetry of the stenotic valve and
LV concentric remodeling and myocardial fibrosis, both calculates AVA with increased confidence level.80–83
contributing to the restrictive physiology. The fibrosis is The inherent ability to present data in 2D cut-planes
mainly subendocardial and is responsible for marked from a 3D data set permits assessment of angulated
attenuation of intrinsic LV systolic function, not evidenced orifices and domed AVs as well as localizes and evaluates
by the LVEF, but rather by other more sensitive parameters the exact individual sites of obstruction in patients
directly measuring LV midwall or longitudinal axis with associated subvalvular, supravalvular, or tandem
shortening.74–77 obstruction (see Figs 79.3 to 79.5).83–86 In low-flow states
as in patients with severe AS and low cardiac output, 3D
Live/Real Time Three-Dimensional TTE may also supplement 2D TTE; the AVA can be directly
Transthoracic Echocardiography inspected and measured following dobutamine infusion,
which obviates reliance on 2D TTE/Doppler gradients
(Figs 79.9 to 79.16) that provide only indirect estimations of AS severity.
Live/real time three-dimensional transthoracic echocardi- Although 3D TTE can be performed in all patients with
ography (3D TTE) with a full matrix–array transducer has AS, it is especially important to perform 3D TTE when
A B
Figs 79.9A to C: Aortic valve stenosis. Live/real time three-

dimensional transthoracic echocardiography. (A to C) Careful
cropping of the parasternal long-axis data set at the flow-limiting
tips of the aortic valve (AV) leaflets demonstrated a bicuspid
morphology and severe stenosis. (LA: Left atrium; LV: Left
ventricle). (Movie clips 79.9A to C Parts 1 to 4).
C UK: Wiley-Blackwell; 2010.
A B
Figs 79.10A to C: Another adult patient with bicuspid aortic valve

stenosis. Live/real time three-dimensional transthoracic echocar-
diography. (A and B) The arrowhead in A points to thickened aortic
valve (AV) leaflets with restricted opening motion viewed in par-
asternal long axis. Short-axis cropping at the AV tip demonstrates
a severely stenotic bicuspid valve; (C) QLab cropping shows a
small orifice consistent with significant AV stenosis. Despite the
presence of a motion artifact, it was possible to assess the orifice
size in this patient. (LA: Left atrium; LV: Left ventricle). (Movie clips
79.10A to C Parts 1 to 4).
C UK: Wiley-Blackwell; 2010.
Fig. 79.11: Mild aortic stenosis. Three-dimensional transesopha- Fig. 79.12: Severe aortic stenosis. Three-dimensional transesoph-
geal echocardiographic reconstruction showing a mildly calcified ageal echocardiographic reconstruction. Demonstrates a heavily
tricuspid aortic valve (AV) with minimal stenosis. (LA: Left atrium; calcified, vertically oriented bicuspid aortic valve (AV) with a very
RA: Right atrium; RV: Right ventricle) (Movie clip 79.11). small orifice indicative of severe stenosis. (LA: Left atrium; RA:
Right atrium; RV: Right ventricle) (Movie clip 79.12).
Fig. 79.13: Severe aortic stenosis. Three-dimensional transesoph-

ageal echocardiographic reconstruction. Demonstrates a heavily
calcified, horizontally oriented bicuspid aortic valve (AV) with a very
small orifice denoting severe stenosis. (LA: Left atrium; RA: Right
A B
Figs 79.14A and B: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. (A) Shows a heavily
calcified tricuspid aortic valve (AV) with small a very small orifice consistent with severe stenosis; (B) In addition, a perforation
(arrowhead) is visualized in diastole in one of the cusps. (LA: Left atrium; RA: Right atrium; RV: Right ventricle) (Movie clip 79.14).
Fig. 79.15: Severe aortic stenosis. Three-dimensional transeso-

phageal echocardiographic reconstruction. Demonstrates severe
tricuspid aortic valve (AV) stenosis with a very small orifice but
only mild calcification. (LA: Left atrium; RA: Right atrium; RV: Right
ventricle) (Movie clip 79.15).
Table 79.2: Indications for Performing Three-Dimensional Echocardiography in Older Adults with Suspected Aortic Stenosis
Increased likelihood of significant pressure recovery:
• Aortic valve doming on two-dimensional echocardiography (in both bicuspid and tricuspid aortic valves)
• Tubular, rather than discrete aortic stenosis
• Narrow or borderline-narrow left ventricular outflow tract diameter (≤ 2.0 cm)
• Small ascending aortic diameter (< 3.0 cm measured at or just beyond the sinotubular junction)
Discrepancy between two-dimensional echocardiography and Doppler findings
Discrepancy between two-dimensional echocardiography/Doppler and clinical findings
A case can be made for performing three-dimensional echocardiography in all patients with suspected aortic stenosis because
of the additional data provided and to avoid the dependence on gradients obtained by technicians; however, this may not be
practical or cost-effective
A B
Figs 79.16A and B: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. (A) Demonstrates
a horizontally oriented severely stenotic bicuspid aortic valve (AV) with a valve area of 0.9 cm2; (B) Diastolic frame shows a perforation
(arrowhead) in the AV. (LA: Left atrium; RA: Right atrium; RV: Right ventricle; RVO: Right ventricular outflow tract) (Movie clip 79.16).
there is reason to believe that 2D TTE/Doppler may not pressures due to AS. The increased muscle mass normalizes
provide an accurate assessment of AS severity (Table 79.2). wall stresses by an increase in left ventricular wall thickness
However, it is important to recognize that in patients with relative to the chamber size by operation of Laplace’s law.87
poor acoustic windows or those with very heavily calcified Unfortunately, this compensatory response is maladaptive
valves, which are more commonly seen in the elderly, 3D because the hypertrophied heart may have reduced
TTE images may not be adequate quality to planimeter coronary blood flow per gram of muscle88 and also exhibit
the aortic orifice. Some investigators have reported that an attenuated coronary vasodilator reserve, even in the
this may occur in as many as 20% of patients, although absence of epicardial CAD.89 The hemodynamic stress
in our experience it is possible to measure the AVA by 3D of exercise or tachycardia can elicit a maldistribution
TTE planimetry in a much greater proportion of patients of coronary blood flow and subendocardial ischemia,
with AS. 3D TEE reconstruction or live/real time 3D TEE which can contribute to systolic or diastolic dysfunction
may supplement 3D TTE in patients with poor acoustic of the LV. Pressure overload left ventricular hypertrophy
windows. (LVH) has been shown to increase sensitivity to ischemic
injury, with larger infarcts and higher mortality rates than
in the absence of hypertrophy.90,91 In geriatric population
Ventricular Response to Aortic Stenosis especially in the female, an excessive or inappropriate
Concentric hypertrophy is a compensatory response to degree of hypertrophy has been observed with wall
chronic elevation of afterload and increased intracavitary thickness greater than is necessary to counterbalance
the high intracavitary pressures. This response maintains regurgitation (AR) was seen in 12.2% of TAVR patients in
the systolic wall stress relatively low and ejection fraction the seminal PARTNER (Placement of Aortic Transcatheter
relatively high, but has been associated with high Valves) trial, a significantly higher figure than seen in the
perioperative morbidity and mortality.92–94 surgical group (0.9%).98 AR, when more than just trace,
In the geriatric patient with AS, the hemodynamic has been shown to be a predictor of both in-hospital and
load to the LV is summation of the resistance offered long-term mortality.101 One of the important predictors
by the stenotic AV and the attenuated systemic arterial of paravalvular AR and postprocedure AR is prosthesis–
compliance. Hence, description of the severity of the AS in annular mismatch. Conventionally, the manufacturers’
elderly is rather simplistic if described merely by AVA and recommendations on size selection had been based on
transvalvular gradients as it does not take into consideration 2D echocardiographic measurement of the AV annulus.
the influence of altered systemic arterial compliance and However, the 2D echocardiographic measurement has
systemic vascular resistance. The global LV hemodynamic been shown to significantly underestimate the AV annular
load can be quantified by “valvuloarterial impedance” dimensions.102–104 Furthermore, there is significant intero-
(Zva), which incorporates the degree of valve stenosis and bserver and intraobserver variability in measurement
the systemic arterial compliance.95,96 Zva has been shown because of uncertainty about where in the dense calcium
to be superior to the conventional indices of AS severity in to place the cursor and also the frequent need for guessing
predicting LV dysfunction and patient outcome.73,95 where the leaflet hinge point is. 3D echocardiographic
measurements may mitigate some of these problems105
Transcatheter Aortic Valve but it still underestimates the annular dimension when
compared to multidetector computerized tomographic
Replacement (Figs 79.17A to E)
(MDCT) measurements. MDCT is considered as the
Standard surgical AV replacement in the elderly carries reference standard for measuring the aortic annulus in
a high mortality in the immediate postoperative period, most centers. In the majority of patients, the AV ring is an
but subsequent to this the mortality in the long term oval structure with sagittal diameter being the minimum
approximates that in the younger patients. However, the diameter and the coronal diameter being the maximum
presence of serious comorbidities and other contrain- diameter of the annulus. Therefore, averaging the
dications often precludes surgery in these patients. In minimum and maximum diameter to get a mean diameter
this regard, the advent of TAVR has been a boon for is a pragmatic way of sizing the annulus. However, in one
the elderly and is now considered a standard of care for third of the patients, the true minimum and maximum
patients with severe symptomatic AS who have a high diameter will be at oblique angles and not in the true
risk or contraindications to conventional surgical AV coronal and sagittal plane, respectively, and this leads to
replacement. This is a catheter-based procedure in which an imperfect and imprecise measurement in up to 33% of
a prosthetic valve mounted on a catheter tip is implanted the patients.106 These shortcomings have prompted many
inside the preexisting stenosed AV with angiographic TAVR centers to use a multimodality imaging approach in
and echocardiographic guidance. Based on data from which all patients receive MDCT along with 2D TTE, and
prospective randomized clinical trials, this procedure 2D and 3D TEE. Some of the complications of TAVR in the
has been shown to confer improvement in survival in elderly such as stroke and paravalvular aortic regurgitation
nonoperative candidates when compared to conventional may be reduced with further impending improvements in
medical treatment97 and confers equivalent survival benefit the technique and development of improved valve design.
compared to open heart surgery in high-risk patients.98
During the procedure, accurate measurement of the AV
annulus or ring is pivotal to select the most appropriate
AORTIC ANEURYSM (FIGS 79.18A AND B)
prosthetic valve size to avoid complications such as An aortic aneurysm represents a pathologically dilated
prosthesis migration, paravalvular aortic regurgitation,99 segment of the aorta that has the propensity to expand
or aortic annulus rupture. Some paravalvular regurgitation and rupture. The Olmsted county study estimated the
leading to postoperative aortic insufficiency (AI) is incidence of thoracic aortic aneurysm (TAA) to be 5.9
common post TAVR and is known to occur in 22–40% per 100,000 person-years compared with 350 cases
of patients.100,101 Moderate or severe paravalvular aortic for abdominal aortic aneurysms (AAA).107 However,
A B
C D
Figs 79.17A to E: Transcatheter aortic valve replacement (TAVR)

in an 85-year-old patient. Two-dimensional (2D) and live/real time
three-dimensional (3D) transesophageal echocardiography. (A)
Pre procedure. 3D short-axis view shows a calcified aortic valve
(arrow) with a very small irregular orifice consistent with severe
aortic stenosis; (B) During procedure. Arrow shows a catheter
in the region of the aortic root and valve imaged using the 3D
approach; (C to E) Post procedure. Arrow in C and D points to a
thin aortic valve leaflet seen in both 2D and 3D long-axis views.
The upper arrow in E points to significant paravalvular aortic
regurgitation clearly seen beyond the confines of the prosthetic
elements visualized in the 2D short-axis view. Lower arrow points
to mild valvular regurgitation located within the ring. (AO: Aorta; LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle)
E (Movie clips 79.17A to D).
more contemporaneous estimates in the era of CT and ascending aorta is affected in 50 to 60% of cases, the aortic
echocardiography indicate the incidence to be at least 10 arch in < 10%, and the descending thoracic aorta in 30 to
per 100,000.108–110 The increment is largely due to improved 40%. The mean age of diagnosis is 59–69 years with a male
diagnostics and case ascertainment.111 Of the TAA, the predominance of 2:1 to 4:1.
A B
Figs 79.18A and B: Aortic arch aneurysm with rupture. Live/real time three-dimensional transesophageal echocardiography. (A) The
top arrowhead points to the aneurysm in a 76-year-old male with a bioprosthetic aortic valve, which contains thrombus (T), and the bot-
tom arrowhead denotes the site of rupture of this aneurysm into the mediastinum. Movie clips 79.18A Parts 1 and 2. In Movie clip 79.18A
Part 2, arrowheads in the right upper panel and in the left lower panel point to en face views of the aneurysm rupture site and mouth of
the aneurysm, respectively. Note the presence of spontaneous echo contrast suggestive of low blood flow state in the aneurysm and
aortic arch (ACH); (B) Ascending aortic aneurysm in a 67-year-old male with rupture into the mediastinum. Arrowhead points to a large
rupture visualized en face measuring 1.75 × 2.34 cm, area 3.01 cm2. It was not possible to visualize the rupture site en face by two-
dimensional echocardiography. Movie clip 79.18B.
Source: Reproduced with permission from Joshi D, Bicer E, Donmez C, et al. Incremental value of live/real time three-dimensional
transesophageal echocardiography over the two-dimensional technique in the assessment of aortic aneurysm and dissection. Echocar-
diography. 2012;29:620–30.
The etiology of aneurysms can be degenerative, relat- of TAA in LDS. TEE can image almost the entire thoracic
ed to cystic medial degeneration (CMD), genetically trig- aorta well and is an attractive alternative to computerized
gered, atherosclerotic, inflammatory, traumatic, or my- tomography or MRI. FTAA/D accounts for 20% of TAA
cotic. CMD is a common denominator in many genetically and exhibits autosomal dominance inheritance with
triggered TAA including Marfan syndrome (MFS). In addi- reduced penetrance, variable age of onset, and variable
tion, normal aging is associated with some degree of CMD expression with regards to location of aneurysm112 with the
and this process is accentuated by hypertension. These pedigree showing TAAs in 66%, AAAs in 25%, and cerebral
changes cause progressive weakening of the aortic wall, aneurysms in 8%. Imaging of the aorta in family members
leading to dilation and aneurysm formation. The geneti- often reveals asymptomatic aneurysms, and the incidence
cally triggered TAA can be syndromic with multisystem of aortic disease increases with advancing age. BAV affects
manifestations [MFS, Loeys–Dietz syndrome (LDS), vas- approximately 1% of the population and the aortopathy
cular Ehlers–Danlos syndrome (vEDS), Turner syndrome associated with BAV is one of the most common causes of
(TS)] or nonsyndromic [familial TAA and dissection syn- ascending aortic aneurysm. The TAA associated with BAV
drome (FTAA/D), aortopathy associated with BAV] that often arises in the proximal to mid-ascending aorta, and
manifests with thoracic aortic disease alone. is not a mere passive post-stenotic dilation, but a direct
The TAA in MFS involves the sinuses of Valsalva consequence of abnormalities of the aortic media. It may
with the ascending aorta above the sinotubular junction be present in absence of AS or AR and may occur late after
usually being normal in dimension. TTE provides excellent AVR. Due to its predilection to form an aneurysm in the
imaging of the aortic root and the sinuses of Valsalva and proximal to the mid-ascending aorta, it is imperative that
is therefore an adequate imaging tool for evaluation and the entire ascending aorta be visualized to evaluate for
surveillance of TAA size in MFS. In contrast, in LDS aortic aneurysms above the sinotubular junction. This implies
root, involvement is less common, with the descending and that imaging with TTE may not be optimal enough and
abdominal aorta and aortic branch vessels more frequen- may require evaluation with TEE or CT or MRI. The
tly involved. TTE alone may, therefore, not be sufficient atherosclerotic TAA have predilection for the descending
enough for adequate evaluation as well as surveillance aorta and generally originate just distal to the origin of
the left subclavian artery. Morphologically, they are either been recommended when the ascending aorta is 3.5 cm
fusiform or saccular and may extend into the abdominal or larger or 2.5 cm/m2 or larger.117 Other modifiers that
aorta (thoracoabdominal aneurysm) or coexist with AAA. determine the timing of intervention include the velocity
TEE is excellent for evaluation of the atherosclerotic TAA of aneurysm growth > 0.5 cm/year, coexisting valvular
but may need additional imaging to evaluate the distal disease and indications for concomitant cardiac surgery,
end of the thoracoabdominal aneurysm or evaluate the and body size; surgery is recommended if the maximal
presence of synchronous AAA. cross-sectional area (in cm2) of the ascending aorta or root
Many factors influence the natural history of TAA; divided by the patient’s height in meters exceeds a ratio of
the most robust determinants of likelihood of rupture 10.115 Prophylactic intervention is indicated in descending
or dissection are the underlying causes of TAA, location TAAs with a diameter > 5.5 cm or thoracoabdominal aortic
of TAA, pre-existing diameter of the TAA, and growth aneurysm > 6 cm.115
velocity. Intervention is generally recommended when
the TAA reaches a certain size threshold in an appropriate AORTIC DISSECTION
candidate. In general, prophylactic surgical intervention is
recommended when the ascending TAA reaches 5.5 cm,
(FIGS 79.19 TO 79.22)
5.0 cm in the setting of BAV, 4.5–5 cm in setting of MFS and Although aortic dissection may occur in genetically
FTAA/D, or 4 cm in LDS patients, as they are phenotypically predisposed young adults such as those with MFS, LDS,
more aggressive.113–116 In TS, prophylactic surgery has vEDS, FTAA/D, BAV, or TS, but it is mainly a disorder of the
A B
C D
Figs 79.19A to D
E F
Figs 79.19A to G: Aortic dissection in an elderly patient. Two-
dimensional transthoracic and transesophageal echocardiography.
(A to G) Transthoracic parasternal long-axis (A) and apical five-
chamber (B) views demonstrate irregularly moving linear echoes
(arrows) in the aorta (AO), resembling a wiggling worm typical of
aortic dissection. Thus, in some cases, a definitive diagnosis of
aortic dissection can be made by transthoracic echocardiography
precluding a transesophageal study, which can subsequently be
done in the operating room. This prevents undue delay in taking the
patient for surgery. In this patient, the dissection flap can be clearly
seen protruding into the left ventricular outflow tract in diastole and
this resulted in severe aortic regurgitation. Transesophageal long-
(C) and short-axis (D) views of the aortic root and ascending aorta
in another elderly patient show the dissection flap (arrow) extend-
ing into the right coronary artery (arrowhead). It is also seen in the
vicinity of the origin of left main coronary artery (LMCA) but does
not extend into it. Examination of the descending thoracic aorta in
short- (E and F) and long-axis (G) views show the perfusing lumen
(PL) surrounded by the nonperfusing lumen (NPL, arrow). Movie
clips 79.19E shows the dissection flap mimicking opening and clos-
ing motion of a person’s mouth as if the patient was begging for
help, the so-called “Help sign.” (LA: Left atrium; LV: Left ventricle;
G RV: Rright ventricle). (Movie clips 79.19A to E).
A B
Figs 79.20A and B
C D
E F
G H
Figs 79.20C to H
Figs 79.20A to I: Aortic dissection rupture into the right ventricular

outflow tract. (A to C) Two-dimensional transthoracic echocardiog-
raphy. (A) Parasternal long-axis view. The arrowhead points to the
site of rupture of the false lumen (FL) into the right ventricular out-
flow tract (RVOT); (B) Color Doppler examination. The arrowhead
on the right points to a communication between the true lumen (TL)
and FL. The arrowhead on the left shows flow signals moving from
the FL to the RVOT. Moderate aortic regurgitation (AR) is also dis-
played; (C) Continuous wave spectral Doppler interrogation of the
rupture site showing continuous flow throughout the cardiac cycle;
(D to G) Live/real time three-dimensional transthoracic echocar-
diography; (D) The arrowhead points to the en face view of the
rupture site upon cropping of the data set. It is roughly elliptical in
shape and measured 0.51 cm2 in area (A) by planimetry; (E) QLab
image of the same data set with the orifice (arrow head) planimeter-
ized; (F) The arrowhead points to compression of the main pulmo-
nary artery (PA) by the FL; (G) Extension of the dissection (arrow-
I head) into the left common carotid artery (LCC) is shown; (H and I)
Computed tomography angiogram. (H) Left anterior oblique (LAO)
view demonstrating the communication (arrowhead) between the
elderly with ascending aortic dissection, most prevalent
FL and RVOT; (I) Cut slab volume rendered image in oblique LAO
between 50 and 60 years of age, and descending aortic view demonstrating PA compression by the dilated FL. The arrow
dissection peaking at 60–70 years of age. Although the in figures H and I points to the dissection flap extending into the
classical presentation of the patient to the emergency aortic arch and the brachiocephalic artery (BR). (A: Anterior; AO:
Aaorta; F: Foot; H: Head; L: Left; LA: Left atrium; LV: Left ventri-
room is severe tearing chest pain radiating to the back cle; P: Posterior; PV: Pulmonary valve; R: Right; RA: Right atrium).
along with symptoms of autonomic activation, this (Movie clips 79.20A–I).
typical presentation may be modified by the location of Source: Reproduced with permission from Hansalia S, Nanda NC,
et al. Live/real time three-dimensional transthoracic echocardio-
dissection and coexisting complications including acute
graphic assessment of aortic dissection rupture into right ventricu-
coronary syndrome (ACS). Aortic dissection and ACS can lar outflow tract: a case report and review of literature. Echocardi-
present synchronously as the aortic dissection can cause ography. 2009;26(1):100–6.
impairment to coronary flow due to a variety of reasons:
the dissection flap may mechanically obstruct the orifice of
the left or right coronary artery, the dissection process may because that would further incur delay and catheter
extend along the walls of a coronary artery significantly manipulation in the aorta may aggravate the dissection
narrowing the vessel lumen, subadventitial hematomas if the catheter passes into the nonperfusing lumen. It
(commonly present and alerts the surgeon to the presence is also important to differentiate aortic dissection from
of dissection as soon as he opens the chest wall) may an ACS to avert an immediate disastrous outcome from
compress the coronary arteries, localized pericardial administration of anticoagulants or antiplatelet agents to
effusion either due to heart failure or partial rupture of the the patient. In addition, it is paramount to be aware, as
dissected aorta into the pericardium may also compress mentioned above, that both may coexist and the ACS may
the coronaries, and finally, hypotension resulting from in fact be related to aortic dissection.
dissection in an elderly patient with preexisting significant CT scan is often used to detect or rule out aortic
CAD may precipitate severe myocardial ischemia and/or dissection because of its easy availability in the emergency
myocardial infarction. It is important that the diagnosis room but occasionally gives false-positive results. In
of acute aortic dissection be made emergently and if it many centers, echo machines are also available in the
involves the ascending aorta and/or aortic arch (DeBakey emergency department and the emergency physicians
type I or type II dissection or Stanford type A dissection), are trained to perform transthoracic studies. A linear
urgent surgical intervention is necessary. Any delay in echo moving in an irregular, chaotic manner in the aortic
doing this substantially increases the mortality rate, lumen (“a worm wiggling in the aorta”) is highly specific
which in acute dissection is very high, up to 1–2% per for aortic dissection. However, a transthoracic echo may
hour reported in the first several hours after dissection.118 be negative in many instances and most often one has
This has led to the recommendation that even a coronary to resort to TEE to make the diagnosis. In addition to
arteriogram should not be performed in an elderly patient finding an irregular flap-like echo in the aortic lumen,
A B
Figs 79.21A to C: Aortic dissection. Transesophageal three-

dimensional echocardiographic reconstruction. (A and B) The
descending thoracic aorta was examined using multiple cut sections
and various viewing angles. Both the true (TL) and the false (FL)
lumens are well visualized, and the dissection flap (F) presents as
a sheet-like structure along the aortic length. The communication
(arrows) between the true and false lumens is viewed en face using
a transverse section in C. (H: Mediastinal hematoma that resulted
from rupture of dissection).
Source: Reproduced with permission from Nanda NC, Khatri G,
et al. Three-dimensional echocardiographic assessment of aortic
C dissection. Echocardiography. 1998;15(8):745–54.
A B
Figs 79.22A and B: Aortic dissection. Two-dimensional (A) and live/real time three-dimensional (3D) transesophageal; (B) echocardiog-
raphy. Arrowhead points to a linear echo in ascending aorta (AO) consistent with dissection. However, instrument artifacts may present
as linear echoes in aortic lumen mimicking dissection. Therefore, in cases where a doubt exists as to the origin of the linear echo, it is
best to do a live/real time 3D study. The dissection flap will then appear as a sheet with finite width when viewed en face reflecting split-
ting of the aortic wall by the dissection process. Thus, aortic dissection can be confidently diagnosed or ruled out. (RPA: Right pulmonary
artery) (Movie clip 79.22).
differential color Doppler flow signals in the nonperfusing dissection. However, some dissections present as virtually
and perfusing lumens. and detection of a communication nonmobile linear echoes and distinguishing them from
between the perfusing and nonperfusing lumens by an artifact becomes very difficult. In these instances, 3D
color Doppler further add to the confidence level of the TTE and TEE are invaluable in making a diagnosis with
diagnosis. Visualization of spontaneous echo contrast certainty. Cropping the 3D data set will demonstrate the
signals in the vicinity of the aorta should alert one to the dissection flap as a sheet of tissue that clearly differentiates
presence of dissection rupture and calls for immediate it from the linearly presenting artifactual echo. Basically,
surgery. In these instances, rupture has occurred but has aortic dissection represents “splitting” of the wall of the
been temporarily closed by a plug of fibrin preventing aorta and should, therefore, present as a tissue with finite
exsanguination. width rather than a linear echo seen on 2D imaging, which
Color Doppler is also useful in not only detecting provides only thin slice-like sections of the aorta at any
the presence of aortic regurgitation but also assessing given time.
its severity. Severe aortic regurgitation resulting from Dissection involving only the descending thoracic aorta
the dissection flap interfering with aortic cusp motion (DeBakey type III or Stanford type B) may be diagnosed by
and/or prolapsing into the LVOT can be diagnosed and 2D TTE using the suprasternal approach and by examining
the surgeon informed sparing the patient concomitant the descending thoracic aorta imaged behind the LV/left
AV replacement. On the other hand, severe aortic atrium in the parasternal long-axis view. Dissection may
regurgitation due to a dilated or distorted aorta in an also be visualized in the proximal abdominal aorta using
elderly patient or due to thickened or retracted valve the subcostal approach. The diagnosis is made by noting
cusps found on the echocardiogram would require AV echocardiographic findings similar to those mentioned
replacement. Over all, TTE has a sensitivity of 78–100% above for dissection involving the ascending aorta/aortic
for type A aortic dissection, but only 31–55% in type B arch. Unlike ascending aorta/aortic arch dissection, these
dissection; therefore, a negative TTE does not exclude patients are generally managed conservatively unless
acute aortic dissection. However, TEE is highly accurate there is evidence of aortic enlargement and other signs of
for the evaluation and diagnosis of acute aortic dissection progression or there is compromise of blood supply to a
and boasts a sensitivity of >98% and specificity of 94–97%. visceral organ or spinal cord with impending paraplegia.
During a transesophageal study, it is important to examine In 15–20% of patients, aortic dissection does not
the proximal coronaries for obstruction produced by the communicate with the aortic lumen forming an IMH. This
dissection flap impinging on the orifices or narrowing presents as a thickened aortic wall and is considered a
produced by extension of dissection along their walls. precursor of aortic dissection. This is especially common
In the older patient, coronary lumen narrowing and in the hypertensive and older patient and most commonly
obstruction produced by atherosclerotic plaques can also involves the ascending aorta. Echocardiography is useful
be visualized by echocardiography and this is important to follow its course over time and any increase in size or
information for the surgeon, especially if a preoperative progression to dissection with communication with the
coronary angiogram was not performed. Depending on vessel lumen warrants consideration for surgery. IMH
the clinical status of the patient, the surgeon may consider has been found to progress to aortic dissection or rupture
placing bypass grafts. The perfusing lumen (which may in up to 45%, regress completely in 34%, or evolve into
occasionally be the false lumen) can be differentiated pseudoaneurysm in 24%.119 Small, localized IMH are
from the nonperfusing lumen by its expansion during generally conservatively managed.
systole. Also, color Doppler flow signals are usually more
prominent in the perfusing lumen as compared to the LEFT VENTRICULAR MASS,
nonperfusing lumen. Artifacts are not uncommonly seen
in the aorta during transesophageal examination and have
DIMENSIONS, AND FUNCTION
been mistaken for dissection in inexperienced hands with LV mass increased monotonically with age in the whole
near-disastrous results. These artifacts are linear in nature Framingham study cohort, but not in a subgroup of normal
and their motion tends to be parallel to the movement of individuals.120 Other studies have also corroborated
the aortic walls. Also, they may extend beyond the aortic that after excluding the influence of coexisting disease,
wall, which easily identifies them as artifacts and not LV hypertrophy is not an inevitable consequence of
aging.121,122 However, LV wall remodels with normal aging and can provide a measurement of the rate of change of
primarily with an increase in relative wall thickness (ratio longitudinal dimension and volume.134 The mitral annulus
of wall thickness to chamber radius) but with little or no velocity profile by TDI during diastole consists of early
increase in overall LV mass.123 This concentric remodeling diastolic movement (E′) that commences simultaneously
parallels the age-related stiffening of the arterial tree, with the onset of mitral inflow and late diastolic velocity
while hypertension induces concentric hypertrophy of the (A′) that corresponds to the late transmitral flow A. The
myocardium with an increase in LV mass. mitral annular TDI velocities are relatively less preload-
Aging induces significant alteration in LVOT geom- sensitive and fall progressively with increasing age.134 In
etry124 primarily due to reduction in the angle between addition, significant decrement in the ratio of early to late
the aorta and the interventricular septum. The age-related myocardial velocities, E′/A′ with increasing age is observed
dilation and lengthening of the aorta may push the sep- mimicking the pattern of normal mitral inflow. However,
tum downward and kink its upper portion, accentuating although reversal of the transmitral E/A ratio occurs in the
the septoaortic angle. This shifts the position of the inter- 60s, reversal of the E′ to A′ ratio occurs in the 40s.134,135
ventricular septum relative to the chest wall and leads to
systematic errors in echocardiographic M-mode measure- ECHOCARDIOGRAPHY IN STROKE
ments across the LV. This anatomical alteration also induc- PATIENTS: ASSESSMENT OF
es a “septal bulge” in >10% of the geriatric population but
CORONARY STENOSIS
is not associated with higher LVOT velocity or increased
LV mass index. LV systolic function remains relatively well (FIGS 79.23 TO 79.25; ALSO SEE
preserved and there are no significant alterations in LV FIG. 78.13 IN THE CHAPTER 78)
ejection fraction with normal aging.125
Ischemic stroke constitutes 70–80% of all strokes and
The Doppler transmitral inflow velocities are used as
is caused by embolic or thrombotic occlusions of the
surrogate indices for echocardiographic assessment of cerebral vessels and accounts for major morbidity and
ventricular diastolic function. Both in the Framingham mortality in the elderly.136 Embolic occlusions can be of
study126 and the Cardiovascular Health study,127 age was arterial or cardiac origin, with 15–30% attributable to
the predominant determinant of Doppler indices of LV cardioembolism (CE). Identification of the specific etiology
diastolic function in normal subjects with decrease in is crucial for risk stratification and in order to tailor the most
peak early velocity (E-wave) and increase in peak late optimal preventive strategy. CE strokes portend a poor
velocity (A-wave) with increasing age. These indices prognosis with increased short- and long-term recurrence,
change gradually and progressively128,129 with decrement higher in-hospital mortality, and a higher index of fatal
in E-wave velocity of approximately 50%126,130 together recurrence versus other causes of stroke.136–138 There is
with a 40% increment of A-wave velocity between 30 and no clear consensus on the indication and the optimal
70 years of age.131 The ratio of the peak E and A is shown echocardiographic approach in the cardiac evaluation
to range from a mean of 2.08 ± 0.55 for subjects in their of ischemic stroke. The European Stroke Organization
third decade to 0.84 ± 0.29 for those in their eighth decade. guidelines recommend the use of echocardiography in
A peak velocity E/A ratio < 1 is abnormal in subjects aged selected patients, while the American Stroke Association
< 40 years, but occurs in most subjects aged ≥ 70 years.126 guidelines do not make any clear recommendation on
These indices are accompanied by a prolongation of the its use..139,140 In real life practice, evaluation for cardiac
deceleration time of the E-wave,132 and an increase in left source for embolism is one of the most common requests
atrial size127 and are present in more than 85% of healthy for the performance of a TEE (>25% of all studies at most
people over the age of 70 years.129 However, mitral inflow institutions).141 TEE identifies possible sources of CE
velocities profiles are affected by loading conditions,133 left including the presence of a patent foramen ovale in >50%
atrial pressure,131 are reflective of flow patterns, and are of patients without clinically known heart disease, in
not synonymous with function. It is therefore desirable comparison, the diagnostic yield with TTE with agitated
to have additional variables to complement mitral saline-injection is only 25%, which drops to 10% without
inflow Doppler velocity in evaluating diastolic function. saline-injection.142 However, the diagnostic superiority
Pulsed wave tissue Doppler imaging (TDI) measures low of TEE does not necessarily translate into altered
amplitude myocardial velocities with a high sampling rate therapeutic decisions. While the diagnosis of thrombus,
A B
C D
Figs 79.23A to D: Ostial left main (LM) and mid-left anterior descending coronary artery (LAD) stenosis in a 72-year-old white male.
Two-dimensional transesophageal echocardiography. (A) Shows an area of flow disturbance in the ostium of the LM (1), preceded by
prominent flow convergence; (B) Color Doppler-guided continuous wave Doppler interrogation shows a very high diastolic velocity of
2.0 m/s (arrows) indicative of severe LM stenosis. Note also the high systolic flow velocity; (C) Demonstrates an area of aliased flow in
the region of mid-LAD (2) with a high diastolic flow velocity indicating significant stenosis; (D) Coronary angiogram showing 95% ostial
LM stenosis and 50% mid-LAD stenosis (arrows). (Ao: Aorta; Cx: Left circumflex coronary artery; LA: Left atrium; PA: Pulmonary artery)
(Movie clip 79.23).
Source: Reproduced with permission from Thakur A, Voros A, Nanda NC, et al. Transesophageal echocardiographic diagnosis of proxi-
mal coronary artery stenosis in patients with ischemic stroke. Echocardiography. 1999;16(2):159–66.
infective endocarditis, and cardiac tumors will have major increase the probability of finding the source of CE with
therapeutic implications, entities like patent foramen TTE alone and may obviate the need for TEE.122 In a study
ovale, atrial septal aneurysm, complex aortic atheroma, of 441 unselected ischemic stroke patients, TEE detected a
and spontaneous echo contrast may not always change source of CE in 56% of the patients. However, in the cohort
patient management despite their robust epidemiological of patients in sinus rhythm and without apparent heart
association with stroke recurrence. Although age has been disease, TEE altered the therapeutic strategy in only 8%
used to select the echocardiographic approach in stroke of patients.144 In another study, the therapeutic impact of
patients, several studies have concluded that it should not TEE was highest only in the cohort of patients in whom
be used as an exclusion criterion in the selection of patients the cause of ischemic stroke remained cryptogenic despite
for TEE.143 Presence of preexisting heart disease may routine diagnostics.145
A B
C D
E F
Figs 79.24A to F
a first coronary event. The American Heart Association/

American Stroke Association recommends that patients
with stroke/TIA who have Framingham Risk Score-
predicted 10-year CHD risk ≥ 20% should be considered
for noninvasive testing for asymptomatic CAD.149 TEE
evaluation of stroke patients provides a unique opportunity
in which the presence of coexisting disease of a major
epicardial artery can be evaluated.152 Direct visualization
of the origins of the left and right coronary arteries is
possible in the transverse imaging plane, basal short-axis
view, just above AV leaflets. With anteflexion and slight
leftward tilting, the left main ostium and the entire length
of the left main coronary artery can be imaged between
G the 1 and 2 o’clock positions. Continued tracking allows
Figs 79.24A to G: Detection of left main (LM), mid-left anterior visualization until the left anterior descending (LAD) and
descending coronary artery (LAD), and left internal carotid artery left circumflex coronary arteries bifurcate. Further inferior
stenosis and demonstration of atherosclerotic plaque in the left tilting or slight probe angulation allows longer segments of
subclavian artery in a 59-year-old black female. Two-dimensional
the LAD to be visualized. The ostium of the right coronary
transesophageal echocardiography. (A) The black arrowheads
demonstrate prominent atherosclerotic plaques in the LM, pro- artery (RCA) can be imaged in the basal short-axis,
ducing 90% stenosis; (B) The arrow shows flow turbulence corre- transverse image plane, and interrogating between the 6
sponding to the stenosis seen in A; (C) Color Doppler-guided con- and 7 o’clock positions.153
tinuous wave Doppler (arrow) demonstrates a very high diastolic Coronary stenosis can be diagnosed by Doppler
flow velocity of 3 m/s consistent with severe stenosis. The systolic
flow velocity is also high at 2 m/s; (D) The lower arrowheads point interrogation of the diastolic flow in the coronary arteries.
to a prominent shadowing effect produced by the heavily calcified The TEE pulsed wave Doppler waveform in the normal
plaque in the LM viewed in short axis (top arrowhead); (E) Color LAD generally consists of a peak velocity of 40 ± 20 cm/s,154
Doppler-guided pulsed wave Doppler interrogation of the mid-LAD which is fairly consistent with normal maximal diastolic
(arrow) demonstrates a high peak diastolic flow velocity exceeding
1 m/s, indicating significant stenosis; (F) Withdrawal of the probe
velocity of 49 ± 20 cm/s and 37 ± 12 cm/s in the LAD and
into the upper esophagus and laryngopharynx demonstrates RCA, respectively.155 In patients with significant coronary
marked narrowing of the proximal left internal carotid artery (LICA, stenosis, pulsed wave Doppler velocities are significantly
arrow), indicative of severe stenosis; (G) The arrow shows a large increased to >100 cm/s, and the color-flow pattern changes
soft plaque occupying 50% of the proximal left subclavian artery
(LSCA) viewed in an oblique axis. (Ao: Aorta; Cx: Left circumflex
from a low-velocity, laminar pattern to the characteristic
coronary artery; LA: Left atrium; LCC: Left common carotid artery; mosaic color flow pattern seen with turbulent flow and
LEC: Left external carotid artery; LV: Left ventricle; PA: Pulmonary higher velocities that exceed the Nyquist limit. However,
artery; RVO: Right ventricular outflow tract). patients with elevated cardiac output, moderate to
Source: Reproduced with permission from Thakur A, Voros A,
severe aortic regurgitation, or hypertrophic obstructive
Nanda NC, et al. Transesophageal echocardiographic diagno-
sis of proximal coronary artery stenosis in patients with ischemic cardiomyopathy normally have augmented coronary
stroke. Echocardiography. 1999;16(2):159–66. artery velocities exceeding 100 cm/s.156,157 Conversely,
extremely severe luminal narrowing (subtotal or high-
grade stenosis) may limit coronary flow so severely that
The prevalence of asymptomatic CAD in patients the velocity within the vessel is actually diminished.
with cerebrovascular disease is very high146–148 and
predominantly accounts for the morbidity and mortality
in patients with stroke or a transient ischemic attack MITRAL ANNULAR CALCIFICATION
(TIA).149 Although recurrent strokes occur more frequently (FIG. 79.7; ALSO SEE FIG. 70.58 IN
than cardiac events over the long term after stroke,
THE CHAPTER 70)
cardiac events still account for a greater proportionate
mortality.150,151 It may, therefore, be pertinent to identify Mitral annular calcification (MAC) is a chronic dege-
asymptomatic coronary artery stenosis that might benefit nerative process of the mitral valve ring that was first
from specific additional therapeutic measures to prevent described in 1908 by Bonninger as associated with
A B
C D
Figs 79.25A to E: Anomalous coronary artery in a 69-year-old
black female. Two-dimensional transesophageal echocardiogra-
phy. The patient presented with pulmonary edema and stroke with
left sided residual weakness. (A and B) Show both, left main (right
arrowhead) and right coronary arteries (left arrowhead) with color
flow in B, arising from a common ostium in the right sinus with the
left main coursing between aorta (AO) and right ventricular outflow
tract (RVO); (C) The arrowhead points to intramyocardial course of
the left anterior descending coronary artery (arrowhead) within the
ventricular septum; (D) Demonstrates the course of right coronary
artery (arrowhead) in the right atrioventricular groove; (E) Coronary
angiogram showing the common origin of the left main (LM) and
right coronary (RCA) arteries. The patient underwent percutaneous
transluminal coronary angioplasty with stent placement in mid RCA
because of 90% stenosis. (Movie clip 79.25) (LVO: Left ventricular
outflow tract; MV: Mitral valve; RV: Right ventricle).
Source: Reproduced with permission from Nanda NC, Bhambore
M, Jindal A, et al. Transesophageal Three-Dimensional Echocar-
diographic Assessment of Anomalous Coronary Arteries. Echocar-
E diography. 2000;17(1):53–60.
complete heart block.158 In the cardiovascular health simulating tumor necrosis and may show some mobility.
study, MAC was found in 42% of patients with a mean age This is essentially a benign condition and does not need
of 76 years.159 In the Atherosclerotic Risk in Communities any surgical intervention. However, some of these patients
(ARIC) study,160 MAC was much less common in younger have been referred for surgical resection in the mistaken
subjects with a mean age of 59 and a prevalence of 4.6% in belief that the lesion is an annular tumor. We have found
females and 5.6% in males. live/real time 3D TTE useful in these cases to make a more
The calcific process in MAC initiates at the attachment confident diagnosis of caseous calcification.162 Careful
points of the annulus and the calcification may extend to cropping of the 3D data set shows the extent and severity
involve the base and body of both mitral leaflets. Mitral of echolucencies and in one particular elderly patient
regurgitation (MR) is commonly associated with MAC, studied by us intraoperatively, a typical telltale tooth paste–
with a reported incidence of up to 63%.161 The putative like appearance was noted when the mass was sectioned
mechanism for MR is accentuated rigidity of the annulus. and viewed en face on the 3D data set. This finding was
Typically, MR is mild to moderate by color Doppler and confirmed at surgery.
not severe enough to require surgical intervention. MAC is associated with traditional CV risk factors
However, severe annular calcification may result in and calcific aortic disease, coronary atherosclerosis
poor penetration of the ultrasonic beam resulting in and chronic kidney disease, and is now considered the
underestimation of the severity of mitral regurgitation. In surrogate marker of atherosclerosis. Several trials have
these cases, it is important to perform a 3D transthoracic evaluated the association between MAC and CV outcomes.
study to assess the regurgitant vena contracta, which Data from the Framingham study suggested that each
can provide a more quantitative estimate of the severity 1-mm increment in MAC increased the composite risk of
of mitral regurgitation. Transesophageal echo may also CVD, CVD death, and all-cause death by approximately
need to be done in difficult cases. Typically, leaflet tips 10%.163 Similarly, MAC was found to incur a significant risk
are spared and this distinguishes it from rheumatic mitral for coronary events in the ARIC study160 and increased CV
disease, where calcification commonly involves the tips morbidity, CV mortality, and all-cause mortality of patients
of mitral leaflets and commissures producing a typical with atrial fibrillation in the Belgrade Atrial Fibrillation
“hockey stick” appearance. In the elderly, calcification Study.164
may occur in the chordae and papillary muscles but, unlike
rheumatic disease, does not produce chordal shortening PROSTHETIC VALVES
and fusion. MAC in the elderly may occasionally become
severe enough to produce significant mitral stenosis
(FIGS 79.26 AND 79.27)
requiring mitral valve replacement. Calcification may Prosthetic valves are common in geriatric population. A
also involve the aortic annulus and rarely the tricuspid suggested estimate of the prevalence of valve prostheses
annulus where fatty deposits are more common. The latter ranged from 0.2 per 1,000 in those aged 44 and under
may mimic a tumor mass. Occasionally, calcification may to 5.3 per 1,000 in those 75 years of age and older.165 3D
involve the whole cardiac skeleton as well as the proximal TTE has been shown to be superior to 2D TTE in the
ventricular walls in the elderly, resulting in various types evaluation of prosthetic valves, especially the mechanical
of heart block and other conduction abnormalities. 2D prostheses, since it allows for the visualization of both
TTE represents the best noninvasive technique to not only leaflets simultaneously, which increases the confidence
diagnose but also assess the extent and severity of mitral in excluding significant abnormalities.166 Although the
annulus calcification as well as calcification affecting current guidelines continue to be based on the unreliable
other areas of the heart. The diagnosis is made by noting Doppler-derived pressure gradients for the assessment
the presence of highly echogenic areas in and surrounding of prosthetic valve dysfunction, 3D TTE is emerging as a
the mitral annulus best seen in the parasternal and apical more robust tool.167,168
planes. A short-axis view at the level of the mitral valve may In conclusion, echo/Doppler techniques represent
show crescent-shaped calcification posteriorly similar to the most useful and most cost-effective noninvasive
fluoroscopy and chest X-ray. One particular abnormality modalities in the assessment of CV disease entities in the
one has to be particularly aware of in the elderly is caseous geriatric patient. In addition, these techniques are also
calcification. With the passage of time, a severely calcified useful in monitoring various structural and physiological
area of the mitral annulus may undergo liquefaction changes that occur in the CV system with aging.
A B
Figs 79.26A to C: St. Jude mitral prosthesis in a 70-year-old female.

(A) Arrow points to a large thrombus on the atrial aspect of mitral
valve replacement (MVR); (B) Arrows point to two thrombi on the
ventricular aspect; (C) Arrow points to a thrombus in the left atrial
appendage (LAA; Movie clips 79.26 Parts 1 to 3). The arrowheads
in Part 1 show thrombi on the atrial aspect of the prosthesis. The
arrowhead in Part 2 denotes an irregular thrombus with some
mobility in the left atrial appendage. Part 3 of the movie clip shows
virtually no motion of the prosthetic valve, only ring motion. (AO:
aorta; LA: Left atrium; LV: Left ventricle; R: Reverberations from
MVR; RV: Right ventricle).
Source: Reproduced with permission from Singh P, Inamdar V,
Hage FG, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in evaluation of prosthetic valve
C function. Echocardiography. 2009;26:1236–49.
Fig. 79.27: Tissue mitral prosthesis in a 85-year-old female patient.

Arrow points to a tear in one of the leaflets of mitral valve replace-
ment (MVR) that is prolapsing into left atrium (LA; Movie clips 79.27
Parts 1 to 3). (AV: Aortic valve). Other abbreviations as in previous
figure.
Source: Reproduced with permission from Singh P, Inamdar V,
Hage FG, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in evaluation of prosthetic valve
function. Echocardiography. 2009;26:1236–49.
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infarction and vascular death after transient ischemic evaluation of prosthetic valve function. Echocardiography.
attack and ischemic stroke: a systematic review and meta- 2009;26(10):1236–49.
analysis. Stroke. 2005;36(12):2748–55. 167. Hage FG, Nanda NC; American Society of Echocardio-
152. Sorrell VL, Nanda NC. Transesophageal echocardiographic graphy’s Guidelines and Standards Committee; American
evaluation of coronary arteries for stenosis in the elderly College of Cardiology; American Heart Association;
patient. Am J Geriatr Cardiol. 2001;10(1):30–41, 49. European Association of Echocardiography; Japanese
153. Samdarshi TE, Nanda NC, Gatewood RP Jr, et al. Usefulness Society of Echocardiography; Canadian Society of Echo-
and limitations of transesophageal echocardiography in cardiography. Guidelines for the evaluation of prosthetic
the assessment of proximal coronary artery stenosis. J Am valves with echocardiography and Doppler ultrasound:
Coll Cardiol. 1992;19(3):572–80. value and limitations. Echocardiography. 2010;27(1):91–3.
154. Yamagishi M, Miyatake K, Beppu S, et al. Assessment of 168. Zoghbi WA, Chambers JB, Dumesnil JG, et al.
coronary blood flow by transesophageal two-dimensional American Society of Echocardiography’s Guidelines
pulsed Doppler echocardiography. Am J Cardiol. 1988;62 and Standards Committee; Task Force on Prosthetic
(9):641–4. Valves; American College of Cardiology Cardiovascular
155. Ofili EO, Labovitz AJ, Kern MJ. Coronary flow velocity Imaging Committee; Cardiac Imaging Committee of
dynamics in normal and diseased arteries. Am J Cardiol. the American Heart Association; European Association
1993;71(14):3D–9D. of Echocardiography; European Society of Cardiology;
156. Fusejima K. Noninvasive measurement of coronary artery Japanese Society of Echocardiography; Canadian Society
blood flow using combined two-dimensional and Doppler of Echocardiography; American College of Cardiology
echocardiography. J Am Coll Cardiol. 1987;10(5):1024–31. Foundation; American Heart Association; European
157. Kisanuki A, Murayama T, Matsushita R, et al. Trans- Association of Echocardiography; European Society
esophageal Doppler echocardiographic assessment of left of Cardiology; Japanese Society of Echocardiography;
coronary blood flow velocity in chronic aortic regurgita- Canadian Society of Echocardiography. Recommendations
tion. Am Heart J. 1996;131(1):101–6. for evaluation of prosthetic valves with echocardiography
and doppler ultrasound: a report From the American registered branch of the European Society of Cardiology,
Society of Echocardiography’s Guidelines and Standards the Japanese Society of Echocardiography, and Canadian
Committee and the Task Force on Prosthetic Valves, Society of Echocardiography. J Am Soc Echocardiogr.
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tered branch of the European Society of Cardiology, the 113(14):1738–44.
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Association, European Association of Echocardiography, a Philadelphia, PA: Lea and Febiger, Inc; 1989:178–90.
CHAPTER 80
How to do Echo for the
Electrophysiologist
Chittur A Sivaram
Snapshot
Echocardiography in Supraventricular Tachycardia
¾¾ Inferior Vena Cava
¾¾
Left Atrium
¾¾ Echocardiography in Ventricular Tachycardia
¾¾
Atrial Septum
¾¾ Echocardiography in Cardiac Implantable Electronic
¾¾
Pulmonary Veins
¾¾ Devices
INTRODUCTION Discussion of ICE will not be included in this chapter since

ICE is by and large limited to the EP laboratory and performed
The sub-subspecialty of cardiac electrophysiology (EP) has
by the electrophysiologist during EP procedures. The reader
made tremendous strides and remarkable progress in the
is also encouraged to cross-reference to other chapters in the
last three decades. Several major therapeutic advances in
book with overlapping information.
EP techniques have resulted in new treatment options,
capable of providing favorable clinical outcomes in patients
with recurrent arrhythmias. Such innovative therapies ECHOCARDIOGRAPHY IN SUPRA
in EP include ablation treatment of atrioventricular (AV) VENTRICULAR TACHYCARDIA
nodal reentrant tachycardia, accessory pathways, and atrial
fibrillation (AF) as well as device therapy for primary and Often the typical patient with supraventricular tachycardia
secondary prevention in ventricular tachyarrhythmias. (SVT) has a structurally normal heart and consequently,
Echocardiography is frequently performed in a completely normal echocardiogram. This is particularly
rhythm disorders for the initial assessment as well as true in AV nodal re-entrant tachycardia (AVNRT).
during follow-up. The appropriate use of transthoracic However, several aspects of TTE have special relevance
echocardiogram (TTE), transesophageal echocardiogram to the preablation assessment in SVT. The preablation
(TEE), and intracardiac echocardiography (ICE) is
TTE should carefully document chamber dimensions
an important responsibility of cardiologists involved
including left atrial (LA) size and volume. It will be helpful
in the ordering, performance, and interpretation of
to the electrophysiologist to have the dimensions of
echocardiographic modalities.1 The following description
will attempt at delineating steps for maximizing the yield coronary sinus (CS) to facilitate catheters placement in
of echocardiography in patients with rhythm disorders the CS during ablation. Dimension of CS may be obtained
through a focused approach to echo imaging relevant to from the apical four-chamber view of TTE with posterior
the EP diagnosis under consideration. This chapter will angulation of the transducer or with TEE in the lower
be predominantly centered on the use of TTE and TEE. midesophageal location.
Fig. 80.1: Parasternal long-axis view of transthoracic echocar- Fig. 80.2: Apical four-chamber view of transthoracic echocardio-
diogram (TTE) showing dilated coronary sinus (CS) in the left gram (TTE) with posterior angulation of transducer demonstrates
AV groove region (arrow). The descending thoracic aorta is seen the dilated coronary sinus (CS).
outside the pericardial layer while CS is intrapericardial. Dilated
CS can be the result of raised right heart pressures or persistent
superior vena cava (SVC) drainage into CS.
CS imaging during TEE is done by advancing the TEE Two important anatomical landmarks in the CS—
probe slightly from the midesophageal four-chamber the Thebesian valve (located at the junction of CS and
view. This view will demonstrate the ostium of CS, CS–RA right atrium) and the valve of Vieussens (located more
junction as well the terminal part of the CS. A slight further proximally within the CS)—are visible during TEE
advancement of the TEE probe can often reveal the middle (Fig. 80.4). The valve of Vieussens frequently causes difficulty
cardiac vein as it drains into the CS. Rarely, ablation of in catheter advancement within CS,2 and as such might be
accessory pathways in preexcitation is performed in the helpful information in the preablation assessment.
middle cardiac vein and assessment of the middle cardiac Imaging in patients with preexcitation requires
vein becomes highly relevant to the electrophysiologist. additional attention to features of Ebstein’s anomaly due
Cannulation of CS is routinely performed in all patients to its association with posteroseptal accessory pathways.
Careful demonstration of attachment of the septal leaflet of
during cardiac ablation. As such, the dimension of CS is an
tricuspid valve relative to anterior mitral leaflet is indicated
important piece of information to the electrophysiologist.
in all patients with pre-excitation. An apparent apical
Normal size of the CS is approximately 6 mm.
displacement of tricuspid valve leaflet insertion relative to
Dilatation of the CS should raise the suspicion of
mitral leaflet insertion in excess of 8 mm/m2 Body surface
persistent left superior vena cava (PLSVC) drainage to
area (BSA) is consistent with Ebstein’s anomaly (Figs 80.5
CS. The additional volume of blood drained from the
and 80.6). The posterior leaflet of the tricuspid valve needs
left upper limb through PLSVC causes an increase in CS special attention and needs to be carefully looked at to
size. CS is easily imaged by TTE in the parasternal long- exclude Ebstein’s anomaly; this can be done using TTE in
axis view as well as the apical four-chamber views with the parasternal right atrium–right ventricle (RA–RV) view
posterior angulation of the probe (Figs 80.1 and 80.2). with additional posterior angulation of the transducer or
Differentiation from descending thoracic aorta is aided by using TEE in the transgastric long-axis RA–RV view.
the fact the CS is within the pericardium, while descending A rare morphological association between pre-
aorta is extrapericardial. Saline contrast injection from excitation and diverticulae of CS has been described
the left arm would confirm the diagnosis further since (Fig. 80.7). While cardiac CT and contrast angiography of
contrast appears in the CS prior to appearance in the right CS are superior to echocardiography in the detection of
heart chambers (Fig. 80.3). The approach to EP procedures CS diverticulae, occasionally TTE or TEE might provide
and device implantation from left subclavian route is clues toward the diagnosis in the preablation patient with
significantly influenced by presence of PLSVC. pre-excitation.
Chapter 80: How to do Echo for the Electrophysiologist 1959
Fig. 80.3: Apical four-chamber view of transthoracic echocardi- Fig. 80.4: Thebesian valve seen with transesophageal echo
ogram (TTE). Saline contrast injection from the left arm shows cardiogram (TEE) at the junction of coronary sinus (CS) to right
opacification of coronary sinus (CS) and right heart chambers. The atrium.
appearance of contrast in CS prior to right heart chambers is diag-
nostic of persistent left superior vena cava (PLSVC) to CS.
Fig. 80.5: Parasternal long-axis view transthoracic echocardio- Fig. 80.6: Apical four-chamber view of the same patient as
gram (TTE), showing dilated right heart chambers in a patient with before. Marked apparent apical displacement of the septal and
Ebstein’s anomaly. The anterior right heart was composed of right anterior leaflets of tricuspid valve is seen, resulting in a severely
atrium (RA) with its atrialized portion of right ventricle (RV). dilated right atrium (RA); there is a large atrialized part of the right
ventricle (RV).
Scanning Tips leaflets brought to focus by changing the degree of

posterior angulation of transducer.
1. TTE is usually completely normal in SVT. 3. Scan carefully to look for CS diverticulae, a rare
2. Look for evidence of Ebstein’s anomaly; need to morphological association in pre-excitation.
demonstrate septal and posterior leaflet insertion to 4. In presence of dilated CS, perform left arm saline
the annulus; RA–RV view from parasternal window contrast injection to exclude persistent left SVC
should be used to delineate septal and posterior drainage to CS.
Presence of spontaneous echo contrast (SEC; “smoke”)

in left atrial appendage (LAA) has been well recognized
as a precursor for development of LAA thrombus and
embolism.4 SEC can be distinguished from high gain
artifact based on the swirling appearance seen only in
SEC. LAA thrombus is often present in patients with AF
and its confirmation is facilitated by demonstration of the
mass abnormality in orthogonal views. Prominent ridges
in the LAA (pectinate lines) should not be confused with
LAA thrombus.
Careful Doppler interrogation of LAA during TEE for
assessing emptying velocities is required as part of the
assessment of LAA function. A pulsed Doppler sample
volume should be placed close to the ostium of LAA.
Normal LAA Doppler signal is quadriphasic, with an
Fig. 80.7: Coronary sinus contrast angiography showing pre
sence of a diverticulum in pre-excitation (photograph courtesy of
emptying and filling signal seen in mid-diastolic and late
Dr Sunny Po, University of Oklahoma). diastole. The mid-diastolic signals frequently have small
velocity and might not be easily apparent. Normal LAA
emptying velocities are above 0.4 m/s. Reduced emptying
LEFT ATRIUM velocities are seen in patients with AF, atrial stunning, and
in low cardiac output states.
In patients with arrhythmias, imaging of the left
An under-recognized application of LAA Doppler is the
atrium provides very valuable information to the
fact that it serves as a surrogate for atrial contraction and
electrophysiologist, particularly if ablation therapies are
being considered. Dilatation of left atrium is frequently atrial activity, and thus could help in analysis of rhythm. In
seen in patients with atrial arrhythmias such as AF, AF and A Flutter, LAA Doppler signal shows characteristic
atrial flutter, and atrial tachycardia. Careful assessment emptying and filling signals at a rapid rate. The rate and
of LA size is critical. Traditionally, the anteroposterior frequency (cycle length) of atrial emptying signal can be
dimension of LA in systole measured using M-mode measured from the LAA Doppler, thus providing clues to
echocardiography from the parasternal long-axis view of rhythm analysis.
TTE is the standard method for reporting LA size. However, The nongeometric shape of LAA poses unique
in many patients there is an obvious discordance between challen ges to the echocardiographer in performing a
LA size measured with M-mode and LA dimension in the comprehensive scanning that evaluates the entire LAA for
superior-inferior axis seen in the apical four-chamber thrombotic masses (Fig. 80.8). To overcome this challenge,
view of TTE. Recent studies have demonstrated a strong at least two orthogonal views of LAA are required to ensure
correlation of both LA volume and LA volume index with adequate visualization. This can be achieved by sequential
cardiovascular outcomes.3,4 Most of the commercially dialing up of the scan plane angle or by preset simultaneous
available echocardiography machines have measurement orthogonal views offered in some of the newer equipment
packages that permit calculation of LA volume using the (Figs 80.9A and B).
Simpson’s biplane method during TTE. Care should be Diagnostic accuracy of combined two-dimensional
taken to obtain stop frame images in systole that clearly (2D TTE) and three-dimensional TTE (3D TTE) when
demonstrates the LA outlines in the apical four-chamber compared with TEE in experienced hands has been
and two-chamber views, as well as exclusion of LA excellent. The differentiation of pectinate lines in LAA from
appendage and pulmonary veins (PVs) from the trace thrombus is also aided by 3D TTE (Figs 80.10A to D).5,6
contours. LAA exclusion is recommended in conjunction
In patients in atrial arrhythmias [AF, A flutter and with mitral valve surgery as well as surgical ablation
atrial tachycardias], a significant risk of thromboembolic of AF for maximal protection against thromboembolic
complications exists. Several findings on TEE correlated to risk in both valvular and nonvalvular AF. Exclusion of
increased thromboembolic events have been described. LAA by clips or suturing offers lesser protection against
Fig. 80.8: Transesophageal echocardiogram (TEE) showing a

thrombus (arrow) in the left atrial appendage (LAA) in a patient with
atrial fibrillation.
A B
Figs 80.9A and B: (A) Transesophageal echocardiogram (TEE) showing left atrial appendage (LAA; midesophageal probe position,
scan plane angle 54°). Left upper pulmonary vein is seen adjacent to LAA, and it appears that a good demonstration of LAA has been
obtained; (B) With additional scan plane angle without any change in probe position, a large additional segment of LAA is now visual-
ized. This underscores the importance of careful scanning of LAA with different scan plane angles to assess the complex shape of LAA.
thromboembolism compared to excision of LAA. Two In patients with AF and A Flutter, worsening of
well-known complications after LAA exclusion are pre-existing SEC might be seen immediately after
recanalization of LAA and persistence of a LAA remnant. cardioversion. This is caused by atrial stunning, which
The presence of LAA remnant is accompanied by a greater results in a reduced atrial transport function even after
risk of thromboembolism compared to LAA excision.7 sinus rhythm has been re-established. Atrial stunning has
Recanalized LAA often is associated with presence of an been known to persist for several weeks postcardioversion.
emptying and a filling signal seen with color flow Doppler
as well as pulsed Doppler (Figs 80.11A and B). Scanning Tips
Rarely other abnormalities of the LAA might be 1. Obtain M-mode measurements of LA size.
present, for example, nonobstructive valves, masses such 2. Obtain LA volumes from apical four- and two-
as lipoma. chamber views.
A B
C D
Figs 80.10A to D: (A) Two-dimensional transesophageal echocardiogram. Arrowhead points to an echodense mass with-
in the left atrial appendage (LAA) consistent with a thrombus; (B to D) Three-dimensional transthoracic echocardiogram. With-
in the LAA there are two echo densities noted. Sequential cropping shows both to be parts of pectinate muscles, which traverse
the LAA. The upper echo density (upper arrowhead) is larger because it represents a short-axis cut through two pectinate muscles virtually
in contact with each other. This most likely represents the “thrombus” seen on the transesophageal echocardiogram. The second
echo density (bottom arrowhead) is smaller because only one pectinate muscle is involved. (LUPV: Left upper pulmonary vein).
Source: Reproduced with permission from Karakus G, Kodali V, Inamdar V, Nanda NC, Suwanjutah T, Pothineni KR. Comparative
assessment of left atrial appendage by transesophageal and combined two and three-dimensional transthoracic echocardiography.
3. Scan LAA in multiple views to demonstrate all areas of of the atrial septum provides important information for
the nongeometric LAA. procedures requiring trans-septal puncture. The fossa
4. Emptying velocity signal of LAA is a surrogate for atrial ovalis region is the thinnest part of the atrial septum. The
activity. septum secundum is slightly thicker and is seen to the
5. Adjust gain appropriately to optimal level for right of the thinner septum primum. Presence of a patent
demonstration of SEC. foramen ovale is confirmed by demonstration of a space
between the septum primum and secundum along the
ATRIAL SEPTUM superior aspect and presence of flow between the two
Abnormalities of atrial septum are occasionally seen in layers of atrial septum using color flow Doppler and saline
patients undergoing ablation. Preprocedural assessment contrast study.
A B
Figs 80.11A and B: (A) Transesophageal echocardiogram (TEE) showing a recanalized left atrial appendage (LAA) after surgical
exclusion; (B) Peak velocity by continuous wave (CW) Doppler was 3.4 m/s with a peak gradient of 46 mm Hg across the recanalized
LAA.
Procedures requiring trans-septal puncture and macro-reentrant tachycardia. Thus, the importance
(e.g. PV isolation for AF) often produce a left-to-right shunt of morphological features of PV for the ablation planning
at the fossa ovalis region immediately postprocedure. has been recognized in such patients undergoing pre-
These shunts are invariably small and the defects close and postablation assessment. TTE has limited ability to
spontaneously over time. ICE guidance is used in most demonstrate PV anatomy. Occasionally, one might be
ablation laboratories for trans-septal puncture. If the able to see the ostia of right and left upper PV in the apical
puncture enters the upper part of atrial septum, significant four-chamber view of TTE. Rarely the left inferior PV might
hematoma might result due to entry into the Waterston’s be seen in the parasternal long-axis view. Other imaging
groove (an extracardiac space between the two atria due to techniques are therefore needed for PV imaging. While
in-folding of the atrial walls). CT angiography and cardiac MR imaging have been well
A frequent abnormality of the atrial septum in patients recognized for their ability in delineating PV anatomy,
undergoing AF ablation is lipomatous atrial septum. the role of TEE has been less well recognized. Experience
Significant thickening of the atrial septum can occur due from centers with high volume of TEE have shown that
to deposition of fat and this might be mistaken for an atrial all four PVs can be imaged in an overwhelming number
tumor. Typically lipomatous atrial septum spares the fossa of patients; ostial size, Doppler velocity profile, and other
ovalis region. Lipomatous atrial septum is more often seen abnormalities can be easily demonstrated.8 A systematic
in patients with obesity. review of published studies shows a diagnostic accuracy
rate of TEE for PV abnormalities comparable to CT
Scanning Tip angiography and cardiac MR imaging.9
• Immediately after AF ablation, there is often a left-to- PV imaging during TEE is generally performed at the
right shunt at the site of trans-septal puncture. midesophageal level. Left upper PV is adjacent to LAA,
while right upper PV is adjacent to SVC. Left lower PV is
anatomically close to the descending thoracic aorta. These
PULMONARY VEINS adjacent anatomical landmarks are helpful in locating
The critical role played by firing from PV in the genesis of AF PVs. Each PV ostium has an oval shape with a larger and
has now conclusively been recognized. This mechanism is a smaller orthogonal dimension. From the midesophageal
the basis for ablative therapies in AF (PV isolation). Many probe position during TEE, progressive scan plane
patients undergoing PV isolation for AF require additional angulation combined with counterclockwise torque of the
procedures including additional ablation for AF, A Flutter, scope will bring up both left upper and lower PVs in a single
Fig. 80.12: Transesophageal echocardiogram (TEE) from mideso- Fig. 80.13: Transesophageal echocardiogram (TEE) from mideso-
phageal level, employing 120° scan plane angle as well as torque phageal level showing right upper, middle, and lower pulmonary
shows left upper and lower pulmonary veins (PVs). veins (PVs). Right upper PV is adjacent to superior vena cava.
view (Fig. 80.12). Right upper PV can be demonstrated in of low velocity flow signals. Localized stenosis involving
the caval view by additional dialing of scan plane angle only one PV is frequently asymptomatic while stenosis
or clockwise torque of the scope. Right lower PV can be of all four PVs will lead to development of symptomatic
imaged by slight additional downward movement of the pulmonary hypertension.10 The presence of high velocities
TEE probe (Fig. 80.13). The ostial dimensions of both lower in PVs immediately after ablative therapies is explained by
PVs are smaller compared to upper PVs. The lower PVs are edema caused by the procedure; this frequently resolves
more difficult to image by and large. over time. Persistent elevation of PV velocities would
PV flow should be imaged using pulsed Doppler with be consistent with development of actual scarring and
the sample volume placed about 1 cm from the ostial site. stenosis of PV.
Occasionally, the localization of PV ostium can be difficult
due to a degree of flaring of the PV as they enter LA with INFERIOR VENA CAVA
the resultant absence of a distinct PV–LA junction. The
typical PV flow pattern consists of a systolic forward flow, Postablation complications involving inferior vena cava
a diastolic forward flow, and atrial reversal. These patterns (IVC) have been described. Routine scanning of IVC is
are influenced by diastolic dysfunction, LA pressures, and indicated pre- and postablation. IVC can be easily visua-
presence of significant mitral regurgitation (MR). It will lized by TTE from the subcostal window. More angulation
be essential to have careful assessment of PV velocities to bring right-sided structures in view will often demon-
preablation for accurate assessment of PV stenosis strate long segments of the IVC easily. Size and inspiratory
development. PV stenosis has become a rare complication collapse of IVC allow prediction of right heart filling
of ablation therapy for AF since focal ablation within PV pressures. IVC can also be demonstrated using TEE; this
is not currently used for AF ablation, but PV isolation by requires advancing the TEE probe from the midesophageal
ablation within LA is employed. However, PV stenosis four-chamber view to close to the transgastric level. With
still does occur after AF ablation and it can be readily clockwise torque of the scope, the IVC–RA junction is
diagnosed by TEE through assessment of PV dimensions brought into view. With additional advancement of probe
post procedure as well as increased PV velocities and dialing about 20° to 40° of scan plane angle, both the
(Figs 80.14 and 80.15). Often color flow Doppler shows IVC and hepatic vein are demonstrated. Ablation might
characteristic focal aliasing within PVs; sampling pulsed be complicated by development of mobile masses in
Doppler at the point of aliasing reveals increased velocity the IVC, close to the IVC–RA junction (Fig. 80.16), very
(usually >1 m/s) and loss of the typical spectral pattern likely representing the development of thrombi from the
A B
Figs 80.14A and B: (A) Stenosis of the right middle pulmonary vein (PV) after atrial tachycardia ablation. Significant aliasing of color
flow is seen at the ostial site of right middle PV; (B) Pulsed Doppler sampling from right middle PV shows marked increase in velocities
at the site of aliasing (peak diastolic velocity 1.3 m/s).
A B
Figs 80.15A and B: (A) Transesophageal echocardiogram (TEE) showing focal aliasing of the left upper pulmonary vein (PV) ostium
after atrial fibrillation (AF) ablation; (B) Pulsed Doppler from the left upper PV shows a peak velocity of approximately 1.5 m/s in diastole
consistent with PV stenosis.
Fig. 80.16: Transthoracic echocardiogram (TTE) subcostal view

showing a floating thrombus in the inferior vena cava (IVC) after an
ablative procedure. Patient did not manifest pulmonary embolism.
procedure. Clinically significant pulmonary embolism • Dilated cardiomyopathy

is infrequent in these patients. No correlation between a. Noncompaction
number of sheaths used and duration of procedure have • Hypertrophic cardiomyopathy
been demonstrated in patients developing IVC thrombi • Infiltrative diseases
after ablation. a. Sarcoid heart disease
• Arrhythmogenic RV dysplasia (ARVD)
ECHOCARDIOGRAPHY IN Since ventricular tachycardia (VT) ablations require
VENTRICULAR TACHYCARDIA placement of catheters in left ventricle (LV), the presence
Echo imaging with TTE provides highly meaningful of LV apical thrombi should be evaluated. Off-axis
information in patients with ventricular tachyarrhythmias. scanning of the LV apex and the use of Definity contrast
As such, imaging with specific diagnostic possibilities in are very helpful techniques for the demonstration of LV
mind is called for in a patient with ventricular ectopy. Most apical thrombi. Some EP laboratories are also interested
patients with ventricular arrhythmias have an anatomical in obtaining information about the degree of aortic arch
substrate (i.e. underlying structural heart disease). atherosclerotic burden since ablation of VT requires
Common substrates of ventricular arrhythmias manipulation of catheters across the arch of aorta.
include:11 The Table 80.1 lists the common conditions associated
• Coronary artery disease with scar from prior myoc with ventricular ectopy and the potential findings that
ardial infarction should be carefully looked at during echo imaging.
Table 80.1: Common Conditions Associated with Ventricular Arrhythmias and Associated Echocardiographic Findings
Disease Characteristic Findings
Coronary artery disease Increased LV dimensions
Reduced fractional shortening
Reduced left ventricular ejection fraction
(LVEF; Simpson’s biplane)
Localized scar (thinning, abnormal wall motion)
LV mural thrombi
Dilated cardiomyopathy Increased LV dimension
Reduced fractional shortening
Reduced LVEF (Simpson’s biplane)
Diffuse hypokinesis
LV mural thrombi
Noncompaction12 Increased LV trabeculations
Ratio of noncompacted to compacted segments > 2
Hypertrophic cardiomyopathy Small LV cavity
Hyperdynamic LV function
Asymmetrical septal hypertrophy
Systolic anterior motion of mitral valve
Mitral regurgitation
Aortic valve midsystolic closure
LV outflow gradient (at rest and/or during provocation, e.g.
Valsalva maneuver)
Sarcoid heart disease Localized basal septal scars
Contd...
Arrhythmogenic RV dysplasia13 Increased right ventricular outflow tract (RVOT) dimensions
(≥ 32 mm in the parasternal long-axis view, ≥ 36 mm in the basal
short-axis view)
Increased echogenicity of moderator band
Prominent branching pattern
Focal outpouching of RV free wall
Contd...
Disease Characteristic Findings
Sarcoid heart disease Localized basal septal scars
Arrhythmogenic RV dysplasia13
Increased right ventricular outflow tract (RVOT) dimensions
(≥ 32 mm in the parasternal long-axis view, ≥ 36 mm in the basal
short-axis view)
Increased echogenicity of moderator band
Prominent branching pattern
Focal outpouching of RV free wall
The presence of fibromuscular bands in LV has in patients with CIED and bacteremia prior to device
been reported in patients with idiopathic LV ventricular explantation. The presence of “ghosts”—casts comprising
tachycardia (Belhassen VT). Fibromuscular bands in inflammatory masses over the leads persisting after lead
this disease typically run across the LV cavity from the extraction—has been demonstrated to be associated with
posterolateral free wall to the basal part of the septum.14 worse prognosis in CIED infections.16
Lead extraction is required in CIED infections and one
of the complications of this procedure is hemopericardium
ECHOCARDIOGRAPHY IN CARDIAC
and cardiac tamponade.
IMPLANTABLE ELECTRONIC DEVICES In summary, in patients undergoing EP procedures,
Cardiac implantable electronic devices (CIEDs) are indi a unique set of knowledge and skills is required for us to
cated in a broad array of conditions including symptomatic provide the relevant information required by the EP team.
bradycardia (pacing), ventricular arrhy thmias and Close collaboration between the EP and echocardiography
sudden cardiac arrest (defibrillators for primary and teams along with a process of continuing learning through
secondary prevention), and severe heart failure [cardiac clinical correlation is required.
resynchronization therapy (CRT)]. Echocardiography
provides significant information in patients undergoing REFERENCES
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features of the underlying structural heart disease and Cardiol. 2011;57(9):1126–66.
2. Corcoran SJ, Lawrence C, McGuire MA. The valve of
to provide relevant quantitative measurements dictated
Vieussens: an important cause of difficulty in advancing
by the guidelines applicable in CIED therapy. Patients in
catheters into the cardiac veins. J Cardiovasc Electrophysiol.
need of CIED therapy for primary or secondary prevention 1999;10(6):804–8.
of VT have underlying structural heart disease (coronary 3. Abhayaratna WP, Seward JB, Appleton CP, et al. Left atrial
artery disease, hypertrophic cardiomyopathy, or dilated size: physiologic determinants and clinical applications.
cardiomyopathy) and reduced ejection fraction (EF). J Am Coll Cardiol. 2006;47(12):2357–63.
Thus, the preprocedural assessment should include 4. Gabriel RS, Klein AL. Managing catheter ablation for
careful estimation of EF by Simpson’s method. Selection of atrial fibrillation: the role of echocardiography. Europace.
patients for CRT is based on symptom level of heart failure 2008;10 Suppl 3:iii8–13.
and QRS duration. The role of echocardiography in patient 5. Karakus G, Kodali V, Inamdar V, et al. Comparative
assessment of left atrial appendage by transesophageal
selection for CRT is highly controversial and not supported
and combined two- and three-dimensional transthoracic
by strong evidence.
echocardiography. Echocardiography. 2008;25(8):918–24.
All patients with CIED infections require echo 6. Kumar V, Nanda NC. Is it time to move on from two-
cardiography to rule out infective endocarditis.15 This dimensional transesophageal to three-dimensional
is due to the fact that infection in CIED patients may be transthoracic echocardiography for assessment of left atrial
restricted to the device pocket in which case the duration appendage? Review of existing literature. Echocardiography.
of antibiotic therapy is shorter. Thus, TEE is required 2012;29(1):112–16.
7. Kanderian AS, Gillinov AM, Pettersson GB, et al. Success 12. Stanton C, Bruce C, Connolly H, et al. Isolated left
of surgical left atrial appendage closure: assessment by ventricular noncompaction syndrome. Am J Cardiol. 2009;
transesophageal echocardiography. J Am Coll Cardiol. 104(8):1135–8.
2008;52(11):924–9. 13. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis
8. Stavrakis S, Madden G, Pokharel D, et al. Transesophageal of arrhythmogenic right ventricular cardiomyopathy/
echocardiographic assessment of pulmonary veins and left dysplasia: proposed modification of the task force criteria.
atrium in patients undergoing atrial fibrillation ablation. Circulation. 2010;121(13):1533–41.
14. Thakur RK, Klein GJ, Sivaram CA, et al. Anatomic substrate
9. Stavrakis S, Madden GW, Stoner JA, et al. Transesophageal
for idiopathic left ventricular tachycardia. Circulation.
echocardiography for the diagnosis of pulmonary vein
1996;93(3):497–501.
stenosis after catheter ablation of atrial fibrillation: a
systematic review. Echocardiography. 2010; 27(9):1141–6. 15. Sohail MR, Uslan DZ, Khan AH, et al. Management and
10. Saad EB, Marrouche NF, Saad CP, et al. Pulmonary vein outcome of permanent pacemaker and implantable
stenosis after catheter ablation of atrial fibrillation: cardioverter-defibrillator infections. J Am Coll Cardiol.
emergence of a new clinical syndrome. Ann Intern Med. 2007;49(18):1851–9.
2003;138(8):634–8. 16. Le Dolley Y, Thuny F, Mancini J, et al. Diagnosis of cardiac
11. Stevenson WG, Soejima K. Catheter ablation for ventricular device-related infective endocarditis after device removal.
tachycardia. Circulation. 2007;115(21):2750–60. JACC Cardiovasc Imaging. 2010;3(7):673–81.
CHAPTER 81
Echocardiography in
Life-Threatening Conditions
Rachel Harris, Elizabeth Ofili
Snapshot

Chest Trauma 
Debakey ClassificaƟon

Blunt Chest Trauma 
The Stanford ClassificaƟon

PenetraƟng Chest Trauma 
Pulmonary Thromboembolic Disease

Acute Mitral RegurgitaƟon 
Air Embolism

Acute Severe AorƟc RegurgitaƟon 
Hypovolemia

AorƟc DissecƟon 
Large Intracardiac Thrombus
INTRODUCTION CHEST TRAUMA

The uses of two-dimensional transthoracic and trans- In 2010, among US persons aged 1–44, unintentional injury
esophageal echocardiography have proven vital in risk was the number one cause of death.5 Cardiac injuries
stratification of patients who present with life-threatening are among the most lethal of thoracic trauma patients,
conditions.1 In evaluating patients with hemodynamic especially in penetrating injuries.6,7 In this same age
instability, new or worsening heart murmur, or a recent group, the majority of nonfatal hospital injury visits were
history of blunt or penetrating trauma, echocardiography secondary to blunt force trauma.8 Although the majority of
has become the mainstay in identifying those with these patients die in the field from their injuries, among
critical injuries in need of surgical or immediate invasive the survivors who present to the emergency department,
intervention. Transthoracic echocardiography is both meticulous detail and a high index of suspicion must be
sensitive and specific for identification of pericardial held for the hemodynamically stable patient who may
effusion, pericardial injury, and peritoneal fluid in the rapidly deteriorate post presentation.
setting of anterior chest trauma.2 The advantages of
transthoracic echo are its bedside availability, noninvasive
characteristics, and ability to provide rapid information
BLUNT CHEST TRAUMA
regarding cardiac structure and function. Transesophageal This type of injury is more commonly the direct result of
echo has greater sensitivity and specificity in evaluating assault, motor vehicle collisions, falls, or fallen objects
valvular and aortic pathology, prosthetic valves, interatrial more commonly. There are numerous injuries that should
shunts, and cardiac sources of emboli but is more invasive be suspected depending on the mode of injury. In the case
(Table 81.1).3 of blunt objects whose force may decrease the chest wall’s
Table 81.1: Perioperative Indications for use of Transesophageal Echocardiogram in Trauma Patients
Category 1 Acute hemodynamic instability in which ventricular function and its determinants are uncertain and have
not responded to treatment
Unstable patient with unexplained hemodynamic disturbances, suspected valvular pathology, or thrombo-
embolism
Immediate evaluation of patient with suspected thoracic aortic pathology: aneurysm, dissection, or disrup-
tion
Pericardial window procedures
Category 2 At risk for myocardial ischemia or infarction or hemodynamic disturbances
Detection of air embolism or foreign body
Suspected cardiac trauma
Evaluation of pericardial effusion
Category 3 Evaluation of thoracic trauma in patient with low-suspicion of injury
Intraoperative assessment of repair of thoracic aortic injury
Category 1: Supported by the strongest evidence or expert opinion
Category 2: Supported by weaker evidence and expert consensus
Category 3: Little current scientific or expert support
Source: Adapted from the 1999 ASE/SCA guidelines for performing a comprehensive intraoperative multiplane transesophageal
echocardiography examination: recommendations of the American Society of Echocardiography Council for Intraoperative
Echocardiography and the Society of Cardiovascular Anesthesiologists Task Force for Certification in Perioperative Transesophageal
Echocardiography.3,4
anteroposterior diameter, puncture injuries from ribs

may result. Moreover, right atrial and ventricular free wall
rupture, massive hemothorax, and cardiac tamponade
have also been documented.9
Cardiac Tamponade
Echocardiography results in the diagnosis of cardiac
tamponade as well as assists in rapid management and
treatment, that is, echocardiographically guided peri-
cardiocentesis with catheter drainage.10 Patients presenting
with hemodynamic instability post penetrating or blunt
force chest trauma should be emergently evaluated for
cardiac involvement, namely, myocardial contusion or
pericardial effusion.11,12
The clinical presentation of diaphoresis, dyspnea, Fig. 81.1: Parasternal long-axis with large circumferential
muffled heart sounds, and hypotension should warrant pericardial effusion (PE). (AO: Aorta; DA: Descending aorta;
IVS: Interventricular septum; LA: Left atrium; PW: Posterior wall)
further evaluation for pericardial effusion.11,13,14 Careful
(Movie clip 81.1).
evaluation using parasternal long- and short-axis,
apical four-chamber, and subcostal windows should be
With Doppler, there may be evidence of exaggerated
undertaken as well as the use of M-mode.15 Evaluation of
diastolic right ventricular collapse can be demonstrated respiratory variation in inflow velocity. The transtricuspid
as well as right atrial collapse/inversion. Diastolic right and transmitral inflow velocities should not vary by more
ventricular collapse is more specific but less sensitive than than 25% or 15%, respectively (Fig. 81.5).10
right atrial diastolic collapse. When evaluating the pulmonary and aortic inflow
While most cases of cardiac tamponade are immediate, velocities, these should not vary by >10%.10 There may
there are rare reported cases of delayed tamponade up to also be evidence of phasic variation in the right and
70 days post injury (Figs 81.1 to 81.4).16 left ventricular outflow tract (LVOT) and exaggerated
Chapter 81: Echocardiography in Life-Threatening Conditions 1971
Fig. 81.2: Subcostal view of anterior pericardial fluid. Arrowhead Fig. 81.3: Parasternal short-axis of right ventricular (RV) diastolic
points to diastolic right ventricular collapse. (L: Liver; LV: Left ven- collapse (arrowhead) and pericardial effusion (PE). (LV: Left ven-
tricle; PE: Pericardial effusion) (Movie clip 81.2). tricle) (Movie clip 81.3).
Fig. 81.4: M-mode demonstrating right ventricular wall (RVW) Fig. 81.5: Tricuspid valve (TV) respiratory flow variation. (LV: Left
diastolic collapse (arrow). (CW: Chest wall). ventricle; RV: Right ventricle).
respiratory variation in inferior vena cava (IVC) flow.17 The biochemical cardiac markers.18 Cardiac contusion can
operator will also likely see a plethoric (distended) IVC (in occur as a direct result of pressure on the myocardium
the absence of hypovolemia). caused by deceleration forces that affect the chest wall
or indirectly secondary to shear stresses and increased
intrathoracic pressures.19–21 Noninvasive attempts to
Cardiac Contusion characterize cardiac contusion have aimed at evaluating
The definition of cardiac contusion is a histological the combination of ECG, cardiac enzymes, use of telemetry,
diagnosis. However, the clinical diagnosis relies on the and echocardiogram (notably transthoracic echo). ECG
sum of echo findings, electrocardiogram (ECG), and changes may be associated with ST depression, but
these findings are not specific for myocardial ischemia. ACUTE MITRAL REGURGITATION
However, should a patient have ECG changes, they should
be monitored at least 24 hours for arrhythmias.20,21 Postpenetrating or blunt injury, myocardial infarction
Both CPK Mb and troponin levels have been evaluated or endocarditis, acute mitral regurgitation (MR) from
in terms of their sensitivity and specificity, and troponin is possible valve, chord or papillary muscle involvement
more specific. Post trauma, the CPK Mb has been shown to must be considered. Careful evaluation of the plane of
be elevated in noncardiac injuries.20,21 the penetrating injury should be performed. Standard
Using bedside transthoracic echocardiogram (TTE), parasternal long-axis, parasternal short-axis, apical four-
the operator can examine for possible focal wall motion and two-chamber as well as subcostal images should be
abnormalities, valvular involvement, focal wall rupture, obtained during transthoracic echo. For transesophageal
pericardial effusion, or great vessel injury (Figs 81.6A and B). echocardiography, transgastric short- and long-axis and
midesophageal four- and two-chamber, and long-axis
views are optimal. M-mode assessment can assist in further
PENETRATING CHEST TRAUMA evaluation of the possible mechanism of the regurgitation
Penetrating chest injury may be caused by a knife, gun, rifle, as well as give useful information regarding volume status
or any sharp or impaling object through the skin. The path of (i.e. M-mode evidence of B-bump on the AC shoulder in
entry to exit should be closely examined with consideration of increased LV end-diastolic pressure states). If possible,
possible cardiac structures and/or vessels involved. Wounds a determination of the mechanism (i.e. perforation of
to the inferolateral left parasternal regions call for close valve leaflet, pap muscle dysfunction vs. rupture, chordal
evaluation of the LV, whereas lower right and left parasternal involvement), severity and repairability of the MR should
wounds call for right ventricular evaluation.22 Involvement be performed. This may involve transesophageal echo
of the interventricular septum is also common as well as evaluation if the patient remains hemodynamically stable
free wall rupture.47 Doppler evaluation of suspected areas (Figs 81.8 to 81.10 and Table 81.2).
of involvement should also be performed.23 If intracardiac
missiles are visualized, they may distally embolize and their ACUTE SEVERE AORTIC
location should be reported.24
In the setting of chest wall emphysema or hemothorax,
REGURGITATION
the sensitivity of transthoracic echo is decreased and Acute aortic regurgitation can occur commonly with
transesophageal echocardiogram (TEE) should be consi- trauma, aortic dissection, and infective endocarditis.
dered if the patient remains clinically stable and passage Close attention must be paid to several clinical factors in
of the TEE probe can be safely conducted (Figs 81.7A to D). considering a patient with acute aortic regurgitation. The
A B
Figs 81.6A and B: Parasternal short-axis view (apical level) in end diastole (A) and end-systole (B). Note the focal inferior wall akinesis
(arrows) and small pericardial effusion (PE) in a patient post trauma. (LV: Left ventricle) (Movie clip 81.6A and B).
Table 81.2: Classification of Severe Mitral Regurgitation

Color Doppler jet area Vena contracta width > 0.7 cm with large central mitral regurgitation (MR) jet [area > 40% of
left atrium (LA) area] or with a wall-impinging jet of any size, swirling in LA
Doppler vena contracta width (cm) ≥ 0.70
Regurgitant volume (mL/beat) ≥ 60
Regurgitant fraction (%) ≥ 50
Regurgitant orifice area (cm2) ≥ 0.40
Source: Adapted from Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 Practice Guidelines for the Management of
Patients With Valvular Heart Disease: Executive Summary. J Am Coll Cardiol. 2006;48(3):598–675.25
A B
C D
Figs 81.7A to D: Short-axis at the level of the mitral valve showing a small ventricular septal defect (VSD) without (A) and with (B)
color (arrow). Three months later, the VSD was noted to be significantly larger, measuring 1.5 cm in diameter shown without (C) and
with (D) color (arrows). (LV: Left ventricle; RV: Right ventricle). Courtesy of Dr Robert Chisholm, St Michael’s Hospital, Toronto, ON, with
permission.47
Fig. 81.8: Color Doppler evidence of severe mitral regurgitation Fig. 81.9: Prolapse of P3 scallop (arrow in Movie clip 81.9A).
(MR). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery) (LA: Left atrium; LV: Left ventricle; MR: Mitral regurgitation) (Movie
(Movie clip 81.8). clips 81.9A and 81.9B).
M-mode is also useful in assessing for premature closure

of the mitral valve (prior to QRS onset) and diastolic
fluttering of the anterior mitral valve leaflet (specific but
not sensitive).25 Doppler may also reveal evidence of
diastolic MR (Figs 81.11 to 81.14 and Table 81.3).
AORTIC DISSECTION
TTE and TEE are both are indicated (Class I) in diagnostic
imaging of suspected aortic dissection.26 The type and
extent of the dissection should be determined.
DEBAKEY CLASSIFICATION
Type I: Originates in ascending aorta, propagates at least
Fig. 81.10: Doppler evidence of severe mitral regurgitation (arrow;
Vmax, 4.67 m/s). (LA: Left atrium; LV: Left ventricle).
to the aortic arch, and often beyond it distally.
Type II: Originates in and is confined to the ascending
aorta.
Type III: Originates in descending aorta, rarely extends
pulse pressure in acute regurgitation may be normal to only
proximally but will extend distally.
mildly increased, given rapid equalization of pressures
versus in a chronic, compensated state [LV dilatation,
eccentric left ventricular hypertrophy (LVH), and a large THE STANFORD CLASSIFICATION
stroke volume] the pulse pressure can be very wide. The LV Type A: All dissections involving the ascending aorta,
and left atrial dimensions should be assessed with normal regardless of the site of origin (DeBakey types I
values indicating an acute decompensation. The standard and II).
transthoracic views to assess regurgitation severity are Type B: All dissection not involving the ascending aorta
the apical three- and five-chamber, suprasternal notch, (DeBakey type III; Table 81.4).
and right parasternal windows. For assessment of the The diagnosis of aortic dissection is visualization
LVOT diameter, the parasternal long-axis view is optimal. of two wall lumina separated by an intimal flap
Table 81.3: Classification of Severe Aortic Regurgitation

Color Doppler jet width Central jet, width > 65% left ventricular outflow tract (LVOT)
Doppler vena contracta width (cm) > 0.60
Regurgitant volume (mL/beat) ≥ 60
Regurgitant fraction (%) ≥ 50
Regurgitant orifice area (cm2) ≥ 0.30
Pressure Half-time (ms) < 200
Color M-mode propagation velocity (cm/sec) ≥ 80
Source: Adapted from Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 Practice Guidelines for the Management of
Patients With Valvular Heart Disease: Executive Summary. J Am Coll Cardiol. 2006;48(3):598–675.25
Fig. 81.11: Aortic valve rupture. Long-axis view reveals flail right Fig. 81.12: Color Doppler examination showing severe aortic
coronary cusp (arrow) resulting in severe acute aortic regurgi- regurgitation (AR). (AO: Aorta; LA: Left atrium; RV: Right ventricle)
tation. (AO: Aorta; LA: Left atrium; LV: Left ventricle) (Movie clip (Movie clip 81.12).
81.11).
Fig. 81.13: M-mode of mitral valve (MV) revealing early diastolic Fig. 81.14: Color Doppler superimposed on M-mode tracing.
closure (arrow) due to severe aortic regurgitation. (AO: Aorta; LA: Evidence of pan-diastolic regurgitation (arrow). (LVO: Left ventricle
Left atrium; LV: Left ventricle). outflow).
Table 81.4: Standard Two-Dimensional Transthoracic Views causes difficulty in visualizing small dissection areas in the
in Evaluating Aortic Dissection27–29 distal ascending aorta and anterior aortic arch that may be
Left Parasternal Ascending aorta encountered if not using a multiplane transesophageal
Apical Ascending aorta probe.31,32
Subcostal Ascending aorta
Right parasternal Ascending aorta PULMONARY THROMBOEMBOLIC
Suprasternal Notch Aortic arch DISEASE
Paraspinal Descending aorta
Right ventricular dysfunction and dilatation may be evident
Two-dimensional echo Doppler and M-mode standard views in massive or submassive pulmonary thrombo-embolic
reveal key portions of the aorta
(PTE) disease. Not all patients presenting with PTE need
urgent echocardiographic evaluation but it may be useful in
(Figs 81.15A and B).30 Evidence of tears may lead to
determining prognosis as well as the need for thrombolysis
propagation of the dissection either proximally or distally.
(Fig. 81.18). Mortality rates for acute PTE can exceed
Characteristics of the true lumen are systolic expansion
15% in the first 3 months post diagnosis.33–35 A common
and diastolic collapse, the absence or low intensity of
spontaneous echocardiographic contrast, systolic jets specific finding on echo is evidence of pressure overload,
directed away from the lumen, and systolic forward flow. (Figs 81.19A to D) described by Nazeyrollas et al. as right
Characteristics of the false lumen are diastolic diameter ventricle/left ventricle End-diastolic dimension (RV/LVEDD)
increase, spontaneous echocardiographic contrast, > 0.5 (parasternal M-mode echo) and tricuspid insufficiency
and reversed, delayed, or absent flow and thrombus (TI) with a jet velocity > 2.5 m/s.36 Furthermore, a
formation.27,29,30 Flow signals within the false lumen RV/LVEDD ratio of 0.9 or greater (left parasternal long-axis
represent signs of communication (Figs 81.16 and 81.17). or subcostal view) is an independent predictor of hospital
There are a few special considerations when performing mortality.37 The findings of akinesia of the mid right
transesophageal imaging. The number of tears should be ventricular free wall but normal motion of the apex are
documented as well as the location of each (distance from referred to as the McConnell’s Sign.38 This phenomenon
teeth to tip of probe).26 Moreover, there is a “blind” zone has a reported 77% sensitivity and a 94% specificity for the
caused by overlap of the trachea and the left main stem diagnosis of acute pulmonary thromboembolism in the
bronchus between the esophagus and the aorta, which setting of right ventricular dysfunction.36,38
A B
Figs 81.15A and B: Descending aortic dissection with intimal flap (arrows). (FL: False lumen; TL: True lumen). Courtesy of Dr Anekwe
Onwuanyi, Morehouse School of Medicine, Atlanta, GA (Movie clip 81.15).
Fig. 81.16: Color Doppler with evidence of flow signals in the Fig. 81.17: Spontaneous contrast in false lumen (FL). (TL: True
false lumen (FL) consistent with communication. (TL: True lumen) lumen) (Movie clip 81.17).
(Movie clip 81.16).
A B
C D
Fig. 81.18: Echogenic structure (arrowhead) in right atrium (RA) Figs 81.19A to D: (A) Patient who presented with submassive
consistent with thrombus in transit in a patient subsequently diag- acute pulmonary thromboembolism and right heart strain. Interatrial
nosed with acute pulmonary thromboembolism. (LA: Left atrium; septum bows into the left atrium (LA) consistent with elevated right
LV: Left ventricle; MV: Mitral valve; RV: Right ventricle) (Movie clip atrial (RA) pressures. (B) “D” sign (arrow) revealing right ventricular
81.18). (RV) pressure overload and pulmonary hypertension. (C) Tricuspid
regurgitant (TR) velocity > 4 m/s; (D) Dilated inferior vena cava
(IVC) with no inspiratory collapse. (LV: Left ventricle) (Movie clip
AIR EMBOLISM 81.19B).
Air embolism can result post multiple injuries including
blunt and penetrating chest trauma (Fig. 81.20). It may Prompt identification is needed to assist in definitive
result in air in the coronary arteries and Doppler findings treatment.
on echo consistent with air in cardiac chambers or major
arteries.39 When a patent foramen ovale is present, a very HYPOVOLEMIA
small amount of air can result in paradoxical emboli.
Patients may present with hemodynamic instability, Consideration of hypovolemia should be part of the
respiratory distress, or postcardiac arrest. Physical exam differential in patients with hypotension. The size of the left and
findings may be the appearance of cutaneous petechiae. right ventricles are not reliable indicators of hypo-volemia;
Fig. 81.20: Air (small echogenic shructures) noted in left

atrium (LA) and left atrial appendage (LAA; Arrows) (Movie clip
81.20).
A B
Figs 81.21A and B: Normal sized left ventricle (LV) with hyperdynamic state in hypotensive patient. End diastole (A) and end systole
(B). (Movie clip 81.21).
however, in severe hypovolemia, the left and right ventricles

are small and hyperkinetic, with evidence of systolic
collapse of the LV (Figs 81.21 and 81.22).40–42 A small IVC
(< 1.2 cm) has a 100% specificity (low sensitivity) for a RA
pressure of <10 mm Hg.43
LARGE INTRACARDIAC THROMBUS

Thrombus is defined as a discrete echodense mass with
defined margins that are distinct from the endocardium
and seen throughout systole and diastole. They can
occur in the setting of low flow or stasis. Cardiac thrombi
may predispose the patient to significant morbidity and
mortality. They may be formed de novo or identified as Fig. 81.22: Transesophageal echocardiogram (TEE). Parasternal
right heart embolism while in transit. long-axis view reveals small left ventricular (LV) diastolic dimen-
There are several causes including atrial fibrillation, sions indicating severe hypovolemia. (AO: Aorta; LA: Left atrium;
RV: Right ventricle) (Movie clip 81.22).
atrial flutter, atrial appendage, LV aneurysm formation,
A B
Figs 81.23A and B: Apical views. Left ventricular (LV) apical mobile thrombus (arrow) in newly diagnosed congestive heart failure
(CHF) in a patient who suffered a stroke 2 weeks later on anticoagulation therapy. (MV: Mitral valve; RV: Right ventricle) (Movie
clips 81.23A and B)
make a formal diagnosis. Transesophageal images are not

superior over transthoracic images for identification of LV
thrombi as the apex is usually foreshortened but is 100%
sensitive and 99% specific in identifying left atrial and left
atrial appendage thrombus (Fig. 81.24).44
The appearance of thrombi can vary from a hetero-
geneous, echolucent, protruding masses (in newly formed
thrombus) to a homogenous, smooth mass in chronic
states.46
SUMMARY
In life-threatening conditions, both two-dimensional
TTE and TEE are essential in rapid risk stratification, via
evaluation of cardiac structures and function, as well as
Fig. 81.24: Left atrial thrombus (LA: arrow) extending into left atrial remain a current noninvasive and more cost-effective
appendage (not shown) in a patient with severe mitral stenosis.
(AV: Aortic valve) (Movie clip 81.24). mainstay in diagnosis and treatment strategies.
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15. Martin M, et al. 2-D echocardiography: emergent use in simplified Pulmonary Embolism Severity Index in
the evaluation of penetrating precordial trauma. J Trauma. hemodynamically stable patients with acute pulmonary
1991;31(7):902–5. embolism: a prospective validation study. Circulation.
16. Rendón F, Gómez Danés LH, Castro M. Delayed cardiac
2011;124(24):2716–24.
tamponade after penetrating thoracic trauma. Asian
34. Torbicki A. Echocardiographic diagnosis of pulmonary
Cardiovasc Thorac Ann. 2004;12(2):139–42.
embolism: a rise and fall of McConnell sign? Eur J
17. Feigenbaum H, Amstrong WF, et al. Feigenbaum’s Echo-
cardiography. 6th ed. Lippincott Williams & Wilkins; 2005: Echocardiogr. 2005;6(1):2–3.
256. 35. Goldhaber SZ, Visani L, De Rosa M. Acute pulmonary
18. Sybrandy KC, Cramer MJ, Burgersdijk C. Diagnosing embolism: clinical outcomes in the International
cardiac contusion: old wisdom and new insights. Heart. Cooperative Pulmonary Embolism Registry (ICOPER).
2003;89(5):485–9. Lancet. 1999;353(9162):1386–9.
19. Wisner DH, Reed WH, Riddick RS. Suspected myocardial 36. Nazeyrollas P, Metz D, Jolly D, et al. Use of transthoracic
contusion. Triage and indications for monitoring. Ann Doppler echocardiography combined with clinical and
Surg. 1990;212(1):82–6. electrocardiographic data to predict acute pulmonary
20. Tenzer ML. The spectrum of myocardial contusion: a embolism. Eur Heart J. 1996;17(5):779–86.
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21. Kaye P, O’Sullivan I. Myocardial contusion: emergency of echocardiographic right/left ventricular end-diastolic
investigation and diagnosis. Emerg Med J. 2002;19:8–10. diameter ratio in patients with acute pulmonary embolism:
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2008;133(2):358–62. 5(6):613–19.
38. McConnell MV, Solomon SD, Rayan ME, et al. Regional 44. Weinsaft JW, Kim RJ, Ross M, et al. Contrast-enhanced
right ventricular dysfunction detected by echocardiography anatomic imaging as compared to contrast-enhanced
in acute pulmonary embolism. Am J Cardiol. 1996;78(4): tissue characterization for detection of left ventricular
469–73. thrombus. JACC Cardiovasc Imaging. 2009;2:969–79.
39. Yee ES, Verrier ED, Thomas AN. Management of air 45. Weinsaft JW, Kim HW, Crowley AL, et al. LV Thrombus
embolism in blunt and penetrating thoracic trauma. J Detection by Routine Echocardiography. Insights Into
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40. Charron C, Caille V, Jardin F, et al. Echocardiographic CMR. JACC: Cardiovascular Imaging. July 2011;4(7):
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2006;12(3):249–54. 46. Pepi M, Evangelista A, Nihoyannopoulos P, et al.; European
41. Feissel M, Michard F, Faller JP, et al. The respiratory Association of Echocardiography. Recommendations for
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fluid responsiveness in ventilated septic patients. Intensive 47. Dehghani P, Ibrahim R, Collins N, et al. Post-traumatic
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size predict right atrial pressures in patients receiving Cardiol. 2009;21(9):483–7.
CHAPTER 82
Lung Ultrasound in Cardiology
Luna Gargani, Eugenio Picano
Snapshot
Physical and Physiological Basis of Lung Ultrasound
¾¾ Acute Respiratory Distress Syndrome
¾¾
Methodology
¾¾ Pneumothorax
¾¾
Pulmonary Interstitial Edema
¾¾ Cardiopulmonary Ultrasound: An Integrated Approach
¾¾
Pleural Effusion
¾¾ Limitations
¾¾
Pulmonary Embolism
¾¾
INTRODUCTION as a hyperechoic horizontal line, moving synchronously

with respiration (Fig. 82.1, Movie clip 82.1). This dynamic
Assessment of the lung has always been considered horizontal movement synchronized with respiration is
off-limits for ultrasound, since it is standard textbook called lung sliding. In an aerated lung, several hyperechoic
knowledge that “because ultrasound energy is rapidly horizontal lines, arising at regular intervals from the pleural
dissipated by air, ultrasound imaging is not useful for the line, can be seen—these are called A-lines and are not
evaluation of the pulmonary parenchyma.”1 However, in pathological. When the air content decreases, the acoustic
recent years, lung ultrasound scan (LUS) has proved to be a mismatch needed to reflect the ultrasound beam is
useful tool for evaluating many different acute and chronic created, and some images appear. In the presence of
heart and lung disease conditions.2 It is especially valuable extravascular lung water (EVLW), the ultrasound beam
because it is a very easy application of echography, far less finds subpleural interlobular septa thickened by edema.
technically demanding than echocardiography,3 rapid to The reflection of the beam creates some comet-tail
perform and interpret, portable, repeatable, non-ionizing, reverberation artifacts, called B-lines or ultrasound lung
and independent of the cardiac acoustic window. Thus, it comets (Fig. 82.2; Movie clip 82.2). B-lines are defined
is suitable for a quick but meaningful evaluation for both as discrete laser-like vertical hyperechoic reverberation
in-patients and out-patients. artifacts that arise from the pleural line, extend to
the bottom of the screen without fading, and move
PHYSICAL AND PHYSIOLOGICAL synchronously with lung sliding.5
The physical basis of the cardiogenic watery B-lines
BASIS OF LUNG ULTRASOUND
also explains the source of pneumogenic fibrotic B-lines,
All diagnostic ultrasound methods are based on the which are found in the presence of interstitial pulmonary
principle that ultrasound is reflected by an interface fibrosis.6 The physical scatterer is represented by water-
between media with different acoustic impedance. In thickened interlobular septa with cardiogenic B-lines, and
normal conditions, with aerated lungs, the ultrasound by connective tissue-thickened interlobular septa with
beam finds the lung air and no image can be depicted pneumogenic B-lines. The two types of B-lines can pose
because the ultrasound beam is rapidly dissipated by the a challenge to differential diagnosis, although several
air.4 The only detectable structure is the pleura, visualized clues may help distinguish the two entities. Cardiogenic
Chapter 82: Lung Ultrasound in Cardiology 1983
Fig. 82.1: Sonographic appearance of a normal lung. Fig. 82.2: Sonographic appearance of multiple B-lines (indicated
by the white arrows).
extravascular lung water very early in the course of lung

injury in pigs, even at a stage when no changes in hemoga
sanalytic parameters can be observed.9
METHODOLOGY
The lung ultrasound examination can be performed
using any commercially available two-dimensional (2D)
scanner (cardiac, convex, microconvex, or linear probe),
also portable, with any transducer frequency. Higher
frequencies, as in linear probes, are useful for the
evaluation of the pleura, but provide a worse definition of
the pulmonary parenchyma. There is no need for a second
harmonic or Doppler imaging mode. The examinations are
performed with patients in a near-supine, supine, sitting,
Fig. 82.3: Sonographic appearance of a consolidated lung. The
echotexture becomes similar to that of the liver. or even standing position, as clinically indicated. Two
main approaches to LUS should be considered—one for
B-lines are always bilateral and are generally more diffuse diagnosis and one for semiquantification and follow-up.
in the right lung than the left lung, with a “hot zone” of In patients admitted to the emergency department, for a
higher density along the axillary lines (in lying patients, prompt diagnosis, LUS can be focused on eight areas, two
as decubitant regions).7 Moreover, cardiogenic B-lines anterior and two lateral per side.5 When the assessment
can be dissolved in a few hours by an acute diuretic load.8 is focused on the evaluation of pulmonary congestion,
For a further reduction in the air content, when the lung in order to achieve a semiquantification that may help
is consolidated, it is possible to visualize the pulmonary for follow-up and prognostic stratification, it would be
parenchyma as a solid parenchyma, just like the liver or appropriate to use the scheme shown in Figure 82.4.7
the spleen (Fig. 82.3). Ultrasound scanning of the anterior and lateral chest is
B-lines are a very early event in the cascade, leading to obtained on the right and left hemithorax, from the second
pulmonary edema, as has been shown in an experimental to the fourth (on the right side to the fifth) intercostal
model of oleic acid-induced lung injury, which mimics spaces, and from the parasternal line to the axillary line.
human acute respiratory distress syndrome (ARDS). The sum of the B-lines found on each scanning site yields
B-lines unmasked accumulation of histologically verified a score denoting the extent of extravascular fluid in the
Fig. 82.4: Thoracic scanning areas for semiquantitative assessment of B-lines.

Source: Modified from Jambrik et al. 2004.
lung. Zero is defined as a complete absence of B-lines in technology for measuring lung edema can be inaccurate
the investigated area. For clinical purposes, B-lines may be (chest X-ray), cumbersome (nuclear medicine and radio
semiquantified from mild to severe degree, similar to what logy techniques), or invasive (indicator dilution), there is
is done for most echocardiographic parameters.10 B-lines great potential for a technology that could quantify lung
have a very satisfactory intraobserver and interobserver edema noninvasively in real time with a radiation-free and
variability, consistently < 10%.7 portable method.
Being a sign of pulmonary congestion, B-lines are
PULMONARY INTERSTITIAL EDEMA useful for the identification of cardiogenic versus noncar
diogenic dyspnea in an acute setting,13 with an accuracy
Diagnosis comparable to that of natriuretic peptides.14–16 B-lines could
The possibility of detecting pulmonary edema before it be a plausible alternative, especially in emergency depart
becomes clinically apparent is so inherently attractive ments where natriuretic peptide analysis is not available,
that the effort to develop and validate such a technique and could aid diagnosis in cases where natriuretic peptide
still continues after many years of tireless and relatively levels are in the “gray zone,” and positive predictive value
unrewarding attempts.11 Chest X-ray remains by far the is not very high, especially in patients with renal failure.
best and most frequently used screening test for the
detection of pulmonary edema, but is difficult to interpret Treatment
and is imprecise, with high interobserver variability.12
The absence of chest X-ray findings does not exclude the The recognition, quantification, and monitoring of pulm
presence of a high pulmonary capillary wedge pressure onary congestion is important for clinicians at all stages of
(PCWP) > 30 mm Hg. According to American Heart care of the HF patient. Accurate assessment of effectiveness
Association/American College of Cardiology guidelines, of medical treatment in reducing pulmonary congestion
serial chest radiographs are not recommended in the is mandatory in these patients. Chest X-ray remains by
assessment of pulmonary congestion in chronic heart far the best and most frequently used screening test for
failure (HF), since they are too insensitive to detect all the detection and in-hospital follow-up of pulmonary
but the most extreme changes in the fluid status. Direct congestion, although showing the previously mentioned
measurement of PCWP via catheterization is the “gold limitations. Another way to monitor congestion is through
standard” to evaluate hemodynamic congestion, but its monitoring body weight. However, this has limited
invasive nature limits clinical utility. Thus, because current reliability as a predictor of congestion status, since body
weight does not take into account systemic water, weight PLEURAL EFFUSION
gain may reflect normal fluctuations in time, and weight
loss due to loss of muscle/fat (cardiac cachexia) may Detection of pleural effusion (PE) is the more established
application of LUS.1 PE can be easily detected directly
obscure increased fluid retention.17
through the intercostal spaces with either a cardiac or
B-lines have already been proposed as a bedside, easy-
convex probe. The effusion should first be sought in
to-use alternative diagnostic tool for clinically monitoring
dependent zones, that is, lateral and posterior chest. It
pulmonary congestion in patients with HF.18 The sign is
rules out other etiologies such as atelectasis, consolidation,
very dynamic, as shown by rapid increase after exercise,
mass, or an elevated hemidiaphragm. It takes less time
in patients with and without left ventricular dysfunction.19
than radiographic methods and can be repeated serially
B-lines are well-related to the degree of dyspnea: the
at the bedside. Ultrasound images PE as an anechoic or
number of B-lines increases, as the degree of dyspnea
hypoechoic space between two pleural layers. The lung
worsens.20 Since B-lines can be dissolved in a few minutes
behind a PE appears either aerated or consolidated in the
by an acute diuretic load, they may be a useful bedside tool
case of large PE. In critically ill patients, LUS is especially
for monitoring diuretic therapy response, in a real time valuable, showing better sensitivity and reliability than
fashion.8,18 bedside chest X-ray for the diagnosis of PE.25,26 Bedside
The simplicity and low-tech nature of this examination chest X-ray rarely detects small effusions and can also miss
also makes it appealing for out-of-hospital office monit effusions of up to 500 mL.27 For the detection of PE, with
oring of HF patients; there would be the possibility of computed tomography (CT) as a gold standard, sensitivity
tailoring pharmacological therapy as soon as the patient, and specificity of ultrasound are 93%.27 Minimal effusions
although asymptomatic, shows echographic signs of can be detected using ultrasound, provided the probe is
pulmonary congestion, in order to avoid new hospita applied over an adequate area of the chest. Ultrasound
lizations for worsening dyspnea, which would likely can detect the effusion, evaluate its volume, provide
appear with some days of delay.21 The possibility of information on its nature, and indicate the appropriate
assessing B-lines with light, portable, hand-held devices area for an eventual thoracocentesis. Ultrasound is
could also allow a cardiologist to evaluate the degree of acknowledged as the method of choice for detecting an
decompensation directly at the patient’s home.3 effusion in a supine patient.
Prognosis PULMONARY EMBOLISM

Persistent hemodynamic congestion that is not adequately The diagnosis of pulmonary embolism has always been a
recognized and treated before discharge is associated with considerable challenge and requires a high index of clinical
adverse clinical outcome in HF patients; on the other suspicion from the emergency physician. In addition,
hand, postdischarge freedom from pulmonary congestion diagnosing pulmonary embolism may require the use of
is associated with a better prognosis.22 It has been shown one or more direct and indirect diagnostic methods. Most
that in patients admitted to the hospital with dyspnea and/ lesions related to pulmonary embolism are localized in
or chest pain, the presence of B-lines identifies a subgroup the lower lobes of the lung and are often associated with
at a higher risk of experiencing events—the higher the an area of pleuritic chest pain. These processes essentially
number of B-lines, the worse the outcome. The 16-month involve the terminal air spaces and result in the evacuation
event-free survival showed a significantly better outcome of air from the affected parenchymal area, creating an
for those patients without B-lines, whereas a worse outcome “ultrasonic window” and allowing ultrasound waves to
was observed in patients with a severe grade of B-lines travel further into the lung. The characteristic sonographic
(total number > 30). In regard to future HF hospitalization findings in pulmonary embolism are multiple, hypoechoic,
alone, and not as part of the combined endpoint, the rate pleural-based parenchymal lesions which adopt a wedge
of new hospitalization for progression of HF was higher in shape.28 These lesions can be visualized at least in three
patients with a severe B-line score and lower in patients fourths of cases.29 The accuracy of LUS for the detection of
with no B-lines (log rank = 24.4, P < 0.0001).23 These data pulmonary embolism was found to be 84% at a prevalence
have also been confirmed in a multicentric study on a of 55% in a large multicenter study series comprising
large number of patients on hemodialysis.24 352 patients with suspected pulmonary embolism.30
X-ray.33 Differential diagnosis between acute cardiogenic

pulmonary edema and ARDS may often be difficult. The
sonographic pattern of multiple diffuse B-lines is present
in both conditions. However, there are some clues that
may help in the differential diagnosis—pleural line
abnormalities, presence of small subpleural consolidations
or coarse appearance of the pleural line (Fig. 82.5), “spared
areas” defined as areas of normal lung pattern surrounded
by areas of multiple B-lines, and lung consolidations are
often found in ARDS, but are not present in cardiogenic
pulmonary edema.34
B-lines can also identify subclinical pulmonary edema
in situations of extreme physiology such as strenuous effort
in ordinary conditions (elite apnea divers or triathletes)35,36
or ordinary effort in extreme conditions such as high
Fig. 82.5: Small subpleural consolidations and multiple B-lines in
a typical sonographic pattern of ALI/ARDS. altitude pulmonary edema in recreational climbers,37
again suggesting that clinical symptoms are only the tip of
the iceberg of pulmonary edema, even outside the acute
In combination with echocardiography and leg vein HF syndrome.
compression sonography, the accuracy of LUS is >90%.31 In
order to be detectable by LUS, the lesions need to extend to PNEUMOTHORAX
the pleural surface, which is usually the case. In addition,
Pneumothorax (PTX) is a frequent, life-threatening
mechanical alterations associated with consolidated lung
complication in patients who have been admitted to the
tissue, increased capillary pressure, and permeability,
emergency department. Bedside chest X-ray may mis
due to the release of inflammatory mediators, also cause diagnose up to 30% of cases.38 Radiographically “occult”
increased exudation of fluid,28 leading to localized pleural PTX may rapidly progress to tension PTX if its diagnosis
fluid collection adjacent to the affected pulmonary region is missed or delayed, especially in patients receiving
or presence of localized multiple B-lines. Exploration of mechanical ventilation.39 Being a “nondependent”
the lesions by color Doppler imaging may provide addi condition, PTX should be sought at first on the anterior
tional diagnostic information. In pulmonary infarction, and lower areas. Absence of lung sliding is a basic and
areas of pulmonary arterial flow cannot be detected on initial step for the diagnosis, and a striking absence of
color Doppler ultrasound, a phenomenon referred to motion arising from the pleural line is observed instead
as “consolidation with little perfusion.”31 On the other of the lung sliding.40,41 The presence of lung sliding allows
hand, recanalization of incomplete infarction resulting PTX to be confidently discounted because the negative
from anticoagulation treatment or intrinsic lysis can be predictive value is 100%.40 The abolition of lung sliding
demonstrated by the reappearance of a blood flow signal can be objectified in M-mode, which gives a characteristic
on follow-up. It is important to note that a normal LUS pattern, the stratosphere sign. However, absent lung sliding
does not exclude the presence of PE.29 does not necessarily mean PTX. Many other situations yield
abolished lung sliding, such as high-frequency ventilation,
ACUTE RESPIRATORY massive atelectasis, pleural adherences, severe fibrosis,
and so on.5 The other conditions needed to diagnose PTX
DISTRESS SYNDROME by LUS are absence of B-lines—the slightest B-line allows
ARDS is a common syndrome of diffuse lung injury prompt ruling out of PTX.40 The pathognomonic LUS sign
and has a high mortality rate.32 In ARDS, LUS provides a of PTX is the lung point, which allows PTX to be confirmed,
sensitivity of 98% and a specificity of 88% in diagnosing with a specificity of 100% and sensitivity of about 65%, a
the presence of the alveolar-interstitial syndrome as seen percentage that falls with major PTX with complete lung
at CT, performing better than either auscultation or chest retraction.42
Table 82.1: The Three B-Line Scenarios: Heart Failure, ALI/ARDS, and Interstitial Lung Disease
Clinical Examples Heart Failure ALI/ARDS Pulmonary Fibrosis
EF Abnormal (decreased) Normal Normal
PASP Abnormal (increased) Normal Normal/Increased
Effect of diuresis/dialysis Reduction in minutes–hours No effect No effect
on B-lines
Other LUS sign Pleural effusion Subpleural alterations Pleural thickening
Theater ER/Cardiology/Internal Medicine Intensive Care Pulmonology/Internal Medicine
(ALI: Acute lung injury; ARDS: Acute respiratory distress syndrome; EF: Ejection fraction; LUS: Lung ultrasound scan; PASP:
Pulmonary artery systolic pressure).
CARDIOPULMONARY ULTRASOUND: LUS is one of the easiest applications of echography,

much easier than echocardiography. Image patterns
AN INTEGRATED APPROACH are readily teachable, and minimal didactic and image
Echocardiography is an essential tool for the management recognition skill sessions are needed.3,45 The learning
of patients with heart disease, providing an enormous curve for B-line evaluation and grading is very short. The
amount of information on both acute and chronic condi complement of LUS to echocardiography would require
tions. The addition of LUS to echocardiography provides only a few minutes in addition to the time needed for a
an additive insight into the presence of EVLW or any other resting echocardiogram.
pulmonary involvement. Presence of multiple, diffuse,
bilateral B-lines associated with left ventricular systolic LIMITATIONS
and/or diastolic dysfunction or valvular heart disease is
highly suggestive of cardiogenic pulmonary congestion. LUS limitations are essentially patient-dependent. Obese
Moreover, for any given level of cardiac dysfunction, patients may be more difficult to examine due to the
thickness of their rib cage and soft tissues. The presence
the response of the pulmonary vascular bed may be
of subcutaneous emphysema or large thoracic dressings
variable. LUS helps identify those patients who, although
alters or precludes the propagation of the ultrasound
asymptomatic, are going to decompensate and require
beams to the subpleural lung parenchyma. The main
more aggressive treatment.43,44 Presence of multiple,
limitation of B-line interpretation is the lack of specificity.
diffuse, bilateral B-lines, associated with a normal heart,
As previously mentioned, they are a sign of interstitial
indicates a noncardiac cause of pulmonary edema such as syndrome; therefore, they are a very sensitive but not
acute lung injury (ALI)/ARDS or interstitial pneumonia. specific sign of cardiogenic pulmonary edema. How to
Alternatively, especially in a chronic setting, it should distinguish the different etiologies of B-lines has been
prompt the suspicion of pulmonary fibrosis. It is important discussed in this chapter. However, it must be emphasized
to distinguish the multiple, diffuse, bilateral B-line pattern that LUS does not rule out pulmonary abnormalities
from focal multiple B-lines, which can be present in (especially consolidations) that do not reach the pleura.
normal lungs or may be seen in the context of many In the next few years, LUS is likely to become an
pathological conditions such as lobar pneumonia, pulmo extension of physical examination in many different
nary contusion, pulmonary infarction, pleural disease, clinical settings, from the emergency department to the
and neoplasia. This further underlines the importance of intensive care unit, from cardiology to pulmonology, and
integrating LUS findings with the patients’ history, clinical nephrology wards and dialysis units.
presentation, and other instrumental data. An example
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detects lung water accumulation in healthy elite apnea
umothoraces: the Extended Focused Assessment with Sono
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CHAPTER 83
The Future of
Echocardiography and Ultrasound
David Cosgrove
Snapshot
Plane Wave Ultrafast Imaging
¾¾ Elastography
¾¾
Trends in Scanners
¾¾ Light and Sound
¾¾
Doppler
¾¾ Therapeutic Applications of Ultrasound
¾¾
Microbubbles
¾¾
INTRODUCTION
Ultrasound has shown a remarkable ability to evolve
and even revolutionize, thanks to the efforts of research
laboratories and commercial companies around the
world. Unlike some industries, ideas developed in one
quarter find their way into clinical scanners surprisingly
rapidly, to the benefit of our patients. Here, some of the
more exciting and intriguing developments and prospects
are briefly covered, but it should be borne in mind that the
most important innovations may well be unpredictable:
expect the unexpected!
PLANE WAVE ULTRAFAST IMAGING

Fig. 83.1: Ultrafast plane wave imaging. The left image shows a
A major trend in ultrasound imaging is likely to be the transverse section of a rat brain, measuring about 1 cm across,
implementation of plane wave imaging1 (Fig. 83.1). This imaged with the conventional pulse-echo technique. Spatial reso-
approach, first mooted in the 1980s, radically changed the lution is greatly increased by using plane wave imaging, shown
on the right, in which multiple interrogation at a different angle of
way scanning is performed.2 Instead of transmitting a series
insonation (compounding) has been used.
of focused ultrasound beams one by one, the transmitted Source: Redrawn with permission from Mace E, Montaldo G,
ultrasound is unfocused, all the elements being activated Cohen I, et al. Functional ultrasound imaging of the brain. Nat
at once. All the focusing is performed on receive, ideally Methods. 2011;8(8):662–4.
using software-driven systems. The potential penalty
in spatial resolution is more than made for by the huge high frame rates. The images are crisper because the
increase in frame rate (up to 20,000 fps, depending on transmission is so rapid that any motion-induced blurring
the required depth) so that multiple pulses can be sent, is “frozen”; this is likely to be especially important in
for example, for compounding, while still retaining very cardiology and particularly for the fetal heart. Doppler
Chapter 83: The Future of Echocardiography and Ultrasound 1991
Fig. 83.2: Ultrafast Doppler. A series of transverse sections of a Fig. 83.3: Smartphone ultrasound system. This USB-based scan-
rat brain using directional power Doppler at an acquisition rate ner from Mobisante uses a Smartphone to create ultrasound
of 20,000/s. The method allows changes in cerebral blood flow images. The implications for usage and training are major. http://
to be displayed after brain stimulation in the same way as www.mobisante.com/product-overview/
functional MRI.
Source: Redrawn with permission from Tanter M.
could also benefit3 (Fig. 83.2) as could elastography4 training and embodies a cruel conundrum: less skilled
and contrast studies could take advantage of the overall users need the best image quality, and this is denied
lower peak power (since there is no transmit focusing) them. They raise concerns for the quality of diagnoses and
which could reduce bubble destruction. In addition, the possible legal consequences. They could also exacerbate
possibility of using extended multipulse methods could the great harm that ultrasound has delivered in some
improve tissue suppression. developing countries: informing on the gender of the
The reason for the slow implementation of what fetus so that females can be selectively aborted, a human
seems like an obvious way to exploit one of the strengths disaster of unprecedented scale. Indeed, it could be argued
of ultrasound, its speed, has been computing limitations. that, on account of this, ultrasound has done more harm
Handling the huge amount of data and capturing and than good worldwide.6
processing the radio frequency (RF) data from over 100 Increasingly, software-driven scanners can be expec-
elements at such high data rates was impossible when ted to predominate, at least at the mid- and high end, with
the approach was first suggested. With the startling speed fast computing and graphics cards replacing fixed function
bumps that the industry has experienced and with the chips for greater flexibility, upgradability and advantages
addition of fast graphics chips (stimulated by the computer in servicing. This could result in price reductions, though
gaming industry), this limitation has been rolled back there has been no sign of this to date. They are also expected
and partial implementations have appeared in clinical
to form the platform for plane wave (ultrafast) imaging.
scanners. Much further development can be expected.
Always the key component of an ultrasound system,
transducers are being improved, chiefly with the intro
TRENDS IN SCANNERS duction of single crystal piezoelectric materials.7 These
Miniaturization is well under way, with laptops and are grown rather like a silicon chip, rather than being a
even smart phones being used, if only for display5 ceramic that requires firing, as is used in conventional
(Fig. 83.3). These devices are likely to find their way into piezo materials. Single crystals have better sensitivity on
every clinician’s white coat pocket, supplementing or both transmit and receive, and wider bandwidths, so that
even replacing their stethoscopes. This degree of miniatu they can operate over wider frequency ranges, meaning
rization comes at a cost: their image quality cannot rival that one probe can work over a wider range of applications.
that of high-end systems. This puts an extra burden on This could be especially important in contrast-enhanced
Fig. 83.4: Live/real time three- and four-dimensional transesopha- Fig. 83.5: Acoustic structure quantification of the liver. This form of
geal echocardiography. En face view of paravalvular mitral pros- tissue characterization looks at the B-mode structure, working in
thetic regurgitation, oriented to be identical to the surgical view. the radio frequency (RF) domain, to assess the image texture. The
The paravalvular (P) defect is localized at 5 o’clock position in result is a moving image in which uniform structures are displayed
this patient with a metallic prosthesis. (AO: Aorta; LAA: Left atrial in blue or green, and heterogeneous structures, here exemplified
appendage; MVR: Mitral valve replacement). by the walls of blood vessels, are depicted as orange and red. It is
Source: Reproduced with permission from Singh P, Manda J, Hsi- likely to be of most value in the liver for assessing steatosis (very
ung MC, et al. Live/real time three-dimensional transesophageal uniform) or severe fibrosis and cirrhosis.
echocardiographic evaluation of mitral and aortic valve prosthetic
paravalvular regurgitation. Echocardiography 2009;8:980–7.
ultrasound (CEUS) where higher harmonics need to be semiconductors onto the back of the cMUT, operating as
detected. Their efficiency is also key to the development preamplifiers, for example. Prototypes have been built, but
of cable-free transducer systems: adequate battery life the field seems to have gone into abeyance.
depends on highly efficient transduction. There is also a Ways to cut conventional PZT arrays into two-dimen
move to replace the lead in Lead zirconate titrate (PZT) sional matrices containing thousands of elements have
transducers by less toxic elements such as barium, driven been developed.10 These engineering wonders pose a major
by concerns over the potential toxicity of lead in discarded challenge in cabling and in handling the huge amount
transducers.8 of data they generate, but they make possible three-
One transducer trend that, sadly, seems to have stalled dimensional (3D) and four-dimensional (4D) transducers
is the capacitance micro-machined ultrasound transducer that have been especially promising in echocardiography
(cMUT); these tiny electrostatic transducers are formed (Fig. 83.4) and may eventually develop into the standard
by silicon etching using a photographic mask, just as for a way to perform ultrasound examinations by replacing the
semiconductor chip, but are built with an air gap between bulky hybrid electromechanical probes that are widely
two electrodes.9 When an electric charge is placed across available.
the gap, the plates are attracted (or repelled, depending on Analysis of the B-mode signal content historically
the charge direction) and the plates move closer together formed the basis for ultrasound tissue characterization, a
(or further apart), exactly as in an electrostatic loudspeaker. subject that had a vogue but lost its momentum. Interest
The symmetrical effect on receipt of an acoustic signal has now been rekindled because the capacity to work with
allows them to act as both receivers and transmitters. Each RF or raw data has arisen with continuing improvements in
element is only a millimeter or so in size and so a matrix of computing power and data storage and the annual Tissue
many thousands is needed. An advantage is that once the Characterization Conference has regained popularity.11
photo mask has been created, they can be made in large There are also commercial implementations that either
numbers in the same way as silicon chips, and they could map tissue texture [e.g. Toshiba’s Acoustic Structure
therefore become cheap and even disposable (of interest Quantification, ASQ12 (Fig. 83.5)] or look at aspects of the
for intravascular ultrasound). It is also possible to build frequency spectrum of the echoes (e.g. Histoscanning).13
interrogated from several angles. The computer can

then correct the result for the beam-to-vessel angle. This
approach can be done with ultrafast imaging, the rapidly
acquired data being reviewed in slow motion so that
spectral measurements can be made postacquisition.16 In
a smart innovation, another approach is to make use of the
lateral modulation that is conventionally ignored to gather
data about both inline and across-line Doppler shifts.17
The resulting color Doppler real time display is direction
independent, with vector arrows to indicate the flow
direction. It is particularly useful for tortuous vasculature,
though the method works less well for deeper vessels.
MICROBUBBLES
Fig. 83.6: Fusion imaging. In order to biopsy this liver lesion, a
previously acquired computed tomography (CT) scan has been
The available microbubbles for ultrasound contrast have
entered into the scanner’s memory and the two image sets aligned excellent properties and, with microbubble-specific
using a position sensor attached to the ultrasound probe. The multipulse techniques at low mechanical index (MI), give
lesion is highlighted in a green circle. This allows the complete good enhancement of both the macro- and microvascular
cross-sectional images of CT to be synchronized with the ultra-
sound images and the latter used for improved targeting of nee-
systems; it has become routine for characterization of
dles for biopsy and interstitial therapy. focal liver lesions (Figs. 83.7A to C), for endocardial border
detection, and is also widely used for assessing myocardial
perfusion.18–20 Many innovative applications are being
An important trend, especially for interventional work, developed, including administration into other body
is fusion imaging, which combines the advantages of the cavities and even intradermal injection for ultrasound
complete cross-sectional display of computed tomography lymphangiography (Fig. 83.8).
(CT) and magnetic resonance (MR) with the real time However, there remains a huge potential for further
interactiveness of ultrasound14 (see Fig. 83.6). In this development, both for diagnostic and for therapeutic
approach, a 3D data set from a CT or MR scan is imported purposes. There is much preclinical work on targeted
into the ultrasound system; the probe is attached to an microbubbles, mainly directed to the endothelium, the first
electromagnetic position sensor and its position in space cell membrane encountered after intravenous injection.21
is linked to the cross-sectional imaging, which is then Three main directions have emerged: targeting activated
resliced and adjusted to match the ultrasound images. endothelium as a means to image acute inflammation,
This allows a needle or ablation antenna to be positioned targeting neovascularization as a means to image the new
using ultrasound together with the CT or MR data. vessels in tumors, atheroma and chronic inflammation
A redesign of the scanner user interfaces to improve and targeting thrombus, all forms of molecular imaging.22
cleanliness by replacing the current button and knob- The first human studies with a microbubble targeted
driven panels with flat wipe-down control surface would to VEG-F2 have begun addressing the need for better
be especially important in sensitive environments where imaging of prostate cancer, with promising results.23
microbial contamination is to be avoided (e.g. intrao One of the difficulties has been the choice of ligand: the
perative ultrasound).15 It would necessitate a radical conventional streptavidin-biotin linker is highly effective
rethink but the benefits could be wide ranging. but is antigenic, so not suitable for human use. For the
VEG-F2 study, Bracco developed a peptide linker that
seems to be well tolerated, and there are also maleamide
DOPPLER
linkers that may prove clinically useful.
Doppler has lagged behind until recently. However, the This target is restricted to the vascular space and
development of methods to mitigate its angle depen the endothelium because microbubbles are too large
dence should simplify its routine use. One method to penetrate into the interstitium, even when it is leaky,
uses compounding, so that each pixel or range gate is as occurs in tumors. So there has been much interest in
A B
Figs 83.7A to C: Liver metastasis. This lesion in the inferior

border of the liver is indeterminate on B-mode ultrasound (arrow
in A) despite the presence of scanty power Doppler signals.
Following intravenous (IV) administration of a 1.2-mL dose of the
microbubble SonoVue, there is enhancement in the arterial phase
at 39 s (B) followed shortly by washout in the late phase at 1 min
07 s (C), seen as an increase and then a reduction in the gold-
colored signals in the left pane. These hemodynamic features are
C characteristic of malignancy, in this case, metastatic disease.
Fig. 83.8: Sentinel lymph node—microbubble lymphangiography.

In this patient with an invasive breast carcinoma, a small amount
(0.4 mL) of SonoVue was injected intradermally in the periareaolar
tissue and the draining lymphatic (seen here as a green curved
line in the left-hand panel) traced to the sentinel lymph node
(arrow), which was then biopsied. The method could improve
preoperative staging in these patients and avoid the need for
frozen section microscopy in theater, thus reducing the need for
repeat surgery when this is falsely negative, as occurs in up to 25%
of cases.
developing particles24 (actually condensed microbubbles ELASTOGRAPHY

with no gas) that are both small enough to do so and can
be re-activated to form microbubbles by sonication once A special opportunity to examine the mechanical
they have reached the desired tissue space or target. properties of human tissue has been the development
Fig. 83.9: Strain elastography. This breast carcinoma shows as Fig. 83.10: Shear wave elastography. A scirrhous breast cancer
a predominantly blue (indicating hard) region in the elastogram, shows as a shadowing mass on B-mode in the lower panel. The
shown in the left panel. Regions of interest can be placed over the shear wave elastogram in the upper panel shows it to be very stiff
surrounding fat and over the lesion to derive a strain ratio, here (red colors) especially in the periphery where values of 225 kPa
calculated at 3.37, indicating a stiff lesion. were measured. This system uses ultrafast plane wave imaging to
capture the shear wave at sufficient speed that real time imaging
is possible.
of elastography.25–27 Tissue stiffness derives from the to use for the heart or abdominal organs) and of being
interlinks between tissue structures, especially connective quantitative, with readouts in meters per second (m/s)
tissues (and ultimately, bone, though this is not amenable that can be converted to kilopascals (kPa), the standard
to ultrasonic interrogation), and this is quite different unit for elasticity.
to the structures that support the longitudinal waves of Much work has been done in refining the classification
conventional ultrasound. The attraction of elastography is of malignancies in the breast, thyroid, and prostate and for
the huge range of tissue stiffness and the marked changes assessing liver fibrosis; in this application, it is beginning
that occur with pathology, malignancies, for example, to reduce the need for liver biopsies.
being stiffer than the host tissue. Two ways to image the The mechanical properties of body tissues are extre
elastic properties of tissue have been developed: strain and mely complex and elasticity also includes a viscosity
shear wave approaches. In strain elastography, the longer component (tissue is stiffer when distorted rapidly) as
established of the two, the tissue is displaced, usually by well as porosity (exemplified by pitting edema). Some of
gentle probe pressure, and the way it deforms is measured these might be amenable to elastography approaches. The
by tracking the changes in the speckle pattern using viscosity component results in a spread of the frequencies
conventional ultrasound (Fig. 83.9). Softer tissues displace of shear waves and measuring this is feasible and could
more, harder tissues less, and a color overlay indicating provide a key to evaluating steatosis of the liver.28
their relative stiffness can be created.
Shear waves are transverse, slow-moving waves that
LIGHT AND SOUND
are generated by any body movements. Their speed is
proportional to the tissue’s stiffness. The commonest way If a laser pulse is focused into soft tissue, the local heating
to generate shear waves is to use acoustic radiation force causes expansion which releases an ultrasound pulse that
impulses (ARFIs) with high MI ultrasound push pulses can be detected at the body surface, a process known as
that are akin to those used in Doppler. The resulting photoacoustic imaging.29 Tissue strongly attenuates most
shear waves travel at right angles to the push beam light wavelengths (it is opaque to most colors of light)
and their speed can be measured using conventional but infrared and near-infrared light can penetrate for a
or plane wave ultrasound (Fig. 83.10). The shear wave centimeter or so. It is strongly absorbed by hemoglobin
method has advantages of being applicable in any organ and melanin (endogenous absorbers), and injected
that is ultrasound-accessible (strain elastography is hard dyes can extend these effects. The precision of the laser
pulses gives excellent spatial resolution and both single and heparin can be delivered. This has the potential for
ultrasound transducers (with CT-like repositioning) and continuous administration of these drugs that otherwise
arrays have been used to image blood vessels, melanomas, must be injected, with the expectation of a better-
and myocardial infarctions in small animals. Extension to controlled therapeutic effect. The process also works in
superficial tissues in man seems possible. the opposite direction such that interstitial fluid samples
The complementary approach, acousto-optical ima can be obtained for analysis, for example, of glucose levels.
ging, relies on the fact that the beam of a laser light passing Ultimately, the combination could facilitate a feedback
through a semi-opaque medium such as tissue is diffracted loop to maintain excellent round-the-clock blood sugar
when the material is agitated by a traversing ultrasound control.
beam. A classic implementation is the Schlieren tank used Microbubbles and nanodroplets can have ligands
to visualize the beam from an ultrasound transducer and attached and can carry payloads such as nucleic acids37
the same principle can be used to image tissues and has and chemotherapeutic drugs for targeted delivery, with the
been applied to small animals.30 In an extension of this expectation that tissue side effects would be minimized.
method, the arrival time of a shear wave can be measured, Short of this development, the coadministration of
and if this is performed at more than one location conventional microbubbles and thrombolytic drugs
(e.g. using an array to generate shear waves from different such as tissue plasminogen activator (tPA) and plasmin
locations), the shear wave speed can be measured with has been used with sonication of the relevant cerebral
great precision.31 artery (usually the middle cerebral, but alternatively the
coronaries). Diagnostic ultrasound alone accelerates
THERAPEUTIC APPLICATIONS thrombolysis, but the combination is more effective and
clinical trials are underway.38 Remaining problems are
OF ULTRASOUND the risk of hemorrhagic stroke and the persistence of
Diagnostic ultrasound is used at low powers, partly with ischemic damage to the downstream microvasculature.
the intention of avoiding heating tissue, but if the power In myocardial infarction, this manifests itself as poor
is increased, heating occurs and this has been exploited in myocardial perfusion at the microvascular level despite
physiotherapy. It is also effective for promoting healing of restoration of flow in the main coronary arteries.
bone fractures and skin ulcers.32 The therapeutic uses of microbubbles and derivatives
If much higher powers are used, tissue temperatures seem likely to become more important clinically than their
can be raised to above 55°C and the tissue coagulated.33,34 diagnostic value.
In this approach, a large transducer with tight focusing
concentrates the beam into a volume of about 2 × 10 CONCLUSION
mm; heating takes a few seconds and then the beam is The prospects for continued clinically significant develo
repositioned to the neighboring location and the steps pments in ultrasonography are strong with many inno
repeated across the desired volume that needs to be vations in the offing. The area continues to surprise us with
ablated. The process is known as highly focused ultrasound innovations in diagnostics while therapeutic opportunities
(HIFU) and is widely used, especially in China, to ablate are also on the horizon.
tumors, both benign and malignant as well as for pain
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CHAPTER 84
A Primer on Cardiac MRI for the
Echocardiographer
Madhavi Kadiyala, Aasha S Gopal
Snapshot

QuanƟtaƟve LeŌ and Right Ventricular Assessment 
Velocity Mapping, Flow and Shunt Assessment

Strain Assessment 
Valvular Heart Disease and ProstheƟc Valves

LeŌ Ventricular Structure 
Pericardial Disease

MyocardiƟs and Sarcoidosis 
Normal Variants and Masses

Cardiac Hypertrophy 
LimitaƟons of Cardiac MRI and CT

Cardiomyopathies
INTRODUCTION 3. Cardiac masses and normal structural variants

4. Intracardiac shunts
Significant progress has been made in the area of 5. Pericardial pathology.
cardiac imaging since the days of M-mode and B-mode While cardiac MRI can be very useful in many
echocardiography. Advances in computed tomography (CT) conditions, its value is limited in some situations, for
and magnetic resonance imaging (MRI) technology, along example, patent foramen ovale, small mobile masses such
with three-dimensional (3D) echocardiography enable the as vegetations, calcium, etc. Flow quantitation in valvular
modern cardiologist to make clinical diagnoses with high heart disease is also possible. However, only imagers
accuracy and precision. Until recently, myocarditis was a with expertise in this area should interpret this useful
presumptive diagnosis in a patient presenting with elevated technique due to the many limitations of the methodology.
cardiac troponins and normal coronary arteries. Today, Additionally, there are technical and patient-related
accurate diagnosis of myocarditis and the extent of myocardial factors that influence the image quality, thus affecting the
involvement can be made without invasive procedures using overall utility of cardiac MRI. In most situations, since it is
cardiac MRI. This chapter discusses the practical applications the echocardiographer who recommends MRI imaging, it
and limitations of cardiac MRI in various cardiac conditions. is important to understand the advantages and limitations
A glossary of common cardiac MRI terms is included at the of cardiac MRI.
end of this chapter.
While echocardiographic imaging is the first line of QUANTITATIVE LEFT AND RIGHT
diagnostic testing for most cardiac conditions, cardiac
MRI is valuable, when it is important to assess: VENTRICULAR ASSESSMENT
1. Tissue characterization: myocardial infarction, inflam- Accuracy of echocardiographic quantitation of the left
mation, and fibrosis ventricle is highly dependent on the image quality and
2. Accurate quantitation of cardiac chambers can be highly variable due to uncertainty of the location of
Chapter 84: A Primer on Cardiac MRI for the Echocardiographer 1999
the imaging plane in 3D space. Cardiac MRI is considered Quantitative assessment of the right ventricle conti-
the gold standard for quantitative assessment of the nues to be a challenge with echocardiographic methods.
ventricles due to high accuracy and reproducibility in Due to the complex morphology of the right ventricle,
measuring ventricular volumes and ejection fraction. no single view or imaging plane can provide adequate
The most common method uses a stack of short-axis information for accurate assessment of the right ventricle.
images acquired through the body of the ventricles Although quantitative assessment by 3D echocardiography
(Fig. 84.1). Analysis is performed using the Simpson’s is better that 2D-based methods,3 it is not widely available
method, where the endocardial area from each slice is and the technique needs further refinement before routine
measured and multiplied by the interslice distance. The use. Accurate right ventricular quantitation is particularly
volumes are traced in end-diastole and end-systole, and important in arrhythmogenic right ventricular dysplasia,
ejection fraction is calculated. Automated software packages atrial septal defects, anomalous pulmonary veins, etc.
are available that assist the image analyst in computing It is standard practice to quantitatively determine RV
accurate volumes and function in 10 to 15 minutes. volumetrics by cardiac MRI. The inter- and intraobserver
Recent advances in 3D echocardiographic methods variability by cardiac magnetic resonance (CMR) are very
enable quantitative assessment with less variability com- low4 and allows for serial monitoring of chamber volumes.
pared to two-dimensional (2D)-based methods. However, Similar to the left ventricle, a short-axis stack of the right
the accuracy of 3D echo quantitation is highly dependent ventricle is used for analysis (Fig. 84.1).
on the image quality. In one study, only 22% of clinical
patients had the image quality required for accurate STRAIN ASSESSMENT
analysis.1 When compared to MRI, 3D echocardiographic
methods can significantly underestimate left ventricular The complex myofiber arrangement in the heart allows it
volumes, as much as by 67 ± 45 mL in end-diastole and 41 to be a highly efficient pump, such that only 10% to 15%
± 46 mL in end-systole.2 Technical advances continue to single fiber shortening is required to generate an ejection
improve the spatial and temporal resolution of 3D echo. fraction of 65% to 70%. In the midwall, the myocardial
However at the present time, resolving trabeculations fibers generally have a circumferential layout. The myofi-
by 3D echo is challenging and can lead to inaccuracies, bers become obliquely oriented in the endocardium and
particularly when image quality is not optimal. Addi- epicardium, but in opposite directions, resulting in a per-
tionally, most MRI labs trace the endocardial borders pendicular arrangement of the endocardial and epicardial
including the papillary muscles in the left ventricular fibers. This arrangement leads to a finely orchestrated
pool, exacerbating the difference with 3D echo methods. deformation sequence in circumferential, longitudinal,
In evaluation of patients, it is important to use the same and radial directions. Circumferential shortening is pri-
method of quantitation and not use the different imaging marily due to midwall fiber shortening. Longitudinal left
methods interchangeably. ventricular shortening is the result of contraction of the
Fig. 84.1: Epicardial and endocardial contours are semiautomatically traced on a stack of short-axis slices of the left ventricle in
end-diastole (shown) and end-systole. Endocardial area from each slice is measured and multiplied by the interslice distance. Thus,
accurate end-diastolic and end-systolic volumes are computed and ejection fraction is calculated (EDV – ESV/EDV) and expressed as
a percentage. To obtain a time volume curve of the cardiac cycle, the endocardial contours need to be traced in all phases of cardiac
cycle.
oblique epicardial and endocardial fibers. In general, the specialized sequences, making it difficult to implement
epicardial fiber contraction is the dominant force and the in routine clinical practice. More recently, techniques
endocardial fibers shorten passively in the same direction such as “feature tracking” have been developed (Fig. 84.2)
as the epicardial fibers. Even though the greatest amount that can assess strain on routine cine MRI images.10 Other
of myocardial deformation occurs in the subendocardium, MRI techniques such as strain-encoded (SENC) MRI and
this is driven by the subepicardial fibers. In the setting displacement-encoded (DENSE) MRI are being evaluated,
of ischemia, which initially affects the subendocar- and discussion of these techniques is beyond the scope of
dium, longitudinal shortening would be affected before this chapter.
circumferential shortening and radial thickening and
therefore be a more sensitive marker.5,6 LEFT VENTRICULAR STRUCTURE
Accurate assessment of myocardial deformation has
When compared to echocardiography, cardiac MRI
potential for clinical use; however, reliable measurements
provides higher definition images of the left ventricular
and standardization of the various echocardiographic
cavity and its structure. The papillary muscles, chordal
methods has been challenging.7 MRI-based tagging was
attachments, and apical anatomy are better appreciated.
the first noninvasive technique to reliably assess myo-
This has resulted in increased diagnosis of conditions like
cardial strain8,9 and is considered the gold standard.
left ventricular noncompaction (LVNC).
Tagging involves selective destruction of magnetization in
vertical, horizontal, or grid patterns along the myocardium
and assessing the deformation between these tag lines Left Ventricular Noncompaction
(Movie clip 84.1). In general, tag lines do not persist LVNC is an unclassified cardiomyopathy characterized
through the entire cardiac cycle and assessment of strain by abnormal myocardium with two distinct layers:
in late diastole can be difficult. noncompacted and compacted layers. The natural
The most common method of tagging is spatial modu- history and the prognosis of this condition are not well
lation of magnetization (SPAMM) and software packages established. Several echocardiographic criteria have
are available for semiquantitative analysis. The tech- been proposed to diagnose LVNC. Commonly used
nique can be time consuming and requires acquisition of echocardiographic methods involve assessing the end-
Fig. 84.2: Strain imaging by CMR. The left panel depicts regional myocardial deformation by harmonic phase analysis (HARP) of
tagged images. The green curve corresponds to the septum. The red curve corresponds to the lateral wall. The right panel depicts
regional myocardial deformation by feature tracking analysis of steady-state free precession (SSFP) cine images. Strain analysis of
seven segments in the four-chamber view is shown.
Fig. 84.3: Left ventricular noncompaction (LVNC) in a young patient with recurrent episodes of syncope and history of atrial septal
defect repair as a child. 2D echocardiogram showed mildly reduced left ventricular function but missed the increased trabeculation, due
to suboptimal endocardial resolution. Contrast echocardiography was able to identify the abnormal myocardium, although in less detail
than CT or MRI. CT angiography demonstrated normal coronary arteries and increased trabeculations. Cardiac MRI was diagnostic
of LVNC. Pathological LVNC is often associated with congenital heart disease as in this patient; (CT: Computed tomography;
MRI: Magnetic resonance imaging).
systolic ratio of noncompacted to compacted myocardium diastolic ratio > 2.3:1 rather than the systolic ratio, as
(ratio > 2:1 is significant), and presence of color flow in the the myocardium is relaxed in diastole and it is easier to
deep intertrabecular recesses.11 The number and location discern noncompacted myocardium from compacted
of the trabeculations are taken into account in other myocardium (Figs 84.4A to C).18 MRI studies reveal that
echocardiographic criteria.12 These criteria are considered some degree of noncompaction in the left ventricular
too sensitive by some,13,14 and others suggest that the apex is very common and does not necessarily indicate
echocardiographic criteria may be too strict. The sensiti- pathology. Due to higher contrast resolution and better
vity of cardiac MRI is higher than echocardiography15–17 apical resolution of cardiac MRI, it is not uncommon to
(Fig. 84.3). The cardiac MRI criteria for LVNC use end- find apical trabeculation(Fig. 84.4C). Care should be taken
A B C
Figs 84.4A to C: LVNC: in addition to prominent trabeculations (A), deep recesses (B) are often seen in LVNC; (C) Physiological
hypertrabeculation: note the prominent apical trabeculation in C, which can be seen in healthy subjects. This does not indicate pathology.
to avoid over diagnosis. Petersen et al. found that up to Tissue Characterization

6 ± 3 myocardial segments can be noncompacted in normal
subjects.19 Patients with pathological noncompaction The ability to characterize tissues by using different
(10 ± 3) and dilated cardiomyopathy (DCM) (7 ± 3) often imaging sequences is a feature unique to MR imaging. The
have a greater number of noncompacted segments, sequences that are most commonly used are T1 weighted,
compared to patients with hypertension, aortic stenosis, T2 weighted, fat suppression, and gadolinium-enhanced
or hypertrophic cardiomyopathy (HCM). sequences. Using various MRI sequences (Table 84.1), it
Table 84.1: Signal Intensity (Brightness on the Images) of Various Tissues in the Common Tissue Characterization Sequences
Used in Cardiac MRI
SSFP T1w T2w Fat Suppression First Pass Gadolinium Gadolinium Other
(Perfusion) Enhancement PSIR Sequences
Adipose    Suppressed None 
Fluid transudate    No Δ 
Fluid exudate Int Int Int No Δ 
Hemorrhagic  
(acute) extra
cellular
methemoglobin
Hemorrhagic  
chronic (de-
oxyhemoglobin,
intracellular
methemoglobin)
Thrombus acute/   (low in  on early and
subacute sub acute) late imaging
Thrombus    
(chronic)
Patchy fibrosis   Multiple patchy T1 mapping
foci in hypertrophic techniques
segments (HCM > AS)
Diffuse myocar-  Diffusely increased T1 mapping
dial fibrosis SI irrelative to normal techniques
myocardium
Edema (inflam-   No Δ Patchy Midmyo-
mation/ cardial Subepicardial
ischemia) Non coronary
Infarction/   
necrosis Subendocardial/
Transmural Coronary
distribution
Collagen/amyloid  TI scout
Calcification   No Δ 
Iron  T2* sequence
Vascularity    
(AS: Aortic stenosis HCM: Hypertrophic cardiomyopathy; PSIR: Phase sensitive inversion recovery; SSFP: Steady-state free precession).
Arrows indicate the signal intensity (SI) relative to myocardium. Details of the sequences are found in the text and the glossary.
is possible to reliably identify fat, fluid, thrombus, edema, Myocardial Infarction and
infarction, and inflammation in most situations. First
pass gadolinium imaging is used to assess vascularity and
Viability Assessment
perfusion. Late gadolinium enhancement (LGE) is perhaps MRI with late gadolinium imaging is the gold standard
the most useful sequence for tissue characterization. in the diagnosis and characterization of myocardial
The location, pattern, and intensity of LGE are helpful in infarction. Increased interstitial space due to myocardial
assessing inflammation, fibrosis, infarction, and infiltrative necrosis and fibrosis in the infarcted segment results in
patterns. delayed gadolinium uptake and slow wash out compared
to the healthy myocardium.18 This results in increased correlation of quantitative assessment of infarct mass by
signal intensity of infarcted segments on late gadolinium LGE with positron emission tomography (PET) infarct
images (10–20 min postgadolinium contrast). MRI is highly size.22
sensitive and small subendocardial infarcts (Fig. 84.5)
can be easily detected on MRI due to the higher spatial
MYOCARDITIS AND SARCOIDOSIS
and contrast resolution, even when echocardiography
and SPECT imaging are normal. Histologically confirmed Cardiac MRI is considered the gold standard for the
subendocardial infarcts were detected by LGE-MRI in diagnosis of myocarditis (Fig. 84.7). Tissue characterization
92%, whereas SPECT imaging detected only 28%.20 is useful in determining the presence and duration of
Infarction typically begins in the endocardium and myocardial inflammation. The location is generally in a
progresses toward the epicardium. Subendocardial or noncoronary distribution and can be patchy or diffuse.
transmural involvement in a coronary artery distribution The pattern is often mid-myocardial or subepicardial and
is characteristic of myocardial infarction (Fig. 84.5). can involve the basal lateral wall or septum. In contrast,
Microvascular obstruction (Fig. 84.6) in acute myocardial ischemic pathology usually begins in subendocardium
infarction (MI) is an exception, where there are areas of and progresses to the epicardium. Presence of edema and
hypoenhancement in the subendocardium surrounded by increased capillary permeability is consistent with the
hyperenhancement. This is due to inadequate penetration acute inflammatory process.23
of contrast into the center of the infarction from plugging Myocardial edema associated with inflammation can
of small vessels by thrombus and debris. The transmural be seen as global or regional increase in signal intensity
extent of enhancement correlates with the thickness of (SI) on T2w images. The sensitivity of detecting edema in
the nonviable scar tissue and is related to the extent of acute phase of myocarditis is high (84%) with a specifi-
functional recovery after revascularization. In general, city of 75%.24 Increased capillary permeability is recog-
when there is less than 50% transmural enhancement, nized by increased contrast uptake on early gadolinium
the myocardial segment is considered viable suggesting enhancement (EGE) imaging, which is quantified as at
high likelihood of functional recovery.21 There is excellent least 45% increase in signal intensity on postcontrast
Fig. 84.5: Small recent subendocardial infarction in the circumflex Fig. 84.6: Anteroseptal myocardial infarction (blue arrows) on late
territory (day 3). Increased signal on T2w images is consistent with gadolinium imaging. The red arrowheads point to a large area of
edema. Edema is more extensive than the areas of necrosis seen microvascular obstruction.
on LGE. Echocardiography shows normal regional wall motion.
(LGE; Late gadolinium enhancement).
Fig. 84.7: Tissue characterization in acute

myocarditis. Bright areas in T2w image
indicate edema. Increased signal intensity
in postcontrast T1 images (>45% relative
to precontrast) and EGE images indicate
increased capillary permeability. The area
of enhancement in LGE indicates necrosis/
fibrosis (less extensive than extent of edema).
(EGE: Early gadolinium enhancement;
LGE: late gadolinium enhancement).
Fig. 84.8: Myopericarditis: early and late gadolinium enhancement images both show multiple foci of hyperenhancement (green arrows)
in the myocardium, consistent with myocarditis. There is evidence of pericardial enhancement (blue arrows) indicating pericarditis. This
appears to be limited to the basal pericardium. The pericardium in the mid-sax images does not show enhancement. A moderate sized
pericardial effusion is present (star).
T1 images compared to the precontrast images. Higher be seen over time due to fibrosis and scar contraction
signal intensity in myocardium relative to skeletal muscle, that is smaller than the threshold of the MRI sequences.
postcontrast (ratio > 4) is also indicative of increased Pericardial effusion and associated pericarditis can also be
capillary permeability. Abnormal enhancement on present (Fig. 84.8).
delayed gadolinium imaging is indicative of irreversible In sarcoidosis, myocardial inflammation is chara-
myocardial damage due to either necrosis or fibrosis. cterized by noncaseating granulomas. Cardiac involve-
Delayed gadolinium enhancement occurs when there ment can be seen in up to 30% of patients25 with pulmo-
is expansion of interstitial space, due to necrosis of nary sarcoidosis and when present, requires aggressive
myocardial cells or infiltration. Small areas of necrosis treatment. The presence of multiple foci of intense hyper-
seen in the acute phase of myocarditis may no longer enhancement (Fig. 84.9) in a noncoronary distribution
Fig. 84.9: Cardiac sarcoidosis: intense hyperenhancement in the basal inferolateral wall on late gadolinium enhancement images in
this patient with history of pulmonary sarcoidosis and arrhythmias is suggestive of cardiac sarcoidosis.
(midwall, subepicardial, or transmural) in a patient with hypertrophy (Figs 84.10 and 84.11), indicating areas
sarcoidosis is consistent with cardiac involvement. It is, of fibrosis.26 Myocardial fibrosis or scarring detected
however, difficult to differentiate sarcoid granulomas by cardiac MRI occurs in up to 33% to 86% of patients
from other forms of myocarditis and definitive diagnosis with HCM. It is suggested that these abnormal areas of
requires tissue biopsy. enhancement increase the risk for arrhythmias and sudden
cardiac death.27 Whether patients with extensive areas of
CARDIAC HYPERTROPHY enhancement need prophylactic defibrillators is an area of
active investigation. Abnormal myocardial deformation in
Cardiac amyloidosis, hypertensive cardiomyopathy, the hypertrophied myocardium can be detected on tagged
HCM, storage diseases such as Fabry’s disease can all imaging (Movie clip 84.2).
cause cardiac hypertrophy and may be indistinguish- While there is no specific pattern of hyperenhancement
able from one another by echocardiography. Tissue in Fabry’s disease, midwall hyperenhancement in the basal
characterization by MRI is useful in further evaluation inferolateral wall has been described in some series.28
of these conditions. It is uncommon to find areas of intense hyper-
Echocardiography is usually the reason to suspect enhancement in hypertrophy from pressure overload. A
HCM with or without obstruction. Morphological features mildly increased heterogeneous signal on LGE imaging is
of HCM, particularly the distribution of hypertrophy often noted in hypertensive heart disease, aortic stenosis,
(septal, midwall, or apical) are well characterized on etc. Pressure overload hypertrophy in these conditions
routine cine MRI images. Apical HCM may be missed generally results in diffuse myocardial fibrosis, which is
on echocardiography due to limited apical resolution. difficult to detect visually. Quantitative methods such as
Contrast-enhanced cardiac MRI images often, but not T1 mapping are being investigated to quantitate diffuse
always, show patchy hyperenhancement in areas of fibrosis in these conditions.
Fig. 84.10: Hypertrophic cardiomyopathy: severe hypertrophy of the septal myocardium with markedly abnormal LGE. Patchy areas
of enhancement with multiple foci in the thickened septum are indicative of extensive fibrosis. (LGE: Late gadolinium enhancement).
Fig. 84.11: Apical variant of hypertrophic cardiomyopathy: focal bright enhancement (blue arrows) in the apex on contrast-enhanced
image in a patient with apical HCM. Apical infarctions are also frequently observed due to coexisting microvascular disease. (HCM:
Hypertrophic cardiomyopathy).
Amyloid heart disease has certain unique features by myocarditis and chronic inflammation can eventually
cardiac MRI, allowing for a noninvasive diagnosis without lead to DCM.29 Accurate chamber quantitation is useful
biopsy. There is marked expansion of the interstitial space in the initial diagnosis and monitoring of the progression
due to amyloid deposition resulting in hyperenhancement of DCM. Late gadolinium images can have a variable
especially on early gadolinium imaging. The pattern is appearance in DCM, and in general, is helpful in excluding
frequently diffuse subendocardial, as amyloid deposits ischemic cardiomyopathy. A frequently observed finding
tends to be endocardial initially, but can be more diffuse in DCM is a mid-myocardial “stripe” pattern30 (Figs 84.13A
as the disease progresses (Fig. 84.12). Biventricular and and B) of enhancement, which is not specific for a particular
atrial involvement are often present. The kinetics of etiology. A subepicardial enhancement pattern may also
gadolinium in amyloidosis is unusual due to early wash be present in patients with DCM and myocarditis.31 LGE is
out of gadolinium from the blood pool, as a result of its noted in half of patients with biopsy proven myocarditis;
binding with the amyloid protein in the blood. Anatomic however, an absence of LGE does not indicate absence
features such as biatrial enlargement, thickened valves, of previous myocarditis.32 Cardiac MRI can be helpful
and pericardial effusion are evident on the cine MRI in guiding the endomyocardial biopsy,33 by directing it
images. Amyloid patients have restrictive diastolic toward the pathological segments.
filling, which is best detected by pulsed wave and tissue Myocarditis associated with eosinophilia can occur
Doppler echocardiography. Echocardiography and tissue in a variety of conditions such as eosinophilic myocardial
characterization by MRI are usually adequate to make a fibrosis, Loeffler’s syndrome, Churg-Strauss syndrome,
reliable diagnosis or to exclude cardiac amyloidosis in etc. The pathophysiology involves eosinophilic infiltration
most patients. of the endomyocardium, followed by necrosis and fibrosis
along with thrombus formation. Any form of eosinophilic
CARDIOMYOPATHIES myocarditis can eventually result in fibrotic contraction
MRI offers additional information in the diagnosis of and restrictive cardiomyopathy. Normal ventricular and
cardiomyopathy beyond size and systolic function. The apical contraction with characteristic subendocardial
role of MRI in dilated and other forms of cardiomyopathies enhancement pattern and apical thrombus on late
is discussed below. gadolinium imaging are the hallmarks of this condition34
DCM may occur in many conditions including viral (Fig. 84.14). While restrictive diastolic filling of the
myocarditis, alcoholic cardiomyopathy, hemochromatosis, ventricles can be appreciated on cine imaging in advanced
thyroid disease, familial cardiomyopathy, etc. Most cases, diastolic abnormalities are best detected on pulsed
commonly, DCM is idiopathic and is a diagnosis of wave and tissue Doppler echocardiography.
exclusion. It is believed that mechanisms specific to viral Myocardial iron overload is an occasional cause of
cardiomyopathy and can occur in conditions such as
thalassemia, myelodysplasia, and hemochromatosis.
Iron overload-induced cardiomyopathy is reversible
if intensive chelation therapy is instituted in time. T2*
imaging is a specialized gradient echo sequence that can
be used to estimate myocardial and hepatic iron load.35
MRI T2* times correlate well with myocardial iron levels.
VELOCITY MAPPING, FLOW AND

SHUNT ASSESSMENT
Phase velocity imaging or velocity mapping is an MRI
sequence that is used to assess velocity and flow. In this
sequence, each point of the imaging plane is encoded
Fig. 84.12: Cardiac amyloidosis: diffuse enhancement in both with a phase shift, which is directly related to the velocity
the ventricles and atria (blue arrows) in a patient with cardiac in that pixel. Velocity encoding can be applied in plane
amyloidosis. Also present are pericardial and pleural effusions (in the direction of flow) or through plane to the flow
(orange arrows).
(perpendicular to the flow). Through plane images are
A B
Figs 84.13A and B: “Mid myocardial stripe” pattern of enhancement on late gadolinium images is seen in this patient with dilated
cardiomyopathy.
outflow. Error due to inaccurate estimation of area is

unlikely with MRI as the through plane area is mapped
and traced in each frame of the cardiac cycle. Pulmonic
and systemic flow should be equal in the absence of a
shunt and are generally within 10% of each other.
In general, the measurement of Qp/Qs by MRI is
accurate and reliable.36,37 When estimating flow, it is
important that velocity mapping is performed as close
to the center of the magnetic field as possible. The great
vessels can almost always be positioned in the center. Any
errors in flow calculation tend to cancel out when deriving
a ratio; therefore, the estimation of Qp/Qs is generally very
accurate. This is, however, not the case when using velocity
mapping to assess valvular flow. There are several technical
Fig. 84.14: Diffuse subendocardial enhancement (green arrows) issues that may limit the accuracy of flow quantitation
in the left and right ventricles along with a thrombus (yellow arrow) across the valves. Some of these technical issues can
in the left ventricular apex in a patient with Loeffler’s syndrome. be addressed by paying attention to proper positioning
of imaging planes, applying background correction
typically used for quantitative analysis. Similar to the techniques, and rigorous internal validation with flow
Nyquist limit, a velocity-encoding window is specified, phantoms. It is however important to realize that there are
which determines when aliasing will occur. Generally, limitations of phase-velocity imaging at the present time
the velocity-encoding window is set as close as possible and the data should be interpreted only by experienced
to the estimated peak velocity. This allows for accurate users, who understand the limitations of the method.
flow analysis while avoiding aliasing. Instantaneous flow Technical improvements in velocity mapping methods are
volume can be calculated by measuring the velocity of underway which will allow for reliable routine assessment
all pixels in an area of interest. Integrating the flow of all flow quantitation.
phases of the cardiac cycle yields the flow volume per beat.
Pulmonic (Qp) and systemic flow (Qs) can be calculated VALVULAR HEART DISEASE AND
by measuring through plane flow in the proximal pul-
monary artery and the ascending aorta (Fig. 84.15).
PROSTHETIC VALVES
Echocardiographic estimation of Qp/Qs is often prone to Cardiac MRI allows for comprehensive assessment of
error due to errors in measuring the diameter of pulmonary valve disease, including valvular anatomy, function, flow
Fig. 84.15: Forward flow through the aorta (Qs) and pulmonary artery (Qp). In velocity- mapped images, pixels with velocity in either
direction are represented in grades of black or white (note the opposing colors in the ascending and descending aorta), whereas
stationary objects are represented in gray. The area under the curve is integrated to obtain the flow in either direction.
quantitation, as well as provides accurate quantitative It has to be noted that the temporal resolution
analysis of the left and right ventricles. The assessment of velocity mapping sequences (25–45 ms) is much
of the ventricles is important to understand the effect of lower than that of Doppler echocardiography (10-fold
the valvular pathology on the heart and to detect higher temporal resolution) and generally results in
progression of pathology in serial evaluation of a patient. underestimation of peak velocities. In addition, magnetic
The role of MRI in the more common valvular conditions field inhomogeneities and eddy currents can result
is discussed below. in variability in measurements. For this reason, direct
planimetry of aortic valve area by is preferred over the
Aortic Stenosis continuity method by MRI.
In general, direct planimetry of stenotic valve area is the
preferred method of assessing severity of aortic stenosis Aortic Regurgitation
by MRI. Valve area can be measured in either cine images Regurgitant jets are seen as flow void on steady-state free
or in through plane velocity-mapped images and has been precession (SSFP) images due to turbulence. There is a
shown to correlate well with Doppler echocardiography modest correlation between the width of the jet and the
and catheterization measures of severity.38,39 A cross- size of flow void with severity of regurgitation. However,
sectional image through the leaflet tips in systole obtained severity is often underestimated when using SSFP
by carefully aligning two perpendicular left ventricular sequences. It is often difficult to detect small amounts
outflow tract (LVOT) views is used to calculate the valve of aortic regurgitation and may be missed by SSFP
area (Fig. 84.16). Peak and mean velocities can be obtained imaging. Quantitative assessment by velocity mapping
by velocity mapping. Through planes that are perfectly is recommended for assessment of significant aortic
perpendicular to the stenotic jet at the tips of aortic leaflets regurgitation.
can be obtained, even in the setting of angulated aortic Quantitative echocardiographic assessment of aortic
roots. regurgitation (AR) can be challenging due to difficulty in
It is also feasible to calculate aortic valve area by MRI visualizing the vena contracta and proximal convergence
using the continuity method similar to echocardiography. of AR jet. Assumptions involving mitral annular area
Through plane velocity maps of the LVOT and planimetered calculation limit the use of the continuity equation in
area of LVOT are obtained by obtaining a perpendicular assessing AR. In contrast quantitative analysis by cardiac
plane through the LVOT and peak velocity obtained at the MRI, velocity mapping is relatively straightforward.
leaflet tips is used to calculate the aortic valve area. Through plane flow is measured (Fig. 84.17) above the
Fig. 84.16: Systolic frames of the aortic valve in long- and short-axis images are represented. The flow is in plane in long axis and
through plane in short axis. Aortic valve area can be traced by planimetry in either standard cine (SSFP) images or phase image
(as shown). In this example, it is easier to trace the contours on the phase image compared to the SSFP image. Respiratory artifacts
and dark signal from calcification render the SSFP image technically difficult to trace. (SSFP: Steady-state free precession).
aortic valve and below the level of the coronary artery ostia,
after carefully selecting the plane using two perpendicular
longitudinal views of the ascending aorta. Forward and
reverse flows are quantitated and regurgitant fraction is
calculated as follows.
Regurgitant fraction (%) = Aortic retrograde flow
(mL/beat) × 100 Aortic forward flow (mL/beat).
The regurgitant volume may be underestimated by
velocity mapping due to the motion of the valve plane
during the cardiac cycle; however, interstudy reproduci-
bility is high and the method is useful for long-term patient
follow-up.40 Quantitative assessment of left ventricular
volumes and function are equally important in long-term
follow-up. In the absence of other valvular regurgitation,
Fig. 84.17: Quantitation of aortic regurgitation: flow below the
the difference between left and right ventricular stroke baseline is forward flow in systole. Flow above the baseline repre-
volumes should equal the amount of aortic regurgitation sents reverse flow. In this example, there is holodiastolic reverse
and this can be used to quickly corroborate the velocity flow consistent with significant aortic regurgitation. The calculated
regurgitant fraction is 39%.
mapping analysis.
Mitral Valve gitation, ventricular volumes, and function. Quantitative

analysis of mitral regurgitation is performed as follows:
When assessing mitral valve with cardiac MRI, standard
In the presence of single valve regurgitation:
long-axis SSFP views are generally adequate for assessing
Regurgitant volume is the extra stroke volume (SV) the
the motion and function of mitral valve. Systematic
affected ventricle has to pump.41 Stroke volumes are
evaluation of individual segments of mitral valve can
calculated by quantitative analysis of the left and right
be done to identify prolapsed segments, when there is a
ventricles at end-diastole and end-systole
specific question of an anatomical abnormality (Fig. 84.18).
The resolution is, however, inferior to transesophageal (SV = EDV – ESV)
echocardiography, such that the anatomic detail is less Mitral regurgitant volume = LVSV – RVSV (LVSV: Left
well appreciated. This is generally true with cardiac MRI ventricular stroke volume; RVSV: Right ventricular stroke
sequences, when assessing any thin structures that move volume)
rapidly like valve leaflets. In the setting of mitral regurgitation, the left ventricle has
to eject a higher stroke volume as it is now pumping into
Mitral Regurgitation both the aorta and the left atrium in systole.
The major contribution of cardiac MRI in the setting of In the setting of other coexisting valve regurgitation:
mitral regurgitation is quantitative assessment of regur- Mitral regurgitant volume = LVSV – Aortic forward flow
Fig. 84.18: Multiplane cine MRI images in a patient with posterior mitral valve prolapse and anterior regurgitant jet. By prescribing
multiple planes through a short-axis image of the mitral valve (last frame), it is possible to accurately localize the pathology.
(MRI: Magnetic resonance imaging).
In systole, the left ventricle ejects into the aorta and and flow calculations are technically feasible, they are
the left atrium. Aortic forward flow is calculated by the routinely not performed.
velocity mapping method. Subtracting this forward
volume from the left ventricular stroke volume yields the Tricuspid Regurgitation
mitral regurgitant volume. This method holds true even in
the setting of coexisting aortic and tricuspid regurgitation. Tricuspid valve anatomy can be assessed in the standard
It is also possible to directly measure the mitral inflow long-axis views. Qualitative assessment of the tricuspid
by through plane velocity mapping of the mitral annulus regurgitant (TR) jet can be difficult because of less
(Fig. 84.19). Subtracting the aortic forward volume from prominent signal void in the setting of lower velocity
the mitral inflow volume yields the mitral regurgitant in the right ventricle (Figs 84.20A and B). Quantitative
volume. However, the excessive motion of the mitral analysis is useful and is performed using the same
annulus during the cardiac cycle results in variable principles as that of mitral regurgitation. The most
inflow quantitation and is not the preferred method of common method is
quantitation. Although not routinely performed, it is Tricuspid regurgitant volume = RV stroke volume –
feasible to evaluate the pulmonary venous flow pattern to Pulmonary forward flow (mL/beat).
further assess severity of MR. As each additional sequence Quantitation of right ventricular volumes and function
adds to the imaging time, this is not routinely performed. is important in the evaluation of the severity of TR and for
long-term follow-up.
Mitral Stenosis
In general, echocardiography is the preferred method
Prosthetic Valves
of assessing the anatomy and function of the mitral Despite the prevalent misconception that prosthetic valves
valve in mitral stenosis. By prescribing through planes are a contraindication to cardiac MR imaging, almost
perpendicular to the mitral leaflets it is possible to perform all prosthetic valves can be safely imaged at strengths of
direct planimetry of the mitral valve orifice. While velocity 1.5 tesla. It should be remembered that the mechanical
Fig. 84.19: Top panel demonstrates estimation of mitral inflow at the level of the mitral annulus. The area under the curve in diastole
yields the mitral inflow. Bottom panel depicts the flow pattern in the left pulmonary vein. The flow is systolic dominant.
A B
Figs 84.20A and B: Tricuspid regurgitation. The severity of valvular regurgitation is usually underestimated by SSFP sequences,
particularly in the case of right ventricle, where the regurgitant jets are of lower velocity. (SSFP: Steady-state free precession).
forces of the cardiac pump have a much stronger effect on

the prosthetic valve compared to the weak magnetic forces
generated by MRI. Visualization of the actual prosthesis is
often limited by the metallic artifact (Fig. 84.21); however,
this is variable and sometimes excellent imaging is
possible. Evaluation of the prosthetic valves is otherwise
similar to native valves and quantitative flow using velocity
mapping and volumetric analysis of the ventricle is valid in
assessing prosthetic valve function.
PERICARDIAL DISEASE
Echocardiography is the primary imaging modality to
evaluate pericardial disease; however, MR imaging offers
additional information that can help in clarification
of pathology and management. Cardiac MRI methods
allow for excellent tissue characterization, understanding
pericardial function, as well as presence of adhesions.
Normal pericardium is 1 to 2 mm thick (Figs 84.22A
and B) and is generally visualized as a layer of low signal
intensity on dark blood and SSFP images and is often Fig. 84.21: Normally functioning bioprosthetic aortic valve.
Artifacts from the bioprosthetic struts are noted. The bioprosthetic
better visualized along the right ventricle, where there is
valve is noted to open normally in systole. Phase velocity images
usually adipose between right ventricular free wall and the can be done in plane (three-chamber) and through plane (sax) to
pericardium. Tagged imaging can be helpful in assessing assess flow. Peak velocity can be calculated from through plane
“slippage” of the pericardium. In normal subjects, as phase velocity images.
the myocardium contracts and relaxes, it slides past the
adjacent pericardium and slippage is clearly visualized distribution. Clear distinction is possible by MR imaging
(Movie clip 84.3). In the setting of adherent pericardium, owing to the different signal characteristics of fat and fluid
the “slippage” sign is absent (Movie clip 84.4). (Fig. 84.23). Additionally, when assessing for pericardial
Differentiation of pericardial effusion from epicardial effusion, MR imaging allows accurate determination of the
fat can be difficult at times on echocardiography, size and extent of the effusion. The type of effusion can be
particularly if the adipose is extensive and has an atypical reasonably determined by assessing the signal intensity of
A B
Figs 84.22A and B: Normal pericardium is identified by a layer of low signal between the layers of epicardial fat which has bright signal
on cine and LGE images. Pericardium along the right ventricle is better visualized.
Fig. 84.23: Pericardial cyst is noted adjacent to the left ventricle (). Fluid has bright signal on SSFP images and has a higher signal
on T2w (T2 > T1) images and short tau-inversion recovery (STIR) sequences. On phase sensitive inversion recovery LGE sequence,
fluid appears dark. In contrast, pericardial fat (blue arrows) has bright signal on SSFP, T1 weighted, and LGE images. Additionally,
signal from fat is suppressed on fat suppression and STIR sequences. (LGE: Late gadolinium enhancement; SSFP: Steady-state free
precession).
the effusion in various sequences. Moreover, tagged images definitive diagnosis of constrictive pericarditis based on
may be helpful in assessing the fluidity and composition. cardiac MRI findings alone. However, the demonstration
For example, in the setting of free-flowing fluid, tag lines of thickened irregular pericardium and/or adhesions on
fade away quickly within the effusion (Movie clip 84.5). tagged imaging by cardiac MRI can corroborate the clinical
On the other hand, when the effusion is organized or is and echocardiographic findings to make an accurate
less fluid, the tag lines tend to persist longer. The effects diagnosis. It should be remembered that constriction can
of the effusion on the cardiac chambers such as chamber also occur in the absence of pericardial thickening. In this
collapse and abnormal septal motion can be appreciated situation, cardiac MRI is of little value.
on cine images similar to echocardiography. Importantly,
the presence or absence of pericardial inflammation NORMAL VARIANTS AND MASSES
can be determined (see Fig. 84.8) using cardiac MRI Due to its superior spatial resolution and tissue
sequences. In the setting of active inflammation, there is characterization, cardiac MRI is often used to evaluate
increased signal on T2w images and hyperenhancement cardiac masses. A full description of cardiac tumors and
on gadolinium-enhanced images. The presence of any their tissue characteristics is beyond the scope of this
concurrent myocarditis can also be assessed. Pericardial chapter. Besides tumors, normal cardiac structures can also
thickening may or may not be present in pericarditis and if produce “mass” like appearance on echocardiographic
thickened, may have an irregular appearance. images. Cardiac MRI is helpful in these situations to
Chronic inflammation of the pericardium results in confirm the benign nature of these “masses” avoiding
pericardial fibrosis, thickening, and occasional calcifi- further invasive procedures. Extracardiac adipose tissue is a
cation eventually leading to constriction and impaired frequent cause of a cardiac mass on echocardiography and
diastolic filling of the left ventricle (Figs 84.24A and B, is clearly discerned by cardiac MRI (Fig. 84.25). Epicardial
Movie clip 84.6). Typical features of constrictive peri- fat can be difficult to differentiate from pericardial effusion
carditis such as tubular elongated appearance of the left by echocardiography, particularly in postoperative
ventricle and diastolic flattening of the septum can be setting. Using tissue characterization techniques such
easily appreciated on SSFP imaging. Paradoxical septal as “fat suppression,” the true nature of the masses can
motion and variation with respiration can be visualized on be identified. Occasionally, right atrial masses seen on
real time imaging sequences. However, it should be kept echocardiography are due to hypertrophic Eustachian
in mind that diastolic function and respiratory variation valves and crista terminalis and their benign nature can be
of inflow are easier to determine with Doppler and readily identified with MR imaging (Fig. 84.25 and 84.26).
tissue Doppler echocardiography than with cardiac MRI. In addition to “fat suppression” sequences, which can
Echocardiographic assessment is essential to confirm detect the presence of adipose tissue, perfusion sequences
constrictive physiology and it is not advised to make a are helpful to determine the vascularity of a mass.
A B
Figs 84.24A and B: Pericardial thickening can be clearly seen on dark blood images along the left ventricle in this patient who had
features of constrictive pericarditis (Movie clip 84.6).
Fig. 84.25: This patient was referred for cardiac MRI for evaluation of multiple right atrial masses on an echocardiogram. There is
extensive amount of epicardial fat that wraps around the right atrium along with lipomatous hypertrophy (blue arrows). Epicardial fat
in the tricuspid annulus often appears as right atrial mass. In addition, there is a prominent eustachian valve (orange arrows). The
eustachian valve is a normal atrial structure, but can be mistaken for a mass, when it is enlarged. A clue for identifying this correctly is
its location at the ostium of the inferior vena cava.
Cardiac thrombi occur more commonly than a higher likelihood of malignancy. There is significant
tumors. Contrast cardiac MRI is particularly useful in overlap of tissue characteristics of the various types of
identifying thrombus. Thrombi are characterized by lack tumors, such that specific tissue diagnosis is not made
of enhancement on first pass imaging, early, and LGE based on cardiac MRI findings alone. Cardiac MRI is
(Fig. 84.27). Intracardiac thrombi generally occur in extremely helpful to evaluate the initial tumor burden and
relation to infarcted segments and atrial appendages extent, as well as in the follow-up of tumors.
(atrial fibrillation patients).
The morphological features, location, presence of LIMITATIONS OF CARDIAC MRI AND CT
adipose tissue, vascularity, and appearance on LGE
images are helpful in characterizing cardiac masses Cardiac MRI is a powerful diagnostic tool in the cardio-
(Fig. 84.28). While there are no specific diagnostic features logist’s armamentarium. However, it is important to
of malignancy, the presence of multiple lesions in multiple recognize the limitations of this modality. Patients with
chambers, pericardial involvement, signal heterogeneity, pacemakers and defibrillators cannot generally have an
areas of hemorrhage, necrosis, hemorrhagic pericardial MRI. Cardiac MRI studies are usually long studies requiring
effusion, or infiltration into other tissue layers indicate patients to be supine and hold their breath. It is difficult to
Fig. 84.26: The mass noted in the right atrium on the echocardiographic images was demonstrated to be hypertrophic crista-terminalis
(blue arrows) and eustachian valve (orange arrows). Notice the thickened crista along the posterior wall of the right atrium that extends
from the inferior to the superior vena cava. This is well characterized on the serial cine planes through the atria.
Fig. 84.27: An unusually large thrombus seen in a young female patient with diabetes, peripheral vasculopathy, nephrotic syndrome
admitted with sepsis. Echocardiographic images in the upper panel demonstrate a large mass attached to the anterior wall, suspicious
for thrombus. There was severe biventricular dilation and systolic dysfunction. The absence of enhancement on perfusion and late gado-
linium enhancement is consistent with thrombus. Additionally, there was diffuse hyperenhancement of the myocardium and abnormal
gadolinium kinetics consistent with cardiac amyloidosis.
Fig. 84.28: Papillary fibroelastoma on the tricuspid valve. Note the low signal of the mass on the cine MRI (SSFP) images due to partial
volume effects. There is no evidence of fat suppression or perfusion. (MRI: Magnetic resonance imaging; SSFP: Steady-state free
precession).
obtain satisfactory studies in patients who are unable to low-velocity flow across a small patent foramen ovale
lie supine, are claustrophobic, or uncooperative. While it (PFO) and cardiac MRI should not be used for the
is possible to sedate patients, the images are generally less diagnosis of PFO. Color Doppler and agitated saline
than optimal in this situation. Most cardiac MRI images contrast echocardiography is the preferred diagnostic
are segmented, that is, averaged over a few cardiac cycles. test. Similarly, when imaging valvular vegetations and
In the setting of arrhythmias with irregular heart rhythms small mobile masses by cardiac MRI, it should be kept
(e.g. atrial fibrillation, frequent ectopy), the image quality in mind that highly mobile small masses are not well
is suboptimal. imaged due to the lower temporal resolution and partial
While the image contrast and tissue characterization of volume effects. Transesophageal echocardiography is
MR imaging is better than echocardiography, the temporal the preferred method. Another consideration is imaging
resolution is much lower (most MR sequences have 20–40 calcific structures. In general, calcium has no signal on MR
frames/s compared to echocardiography, which can sequences due to lack of water content and is always dark
image in the range of 30–100 frames/s). The slice thickness on all sequences. Calcification is best imaged on X-ray or
of MR images is usually 5 to 8 mm and the images have CT imaging.
partial volume effects (Fig. 84.28). Generally, slices thinner
than 5 mm do not have adequate signal and are not used. CONCLUSION
These limitations may be overcome by improvement in
MR technology and development of newer sequences. Cardiac MRI has become a standard diagnostic modality
Cardiac MRI is extremely helpful in many conditions; in today’s cardiology practice, although availability of this
however, technical considerations may limit its value technique is still limited to a few major centers. It is the
in some situations. For instance, neither SSFP nor flow gold standard in the determination of cardiac volumes
sequences are sensitive or specific enough for detecting and evaluation of right ventricle, particularly when
echocardiographic visualization is not adequate. Tissue Flow Imaging (Phase Velocity/Velocity Mapping)
characterization by cardiac MRI is extremely useful in
the diagnosis of myocardial infarction, myocarditis, This sequence is used to assess velocity and flow. Each point of
the imaging plane is encoded with a phase shift, which is directly
cardiomyopathies, and cardiac masses. It is important for
related to the velocity in that pixel. Velocity encoding can be
the cardiologist and the cardiac imager to understand the
applied in plane (in the direction of flow) or through plane to the
unique advantages and limitations of cardiac MR imaging.
flow (perpendicular to the flow). This is helpful in determining
Qp/Qs and also in assessing valvular function.
GLOSSARY OF CARDIAC MRI SEQUENCES
Cine Imaging Contrast CMR (Gadolinium)

First pass imaging (perfusion): Images are acquired in rapid
Steady-State Free Precession (SSFP) succession during the first pass of intravenously injected
gadolinium. This sequence can be used to assess qualitative and
This is the work horse cardiac MRI sequence. Images have a very
quantitative myocardial perfusion. First pass imaging is also
high signal-to-noise ratio and excellent contrast between the
useful in assessing vascularity of cardiac masses.
myocardium and blood. This is a gated sequence acquired over
several cardiac cycles with final display being an average of the Late gadolinium enhancement (LGE)/phase sensitive inversion
collected data. Respiratory motion and arrhythmia can degrade recovery (PSIR): This is a T1-dependent inversion recovery
the image quality. sequence that highlights increased interstitial space, where
gadolinium accumulates and has a delayed washout. Gadolinium
decreases T1 relaxation time and when delayed images are
Real Time imaging
obtained 10 to 20 minutes after gadolinium, the contrast between
This is a nongated imaging technique used in the setting of the healthy myocardium and abnormal myocardium (e.g.
arrhythmia or respiratory artifacts; however, image quality is infarction, inflammation, fibrosis, infiltration) is at its maximum.
inferior compared to SSFP images. The PSIR sequence further increases the contrast of the LGE
sequence.
Dark Blood Imaging (Spin Echo) Early gadolinium enhancement (EGE): EGE is performed
using the same T1 inversion recovery sequence as in LGE,
Spin echo sequences yield high quality images at a given but performed earlier (<10 min after gadolinium). It is useful
time point in cardiac cycle and are generally obtained as for imaging increased capillary permeability in myocarditis,
dark blood images. These sequences are very useful for tissue amyloidosis, and thrombus imaging.
characterization.
T1 weighted: In this sequence, tissues with short T1 relaxation
time appear bright: Example: adipose tissue (TI ~ 200 ms). Signal
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CHAPTER 85
Cardiac CT Imaging
Satinder P Singh, Sushilkumar K Sonavane
Snapshot

Challenges for Cardiac Computed Tomography 
Coronary Plaque

RadiaƟon Dose 
PrognosƟc InformaƟon from Coronary Computed

PaƟent SelecƟon Tomography Angiogram

Technique 
Cardiac FuncƟon

Image Postprocessing 
Myocardial Perfusion

Image Analysis 
How to Improve Accuracy of Computed Tomography

Piƞalls and ArƟfacts Angiogram in Determining Flow LimiƟng Disease

DiagnosƟc Accuracy of Coronary Computed 
Clinical IndicaƟons
Tomography Angiogram
INTRODUCTION such as esophagitis, pneumonia, pulmonary embolism,

aortic dissection, as well as chest wall abnormalities can
In the 1980s, the electron beam computed tomography also be evaluated in the same setting. In the last 4–5 years,
(EBCT) was introduced and was able to freeze cardiac we have also seen dramatic improvement in postprocessing
motion with a temporal resolution (TR) of 50 ms. EBCT and segmentation capabilities due to the availability of
was used extensively for early coronary artery calcium powerful computers and their supercomputing powers.
(CAC) evaluation. However, its role for CT angiography was Coronary artery disease (CAD) is a leading cause of
limited due to excessive noise and poor spatial resolution mortality and morbidity in industrialized nations. The
(SR) from 3 mm to 4 mm thick images. Multidetector management decisions often depend upon accurate
computed tomography (MDCT), which was introduced evaluation of coronary artery lumen. Catheter coronary
in the late 1990s, has rapidly evolved over the past few angiography is considered the gold standard for coronary
years. A 64-MDCT is capable of providing TR of 165 ms arterial evaluation due to its superior temporal and SR.
(85 ms with dual source scanner), SR of 0.4, slice thickness Although not insignificant, there is a complication rate
of 0.6, and gantry rotation time of 330 ms or even less of about 3.6% and a mortality of 0.1% associated with
(Table 85.1). MDCT can provide information about this procedure.1 It gives accurate luminal information
coronary artery patency, coronary calcium, left ventricular but lacks information about vessel wall and presence and
(LV) function, and to some extent even myocardial type of plaque. Coronary computed tomography (CCT)
perfusion. In addition, extracardiac sources of chest pain angiography not only provides anatomical information
Table 85.1: Comparison of Spatial Resolution (SR) and Temporal Resolution (TR) for Different Modalities
Modality Spatial Resolution (SR) [mm] Temporal Resolution (TR) [ms]
Electron beam computed tomography (EBCT) > 0.6 50–100
16-Multidetector computed tomography (MDCT) 0.5 250–400
64-MDCT 0.4 165–250
Dual Source 0.4 83
Catheter Angio 0.2 5–20
Cardiac magnetic resonance imaging (CMRI) 0.7 20
regarding the coronary artery lumen and presence of on the type of material used in the detectors. The SR of
stenosis but also information regarding the vessel wall CT is excellent at around 0.4–0.5 mm and is the primary
and remodeling not seen on conventional catheter strength of CT (Table 85.1). For most practical purposes,
angiography. Due to its near-isotropic image resolution, this resolution currently is good enough to evaluate larger
cardiac CT is well suited to evaluate complex congenital epicardial coronary arteries and their branches. However,
heart diseases (CHDs). It provides information about to optimally evaluate calcified smaller coronary artery
cardiac chambers, valves, pulmonary and coronary veins, branches or patency of coronary artery stents, a SR of
and any extracardiac pathology in the mediastinum/hilum 0.2 mm or less is required. Higher SR also increases image
or visualized lungs. noise and to maintain sufficient signal to noice ratio
Advantages of cardiac CT include its noninvasive (SNR), radiation exposure needs to be increased. Flat
nature, excellent SR, good TR, excellent patient accep- panel volume CT scanners have much higher SR but their
tance, wide availability, simple and fast technique, and no contrast and TR are much inferior to current MDCT.
contraindication to existing hardware.
Temporal Resolution
CHALLENGES FOR CARDIAC
TR of a CT scanner is determined by the speed of rotation
COMPUTED TOMOGRAPHY of the gantry. Since images may be reconstructed from
Small size of coronary arteries, complex cardiac anatomy, a 180° rotation rather 360° rotation, the TR is equal to
and cardiac motion are the three main challenges for CT half the gantry rotation speed. The current generation of
evaluation of heart and coronary arteries. Several factors scanners has a very fast gantry rotation time of 0.27–0.30
defining CT performance include: (a) Volume coverage ms. Excessive mechanical forces and G forces are the
per second, defined by detector coverage, pitch, and limiting factors in further increasing the gantry rotation.
gantry rotation time; (b) SR defined by detector size in Two possible ways to improve TR are multisegment
longitudinal direction, scan field of view (FOV) and image reconstruction and use of dual source CT scanner.
matrix in axial plane, and slice reconstruction increment; Multisegment reconstruction selects small portions of
(c) TR defined by gantry rotation time and reconstruction projection data from various heart cycles and combines
method. all projections to obtain sufficient data for image
reconstruction (TRmax = TR/2 × M). However, variable
Spatial Resolution heart rhythm can cause image degradation due to
misregistration. Dual source CT design uses two X-ray
It refers to the ability to resolve as separate forms, small tubes and two detectors mounted on the gantry with a 90°
objects that are very close together. Submillimeter SR with angular offset and leads to high TR of 83 ms (one-fourth
isotropic imaging (i.e. equal resolution in all three planes) rotation time; Table 85.1).
is desirable to delineate small coronary artery branches.
The SR in CT depends on the size of the three-dimensional
pixels (voxel) in the image as seen on the monitor. The
Cardiac Gating
smaller the size of voxel the less partial volume averaging Electrocardiography (ECG) gating of the tomographic
and better SR. The voxel size depends on the resolution of images is commonly performed in cardiac CT. Gating
the X-ray sensors and the focal spot size. It may also depend not only minimizes cardiac motion artifacts and allows
Chapter 85: Cardiac CT Imaging 2025
localization of an image to a particular phase of cardiac RADIATION DOSE

cycle but also improves visualization of smaller structures
(Figs 85.1A and B). Both prospective and retrospective X-Rays have been classified as carcinogens by WHO and
electrocardiography (ECG) gating are available. In CDC. Per-capita radiation dose from clinical imaging
prospective gating, imaging is done in axial scan mode at exams in the United States increased almost 600% from
a predetermined interval from the preceding R-wave, and 1980 to 2006. Globally, 93 million CTs per year are done at
usually image acquisition occurs in late diastole when the 16/1,000 persons rate, which includes 58 million CTs yearly
cardiac motion is minimal. Images are obtained every in the United States and 3 million CTs performed annually
other heart beat with table moves in between; therefore, in children < 15 years. CT scans deliver almost half of the
such an acquisition is also known as step and shoot mode. estimated collective radiation dose in the United States.9,10
Since radiation exposure occurs only for a short period in In an article published in the New England Journal of
diastole, this reduces the radiation exposure to the patient. Medicine, the authors Brenner and Hall predicted that in
Prospective gating can only be done in patients with low a few decades, 1.5–2% of all cancers in the United States
and regular heart rates, and since no systolic information may be due to the radiation exposure from CT scans
is gathered, cardiac functional analysis cannot be being done now.11 They used dose-response information
performed. In retrospective gating, imaging is done in from the atomic bomb survivors to calculate the risk for
helical mode and continues throughout the cardiac patients undergoing CT procedures. This claim has been
cycle. Therefore, the radiation exposure to the patient disputed by American College of Radiology (ACR) using
is significantly higher. Since both diastolic and systolic the following arguments: (a) CT exams result in limited
data are captured, functional analysis of the CT data can radiation exposure to the body parts, whereas the atomic
be performed to determine ejection fraction (EF), stroke bomb survivors experienced instantaneous radiation
volume, and ventricular volumes. The accuracy of left and exposure to the whole body; (b) CT exams expose patients
right ventricular function by MDCT has been validated solely to X-rays, while atomic blast survivors were exposed
with echocardiography and magnetic resonance (MR).2–4 to X-rays, particulate radiations, neutrons, and other
In addition, qualitative assessment of regional LV function radioactive nuclei. Therefore, the known biological effects
is performed by visualizing changes in wall thickness are very different for these two scenarios. Nonetheless,
using a cine loop display of multiple cardiac phases ACR supports the “as low as reasonably achievable”
(10–90%). Regional wall-motion abnormalities are also (ALARA) concept and urges providers to use the minimum
shown to correlate with MR and echocardiography.5–8 level of radiation needed in such exams to achieve the
A B
Figs 85.1A and B: (A) Nongated versus (B) Gated axial computed tomography (CT) image. Gated image showed minimal cardiac
motion related artifact, which helps in visualization of small size structures such as coronary arteries. (AO: Aorta; LAA: Left atrial
appendage; PA: Pulmonary artery).
necessary results. The typical radiation dose exposure Methods to Reduce Radiation
from commonly performed procedures is show in
Table 85.2.
Exposure (Table 85.4)
Two radiation parameters are available from the • Individualized protocols: The technique of computed
CT study—the CT dose index volume (CTDIvol) and tomography coronary angiography (CTCA) is
the dose length product (DLP). The DLP is obtained by particularly amenable to “dose optimization” by
CTDIvol multiplied by the total scan length. Effective tailoring the scan protocol to the individual patient.
dose, measured in milliSieverts (mSv), is calculated by Adjustments are made to the tube current (mAs)
the DLP multiplied by tissue weighing factor (k) that takes and tube voltage (kVp) according to patient size and
into account the relative sensitivity of a particular body anatomical shape. The range of mAs (400–800) and
region. For cardiac imaging, the most commonly used kVp (80–120) are utilized in our practice.
k-factor is 0.014. It reflects the relative risk from exposure – The CT radiation dose changes in proportion of
to ionizing radiation and accounts for the characteristics square of the tube voltage. Thus, small changes in
of exposed tissues. The effective radiation dose in mSv tube voltage have more impact on the radiation
allows us to compare different forms of radiation such as dose. In the PROTECTION I study, the use of the
X-rays and isotope exposures in nuclear medicine studies 100-kV tube voltage protocol was associated with
(Table 85.3). 53% reduction in radiation dose, when compared
Table 85.2: Typical Effective Doses of Common Examinations

Modality Effective Radiation Dose (mSv)
Chest radiograph (CxR) PA/Lateral 0.02–0.04
Mammogram 0.4
Head computed tomography (CT) 1–3
Chest computed tomography angiogram (CTA) for (pulmonary
thromboembolism (PTE) 8–10
Abdomen CT routine 10–12
Multiphase abdomen/pelvis CT 22–34
Catheter Angio 3–7
SPECT (7–9) Rest or Stress only
(18–23) Rest and Stress
Coronary CTA 6–13 (retrospective)
1–5 (prospective), < 1 (High Pitch)
Thallium 201 21–23
Trans Atlantic Flight 3.0
Average US background 3.6–4.5
Table 85.3: Computed Tomography Radiation Dose Descriptors

Volume computed tomography (CT) dose index CT dose index volume (CTDIvol) [mGy]
Average dose within the scan volume (CTDI/Slice)
Dose length product Dose length product (DLP) [mGy.cm]
Integrates CTDI vol over the scan length (DLP/Scan)
Reflect biological effects attributable to a complete scan acquisition
Effective dose E (mSv)
Table 85.4: Methods to Reduce Radiation Dose in Cardiac PATIENT SELECTION

Computed Tomography
The revised 2010 appropriate use criteria for cardiac CT
Choose correct protocol for a given question
were published in 2010.20 Use of noncontrast CCT for
kVp: 80, 100, 120, 140
calcium scoring is rated as appropriate in intermediate
mA: Lower the better and selected low-risk patients. CCTA is considered appro-
Dose modulation priate in: (a) Patients with suspected anomalous coronary
Iterative reconstruction (ASIR, iDose, SAPHIRE, VEO) artery, symptomatic patients with low to intermediate
High pitch computed tomography angiogram (CTA; only pretest probability for CAD who have normal ECG and
applicable with dual source scanners) cardiac biomarkers, uninterpretable or nondiagnostic
ECG or equivocal biomarkers; (b) New onset heart failure
with reduced left ventricular ejection fraction (LVEF) in
with the conventional 120-kV scan protocol.12
low to intermediate probability for CAD and preoperative
It is important to reduce both tube current and
coronary assessment prior to noncoronary cardiac surgery
more so the tube voltage whenever possible while
and intermediate pretest probability; (c) Patients with
maintaining the image quality. At our institution prior equivocal stress testing or when there is discordance
we try to apply 100 kVp to patients with body mass between the stress test and clinical suspicion for CAD;
index (BMI) < 26. (d) Evaluation for bypass patency after coronary artery
• FOV: Since the radiation dose is directly proportional bypass graft (CABG) and prior left main (LM) coronary
to the scan length, restriction of FOV in Z-axis to the stenting in asymptomatic patients; (e) Cardiac structure
heart, just above origin of coronary arteries to just and function category, with appropriate indications
below the inferior wall of the left ventricle is of vital including coronary anomalies, CHD, evaluation of right
importance. Preceding calcium scan if done can be ventricle (RV) function, evaluation of LV function when
used as a reference to optimize FOV for computed imaging from other modalities is inadequate or evaluation
tomography angiogram (CTA). In cases where there are of prosthetic heart valves; (f ) Preablation pulmonary valve
no calcium scans done, we start just below the carina. (PV) mapping or prior to redo sternotomy in reoperative
This limits excessive exposure and further application cardiac surgery.
of proper filters limit X-ray scatter toward detectors.
Hausleiter demonstrated that a 1% increase in scan Contraindication for Computed
length was associated with 5% increase in radiation.13
Tomography Angiogram
• Prospective versus retrospective gating: The current
trend is more in favor of using prospective gating due to Some of the contraindications for CCTA include: (a) renal
tremendous radiation dose savings. PROTECTION I, a insufficiency; (b) IV contrast allergy; (c) inability to hold
large international multicenter study in 2009 involving breath or follow verbal commands; (d) persistently elevated
more than 50 sites reported a mean effective dose of heart rate, frequent premature ventricular contractions
12 mSv where only 6% of studies were prospectively (PVCs) or premature atrial contractions (PACs). Presence
gated.13 Whereas a study by Freeman et al. showed of atrial fibrillation (AF) is a relative contraindication
depending on the ventricular rate (VR).
a mean effective radiation dose of 3.39 mSv using
prospective gating.14
• High pitch protocols: Several recent studies done on a TECHNIQUE
dual source scanner using a high pitch of 3.4 (compared Important steps to prepare patients for cardiac CT/
to standard 0.2 in conventional scanners) have repo- CTE are listed in Table 85.5. A radiologist or cardiologist
rted coronary computed tomography angiogram should monitor and supervise all CCTA studies for
(CCTA) acquisition with extermely low radiation dose optimization of protocol and best results. The type of
of around 1 mSv.15–19 This technique is only applicable gating and technical parameters should be chosen based
for dual source scanners and requires a low heart rate on the clinical question asked, patient BMI, and heart rate
of < 60 bpm to avoid motion artifacts. (Tables 85.6 to 85.11).
Table 85.5: Patient Prep for Computed Tomography Angiogram

Labs and screening For any C/I (renal failure, contrast allergy), note any pacemakers, details of bypass surgery
Oral -blockade 3 days preferred
Patient education and instructions Explain what to expect
No solid food × 4 hours
No coffee or stimulants
Arrive at least 30–60 minutes before exam
IV access Prefer anticubital fossa, at least 20 ga, test flush with saline
Electrocardiography (ECG) electrodes Proper placement
Heart rate If > 70 bpm, give oral or iv -blocker; ideal close to 60 bpm
Practice breath hold When patient is on the table
Table 85.6: Coronary Calcium Score Protocol Table 85.7: Coronary Computed Tomography Angiogram
Protocol on 64 Detector Scanner
Scanning Mode Axial
Scanning Mode Helical
kV 120
kV 80–120
mAs 140–240 mAs >600
Rotation time 0.5 Rotation time 0.4
Collimation 64 × .65 Collimation 64 × .625
Pitch 0.2 Pitch 0.2
Field of view (FOV) 220 Field of view (FOV) 220
Filter CB (standard cardiac) Filter CB (standard cardiac), sharper (stent)

Slice thickness 0.67 mm
Slice thickness 2.5 mm
Increment 0.33 mm
Increment 2.5 mm
Acquisition Helical
Acquisition Axial
IV contrast yes, 80–100 cc, 4–5 cc/s
Gating Prospective Saline chaser 40 cc at 4–5 cc/s
IV contrast none Gating Retrospective
Effective Dose 1–3 mSv Effective Dose 10–15 mSv
Table 85.8: Preablation Pulmonary Valve Mapping necessitate evaluation of CCTA on a workstation capable
(Prospective Gating) of two- and three-dimensional (2D and 3D) display.
Slice Thickness 1.4 mm The interpreting physician must know how to do the
Increment 0.7 mm postprocessing and not just rely on processed images by
the technologist. The common reformation methods used
Region of interest (ROI) Left atrium
are: multiplanar reformation (MPR), maximum-intensity
Contrast 40–80 cc at 4 cc/s*
projection (MIP), shaded surface display (SSD), and direct
*Depends on body mass index (BMI) and if patient is getting volume rendering (DVR).
ablation the same day.
Transaxial Images
Series of 2D images stacked in the longitudinal (Z-axis)
IMAGE POSTPROCESSING direction in which they are acquired provide minimum
Complexity of coronary anatomy, cardiac motion, calcium- distortion or errors and maximum resolution and
related artifacts, and the subtle nature of coronary lesions gray-scale rendering. Since tortuous vessels will move
Table 85.9: Transcatheter Aortic Valve Replacement (Retrospective Gated Chest and Nongated Abdominal/Pelvis Using Same
Contrast Bolus)
Contrast 60–100 cc at 3–3.8 cc/s [depending on renal function and body mass index (BMI)]
Saline chaser 70 cc at same rate as contrast
Bolus technique With threshold set for 100 H.U. in the ascending aorta
Table 85.10: Contrast Media and Injection

Concentration 300, 320, 370
Flow rate 4–7 cc/s Weight/ body mass index (BMI) based verus fixed rate
Phases Two phase (contrast + saline)
Three phase (contrast + contrast/saline + saline)
Bolus technique Based on predetermined threshold (usually 150 H.U.)
ROI in ascending or descending aorta
Time bolus Inject a 15- to 20-cc test bolus and calculate time to peak
Table 85.11: Optimization of Cardiac Computed Tomography positive and -negative stenosis, viewing from multiple
Angiogram different view points is required (double oblique method),
Before Decrease heart rate (most crucial) and only one branch of a vessel is displayed at a given time
During Tailor exam according to indication (Figs 85.2A and B).
Breath hold
Do not use dose modulation if large patient or Curved Multiplanar Reformation
variable heart rate
This allows to follow the course of a tortuous vessel for
Optimum contrast opacification longer distances as it changes direction. It requires the
After Electrocardiography (ECG) editing centerline to be tracked correctly (done manually or
Use optimal W/L automatically) and, therefore, allows visualization of
Proper phase selection and postprocessing the entire course of the vessel in one image (Fig. 85.3).
method However, inaccurate centerline tracking may lead to
pseudo lesions (Figs 85.4A and B)
in and out of plane as the slice thickness is not variable,
it requires the reader to mentally reconstruct the 3D Maximum-Intensity Projection
anatomical relationships of the vessels and other structures
This involves projection of highest-attenuation voxels
(Movie clip 85.1).
within volumetric data (all points below this value are
ignored). It uses thicker sections to include vessel lumen
Multiplanar Reconstruction and its wall (usually 4–5 mm for coronary), optimizes
A plane is defined inside the 3D volume, and only data visualization and tracking of contrasted structures and is
in this plane is displayed. It is performed by using either especially useful for blood vessel depiction (Fig. 85.5). The
straight or curved planes; thickness is set to zero as a advantages include: visualization of a longer segment of
default to optimize image quality, but can be changed a vessel’s course, reducing perceived image noise, good
(slab MPR). Its advantages include ease of use and speed, differentiation between vessels and background, and
provision of images containing all available information finally is useful when metal is present because of decreased
(all Hounsfield unit values retained), usefulness in artifacts with this rendering. Loss of lesion information
delineating the morphology of the plaque, and its effect on within the slab volume (as MIP does not provide in-depth
the lumen and adjacent vessel wall. Several disadvantages information) and interference from overlapping structures
include its operator dependence, prone to introduce false- are its main limitations.
A B
Figs 85.2A and B: Double oblique method. A 54-year-old male with chest pain and abnormal stress MPT: Mixed plaque is seen in the
proximal left anterior descending (LAD) and more noncalcified plaque in mid-LAD after the origin of D1 (red arrow). Using the double
oblique method, the area of narrowing can be validated in three different planes.
Volume Rendering illustrations for patients (Movie clip 85.2). VR images still
are somewhat operator-dependent and should not be used
This used to be a very tedious and time-consuming for assessing vessel narrowing, which can be misleading
process, but now with advanced fast computing powers (Movie clip 85.3).
of modern processors this process is done with one or
few clicks. Due to its capacity to display multiple tissues
and their relationships to one another, VR is useful for
Virtual Endoscopy
visualizing spatial relationships such as defining the In this technique, the dense contrast within the vessel is
course of coronary anomalies, presence and course of made transparent while the wall of the vessel is opaque,
the bypass grafts as well as in the analysis of thoracic viewed from the point of view of an observer positioned
CV structures and complex CHD (Figs 85.6A and B). VR within the vessel. It creates dramatic images but its clinical
images are impressive and often used for teaching and utility is limited at this time.
Fig. 85.3: Curved multiplanar reformation (MPR). Centerline tracking allows visualization of the entire opacified vessel in one image.
Most current vendors provide semiautomatic centerline placement with one or two points of seeding.
A B
Figs 85.4A and B: The centerline point shown in green is outside the vessel in image (A) causing an area of false stenosis on the curved
multiplanar reformation (MPR). After correctly placing the seed in the lumen of the coronary artery, the pseudostenosis disappears.
Fig. 85.5: Maximum-intensity projection (MIP) images optimize

visualization and tracking of contrasted structures such as coronary
arteries and aorta. (AO: Aorta).
A B
Figs 85.6A and B: (A) Normal coronary artery. The frontal view demonstrates the most anterior chamber as the right ventricle and right
coronary artery coursing in the right artrioventricular (AV) groove (arrow). The broad pyramidal-shaped right atrial appendage (RAA) is
nicely seen. The aorta (AO) is to the right of the pulmonary artery (PA), which is more anterior and to the left of the aorta. (AO: Aorta;
PA: Pulmonary artery; RA: Right atrium; RV: Right ventricle); (B) The axial volume rendered image shows the anomalous origin of the
right coronary artery from the left cusp (red arrow) with interarterial course (yellow arrow). (L: Left cusp; LA: Left atrium; NC: Noncoronary;
PA: Pulmonary artery; R: Right cusp).
IMAGE ANALYSIS two major branches, the left anterior descending (LAD)
and the left circumflex (Cx) artery (Fig. 85.8). Sometimes
Coronary artery evaluation should be done using the there is an additional third branch known as ramus
standard AHA 17-segment evaluation. The coronary intermedius, which courses between the diagonals
arteries originate from superior portions of the sinuses and the obtuse marginal (OM) branches and supply a
just below the sinotubular junction (Fig. 85.7). The normal territory of the LV that might otherwise be supplied by
coronary artery origin is often situated at a right angle to a diagonal or OM branch. When the ramus branch is a
the aortic root wall, whereas anomalous coronary artery large branch, the corresponding diagonal or OM branches
origins are often at acute angles. The left coronary artery are often small in size. LAD courses in the epicardial
originates from the left sinus of Valsalva and courses space along the anterior interventricular groove between
between the right ventricular outflow tract (RVOT) and the right and left ventricles and supplies blood flow to
the left atrial appendage (LAA). It typically divides into the interventricular septum via perforators and to the
Fig. 85.7: Normal coronary artery origin volume rendering (VR). Fig. 85.8: Volume rendered computed tomography (CT) image of
Normal coronary arteries originate from coronary sinuses just aortic root and coronary arteries in left anterior oblique (LAO) pro-
below the sinotubular junction at near-right angle. (L: Left cusp; jection view show the RCA, (LM: Left anterior descending (LAD),
R: Right cusp). and Cx arteries with their major branches. (AO: Aorta; LV: Left
ventricle).
anterior and anterolateral walls of the LV through diagonal This is most commonly seen in the LAD and is more
branches. The branching pattern of LAD is quite variable. often visualized on CTA studies in comparison to catheter
The left circumflex (LCx) courses in the left atrioventricular angiograms. The clinical significance of this variation is
groove between the left atrium (LA) and LV, and provides not clear but according to some it may have a protective
flow to the lateral and posterior lateral walls of the LV effect on the tunneled portion.21 Coronary artery stenosis
through OM branches. The right coronary artery (RCA) should be described in detail and should include its
arises from the right sinus and courses within the right location, degree, number, quantification, factors affecting
atrioventricular groove between the right atrium (RA) evaluation, any positive (Figs 85.11A and B) or negative
and RV. It supplies acute marginal branches to the free (Figs 85.12A and B) remodeling, and features of plaque
RV wall. The conus artery typically arises as a first branch including its size, density, and presence of ulceration.
from the proximal RCA and courses around the RVOT and
terminates on the anterior aspect of the heart. In 50–60% Proper Phase Selection
of patients, the sinonodal artery arises from the RCA and
In retrospective gated studies, it is important to always
courses posteriorly between the aortic root and RA toward
scroll through all phases to select the best phase with the
the cavoatrial junction. The RCA continues to the crux
least motion. All coronary vessels are usually best seen in
of the heart and gives rise to the posterior descending
diastolic phase image (60–80% of R–R interval), whereas
artery (PDA), which supplies the posterior aspect of the
RCA sometimes is better seen in the systolic phase
interventricular septum. RCA then continues to supply the
especially in patients with higher heart rates (Figs 85.13
posterior LV via Posterolateral (PL) LV branches. Coronary
and 85.14).
dominance refers to the supply of the PDA and PL LV
branches. The most common coronary system is right
dominance (> 85%) where both PDA and PL LV branches
Extracardiac Findings
are supplied by RCA (Fig. 85.9A). In a left dominant system It is important for the imager to look at the entire FOV
(8–10%), the LCx supplies the posterior LV branches for any given patient. In our experience we have found
(Fig. 85.9B). In a codominant or balanced system (5–10%), unexpected lung nodules, lymph nodes, pulmonary
the RCA supplies the PDA and LCx supplies the PL LV embolism, pneumonia, esophagitis, and aortic dissection
branches (Fig. 85.9C). Myocardial bridging is a congenital (Figs 85.15 to 85.18). In a study of 6,920 patients who
variation where a segment of the epicardial coronary underwent diagnostic CCTA, the authors found presence
artery is tunneled through the myocardium (Fig. 85.10). of extracardiac findings in almost one fourth of all patients.
A B
Figs 85.9A to C: Coronary dominance (A) Right dominance. Right

dominance, seen in the majority of the population is shown in the axial
maximum-intensity projection (MIP) image. Both posterior descend-
ing artery (PDA) and PL LV branches are arising from RCA; (B) Left-
sided dominance. Left-sided dominant circulation, where the PDA
and PL LV branches arise from the left circulation; (C) Codominance
pattern of coronary circulation. Inferior volume–rendered view of
the heart shows the codominance pattern of coronary circulation.
The PDA (yellow arrow) is supplied by the RCA while the PL LV
(white arrow) territory is supplied by the Cx. The codominance
pattern is the second most common circulation pattern after the
C most common right circulation pattern.
In their study, several serious diagnosis were missed when

reviewing only the limited FOV images and use of broad
FOV led to more workup and follow-up.22
PITFALLS AND ARTIFACTS

Cardiac Motion Artifact
Lower and steady heart rate is very critical for obtaining a
good quality CTA. Even with the newer 256, 320 detector
as well as dual source scanners, the emerging consensus
is to have heart rate as low as possible preferably close to
60 bpm to optimize image quality. The RCA shows
maximum motion during the cardiac cycle followed by
LAD and Cx (Movie clips 85.4A and B). The TR of current
Fig. 85.10: Myocardial bridge. The proximal left anterior descend- scanners is getting better but is not closer to 50 ms required
ing (LAD) courses through the myocardium (red arrow) for a short to achieve motion-free coronary artery imaging. Misali-
distance and then follows the normal epicardial course.
gnment or slab artifact results from a high heart rate,
A B
Figs 85.11A and B: Coronary computed tomography angiogram (CCTA) 4 mm thick axial maximum-intensity projection (MIP) image
shows tight narrowing in the mid-left anterior descending (LAD; red circle), which was confirmed at catheter angiography. The outer wall
to outer wall size of the vessel at the site of noncalcified plaque is larger as compared to vessel proximal to stenosis suggesting positive
remodeling, which is one of the features of vulnerable plaque.
A B
Figs 85.12A and B: In this young 47-year-old male with acute chest pain, the coronary computed tomography angiogram (CCTA)
demonstrates significant stenosis of the mid-left anterior descending (LAD) near the origin of the septal branch. The outer to outer size
of the vessel in this region is actually decreased in comparison to the proximal vessel suggesting the presence of negative remodeling.
Catheter angiogram confirmed the presence of fibrotic narrowing in the LAD.
A B
Figs 85.13A and B: Proper phase selection. In studies done using retrospective gating, the entire cardiac cycle images are available
from 0% to 90% RR intervals and can be reconstructed at 5–10% increment. Since cardiac motion is minimal in the mid to late diastole,
the left coronary circulation is best visualized during the diastolic phase as shown in these images. The right coronary artery is also seen
best in diastole except in a few instances where it is actually better seen at end-systole.
A B C
Figs 85.14A to C: Proper phase selection. RCA in 30%, 40%, and 80% phase. The last image also shows misregistration artifact
(arrow).
irregular heart rate, or presence of arrhythmia and is often preferably exercised to hold her/his breath before image
best seen on coronal or sagittal reconstructed images acquisition. Unlike ECG editing, which can improve image
(Figs 85.19A and B). quality in patients with mild arrhythmia, one cannot do
much with respiratory motion artifacts (Figs 85.20A and B).
Respiration Motion Artifact
With the current scanners, very short 4–10 seconds breath Blooming Artifacts
hold is required and is easily tolerated by majority These occur with high attenuating structures such as
of patients. The patient still needs to be instructed and coronary stents or calcification due to partial volume
A B
Figs 85.15A and B: Extracardiac findings. In limited field of view (FOV) computed tomography angiogram (CTA), the right hilar and
subcarinal nodes (arrows) are barely visible at the edge, but become obvious with wide FOV image (B).
A B
Figs 85.16A and B: The right lower lobe pulmonary embolisms (arrow) seen in wide field of view (FOV) image (B) can be missed
altogether if only limited FOV images (A) are reviewed.
A B
Figs 85.17A and B: The nodule in the left upper lobe is difficult to appreciate in image (A) and can mimic an end on vessel. It is easy
to find on the wide field of view (FOV) image (B). The nodule in this patient was proven to be metastasis from renal cell carcinoma.
A B
Figs 85.18A and B: Extracardiac findings. A 45-year-old female presented with severe chest pain. A gated cardiac computed tomog-
raphy (CT) was performed and showed normal coronary arteries but there was severe thickening of the entire thoracic esophagus
(arrows), which is better appreciated in wide field of view (FOV) image (B).
A B
Figs 85.19A and B: Misregistration artifact due to variability in heart rate is better appreciated on coronal image (B, arrow) than the
axial image (A).
averaging and cause excessive blooming and overesti- Beam-Hardening Artifacts

mation of coronary luminal narrowing (Figs 85.21A
to C). A dual energy technique can provide images An X-ray beam passing through a high-density structure
without calcifications and may prove to be useful in better gets attenuated with most of its low energy photons
assessment of coronary luminal narrowing by enhancing absorbed. In locations near such high attenuating
vessel visualization. Similarly, monochromatic imaging at material, the X-ray beam maintains its low as well as high
different keV has been shown to decrease blooming from energy photons, resulting in an area of low density in the
calcium at 140 keV in comparison to lower keV.23 Coronary image. A common location for such an artifact is LV apex
stent-related artifacts can be improved by using a sharper and posterolateral wall of LV near the descending thoracic
kernel for reconstruction as well as with thinner images, aorta. It is important to recognize this artifact to avoid
although at the cost of slightly increased image noise misinterpretation (Figs 85.23A and B). Few vendors now
(Figs 85.22A and B). provide beam-hardening correction algorithms.
A B
Figs 85.20A and B: Respiration artifact. Breathing-related artifacts can degrade computed tomography angiogram (CTA) studies and
cannot be reversed with editing. These images are from the first patient scanned as gated computed tomography (CT) study on 40
detector scanner at our facility. The first image (A) showed a sudden cutoff of the mid-left anterior descending (LAD) which, of course,
was not seen at the subsequent cath. When you look at the lung window images at the same level, respiration motion-related artifact is
clearly visible as a ghost shadow in the pulmonary vasculature (arrow).
A B
Figs 85.21A to C: Blooming artifacts. Calcium-related blooming

artifact (A) remains a problem and is the common cause for calling
overstenosis on computed tomography angiogram (CTA). Some
newer scanners have different detector material, which helps in
suppressing this artifact as shown in image (B). It is not possible
to determine stent patency due to severe metal blooming artifact
C in image (A).
A B
Figs 85.22A and B: Stent evaluation. Sharper filter or kernel (B) often are very useful to look at the stent lumen and restenosis. The
images are very sharp and show the lumen, although at the cost of slightly increased noise in comparison to standard smooth filter
use (A).
A B
Figs 85.23A and B: Beam-hardening artifact. The hypodensity noted near the LV apex (arrow) in this patient was not visualized on the
coronal images. This artifact can easily be misinterpreted for myocardial hypoperfusion.
DIAGNOSTIC ACCURACY OF to the high spatial and TR of the current MDCT scanners.
The overall performance of the 64-detector CCTA for
CORONARY COMPUTED
detecting coronary artery stenosis in comparison to
TOMOGRAPHY ANGIOGRAM catheter angiography on a patient-based analysis results
CCTA has become a robust and accurate clinical tool for in sensitivity, specificity, positive predictive values, and
the noninvasive evaluation of the coronary arteries due negative predictive values of 85–100%, 64–100%, 64–100%,
and 83–100%, respectively.24–31 Improved TR of the dual angina, and found that in the ACS group the mean density
source scanners has increased the number of evaluable of the plaque was 25 ± 15 H.U. whereas it was 71 ± 16 H.U.
coronary artery segments even in patients with high heart in patients with stable angina.42
rates.32–38 In their study of plaque evaluation with CT attenuation
values, Sun et al. showed clear accurate characterization
CORONARY PLAQUE of calcified plaques, but there was a significant overlap
between CT attenuation values of lipid rich and fibrous
CCTA is excellent at detecting the presence of athero-
plaques.43 The authors concluded that at this time, the
sclerotic plaques and shows good correlation with
CT attenuation value is not able to accurately distinguish
intravascular ultrasound (IVUS).39 However, CTs tend
between lipid-rich and fibrous plaques.
to underestimate the volume of a plaque. Leber et al.
The relationship between coronary artery remodeling
compared plaque assessment by CTA to IVUS and found
and plaque composition has been studied and reported
that the vessel plaque volume is underestimated by
by Varnava et al.44 These authors studied 88 male subjects
64-MDCT and its correlation with IVUS was only
who died with CAD and examined 108 plaques post-
moderate.40
mortem; 59% had no or positive remodeling and 41% had
negative remodeling. They found that there was higher
Plaque Characterization lipid content and macrophage count in plaques with
An acute coronary event often results from plaque rupture positive remodeling (a marker of plaque vulnerability).
and subsequent luminal thrombosis. It is also now In another study, Reilly et al. looked at the effect of
suggested that most acute myocardial infarctions result statins on human coronary atherosclerotic plaque mor-
from rupture of nonstenotic vulnerable plaques.41 A phology.45 The authors retrospectively reviewed the arte-
vulnerable plaque is often large in size, is lipid rich, rial sections from native hearts of patients with end-stage
has an ulcerated irregular surface with areas of spotty ischemic heart disease who received cardiac transplanta-
calcifications, and often shows positive remodeling and tion (Tx). Thirty-three of 44 study group patients received
active inflammation. Many of the features of vulnerable pre-Tx statins, and 11 did not (which served as control).
plaque can be determined from CCTA (Figs 85.24A and B). Both groups were similar in total and low-density lipid
Sato et al. evaluated plaques with CTA in patients with (LDL) cholesterol levels, and available number of arterial
acute coronary syndrome (ACS) and those with stable sections per patient. The prevalence of low-grade fibrous
A B
Figs 85.24A and B: Plaque characterization. Plaque characterization has been investigated and although lower computed tomo-
graphy (CT) attenuation plaques are presumed to be lipid rich and those with CT attenuation between 40 and 120 are fibrous, there is
considerable overlap. At this time it is best to use terms calcified, noncalcified, or mixed plaque for CT descriptions. The CT images in
this Figure. belong to two different patients; (A) one with a positive remodeling has noncalcified plaque of CT values of 43, and (B) the
other has noncalcified plaque of CT value of 157 and associated negative remodeling.
plaques was much higher (45.7%) in patients receiving population included symptomatic patients, those with
statins than those who were not on statins (11.3%). The abnormal stress or rest test as well as asymptomatic
high-grade lesions were found more frequently in the ones with peripheral vascular disease (PVD) or multiple
control group (66.3%) than the study group (34.6%). The risk factors for CAD. The authors identified several
authors concluded that statin therapy substantially prognostically valuable CCTA indices based on visual
enhances plaque stabilization due to reduction in plaque estimate, modified Duke prognostic CA score, and clinical
inflammation. coronary plaque score.
PROGNOSTIC INFORMATION CARDIAC FUNCTION

FROM CORONARY COMPUTED LV function as reflected by the EF is one of the most
TOMOGRAPHY ANGIOGRAM important prognostic parameters in patients with CAD. In
patients getting retrospective gating, LV assessment can be
The prognostic value of coronary calcification by CT has easily performed with CT data available throughout the
been well described but the prognostic value of CCTA cardiac cycle (Fig. 85.25).
is less well reported and remains a hot topic.46 Several In a study comparing cardiac function analysis from
smaller studies have reported variable outcome from CTA MDCT with echo, single-photon emission computed
data.47–56 In a recent meta-analysis of 18 studies evaluating tomography (SPECT), and magnetic resonance imaging
9,592 patients with a mean follow-up of 20 months, the (MRI), the authors found that there was agreement and
authors concluded that major adverse cardiovascular similar correlation between CT and MR for EF, end-
events (MACE) among patients with normal CTA diastolic volume (EDV), ESV, and LV mass parameters.59
are rare and there is incrementally increasing future The standard deviation of EF difference between CT and
MACE with increasing CAD by CTA.46 In another study, MR was significantly less than that between echo and MR
Russo et al. compared the prognostic value of CCTA with or between SPECT and MR. The evaluation of RV function
that of coronary calcium scoring and clinical risk factors.57 is more challenging not only because of the shape of RV
The authors evaluated hard cardiac events in 441 patients but also often less than optimal contrast enhancement of
who underwent CCTA and calcium scoring for about the RV when using CCTA protocol.
32 months. Patients with normal CCTA had a very low
hard annualized event rate of < 0.89%, in comparison
to 3.89% in patients with any CAD. Obstructive CAD MYOCARDIAL PERFUSION
(P < 0.003) and presence of noncalcified or mixed plaque Although CT is currently not the method of choice to
(P < 0.0001) were independent predictors of hard events evaluate myocardial perfusion, one can sometimes see
on multivariate analysis. perfusion abnormalities in the myocardium in the resting
In another study of 432 patients followed for 24 months, state (Figs 85.26A and B).
van Werkhoven et al. looked at the incremental prognostic
value of CCTA compared with coronary calcium score Stress Myocardial Imaging Using
alone.58 In this study, multivariate analysis demonstrated
that the extent of disease and plaque characterization were Computed Tomography
predictive of cardiac events, specifically plaque burden In standard current clinical practice, patients with signi-
and plaque composition provided incremental prognostic ficant coronary artery stenosis detected on MDCT often
value over clinical variables and coronary calcium scoring. require additional functional assessment to determine
They also found that 20% of patients with a zero coronary the significance of coronary stenosis, thereby adding more
calcium score had noncalcified plaques and 4% patients tests, cost, radiation, and patient inconvenience. With
with no coronary calcium had significant CAD stenosis improved spatial and TR along with decreased scan time
(> 50%) by CTA, therefore suggesting that coronary and less radiation exposure, the role of MDCT in evaluating
calcification alone may not be adequate to accurately myocardial perfusion is emerging. Several research cardiac
assess prognosis. James Min et al. looked at the prognostic CT perfusion studies in animals and human subjects have
value of CCTA for prediction of all-cause mortality in been published and are proposing MDCT as a useful
more than 1,127 patients older than 45 years.55 The patient alternative modality in the evaluation of myocardial
Fig. 85.25: Cardiac functions. In retrospective gated CTA, cardiac function can be derived using CT threshold method and can be
displayed in bulls-eye or graphic representation.
perfusion.60–65 Since X-rays are attenuated proportional in shuttle and high-pitch mode to derive myocardial
to contrast concentration in the area of interest, in the perfusion imaging (MPI).64
absence of beam-hardening artifacts the hypoattenuated In a study of 35 patients with a high suspicion of CAD,
myocardial area on CT represents decreased perfusion. Rocha-Filho and his group evaluated CTA alone and in
Similar to SPECT, one can also use stress induced by combination with adenosine stress perfusion CT for the
adenosine, ragadenoson, or dipyridamole to accentuate diagnosis of obstructive CAD on catheter angiography.66
the differential myocardial blood flow (MBF) between The initial diagnostic performance of CCTA to detect
normal and ischemic areas. 50% stenosis had a sensitivity of 83%, specificity of 71%, a
Computed tomographic perfusion (CTP) imaging can positive predictive value of 66%, and a negative predictive
be performed qualitatively with the more widely available value of 87%. After including the CTP information,
64-detector scanners or more quantitative dynamic each of these indices improved to 91%, 91%, 86%, and
imaging can be done now with 256- and 320-detector 93%, respectively. Findings were also similar in the 70%
scanners. With the latter, time attenuation curves (TACs) coronary stenosis group. The same group also compared
of the aorta, LV, and myocardium can be constructed the CTP data to SPECT after including a delayed 7-minute
to provide information regarding MBF and myocardial prospective CT to determine delayed enhancement and
blood volume (MBV) using mathematical models.63 The found similar comparative diagnostic accuracy for 70%
second generation dual source scanners can also be used stenotic lesions.67
A B
Figs 85.26A and B: (A) Resting myocardial perfusion abnormality. This 45-year-old-male came to the emergency department after
being involved in a motor vehicle collision. He had a routine chest/abdominal/pelvis computed tomography (CT). The representative
image of the chest CT shows a clear area of hypodensity in the mid to distal septum and apex with CT attenuation values of 33 in com-
parison to remote lateral wall LV myocardium with a CT value of 68. The wall thickness was maintained. These findings are suggestive
of acute myocardial ischemia/infarction and follow-up serial cardiac biomarkers and the electrocardiography (ECG) showed STEMI in
the left anterior descending (LAD) distribution; (B) In chronic myocardial infarction there is a subendocardial hypodense defect (arrows)
in a vascular territory associated with wall thinning and remodeling as shown in image (B).
HOW TO IMPROVE ACCURACY OF CLINICAL INDICATIONS

COMPUTED TOMOGRAPHY Coronary Calcium Scoring
ANGIOGRAM IN DETERMINING
Quantification of CAC burden on CT is shown to be
FLOW LIMITING DISEASE an independent, noninvasive marker and predictor of
In a recent study done in 381 patients using a 320-detector adverse cardiovascular (CV) events in asymptomatic
scanner, Lima et al. have shown the added value of CT individuals.70 The risk assessment on CT CAC extends
perfusion data to routine CCTA to increase the diagnostic beyond that provided by the Framingham risk score to a
accuracy of the CTA to define flow-limiting CAD at a lower population with a wide ethnic background.70 Thus, CAC
radiation dose when compared with conventional invasive has the potential to restratify intermediate risk groups into
angiogram and MPI combined.68 lower or higher groups.
CT-based fractional flow reserve (FFR-CT) calculation Prospective ECG-gated cardiac images are acquired
is another upcoming promising method to determine with a slice thickness of 2.5–3 mm without intravenous
noninvasively the significance of any coronary artery contrast. The FOV extends from just below the carina to
stenosis. Computational fluid dynamics (CFDs) is used cardiac apex. Coronary calcium is defined as an area of at
to calculate pressure gradients across stenotic areas on least three adjacent pixels in the axial plane in the course
standard CCTA studies. In a multicenter DeFACTO study, of a coronary artery, with an attenuation threshold value
the authors compared FFR-CT with invasive FFR in 252 of 130 H.U. or greater. Three in-axial-plane face-connected
patients and found that there was improved diagnostic pixels correspond to a minimum lesion area > 1 mm2,
accuracy and ability to determine functionally significant which is used as a reference value in calcium scoring.71
stenosis by using FFR-CT in conjunction with CCTA over There are currently two CT calcium scoring systems widely
CTA alone (AUC: 0.81 vs 0.68 respectively, P < 0.001).69 This used: the original Agatston method and the volume scoring
promising method is currently limited by the long time (up method developed by Callister et al.71,72 These images
to several days) taken to compute huge data but hopefully are postprocessed on a dedicated 3D workstation with
will be available one day soon for it to be applicable in appropriate software that automatically detects calcium
clinical practice. on all CT slices (Fig. 85.27).
consensus regarding the calcium score beyond which

CCTA should not be performed. Some authors suggest
a value of 600 while others, 1,000. In our opinion what
is more important is the distribution of the coronary
calcium, since a CCTA can still be useful if the calcium is
concentrated in a few coronary segments leaving many
other segments evaluable as shown in Figure 85.28.
Acute Chest Pain Evaluation with

Multidetector Computed Tomography
in the Emergency Department Setting
Despite recent advances in medical sciences, the eval-
uation of acute chest pain presenting in the emergency
Fig. 85.27: Coronary calcium score. Calcium scores are calcu-
lated by the computer based on selected H.U. threshold calcifica- department (ED) is difficult and remains a diagnostic
tions and the numbers tabulated in different vascular distribution challenge. In a study of more than 10,000 chest pain
as shown here. ED patients, 2.1% with acute MI and 2.3% with acute
unstable angina were inappropriately discharged.75 Due
In Agatston’s scoring method, the calcium area is to medicolegal issues, many of these patients with chest
multiplied by a number related to CT attenuation. Partial pain get admitted unnecessarily for further tests and
volume effects lead to higher peak values for small calcific investigations leading to billions of dollars of excessive
lesions but not for large ones. Whereas, the volume cost each year.76 In this scenario, a test with a very high
scoring method appears to somewhat resolve the issue of a negative predictive value, such as CCT angiography, can
section dependent on minor changes in section thickness. be very useful in effectively triaging this cohort of patients.
However, excellent correlation has been demonstrated A major limitation, especially in acutely ill patients,
between the two scoring methods.73 Both methods is the quality of the images especially if the patients have
calculate lesion-specific scores within the left main, LAD, tachycardia. Administration of -blockers, which is a
left circumflex, and right coronary arteries and provide standard practice for CCT angiography, is time consuming,
total scores for an individual artery as well as total score is limited by contraindications, and may not be effective
across all four arteries. in many acutely ill patients. Initial studies of dual source
When CAC scan is performed just preceding the CCTA, CT have shown a very good diagnostic accuracy of CCTA,
it can serve as a gatekeeper to exclude cases with heavy even with a high heart rate.33,77
calcification because of low diagnostic yield. However, Improved assessment of coronary arteries with CT
there is no established absolute number of CAC score and easy accessibility and close proximity of ED to such
above which CCTA should not be performed. Secondly, it scanners has increased the interest in MDCT evaluation
also serves as a reference scan to tighten the CCTA FOV in of chest pain. With 64-detector CT, sensitivity and
craniocaudal direction. specificity rates of > 90% have been reported with few
The expert consensus document by the American nonevaluable segments than with earlier generation
College of Cardiology Foundation and the American Heart MDCT scanners.28,30,78
Association regarding the current role of CAC testing A recent blinded prospective study of 103 patients
in clinical practice among asymptomatic individuals with chest pain who presented to the ED showed
states, “It may be reasonable to consider use of CAC absence of significant coronary stenosis and presence of
measurement in asymptomatic individuals who are at nonsignificant atherosclerotic plaque by MDCT, suggesting
intermediate risk.”74 However, the committee did not find absence of ACS, giving a negative predictive value of
enough evidence regarding the utility of CAC testing in 100%.79 In another study, MDCT compared favorably
further risk-stratifying in those considered at high risk with radionuclide perfusion imaging for detecting ACS in
of developing CAD in the next 10 years.74 There is no 92 low-risk patients with chest pain in the ED.75
Fig. 85.28: Coronary calcification. The cutoff coronary calcium is reported to be between 600 and 1,200. However, computed tomog-
raphy angiogram (CTA) still could be useful if the calcification score for not performing CTA is limited to a few segments and it can
also show noncalcified lesions elsewhere as in these images. This patient had a calcium score of 1,060 causing mostly nonobstructive
disease, but there was additional more significant stenosis in the distal RCA due to mostly noncalcified plaque (red circle).
Meijboom et al. evaluated the role of 64-MDCT in Goldstein et al. compared the safety, diagnostic efficiency,
254 symptomatic patients with an estimated pre-test pro- and cost effectiveness of 64-detector CT with standard-
bability of CAD to be high (N = 105) in 87%, intermediate of-care diagnostic evaluation.81 In 75% of patients,
(N = 83) in 53%, and low (N = 66) in 13%, using the physicians were able to rapidly triage by immediately
Duke Clinical Scoring System.80 The estimated post- excluding or identifying coronary disease using MDCT.
test probability of the presence of significant CAD after In the remaining 25% of patients, further nuclear stress
a negative CT scan was 17%, 0%, and 0%, respectively, testing was performed due to either nondiagnostic MDCT
and after a positive CT scan was 96%, 88%, and 68%, scans or the presence of intermediate severity lesions. No
respectively. Based on these findings, the authors adverse coronary events were identified for 6 months in
suggested the usefulness of MDCT in symptomatic patients who underwent MDCT comparable to the other
patients with low or intermediate estimated pre-test group with the standard-of-care approach. The average
probability of having significant CAD. CT did not provide time in the diagnostic evaluation of patients was 3.4 hours
additional relevant diagnostic information in high pre-test in the MDCT group compared to 15 hours in the standard-
probability symptomatic patients. of-care evaluation group. The average cost of patients
In a prospective randomized trial of 197 patients with was modestly lowered $1,872 to $1,586, when the MDCT
acute chest pain at low risk for ACS presenting to the ED, algorithm was used.
Several authors recently have investigated the value TIMI score, and no other obvious indicators of ACS such
of calcium scoring, especially with EBCT to triage ED as elevated biomarkers or arrhythmia. Over 6 months of
chest pain patients.82–84 In one such study of 105 patients follow-up, the patients imaged with CCTA were diagnosed
with possible ACS with normal cardiac enzymes and a 54% faster than those imaged with MPI (median 2.9 hours
nondiagnostic ECG result, Laudon et al. found the overall vs 6.3 hours, P < 0.0001). The total costs of care were
sensitivity, specificity, and negative predictive value for 38% lower with the CCTA group (median $2,137 vs $3,458,
a positive finding (non-zero calcium score) at EBCT to P < 0.0001), even though the cost of each MPI test itself
be 100%, 63%, and 100%, respectively.82 No patient with was only a little more than the cost of each CCTA ($538
a negative EBCT had any cardiac event at 4-month vs $507). The diagnostic strategies made no difference
follow-up. in MACE rate (0.8% in the CCTA arm vs. 0.4% in the MPI
arm, P = 0.29). The CCTA patients were also exposed to
Techniques of Multidetector Computed less radiation than the MPI patients (11.5 mSv vs 12.8 mSv,
P = 0.02).
Tomography for Acute Chest Pain
Evaluation in Emergency Department
Anomalous Coronary Artery Evaluation
Two different protocols are described. First is a dedicated
standard CCT angiography protocol, which includes Coronary artery anomalies are rare (0.3–1%) and are
prospecitve gating and small FOV. The advantage of this often asymptomatic. Malignant form of anomalies can be
protocol is improved SR to evaluate coronary arteries symptomatic presenting with syncope, arrhythmia, chest
with less contrast and radiation dose. The disadvantages pain, or sudden death.
include nonvisualization of other potential causes of chest MDCT is now considered the gold standard method to
pain in the lungs and mediastinum not included in the evaluate patients with suspected coronary artery anomalies
FOV. with an appropriate use criteria score of A 9.20 Anomalies
In the second “triple rule out” protocol, the entire of coronary arteries can be classified as anomalous origin,
chest is scanned using a gated technique, but with a large anomalous proximal course, or anomalous termination
FOV leading to increased contrast dose, and increased (Figs 85.29 to 85.32). Among the abnormal courses, the
scan time. The advantage of this protocol is evaluation of interarterial course is most dangerous and has been
other potential causes of chest pain as described above. implicated as one of the causes of sudden death in young
There is no consensus regarding use of these protocols or
regarding a preference to use these protocols. However, in
a given patient, if there is a strong suspicion for CAD or
the main issue is excluding CAD, a dedicated CTA protocol
is preferred. On the other hand, in patients with atypical
chest pain, without definitive CAD suspicion, a “triple rule
out” may suffice.
Two recently published large trials ACRIN-PA and
ROMICAT II collectively recruited 2,370 low- to inter-
mediate-risk patients presenting with suspected ACS
to the ED in the United States and compared protocols
including an early CT with traditional care group.85,86 The
authors concluded that there was a significant reduction
in the length of stay and increased discharge rate from the
ED in the CT group without any adverse outcome.
In CT-STAT trial, 699 patients at 16 EDs were Fig. 85.29: Coronary artery anomaly. The most common
randomized to either CCTA or MPI.87 All of the patients anomaly is high origin of the right coronary artery above the sino-
had symptoms of ischemia but a normal or nondiagnostic tubular junction as shown in this image (arrow). (L: Left cusp;
R: Right cusp).
rest ECG, no previous known coronary disease, a low
Fig. 85.30: Single coronary artery. In this patient presenting with Fig. 85.31: RCA from left cusp. Sagittal oblique CT image shows
atypical chest pain, coronary computed tomography angiogram anomalous origin of the right coronary artery from the left cusp
(CCTA) showed a large single coronary artery (arrow) arising from and has interarterial course between aorta and pulmonary artery
the right cusp and supplying all territories of right and left circula- (arrows). (L: Left cusp; NC: Noncoronary; PA: Pulmonary artery;
tion. Whenever one of the coronary artery is significantly larger R: Right cusp).
than normal, one should look for obstructive disease in other ves-
sels, coronary artery fistula, or anomalous coronary artery origin
from a low pressure system such as a pulmonary artery.
Fig. 85.32: Volume rendering (VR) image clearly shows the empty Fig. 85.33: Anomalous origin of left coronary artery from pulmo-
right cusp and anomalous origin of the right coronary artery from nary artery (ALCAPA). A 25-year-old female with increasing short-
left cusp. Due to the proximity of the anomalous vessel to commis- ness of breath. Oblique volume–rendered image demonstrates
sure and proximal intramural course unroofing procedure can be the anomalous origin of the left coronary artery from the pulmo-
done in such cases with good results. (L: Left cusp; NC: Noncoro- nary artery (red arrow). The RCA (white arrow) is much larger.
nary; R: Right cusp).
patients. The prepulmonic course is often seen in patients presents early in life with chest pain or dilated ischemic
with tetralogy of Fallot (TOF) and should be identified cardiomyopathy (Fig. 85.33). Table 85.12 describes the
preoperatively so as to avoid directly cutting into it on the methods to optimize evaluation of a suspected coronary
operating table. artery anomaly.
Anomalous origin of left main coronary artery from the Coronary artery fistula is rare, commonly congenital,
pulmonary artery (ALCAPA) is a rare anomaly and often and can be coronary-cameral fistulas (between coronary
Table 85.12: Optimization of Anomalous Coronary Artery

Computed Tomography Angiogram Protocol
In a younger age, restrict field of view (FOV)
For interarterial course, show pulmonary valve relation (good

to have some contrast in right circulation)
Volume rendering (VR) images show spatial relationship nicely
Multiplanar reformation (MPR)/thin maximum-intensity
projection (MIP) images needed to document intramural
course and oblique narrow takeoff of the anomalous vessel
artery and cardiac chambers), coronary arteriovenous (AV)

malformation (between coronary artery and low pressure
veins), drainage often to RV, RA, or pulmonary arteries,
and less frequently to superior vena cava (SVC), coronary Fig. 85.34: Coronary artery (CA) fistula. LV to OM branch fistula
sinus, or LA (Fig. 85.34). Rarely, coronary artery fistula in a patient after MI is seen on the axial computed tomography
develops from acquired causes such as trauma or invasive angiogram (CTA) image. (LV: Left ventricle; RV: Right ventricle).
procedures (pacemaker placement, endomyocardial
biopsy) or CABG. Most are small and the patient, asympto- Role in Asymptomatic
matic. On examination, a continuous murmur can be
heard at the left lower sternal border and the CA branches Intermediate Risk Patient
proximal to the shunt become enlarged. Smaller fistulas Currently, CTA is not indicated for asymptomatic indivi-
in children tend to grow with age. If left untreated, they duals and this is a topic of hot debate. One school of
can cause symptoms in 19% of patients under the age of thought leans toward getting CTA in patients with
20 years and in over 60% of older patients. Small fistulas significant risk factors including family history of prem-
in an asymptomatic patient should be followed clinically ature atherosclerosis.
for signs of growth and increasing flow. The larger hemo- In a large multicenter registry study, Chinnaiyan
dynamically significant fistulas should be closed either et al. presented their research data from the advanced CV
surgically or via transcatheter occlusion techniques imaging consortium (ACIC) in Michigan looking at the
especially if the fistula is short and less tortuous. CCTA in asymptomatic individuals.88 A total of 21,573
Sequelae of coronary artery fistula may include chronic patients without known CAD participating in ACIC at
angina, congestive heart failure (CHF), cardiomyopathy, 43 institutions in Michigan were included and of these
myocardial infarction (MI), hypertension (HTN), endocar- 11.8% were asymptomatic and 88.2% were symptomatic.89
ditis, and rarely fistula rupture. The asymptomatic group patients were older, had more
males with higher Framingham risk scores, and higher
Preoperative Computed frequency of abnormal stress tests (38% vs 21%). CCTA
in these patients showed a lower frequency of normal
Tomography Angiogram coronary arteries (38% vs 51.2%) and higher frequency
When catheter angiography is considered dangerous, of both < 50% and > 50% luminal stenosis. The authors
difficult, or contraindicated, such as patients with aortic concluded that the asymptomatic patients had worse risk
vegetations, ascending aortic dissection and those with profiles and a higher CAD burden on CCTA. These findings
massive aortic dilatation making it very challenging to have broad implication on identification of candidates for
canulate coronary ostia (Figs 85.35A to C). aggressive secondary prevention.
The main hurdle in our view has been the issue of
Re-do Coronary Artery radiation exposure, which is understandable. But with
the recent advances and availability of very low dose CTA
Bypass Graft protocols it seems logical and appropriate to use CTA
This is another common indication and can be performed selectively in such high-risk populations with radiation
with or without intravenous contrast depending on the exposure close to or < 1 mSv. In fact, with such low-dose
clinical question (Figs 85.36A and B). scanning capability, it is possible that in the near future
A B
Figs 85.35A to C: Preoperative computed tomography angiogram

(CTA). Preoperative CTA is done when catheter angio is difficult
or dangerous; (A) ascending aortic aneurysm and dissection in
a patient with previous coronary artery bypass graft (CABG);
(B) ascending aortic dissection narrowing the LM ostium (arrow);
(C) aortic valve endocarditis as seen on echo and computed
C tomography (CT) images (arrow).
CTA might play an important role as a screening modality Coronary Stent Evaluation
for CAD somewhat similar to where we stand now for
screening of lung cancer using low-dose CT. The population needing follow-up of PCI is growing
and follow-up of stents with catheter angio is expensive
and carries risks. Currently, clinical symptoms and/or
Coronary Artery Bypass Graft provocative tests are noninvasive tools with no direct stent
Gated cardiac CT is well suited to evaluate patency of imaging capability available. In a study comparing the
bypass grafts noninvasively, since these grafts are usually diagnostic accuracy of coronary in-stent stenosis using a
larger diameter structures and there is less cardiac motion- 64-detector CT with invasive coronary angiography, the
related artifacts. The reported sensitivity and specificity authors reported high sensitivity, specificity, and negative
for detecting stenosis or occlusion is 95–100%.90 With the predictive values of 92% and 90%, 81% and 79%, and 98%
newer scanners, the anastomosis can be well evaluated and 96% per segment/per patient basis, respectively.91 The
with less motion, less metal clip hardware artifact, and positive predictive value, however, was only modest at 54%
better vascular opacification (Figs 85.37A to C). The CTA and 58%, respectively. Several steps that can be taken to
protocol needs to include the entire chest, especially improve in-stent visualization are the use of higher mAs
the subclavian arteries if the patient has had internal to improve contrast resolution, use of high intravascular
mammary grafts. More contrast is often needed and the attenuation, use of sharper kernel, and lowering of the
breath hold is slightly longer (Figs 85.38A and B). heart rate (Figs 85.39A to C).
A B
Figs 85.36A and B: Redo sternotomy. Performing computed tomography (CT) is an appropriate indication for a patient being evaluated
for redo CABG or noncoronary cardiac surgery. CT can accurately detect the distance of vital structures near the sternum as well as the
presence of any adhesions. In the images here, this patient had developed a pseudoaneurysm (PSA) at the site of aortic cannulation
(arrows) done for cardiopulmonary bypass. Computed tomography angiogram (CTA) clearly showed the close proximity of the PSA to
the sternum, which is useful information for the surgeons in planning a safe surgical approach.
Cardiac Masses lipomas are usually small and most commonly seen in
the LV or along the septal wall or roof of the RA. Usually,
Primary cardiac tumors are rare but metastases to the these are asymptomatic and detected incidentally on
heart from other locations are much more common. cross-sectional imaging or rarely can cause symptoms if
Primary benign tumors are much more common than large in size, usually by obstructing a cavity, extrinsic mass
primary malignant neoplasms. Myxoma is the most effect, or causing arrhythmia. On CT, these tumors are well
common primary benign cardiac neoplasm in adults. defined, noncalcified, and nonenhancing masses with fat
Most myxomas arise in the LA near the fossa ovalis and CT attenuation values (Figs 85.41A and B).
less commonly from the atrial free wall or atrioventricular Fibroma is the second most common primary benign
valve. Less commonly they can arise in the RA or RV. On tumor of the heart in children after rhabdomyoma. These
CT, myxomas are round, smooth, and lobulated masses tumors are less commonly seen in adults and more
often with a pedicle and demonstrate patchy postcontrast commonly arise from LV than RV in the interventricular
enhancement. Often these masses have areas of calcifi- septum or anterior ventricular wall. On CT, they are
cations as well as low attenuation (Fig. 85.40). On a homogenous intramyocardial masses, often calcified with
retrospective gated CT, the pedunculated myxoma can be little or minimal enhancement after contrast administration
seen to prolapse through the mitral valve. Symptoms from (Fig. 85.42). Patients can present with dysarrhthymia,
myxoma are usually due to hemodynamic obstruction, chest pain, or sudden death. Several syndromes are asso-
embolism, or rarely constitutional symptoms. Multiple ciated with cardiac fibromas including Gardner, Gorlin,
cardiac myxomas can be associated with pigmented skin and familial adenomatous polyposis.
lesions, extracardiac tumors (schwannoma, pituitary ade- Fibroelastomas account for approximately 10% of
nomas), and endocrine abnormalities (Carney’s complex). cardiac tumors and 75% of valvular neoplasms.92 Most
Lipoma is the second most common primary benign arise from the endocardium of aortic, mitral, tricuspid,
cardiac tumor. It can either arise from the subendocardial and pulmonary valves (in decreasing order) and are often
layer or subepicardial/myocardial layer. The endocardial solitary, small nodular masses, mimicking vegetation,
A B
Figs 85.37A to C: Coronary artery bypass graft (CABG) volume-

rendering (VR) image. (A and B) Proximal and distal anastomosis
(circle) with good distal flow from LIMA to posterior descending
artery (PDA) graft; (C) Multiple grafts are seen including left
internal mammary artery (LIMA) and two venous grafts. Metal clips
are actually not causing much metal streak artifact. (LSC: Left
C subclavian artery).
A B
Figs 85.38A and B: Coronal oblique computed tomography angiogram (CTA) image (A) shows the opacified LIMA with patent distal
anastomosis with diagonal branch (circle) and distal flow. The corresponding cath angio view (B) confirms these findings.
A B
Figs 85.39A to C: (A) Mild in-stent narrowing in mid-left anterior

descending (LAD) stent (arrows); (B and C) Stent occlusion in
saphenous venous graft (SVG) graft as shown in two orthogonal
C images.
thrombus, and myxoma on imaging. Due to their small

size, these tumors are difficult to visualize on CT. In
comparison to a vegetation, fibroelastoma usually spares
the valve-free margin. They can be detected incidentally
in an asymptomatic patient or rarely can cause symptoms
due to embolization or rarely coronary ostial obstruction.
Metastasis is the most common malignancy of the
heart occurring in approximately 10–12% of patients
with extracardiac malignancy.93 Several tumors have
the propensity to metastasize to the heart including
melanoma, lung, breast, thyroid, renal, and esophageal
cancers (Figs 85.43A and B). Tumors can spread to heart
via hematogenous, direct, transvenous, or translymphatic
invasion. Direct invasion occurs usually with lung, breast,
Fig. 85.40: Left atrium (LA) myxoma. Axial computed tomography or other mediastinal tumors and usually manifests as
(CT) image shows a homogenous noncalcified mass (arrow) in pericardial effusion and thickening. Venous extension of
the left atrium attached to the interventricular septum. (AO: Aorta; the renal and adrenal carcinoma occurs via the inferior
LV: Left ventricle RV: Right ventricle).
vena cava (IVC), whereas lung cancer can extend into the
A B
Figs 85.41A and B: Lipoma. A large fat-computed tomography (CT) density mass (arrow) is seen in the right atrium extending up to
the superior vena cava (SVC) to right atrium (RA) junction superiorly as seen in image (B). (AO: Aorta; LV: Left ventricle; PA: Pulmonary
artery).
sarcoma occurring more commonly in females between

20 and 50 years of age. The most common site of cardiac
angiosarcoma is RA, while other sarcoma types tend to
occur more on the left side (Figs 85.44A and B). These
tumors appear as irregular nodular soft tissue masses
with infiltration into the pericardium with postcontrast
heterogenous enhancement. They also tend to involve
more than one chamber by direct infiltration. Often there
is associated pulmonary metastasis in approximately
two-thirds of patients.
Pericardial cysts are the result of blind-ended ventral
parietal pericardial recesses. Most patients are asympto-
matic and only rarely cause chest pain or dyspnea. The
most common location is cardiophrenic angle, 80% on
the right side. On CT, they appear as well-defined fluid
Fig. 85.42: Cardiac fibroma. There is a calcified intramyocardial
mass arising in the left ventricular lateral wall (arrow). Cardiac density smooth masses, which show no enhancement
fibromas are common in pediatric age groups. after contrast (Figs 85.45A and B).
Lipomatous hypertrophy of interatrial septum
occurs usually in older obese patients and are mostly
heart via pulmonary veins. Solitary cardiac metastasis
asymptomatic. On CT, the interatrial septum is thickened
is rare and hematogenous metastasis often is associated
> 20 mm with fat and is dumbbell-shaped with sparing of
with other organ metastasis. The masses are often irregular
the FOV (Figs 85.46A and B).
and demonstrate heterogenous enhancement.
Lymphoma of the heart is often secondary rather than
a primary cardiac tumor. Primary cardiac lymphoma is Pulmonary Vein Ablation
often the more aggressive non-Hodgkin B-cell, tends to Atrial fibrillation (AF), the most common sustained
occur at an older age and in patients with HIV infection. cardiac arrhythmia, is a major cause of stroke and the most
Common sites of involvement include pericardium, RA, common cardiac arrhythmia requiring hospitalization.
and AV groove. AF usually begins as paroxysmal AF, with approximately
Sarcoma is the most common primary cardiac 60% of patients converting spontaneously to normal sinus
malignancy. Angiosarcoma is the most common type of rhythm. Approximately 40% of patients develop persistent
A B
Figs 85.43A and B: Metastasis. Metastasis from melanoma involving the pericardium (star) aortic root and interventricular septum
(arrow).
A B
Figs 85.44A and B: Angiosarcoma. Angiosarcoma of the heart (star) involving right ventricle (RV) and extending across the right
atrioventricular (AV) to involve the right atrium (RA).
A B
Figs 85.45A and B: Pericardial cyst. A well-circumscribed water density nonenhancing mass is seen along the right atrium (RA).
A B
Figs 85.46A and B: Lipomatous hypertrophy of the interatrial septum. Lipomatous hypertrophy of the interatrial septum often spares the
fossa ovalis and produces a dumbbell-shaped appearance as shown in image (red circle). The fatty component can extend into the right
atrial wall to a variable degree and can sometimes encroach on the cavoatrial junction (arrow), although usually without any hemodynamic
consequences. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium: RV: Right ventricle; RVOT: Right ventricle outflow tract).
AF requiring medical or procedural intervention to restore length of the pulmonary trunk. MDCT is useful in detecting
normal sinus rhythm. Up to 50% of patients develop these pulmonary vein variations, knowledge of which is
recurrent AF within the first year of initial onset. vital for successful and complete ablation procedure.
Radiofrequency catheter ablation of the pulmonary vein Paroxysms of AF are initiated by trains of spontaneous
ostia has become the procedure of choice for treating AF. activity originating from the pulmonary veins (90–96%),
CT plays an important role in the evaluation of these with almost half arising in the left superior pulmonary
patients including pulmonary venous anatomy and any vein.
variation before the procedure, and detecting postablation In most individuals, sleeves of the left atrial myocar-
complications. Prospective gating is suitable for the dium extend into the pulmonary veins for a distance of
majority of patients except those with a very high heart 2–17 mm.
rate or obese patients, where either retrospective gating The myocardial sleeve of the LA surrounding the
with tube current modulation or a nongated chest CTA is proximal pulmonary venous segment and venoatrial
an option since motion-related artifacts are not an issue junction are believed to be the source of initiation and
for pulmonary veins and LA. maintenance of AF.94 Since the myocardial sleeve is
The most common normal pulmonary venous pattern longest around the left superior pulmonary vein, this
(75–80%) is two right and two left pulmonary veins vein alone contributes to > 50% of all ectopic beats for AF.
(superior and inferior) with a separate ostia for each vein. Radiofrequency ablation is performed at or within 5 mm
The pulmonary ostium is defined as the junction point of the pulmonary vein ostia; therefore, it is important to
of the pulmonary vein and LA at the point of parietal know the number of pulmonary veins and branching
pericardium reflection over the LA. The pulmonary vein pattern to ensure ablation of all ostia. Successful ablation
segment from the ostia to its first branch is called pulmo- of all electric connections to these veins can eliminate
nary vein trunk and the region of left atrial wall in between paroxysmal AF.
the two separate ipsilateral pulmonary veins is called the Because radiofrequency energy is preferably applied
intervenous saddle. The pulmonary vein ostia and the at the venoatrial junction of all the pulmonary veins to
intervenous saddle together are known as pulmonary avoid stenoses and eliminate ostial remnants that may
venous vestibule. Pulmonary venous branching is more contribute to recurrent AF, knowledge of how many
variable than pulmonary artery (PA) branching variations pulmonary veins are present, and their ostial locations, is
and includes variation in number, branching pattern, and important to ensure that all the ostia are ablated. However,
it is difficult and time-consuming to locate the ostia with Transcatheter Aortic

conventional angiography at the time of ablation. It is
Valve Replacement
important to know ostial orientation and the distance from
each ostium to the bifurcation of each pulmonary vein, Transcatheter aortic valve replacement (TAVR) is rapidly
because pulmonary vein narrowing seems to be critically becoming a popular alternative procedure for older
patients with severe symptomatic aortic stenosis, who are
dependent on catheter position and not on duration of
at high risk for open surgical repair. Several prosthetic
radiofrequency energy application.
valves are available including the balloon expandable
Planning CT is done to determine the number of PVs
Edwards Sapien Valve (Edwards Lifesciences Inc, Irvine,
and ostia, PV diameter and length to its first order branch,
CA, USA) in 23, 26, and 28 mm sizes and self-expanding
accessory veins and the presence of any LAA thrombus CoreValve (Medtronics Inc, Minneapolis, MN, USA)
(Figs 85.47 to 85.49). available in 23, 26, 29, and 31 mm sizes. In the United
States, only the 23- and 26-mm Edwards valves are
currently approved. The most common implantation route
is transfemoral. If this is not feasible, the Edwards Sapien
valve can be implanted via direct transapical approach or
rarely via a transaortic approach. Currently, this system is
not approved for subclavian artery approach.
Imaging is necessary before the procedure to deter-
mine annulus size, annulus area, degree of aortic calci-
fication, access route, and suitable fluoroscopic projection
angles that permit exact orthogonal views onto the valve.
Preprocedural assessment of the aortic root for TAVR
requires knowledge of the orientation of the aortic root
relative to the body axis. For correct deployment of a
percutanous valve prosthesis, the root orientation is
usually assessed by repeated X-ray aortograms in one
or two orthogonal planes. To simplify the procedure, 3D
Fig. 85.47: Segmented left atrium posterior view shows the data sets obtained by MDCT may allow the prediction of
draining pulmonary veins. (LLL: Left lower lobe; LUL: Left upper angiographic projections orthogonal to the valve plane
lobe; RLL: Right lower lobe; RUL: Right upper lobe).
(Figs 85.50A to C).
A B
Figs 85.48A and B: PV ablation. Computed tomography angiogram (CTA) images are loaded into PV ablation software in the cath lab
and are fused with cath images to show the catheter location in relation to the left atrium (LA) and its veins. The electrical map is then
generated and shows as red dots (B), which need to be ablated. (LIPV: Left inferior pulmonary vein; LSPV: Left superior pulmonary vein;
RIPV: Right inferior pulmonary vein; RSPV: Right superior pulmonary vein).
Fig. 85.49: Left atrial appendage (LAA) thrombus. This image

shows a patient with LAA thrombus (*) with chronic atrial fibrilla-
tion. Presence of thrombus is a contraindication for an ablation
procedure. (AO: Aorta; PA: Pulmonary artery).
A B
Figs 85.50A to C: Computed tomography angiogram (CTA) for

transcatheter aortic valve replacement (TAVR). CT plays an impor-
tant role in preoperative planning of TAVR patients, annulus size
(A) and area can be determined along with distance of LM and right
C upper lobe (RUL) to annulus (B) and (C).
CT imaging is the preferred method to evaluate the and (e) determine the cardiac position (position of the
aortic root, entire aorta, and iliac as well as common heart within the chest, orientation of the apex). Due to
femoral arteries preferably with single contrast injection. complexity of CHD, CT images often require evaluation on
Since most such patients are older and have some renal a dedicated 3D workstation.
impairment, it is preferable to do complete analysis with a
single contrast bolus. At our institution, we use 70–100 cc Atrial Septal Defect
of contrast for a retrospective gated chest from arch till
diaphragm and then a nongated CTA of the abdomen Atrial septal defect (ASD) accounts for about one-third of
and pelvis, (Table 85.9). Given the advanced age of the cases of CHD detected in adults.99 Ostium secundum atrial
patient, radiation exposure is of lesser concern after some septal defects are the most common type of interatrial
modifications for minimum recommended vessel lumen communication located at the fossa ovalis (Figs 85.51A
size for different delivery systems.95 to D) and ostium primum atrial septal defects are the next
Accurate annulus size measurement is very important most common type, the least common type of interatrial
in choosing the correct size of the prosthetic valve. A valve communication is a sinus venosus defect, which is located
that is too large can cause rupture of the annulus, which at the junction of either the superior or the inferior vena
is often fatal and if the valve is too small, there is a risk of cava with the RA. In patients with a superior sinus venosus
embolization and paravalvular leak.96–98 defect, there is frequently an abnormal connection of one
or all of the right pulmonary veins (Figs 85.52A and B).100
CT plays an important role in the evaluation of patients with
Anomalous Pulmonary Venous Connection
known or suspected CHD. With the advances in surgical Patients with total anomalous pulmonary venous conn-
techniques and CV anesthesia along with perioperative ection are often evaluated in early childhood, especially
care, many patients with complex CHD now survive the infracardiac variety, which is often associated with
into adulthood. Majority of these patients need lifelong obstruction of the vertical vein at the diaphragm leading
care including reoperations from underlying anatomy or to pulmonary venous HTN and edema. In partial anom-
previous surgical palliative procedures. alous pulmonary venous connection, one or more (but
Careful preparation is important for CT evaluation not all) pulmonary veins are connected to the vena cava
of pediatric and adult CHD. The CT protocol should or RA (Fig. 85.53). Abnormal connection of the right
be selected on a case by case basis depending on the upper pulmonary vein to the SVC occurs frequently
suspected anomaly. In neonates/infants, cardiac gating in the presence of a sinus venosus defect. Anomalous
is a challenge due to higher heart rates. To minimize connection of the right pulmonary vein or veins to the
other artifacts, we routinely intubate them and suspend IVC, often seen in association with hypoplasia of the right
their respiration during the few seconds of nongated CT lung and anomalous systemic arterial supply through
scanning. To avoid physical movement, the child is also aortopulmonary collateral vessels, is called scimitar
given some form of sedation or muscle paralytics. We use syndrome.
approximately 1 cc/lb of contrast and dilute it with 50%
saline, and the injection is made with hand. A central
Transposition of Great Arteries
umbilical venous catheter (UVC) catheter positioned
just below the cavoatrial junction is preferred, although The majority of adult patients with this condition have
a peripheral access also suffices the need for vascular undergone corrective surgery either atrial or arterial
opacification. In adults, the protocol includes a gated CTA switch operations. In the atrial switch procedure, an atrial
preferably with controlled heart rate. baffle redirects systemic venous blood to the anatomical
A systematic segmental approach is the best method left pulmonary ventricle and pulmonary venous blood to
to evaluate complex CHD by CTA as follows: (a) determine the anatomical right systemic ventricle, with a resultant
atrial situs derived from bronchial branching pattern functional atrial switch. The Mustard operation usually
(b) locate the three segments (the atrial chambers, is performed with pericardial tissue or a tissue graft used
ventricular chambers, and the great arteries), (c) identify for the baffle, whereas in the Senning procedure, the atrial
the cardiac connections (venoatrial, atrioventricular, and septum is reconstructed to form the baffle (Figs 85.54A
ventriculoarterial), (d) assess associated malformations, to D).101 In the arterial switch operation, the aorta and the
A B
C D
Figs 85.51A to D: Atrial septal defect (ASD) and Eisenmenger syndrome. Chest radiograph (A) shows an enlarged heart, dilated pulmo-
nary arteries, and shunt vascularity. Axial computed tomography (CT) image (B) shows a large septum secundum defect (circle) along
with a dilated right atrium. The main pulmonary artery is dilated (C) and there is mosaic perfusion in both lungs (D). Findings are sugges-
tive of secondary pulmonary HTN. (LA: Left atrium; LV: Left ventricle; RA: Left atrium; RV: Right ventricle; MPA: Main pulmonary artery).
A B
Figs 85.52A and B: Sinus venosus atrial septal defect (ASD). A connection is seen between distal superior vena cava (SVC) and roof
of the left atrium (A, red arrow) along with anomalous right upper lobe (RUL) pulmonary vein drainage into the SVC (B, yellow arrow).
(AO: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle; RAA: Right atrial appendage; PA: Pulmonary artery; PV: Pumonary
valve).
Fig. 85.53: Coronal computed tomography (CT) angio view.

Partial anomalous pulmonary venous return (PAPVR) of right lung
into inferior vena cava (IVC; arrow) is responsible for secondary
pulmonary artery hypertension (PAHTN) in this 48-year-old
female presenting with progressive shortness of breath and was
responsible for secondary pulmonary HTN. This patient was
operated successfully. (RA: Right atrium).
A B
C D
Figs 85.54A to D: Transposition of great arteries (TGA) status post (s/p) Senning operation. The pulmonary artery (PA) is to the left and
slightly posterior to the aorta (AO). It is connected to the morphological left ventricle (LV). An intra-atrial baffle (B) is connected to the
LV. The aorta is connected to the morphological right ventricle (RV), which is hypertrophied and dilated. The morphological left atrium
is surgically connected to the RV, thereby correcting the blood flow: systemic venous blood flows via the baffle (star) into the LV then
into PA and lungs. The oxygenated blood returns via the pulmonary veins into the LA and is directed into the RV and then into the aorta.
PA are transected above the semilunar valves and moved Tetralogy of Fallot
to the correct circulatory position. The coronary arteries
Because the surgical repair is usually performed during
are excised from the right side with a button-like margin
early childhood, the role of CT in diagnosing TOF is
of tissue around each artery and are implanted just above
minimal. In postsurgical evaluation, the main purpose
the valve on the left side of the heart; the areas from which
of CT is to visualize extracardiac complications, to depict
the coronary arteries were excised are then patched with
the morphological characteristics of the main PA and its
pericardium. Postoperative evaluation after an atrial
branches (to identify any obstruction, distortion after
switch procedure may be difficult with echocardiography
previous palliative shunt creation, or aneurysm), and to
because of the retrosternal position of the pulmonary
detect any right ventricular enlargement due to chronic
trunk and pulmonary arteries.
volume overload in the presence of severe pulmonary
regurgitation (Figs 85.56A and B).
Congenitally Corrected Transposition of the
Great Arteries Coarctation of the Aorta Congenital
In this condition, there is discordant atrioventricular and Heart Disease
ventriculoarterial connections, such that the morph- The most common type of coarctation seen in adults is
ological RA is connected via the mitral valve to the postductal, where the narrowing is located just distal
morphological right-sided LV and then to the PA. The to the left subclavian artery. The aorta just distal to the
morphological LA is connected via the tricuspid valve coarctation is typically dilated and there is often dilatation
to the morphological left-sided RV and then to aorta of the left subclavian artery (Fig. 85.57). There is slightly
(Figs 85.55A and B). In patients with congenitally corrected higher incidence of associated bicuspid AVs in patients with
transposition, the great arteries are parallel (side-by-side Turner syndrome. Preoperatively, CT can easily establish
arrangement). In the absence of associated anomalies the diagnosis of coarctation and provide additional
(e.g. ventricular septal defect, pulmonary outflow tract information about collateral flow. Postoperatively, CT can
obstruction), congenitally corrected transposition of the be used to evaluate restenosis, patch dilatation, or patency
great arteries is often asymptomatic until adulthood. of endo-stent depending on the type of treatment used.
A B
Figs 85.55A and B: Corrected transposition. In congenitally corrected transposition, there is atrioventricular and ventriculoarterial discord-
ance such that the left atrium (LA) is connected to right ventricle (RV), which is connected to the aorta. The right atrium (RA) is connected
to left ventricle (LV) and is then connected to the pulmonary artery (PA). The circulation circuit in these patients is: systemic blood return
morphological RA morphological LV pulmonary arteries. Oxygenated blood from lungs return via pulmonary veins morpho-
logical LA morphological R V aorta.
A B
Figs 85.56A and B: Tetralogy of Fallot (TOF) after palliation treatment. A 64-year-old female with prior Blalock–Taussig palliation for
tetralogy of Fallot. Oblique coronal three-dimensional (3D) volume-rendered reconstruction demonstrating right ventricular (RV) hyper-
trophy, overriding aorta (AO): and subaortic ventricular septal defect (VSD; asterisk). 3D volume-rendered slab reconstruction illustrating
the right ventricular outflow tract obstruction (arrow). (PA: Pulmonary artery).
Fig. 85.57: Coarctation of aorta. Volume-rendered sagittal oblique Fig. 85.58: Left ventricular assist device (LVAD). Typical loca-
view of the thoracic aorta demonstrates focal narrowing of the tion of inflow cannula (A) in left ventricle (LV) midcavity, pointed
descending aorta (circle) beyond the origin of the left subclavian forward the mitral valve. The outflow cannula (B) is connected to
artery (LSA). A few of the dilated collateral vessels are also seen the ascending aorta.
in this image (arrows).
Left Ventricular Assist Device worsening right heart failure, arrhythmias, hypotension,
and hemolysis can be indicators of suboptimal LVAD func-
There has been a steady increase in the number of contin- tion or related complications such as tamponade or aortic
uous flow left ventricular assist devices (LVAD) implanted regurgitation. Accurate early recognition and manage-
annually, since the Heartmate II LVAD was approved for ment of these symptoms is important. CT has been used
bridge to transplant in 2008 and destination therapy in successfully to diagnose several common LVAD compli-
2010. Given the persistent donor shortage and improve- cations. CTA can delineate inflow and outflow cannula
ments in LVAD technology, patients are increasingly likely position, abnormal angulation, cannula thrombi, perican-
to undergo LVAD placement and remain on LVAD therapy nula fluid collection, pericardial effusion, and driveline
for prolonged periods of time. Persistent left heart failure, position (Figs 85.58 to 85.60). 3D reconstruction, a wide
A B
Figs 85.59A and B: Outflow cannula thrombosis (B) in this patient with chronic heart failure and post-left ventricular assist device
(LVAD) complication. This was also seen in the aortic root injection angiogram done in an attempt to remove the thrombus.
contains 15–35 mL of serous fluid.105 The normal peri-

cardial thickness on CT/MR is < 2 mm.106,107 On CT, it is a
linear isodense structure between the epicardial and
pericardial fat.
Pericardial Duplication Cyst

These are congenital encapsulated cysts in close proximity
to the pericardium that usually do not communicate
with the parent cavity. The incidence is rare, about 1 in
100,000.108 They can occur anywhere around the heart, but
the right cardiophrenic angle is the commonest site.109 On
imaging, these are seen as round or elliptical, thin-walled
cystic lesions with low-density fluid within (H.U. 0–10;
Figs 85.45A and B). However, higher attenuation contents
Fig. 85.60: Left ventricular assist device (LVAD) obstruction.
Abnormal position of inflow cannula (A), which is abutting the left
could be seen with infection or hemorrhage.
ventricle (LV) lateral (arrow) wall causing obstruction. (RV: Right
ventricle). Pericardial Defect
FOV, and reproducibility of measurements are advantages It is a rare anomaly with the spectrum ranging from a
of CTA over echocardiographic techniques. In addition, small defect to complete absence of the pericardium.110
gated and dynamic CTA can provide functional infor- It is commonly seen along the left side of the heart and
mation, including right ventricular function and cardiac may have associated congenital abnormalities such as
output.102–104 ASD, PDA, or a bicuspid AV.111
The lack of visualization of normal pericardium is a
direct sign. However, it is difficult to visualize the normal
Pericardial Diseases pericardium along the left side of the heart. The indirect
Pericardium is a dual layer fibrous sac surrounding the signs include levorotation of the heart, and interposition
heart with inner visceral layer adherent to the epicardium of lung tissue between the aorta and main PA or between
and the outer parietal layer. The potential space between inferior border of heart and diaphragm. Excessive motion
these two layers is called the pericardial cavity that of the cardiac apex is seen on cine MR images.112 Complete
absence of pericardium is usually asymptomatic. Partial further assessment with clinical history, echocardio-
absence may cause complications such as herniation with graphy, or cardiac MR is indicated.
entrapment of a cardiac chamber or structure.
Constrictive Pericarditis
Pericardial Effusion Thickening and fibrosis of pericardium from inflammatory
Pericardial effusion may be transudate, exudates, or conditions mentioned above can lead to constrictive
hemorrhage. The role of imaging is to determine its pericarditis. Pericardial calcification may be seen in up to
presence, pericardial thickening, and mediastinal or 50% of these cases.114
thoracic abnormalities to explain the cause. There are no Physiologically, there is impaired diastolic filling of
specific criteria for distinguishing physiological versus the ventricles that leads to equalization of right and left
pathological pericardial fluid. Visual evaluation and ventricular diastolic pressures.115 The patient may present
categorization as mild, moderate, or severe is routinely with right heart failure. The treatment is surgical stripping
performed (Fig. 85.61). Pericardial thickness of > 4 mm is of the pericardium. It is important to distinguish this from
considered abnormal.113 Mediastinal lymphadenopathy or restrictive cardiomyopathy, since they may have the same
presence of thoracic malignancy may raise the suspicion physiology although the treatment is completely different.
for malignant effusion. Hemorrhagic effusions are seen Flattening of the RV free wall and tubular configuration of
in the setting of trauma, dissection, postsurgery, and ventricles are other features that may be seen.
will show CT attenuation value between 40 and 80 H.U.
(Fig. 85.62). Pericardial Tumors
Primary pericardial tumors are extremely rare and
Pericardial Calcification metastases are far more common (see Fig. 85.43).116 Focal
Calcification of pericardium is secondary to a prior insult. or generalized pericardial thickening, enhancement,
Postcardiac surgery, hemopericardium, exudative effusions nodularity, and hemorrhagic pericardial effusions are
such as tuberculous, postradiation are some of the common some of the features that may be seen on CT. The common
causes of pericardial calcifications (Fig. 85.63). Pericardial primary malignancies that metastasize to the pericardium
calcification may result in constrictive physiology and are lung, breast, melanoma, and lymphoma.117
Fig. 85.61: Pericardial effusion and tamponade. Large amount Fig. 85.62: Hemopericardium. High attenuating fluid (45 H.U.) in
of pericardial effusion (stars) producing a mass effect leading to pericardial space is suggestive of blood. The cause of hemoperi-
compression of right ventricle (RV) and dilation of inferior vena cardium in this patient was uremia.
cava (IVC), hepatic veins, and coronary sinus (CS) (not shown),
supports the presence of tamponade physiology. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium).
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Fig. 85.63: Pericardial calcification. The pericardium is thickened with ECG-gated multislice spiral CT: value of different
measuring 4 mm and shows areas of spotty calcification (arrows). image reconstruction algorithms in comparison to MRI.
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Index
Page number followed by f and t indicate figure and table respectively.
A overview, 1856 spared areas, 1986

rheumatic heart disease, 1859 subclinical pulmonary edema, 1986
Abdominal aortic aneurysm, ultrasound vegetations in, 1858–1859 Acute rheumatic fever (ARF), 931
stethoscope for screening of, 294– location for, 1958 diagnosis of carditis in, 765–775
295 size and embolic risk, 1958–1959 echo and Doppler studies, 768–770
Aberration, 81 ACS. See Acute coronary syndromes echocardiography vs. clinical
Abiomed Impella, 1224f (ACS) examination, 767–768
Abnormal mitral arcade, 1613–1614 AcuNav Diagnostic Ultrasound echo interrogation, 767, 769t–770t
Absorption, 56–57 Catheter, 644, 645f. See also M-mode echo in, 770
ACC. See American College of Cardiology Intracardiac echocardiography myocarditis, 766
(ACC) (ICE) pericarditis, 766
Accessory mitral orifice, 1615 Acute aortic regurgitation, 1972 pitfalls in Jones criteria, 766–767
ACCF. See American College of acute decompensation, 1974 regurgitation, 766
Cardiology acute regurgitation, 1974 rheumatic vs. nonrheumatic
Foundation (ACCF) aortic dissection, 1972 regurgitation, 768
Accreditation Council of Graduate diastolic fluttering, 1974 secondary prophylaxis, fidelity of, 775
Medical Education (ACGME), 754 infective endocarditis, 1972 two-dimensional echo, 770–772
Acetylcholine (Ach), 450, 451–452, 451f, left ventricular hypertrophy, 1974 overview, 765
455 post trauma, 1972t physiological vs pathological
Ach. See Acetylcholine (Ach) suprasternal notch, 1974 regurgitation, 772–773
ACM. See Alcohol-induced transthoracic views, 1974 Vijaya's echo criteria, 771, 771t
cardiomyopathy (ACM) ventricular septal defect, 1973f Adenosine stress test, 1333–1334
Acoustic energy safety, 111 Acute chest pain, CT scan for, 2045–2047 ADMA (asymmetrical dimethyl arginine),
Acoustic enhancement, 734, 734f Acute coronary syndromes (ACS) 454
Acoustic radiation force impulses (ARFI), contrast echocardiography for, 443 Adriamycin-induced cardiomyopathy,
1995 echocardiography role in, 1292–1294 1397f
Acoustic shadowing, 61, 62f, 733–734 evaluation of, by CONTISCAN Adult congenital heart disease (ACHD),
Acquired heart diseases, in childhood, transducer, 226
1791–1855
1856–1864 Acute heart failure syndrome, 1986
aortic root in, 1797, 1797f
aortic insufficiency, 1860f Acute LV remodeling (ALVRM), 1325
complex congenital heart defects,
complications of, 1859 Acute myocardial infarction, three-
1826–1848
coronary ectasia and aneurysms, dimensional speckle tracking and,
echocardiography, key concepts of,
1861–1863 376t
Duke criteria for, 1857 1793–1797
Acute pericarditis, 1436
echocardiographic findings, 1857– abdominal situs, 1795
Acute respiratory distress syndrome
1859 (ARDS), 1983, 1986 assessment of shunts, 1795–1796
infective endocarditis, 1856–1857 acute heart failure syndrome, 1986 atrial situs solitus, 1794
infective endocarditis prophylaxis, chest X-ray, 1986 atrioventricular relationship, 1795
1859 differential diagnosis, 1986 position of apex, 1794
Jones criteria for, 1859–1861 diffuse lung injury, 1986 pregnancy and, 1796–1797
acute valvulitis, 1860 lung ultrasound scan, 1986 simplified segmental approach,
chronic RHD, 1860–1861 sonographic pattern 1793–1795
minor criteria, 1860 multiple diffuse B-lines, 1986, 1986f VA connections, 1795
Kawasaki disease, 1861, 1862t small subpleural consolidations, indications for TEE, 1792t
mitral valve insufficiency, 1860f 1986, 1986f overview of, 1791–1793
I-II Comprehensive Textbook of Echocardiography Index I-III
simple congenital heart defects, AmplatzerTM multifenestrated septal acute aortic diseases, 976–982 penetrating aortic ulceration, 961, echocardiographic measurements in, ACHD and, 1797
1798–1813 occluder, 553, 554f aortic aneurysm, 974–976 962f 1640–1641, 1640f anatomy of, 897
surgical terms in, 1794t AmplatzerTM ventricular septal occluder, aortography, 980 TEE characterization of, 960–961 postoperative evaluation of, 1641– echocardiographic assessment of
terminology in, 1793t 558 atherosclerotic disease, 971–974 Aortic atherosclerosis 1644, 1642f dimension, 1797
valvular disease, 1813–1826 Amplifier, 60 celiac artery, 970 in elderly, 1921–1923, 1922f ventricular septal defect, 1633–1634, parasternal long-axis view of, 1797f
Aging, effect of Amplitude Doppler. See Power Doppler descending thoracic aorta, 970 epiaortic ultrasonography in, 1633f–1634f Aortic sclerosis, echocardiography of,
on HV Doppler, 303 Amptazer duct occlude II (ADO II) series, human anatomic cross-section, 968f 640–641, 641f Aortic regurgitation (AR), 806–812, 899–900
on pulmonary venous flow, 329–330, 1598 intramural hematoma, 978 in female, 1896f 930–944, 1628–1630, 1630f Aortic stenosis, 802–806, 896–918
330f AMVL. See Anterior mitral valve leaflet left carotid and subclavian artery, Aortic balloon valvuloplasty (ABV), 541 in adults, 1824 in adults, 1824
Air embolism, 1977 (AMVL) 970, 971f Aortic coarctation. See Coarctation of aortic root dilatation, 1629–1630 aortic jet velocity, 803–804
coronary arteries, 1977 Amyloid cardiomyopathy, 1398–1401, magnetic resonance imaging and, aorta (CoA) aortic valve surgery, timing of, aortic valve Doppler examination,
hemodynamic instability, 1977 1399f–1400f 979 Aortic dissection, 954–958, 1974 941–942 904–912
paradoxical emboli, 1977 Amyloidosis, 1872–1874, 1873f–1874f orientation, 968–970 aorta distal to left subclavian artery, cardiac MRI for, 2010–2011, 2011f appropriate examination, 904–905
postcardiac arrest, 1977 velocity vector imaging in, 399 real time 3D, 981 954 causes of, 1628–1629, 1629t mean and peak gradients, 906
post multiple injuries, 1977 Analogue-to-digital converter, 67 transgastric level, 969 in ascending aorta, 954 continuous wave Doppler, 810–811 pressure recovery, 912
respiratory distress, 1977 Angiosarcomas, 1485–1486, 2055f Aortic abscess diagnostic imaging, 1974 signal of, 1279 valve area measurement, 906–907
Air microbubble contrast, 436 Angle of insonation, 66 in native valve, 1045f dissection, 1974 3DE VCA measurement of, 280 aortic valve replacement for, 540–546
AIUM. See American Institute of Annuloaortic ectasia, 974, 1695 transesophageal approach, 1053f in elderly, 1937–1942, 1937f–1938f, diastolic fluttering of AML, 807–808 associated conditions, 806
Ultrasound in Medicine (AIUM) Annuloplasty ring dehiscence, 608 Aortic aneurysms, 951–954, 1695–1696, 1941f echocardiography asymptomatic patients with normal
ALa. See Anterior leaflet angle (ALa) “Annulus paradoxus,” 1446–1447 1797 acute coronary syndrome and, 1940 exercise, 811 LV function, 912–923
Alcohol-induced cardiomyopathy (ACM), Anomalous coronary artery (ACA), anomalous origin of PA from color Doppler flow in, 1942 indications for, 807t cardiac MRI for, 2010, 2011f
1397 1763–1766 ascending aorta, 1695–1696, descending thoracic aorta, 1942 role of transthoracic, 811 and carotid ultrasound findings, 693
Alcohol-induced necrosis, 1364f perfusing lumen, 1942 three-dimensional, 812 with coexistent subvalvular stenosis,
computed tomography for, 2047– 1696f
Alcohol septal ablation rupture, 1938f–1940f etiology of, 930–935 903–904
2049, 2047f echocardiographic evaluation of,
in HCM, 1362–1363 epidemiology, 954 acute rheumatic fever, 931 critical neonatal, 1622–1623
in elderly, 1947f 951–954, 952f–954f
for hypertrophic obstructive intramural hematoma, 957–958 aortic root and aortic annulus, 3DE assessment of, 279
Anomalous origin of left main coronary in elderly, 1934–1937
cardiomyopathy, 571 TEE diagnosis of, 955–957 diseases of, 935, 936f echocardiography in, 898–904
artery from pulmonary artery cystic medial degeneration and, 1936
Alfieri stitch, 539 TTE evaluation, 955 aortic valve cusps, diseases of, aortic measurements, 902–903
(ALCAPA), 2048, 2048f etiology of, 1936
Aliasing, 70, 137, 736. See also Artifacts Type A dissection, 954, 955–957 930–935, 932f–935f aortic sclerosis, 899–900
Anomalous papillary muscle, 1354f natural history of, 1937
Alveolar-interstitial syndrome, 1986 Behçet's disease, 935 bicuspid aortic valve, 902
Anomalous pulmonary venous drainage, with rupture, 1936f Type B dissection, 954
ALVRM. See Acute LV remodeling congenital cardiac defects and aorta criteria for, 914t–916t
1676 epidemiology of, 951 Aortic dissection, epiaortic
(ALVRM) abnormalities, 930–931 indications and appropriateness for,
Antegrade velocity of mitral inflow, 869 pathophysiology of, 951 ultrasonography for detection of,
Ambulatory echocardiography, 227–229, infective endocarditis, 931 913, 914t
Anterior circulation, 665–668, 667f–669f pulmonary artery sling, 1696 641
229t systemic illnesses, 931 M-mode, 898–899, 899f
Anterior leaflet angle (ALa), 1423 transesophageal imaging of, 974–976 Aortic insufficiency, 1860f
American College of Cardiology (ACC), flow convergence, 809 recommendations for, 802t
Anterior mitral valve leaflet (AMVL), 777 Aortic arch (AoA), 664–665, 665f, 666f, and carotid ultrasound findings, 693
750, 751–752, 757, 758 follow-up in, 812 stress, 805–806, 912–913
Anterior pericardial effusion, 170 1538f, 1795f Aortic isthmus, 1538f
American College of Cardiology grading of, 810f three-dimensional, 806, 900–902
Anthracycline exposure, 395 abnormities, 1690–1692 Aortic leiomyosarcoma, 1489f–1490f
Foundation (ACCF), 758, 2045 left ventricle adaptation to, 941 two-dimensional, 899
Aorta cervical aortic arch, 1691 Aortic lumen to pulmonary artery fistulas
“American correction,” 580 left ventricular outflow abnormality, in elderly, 1924–1934
echocardiographic evaluation, right aortic arch, 1690–1691 (APAF), 1773 1628–1629 aortic valve area, estimation of, 1926
American Heart Association (AHA), 6, 758
American Institute of Ultrasound in 945–951 vascular rings, 1691 Aortico-left ventricular tunnel, 1632 pulsed wave Doppler, 810 2D echocardiography, 1924–1928,
Medicine (AIUM), 110 basic principles, 945 anomalies, 665 Aortico-right atrial tunnel, 1632 regurgitant jet size, 808–809 1925f, 1927f
American Registry of Diagnostic Medical normal aortic size, 951 coarctation of aorta, 1692–1694 Aortico-right ventricle tunnel, 1632 severity, quantification of, 936–941 3D echocardiography, indications for,
Sonography (ARDMS), 754 PLAX view, 945 interruption of, 1694–1695, 1695t Aortic override, 1633–1644 color Doppler methods, 936–939 1933t
American Society of Anesthesiologists PSAX view, 946 types of, 664–665, 665f aortic arch, 1638–1639 continuous wave Doppler methods, LFLG-AS with low EF, 1929f
(ASA), 638 transesophageal echocardiography, Aortic arch anomalies, 1770–1773 aortopulmonary collaterals, 1639– 940–941, 941f live/real time 3D TTE, 1930–1933
American Society of Echocardiography, 949–950, 950f, 951f aortic arch to pulmonary artery 1640 mitral valve findings, 941 mild, 1921f
907 transthoracic echocardiography, fistula, 1773 coronary artery anomalies, 1640 M-mode and 2D findings, 936, 937f PLFLG-AS with preserved EF,
American Society of Echocardiography 945–949, 946f, 947f–949f coarctation of aorta, 1770–1773 patent arterial duct, 1639–1640 parameters to grade, 937t 1929–1930
(ASE), 91, 132, 296, 516, 638, 753, and esophagus, 969 Aortic arch scanning protocol, carotid pulmonary arteries, 1637–1638, 1638f pulsed wave Doppler methods, prevalence and pathophysiology,
1115, 1323 genetic syndromes affecting, 958–959 ultrasound, 676f pulmonary stenosis, 1634–1637 939–940, 940f 1924
A-mode echocardiography, 57, 58f bicuspid aortopathy, 959 Aortic atheroma, 959–961 tetralogy of Fallot with absent severity of, 808, 808t, 812, 1630 severe, 1931f–1932f, 1933f
Amplatzer PFO Occluder, 557 Marfan syndrome, 958–959 appearance on TTE and TEE, 959, pulmonary valve, 1637, 1637f sinus of Valsalva aneurysm and, 1631 transcatheter aortic valve
AmplatzerTM atrial septal occluder, transesophageal imaging of, 967–982, 960f cardiac catheterization, indications vena contracta imaging in, 938 replacement, 1934, 1935f
552–553, 554f 969f–970f grading, 961t for, 1641 Aortic root, 1621 ventricular response to, 1933–1934
I-IV Comprehensive Textbook of Echocardiography Index I-V
etiology of, 897–898 3D echocardiography, 812 color flow mapping, 1603 Arterial–ventricular coupling, 1121–1122 radiofrequency catheter ablation, and PV Doppler, 342, 342f
low-gradient, low stroke volume, diastolic fluttering of AML, 807–808 types of, 1602 Artifacts, 61, 732–749 2056 Qp/Qs ratios in, 1589–1590
severe, 908–911 exercise echocardiography, 811 APAF. See Aortic lumen to pulmonary acoustic shadowing and and HV Doppler, 309, 309f secundum, 1734–1742
aortic valve calcium score, 911 flow convergence, 809 artery fistulas (APAF) enhancement, 61, 62f, 733–734, and PV Doppler, 333, 333f sinus venosus, 1586, 1742–1743
dobutamine infusion, 910 follow-up in, 812 Aperture 734f TD imaging in, 353 stepwise evaluation for, 1586t
hypertension and, 911 grading of, 810f 3D ultrasound imaging and, 80 aliasing, 736 Atrial flutter, and PV Doppler, 333, 333f three-dimensional speckle tracking
low transvalvular gradients and, 913 pulsed wave Doppler, 810 and TEE image quality, 104, 106f from assumptions made by Atrial myxoma, 1468 and, 376t
mean transaortic pressure gradient, regurgitant jet size, 808–809 Apical approach, of three-dimensional ultrasound equipment Atrial septal defects (ASD), 1134, 1279, types of, 1585–1586
804–805 role of TEE in, 811 echocardiography, 244, 254f, 255f software, 749t 1585–1591, 1734 in women, 1900–1901, 1900f–1901f
normal aortic valve anatomy, 896–897 severity of, 808, 808t, 812 Apical hypertrophic cardiomyopathy, attenuation and enhancement, 61 in adults, 1799–1802, 1801t Atrial septum, 1248, 1962
aortic root, 897 aortic stenosis, 802–806 1356 categorization of, 733 cardiac catheterization, 1802 ablation,patients, 1962
aortic valve, 896–897 aortic jet velocity, 803–804 Apical window, TTE, 146, 148 double image, 736 closure of, 1802 abnormalities, 1962
oveview, 896 associated conditions, 806 five-chamber plane, 153–154, 153f, 3D ultrasound imaging and, 80–81 contrast echocardiography, 1801– frequent, 1963
“pseudosevere group” of, 911 3D echocardiography, 806 154f, 154t effects of tissues in ultrasonic beam, 1802 atrial tumor, 1963
sclerosis to stenosis, 897–898 mean transaortic pressure gradient, Doppler imaging, 153–154, 154f, 154t 732, 733f echocardiography, 1799 fossa ovalis region, 1962
severity, estimation of, 803, 907–908, 804–805 technique, 153, 153f encountered in day-to-day clinical exercise testing, 1802 lipomatous, 1963
907t severity of, 803 four-chamber plane, 148–153, practice, 738f–748f MRI/CTA for, 1802 obesity, patients, 1963
definition of, 907, 907t stress echocardiography in, 805–806 148f–153f, 149t identification and elimination of, 737 postoperative adult in, 1802 saline contrast study, 1962
methodological limitations in, 908, valve area, 805 chamber quantification, 150–151, mirror image, 735–736 transesophageal echocardiography, septum primum, 1962
908t, 909t role of 3D TEE in operating room in, 151f, 152f on pulmonary venous Doppler, 1800–1801, 1800f septum secundum, 1962
pitfalls in estimation of, 908 582–588, 594f–600f diastolic function assessment, 343–345, 344f–345f types of, 1799–1800, 1799f trans-septal puncture, 1962
technical errors in, 908 stress echocardiography in, 1318 149–150, 150ff, 151f range ambiguity, 736 atrial septum, evaluation of, 1590 Waterston’s groove, 1963
strain in evaluation of, 913 recognition and interpretation of, 732 basal long-axis or bicaval view,
Aortic valve Doppler examination, Doppler imaging, 149, 150f Atrioventricular and ventricoarterial
subvalvular, 1624–1626 reflection, 62, 62f 1590–1591
904–912 intracardiac shunts assessment, 153, discordance, 1664–1670
supravalvular, 1626–1628 reverberation, 62, 62f, 734–735, 735f basal short-axis view, 1590
Aortic valve fibroelastoma, 1479f 153f abnormalities of RVOT, 1669
valve area, 805 side lobe artifacts, 62–63, 63f four-chamber view, 1591
Aortic valve pseudoaneurysm, 1046f right heart assessment, 151–152, 152f associated defects, 1664
valvular, 1618–1624 in three-dimensional cardiac catheterization in, 1590
Aortic valve sclerosis, 1923f technique, 148, 148f, 149t associated malformations, 1667–1669
vs. hypoplastic left ventricle, 1623 echocardiography, 736–737 closure of
in elderly, 1923–1924, 1923f two-dimensional anatomic imaging, tricuspid value abnormalities,
Aortic trauma, and free rupture, 961–963 transesophageal echocardiography Amplatzer ASD occluder, 557f
bicuspid, 1931f 148–149, 149f 1667–1669, 1668f
Aortic valve (AV), 582–584 and, 107–110, 113–116, 114f ASD closure using intracardiac
echocardiographic findings for, long-axis or three-chamber plane, ventricular septal defect, 1667, 1668f
anatomy of, 896–897 ASD. See Atrial septal defect (ASD) echocardiography, 559f
1923–1924, 1930f 155, 156f coarctation of aorta, 1669
3DE assessment of, 278f, 279, 279f ASE. See American Society of ASD sizing ballon, 556f
normal hemodynamics in, 1924 two-chamber plane, 154–155, 154f, coronary artery anatomy in, 1664–
3D echo of, 520–522, 520f Echocardiography (ASE) closure devices, 552–553, 555f
pathophysiology of, 1923–1924 155f 1667, 1665f–1666f
intermittent opening of, 1242f Asymptomatic patients, with normal LV 3D TEE diagnosis of ASD, 551, 553,
prevalence of, 1923–1924 chamber quantification, 154–155 553f four-chamber views, 1665–1666
short axis of, 1536f function, 912–923
systolic murmur, 1923 Doppler imaging, 155 3D TEE monitoring of, 554–555, 557f, PLAX view, 1667
ventricular assist devices, 1231, 1231f Atherosclerosis, 959–961
Aortic valve stenosis (AS) technique, 154, 154f grading, 961t 558f segmental analysis, 1664–1665
Aortic valve, in adults, 1821–1826
aortic regurgitation, 1824 in adults, 919 two-dimensional anatomic imaging, transesophageal imaging of, 971–974 3D TEE sizing of ASDs, 556f subcostal sagittal view, 1666–1667
aortic stenosis, 1824 calcific, 919 154, 154f, 155f Atherosclerotic plaque, mild, 960f 3D TEE visualization of ASD rim, 557f left ventricular outflow tract
bicuspid, 1821–1824 dobutamine stress echocardiography, Application specific integrated circuits Atherosclerotic Risk in Communities ICE imaging during, 648, 650, 650f obstruction, 1669
cardiac catheterization, 1822–1823 response to, 921 (ASICs), 77, 77f, 78 (ARIC) study, 1948 indications for, 551 Atrioventricular canal defect (AVCD),
echocardiography, 1821–1822 invasive evaluation of, 923 ARDMS. See American Registry of Atresia, of left main coronary artery, 1689 secundum ASDs, 552, 554f 1535, 1544f, 1546f
MRI/CT for, 1823 invasive vs. noninvasive evaluation Diagnostic Medical Sonography Atretic coronary sinus ostium, 1683 computed tomography for, 2059, Atrioventricular connection,
postoperative adult, 1824 of, 922–924 (ARDMS) Atrial and atrioventricular valve 2060f echocardiography of, 1558t,
stress testing, 1823–1824 SAVR role in, 926–927 Area under the curve (AUC), 452, 452f abnormalities, 1773–1776 coronary sinus, 1586 1577–1579, 1578f
subaortic stenosis, 1824–1825 severity grade, 919 ARFI. See Acoustic radiation force cor triatriatum sinister, 1773–1775 degree of left-to-right shunt in, 1590 concordant, 1579
supravalvular aortic stenosis, three main entities of severe, 926t impulses (ARFI) Ebstein’s anomaly, 1775–1776 direction of shunt, 1588–1589 discordant, 1579
1825–1826 Aortocameral communications, 1632 ARIC. See Atherosclerotic Risk in isolated mitral valve cleft, 1775 3D TEE for, 513 isomeric, 1580
Aortic valve annulus, 1621 aortico-left ventricular tunnel and, Communities (ARIC) study Atrial balloon septostomy, 531 echocardiographic imaging in, and loop rule, 1580
Aortic valve area (AVA), 279, 1622 1632 Arrhythmogenic right ventricular Atrial ball valve thrombus, 845 1586–1588 uniatrial and biventricular
calculation of, 543, 585–586 aortico-right atrial tunnel and, 1632 cardiomyopathy (ARVC), 1395– Atrial fibrillation (AF), 1259–1260 fossa ovalis, 1586 connection, 1580
Aortic valve calcium score:, 911 aortico-right ventricle tunnel and, 1396 computed tomography for, 2054–2057 for percutaneous device closure, 1591 univentricular, 1579–1580
Aortic valve diseases, 802–816, 1318 1632 Arrhythmogenic right ventricular myocardial sleeve of LA, 2056 objectives, 1585 Atrioventricular dissociation
aortic regurgitation, 806–812 Aortopulmonary window (APW), 1751, dysplasia (ARVD), 395–396, 1395– paroxysms of, 2056 ostium primum, 1586 and HV Doppler, 308, 308f
continuous wave Doppler, 810–811 1753f–1754f 1396, 1396f planning CT, 2057 patent foramen ovale, 1585–1586 and PV Doppler, 331–332, 332f
I-VI Comprehensive Textbook of Echocardiography Index I-VII
Atrioventricular septal defects (AVSD), BART (blue away red toward), 64 Block matching, 275 with carcinoid syndrome, 1408 coronary calcium scoring, 2044–2045, stress myocardial imaging using,
1604–1610, 1746–1747 Basilar artery, 668 Blood velocity, and Doppler signal, 66 cardiac involvement in, 1408 2045f, 2046f 2042–2043
in adults, 1809–1810 Battery-powered ultrasound imagers, Blooming, 433, 434f 2D transthoracic echocardiography, coronary stent evaluation, 2050 technique, 2027, 2028t, 2029t
echocardiography, 1809–1810 291–292, 292f BLSVC. See Bilateral superior vena cava 1007f left ventricular assist device, 2063– tetralogy of fallot, 2062, 2063f
postoperative adult, 1810 Beam width artifact, 733, 734f (BLSVC) echocardiographic features, 1409– 2064 transcatheter aortic valve
AV valve regurgitation, 1610 Behçet's disease, 935 Blunt chest trauma, 1969 1413 pericardial diseases, 2064–2066 replacement, 2029t, 2057–2059
with common valvular orifice, Benign cardiac fibromas, 1480 assault, 1969 endocardial deposits, 1409, pulmonary vein ablation, 2054–2057 transposition of great arteries,
1606–1610 Berlin Heart INCOR Assist Device, 1228 blunt objects, 1969 1410f–1412f re-do coronary artery bypass graft, 2059–2062, 2061f
complete, 1606–1610 Bernoulli equation, limitations of, 1574 cardiac trauma, 1970t left-sided valvular involvement, 1409, 2049 congenitally corrected, 2062, 2062f
complex, 1608 Biatrial myxoma, 1470f hemodynamic disturbances, 1970t 1410f constrictive pericarditis, 2065 Cardiac contusion, 1971
3D TTE for, 1746–1747 Bicuspid aortic valve (BAV), 898, 1766– hemodynamic instability, 1970t pulmonary valve cusps, 1409 contrast media and injection and, biochemical cardiac markers, 1971
echocardiography in, features of, 1770. See also Aortic valve infarction, 1970t tricuspid regurgitation, 1412 2029t echocardiogram, 1971
1605t in adults, 1821–1824 myocardial ischemia, 1970t Carcinoid tumors, 1874–1875, 1876f coronary calcium score protocol, echo findings sum, 1971
hemodynamic assessment of, 1610 cardiac catheterization, 1822–1823 intraoperative assessment, 1970t Cardiac amyloidosis, 352, 373, 1261 2028t electrocardiogram, 1971
LAVV regurgitation and, 1747 echocardiography, 1821–1822 massive hemothorax, 1970 Cardiac MRI for, 2008f FOV for, 2027 myocardium, 1971
LVOT obstruction in, 1608, 1609t MRI/CT for, 1823 pericardial effusion, 1970t Cardiac calcified amorphous tumor image analysis, 2032–2034 post trauma, 1972
needs for evaluation of, 1607 postoperative adult, 1824 puncture injuries, 1970 (CAT), 1512–1514 extracardiac findings in, 2033–2034, Cardiac fibroma, 2054f
overview, 1604 stress testing, 1823–1824 thoracic aortic pathology, 1970t Cardiac catheterization 2037f Cardiac hemangiomas, 1482–1484
partial, 1604–1606, 1610 “aortopathy” or aortic enlargement thoracic trauma, 1970t in atrial septal defects, 1590 proper phase selection, 2033 Cardiac hypertrophy, cardiac MRI in,
pulmonary stenosis and, 1610 in, 902 vehicle collisions, 1969 of bicuspid aortic valve in adults, image postprocessing, 2028–2032 2006–2008
right ventricular outflow tract echocardiography of, 902 B-mode echocardiography, 57, 58f 1822–1823 curved multiplanar reformation, 2029 Cardiac imaging, 3–4. See also
obstruction in, 1608 in female, 1913f–1914f BNP. See Brain natriuretic peptide (BNP) in CCTGA, 1841 maximum-intensity projection, 2029, Echocardiography
types of, 1604–1610, 1747–1751 M-mode echo for, 7, 7f Bolus infusions, 421 in coarctation of aorta, 1829 2032f Cardiac implantable electronic devices
Atrium, identification of, 1576–1577 progressive degenerative changes in, Bom, Nicolaas, 8 double outlet right ventricle, 1845 (CIED), 1967
multiplanar reconstruction, 2029,
aorta-IVC and abdomen, 1577 902 Bovine arch, 665, 666f in D-transposition of great arteries, cardiac arrest, 1967
2301f
atrial appendage, 1576 Bicuspid aortopathy, 959 Bovis Streptococci, 1043 1838 cardiac resynchronization therapy,
transaxial images, 2028–2029
atrial septum, 1576 Bicycle stress echocardiography, 269 Brachial/radial artery occlusion and echo gradients during, 1574, 1575f 1967
virtual endoscopy, 2030
coronary sinus, 1577 Bilateral superior vena cava (BLSVC), release, 453 indications for, tetralogy of Fallot and, echocardiography, 1967
volume rendering, 2030
venous drainage, 1576 1543f, 1683 Bradykinin, 455 1641 infective endocarditis, 1967
major adverse cardiovascular events
Attenuation artifacts, 56, 61, 431–433, Bioprosthetic aortic valve Brain natriuretic peptide (BNP), 295, 1422 truncus arteriosus, 1844 heart disease, 1967
in, 2042
432f, 433f with anterior paravalvular aortic British Society of Echocardiography, 750 univentricular hearts, 1847–1848 coronary artery disease, 1967
in myocardial perfusion, 2042–2043
Auenbrugger, Leopold, 4 regurgitation, 1088f Brockenbrough needle, 533 Cardiac computed tomography, 2023– dilated cardiomyopathy, 1967
overview, 2023–2024
Augmentation index (AIx), 465, 467, 467f flow velocity across, 1091f Budd-Chiari syndrome, 319 2070 hypertrophic cardiomyopathy, 1967
patient preparation for, 2028t
Aureus Staphylococci, 1043 with posterior mass, 1089f advantages of, 2024 lead extraction, 1967
patient selection for, 2027
Autocorrelation detector, 71 severe stenosis and, 1090f anomalous pulmonary venous preprocedural selection, 1967
AVCD. See Atrioventricular canal defect Bioprosthetic mitral valve
C connection, 2059
pericardial calcification, 2065, 2066f
symptomatic bradycardia, pacing,
pericardial defects, 2064–2065
(AVCD) transesophageal echocardiogram of, Calcific aortic stenosis. See also Aortic aorta congenital heart disease, 1967
AV fistulas, 699–700 1088f coarctation of, 2062, 2063f pericardial duplication cyst, 2064 ventricular arrhythmias, 1967
stenosis
Axial resolution, 59, 59f, 733, 733f vegetations on leaflets, 1089f atrial septal defect, 2059, 2060f pericardial effusion, 2065, 2065f Cardiac lipomas, 1481–1482, 1483f
2D echocardiography in, 900
Bioprosthetic tricuspid valve effective orifice area in, 900 cardiac function, 2042, 2043f pericardial tumors, 2065–2066 Cardiac magnetic resonance imaging, in
with severe stenosis, 1091f CCTA, contraindications for, 2027 pitfalls and artifacts, 2034–2040 ICM/NICM, 1427–1428
B struts and, 1085f
geometric orifice area in, 900
Calcium channel blockers (CCB), 1073t challenges for, 2024–2025 beam-hardening artifact, 2038, 2040f Cardiac masses, role of 3D TEE in
Balloon mitral valvotomy, in mitral transesophageal echocardiogram of, Calcium-related factors, in cardiac cardiac gating, 2024–2025 blooming artifacts, 2036–2038, 2039f operating room in, 617–627,
stenosis, 839t, 842f 1089f motion, 1202 spatial resolution, 2024, 2024t cardiac motion artifact, 2034–2036 625f–634f
complications of, 840–841 Bioprosthetic valves, 1081–1082 Cannon wave, 308, 308f temporal resolution, 2024, 2024t misregistration artifact, 2038f Cardiac motion, 1176–1209
echocardiography in patients for, aortic, 1088f Cannula flow velocities, 1248 clinical indications for, 2044–2066 respiration motion artifact, 2036, calcium-related factors in, 1202
838–840 mitral, 1088f Capacitance micro-machined ultrasound acute chest pain, 2045–2047 2039f clinical implications, 1201
evaluation of patient, 838–840, 839t stented porcine, 1081t transducer (cMUT), 1992 anomalous coronary artery, 2047– plaque characterization, 2041–2042, definitions, 1183–1184
mitral regurgitation in, 840–841 unstented porcine, 1082t Capacitive micromachined ultrasonic 2049, 2047f 2041f diastolic dysfunction, 1201–1202
post, 841f, 843f, 844f Biplane method of discs, 1118–1119 transducer (CMUT), 115f, 116, 116f asymptomatic intermediate risk preablation pulmonary valve dilated cardiomyopathy and,
Balloon valvuloplasty, monitoring of, by Biplane transesophageal echo (TEE) Capillary blood volume (CBV), 421–422 patient, 2049–2050 mapping, 2028t 1202–1203
CONTISCAN transducer, 233, 233f, probe, 100f Capillary wedge pressure (CWP), 1063 cardiac masses, 2051–2054 radiation dose, 2025–2027 heart function, basic, 1177–1180,
234f Biventricular apical hypertrophy, 1554f Carcinoid heart disease, 1006, 1407–1413, congenital heart disease, 2059–2063 of common examinations, 2026t 1177f–1180f
Barlow’s syndrome, 1860 Biventricular pacing, 234, 1378f–1379f 1500 coronary artery bypass graft, 2050, descriptors, 2026t MRI phase contrast velocity mapping
2052f methods to reduce, 2026–2027, 2027t of, 1179f
I-VIII Comprehensive Textbook of Echocardiography Index I-IX
myocardial fiber organization, 1180f tricuspid regurgitation, 2013, 2014f left ventricular thrombus, 1504–1506 in neuromuscular disorders, 396 Carotid intima-media thickness (CIMT), Celiac artery, transesophageal imaging
time frames of systole and diastole, velocity mapping, flow and shunt malignant primary cardiac tumors, peripartum, 1381–1384, 1381f–1384f 471, 676–677, 678f of, 970
1177f–1178f assessment, 2008–2009 1484–1511 primary, 1370 Carotid ultrasound examination. See Central processing unit (CPU), 60
during isovolumic contraction, 1185f viability assessment, 2003–2004 angiosarcoma, 1485–1486 sarcoidosis, 1405, 1405f Peripheral vascular ultrasound Certified Cardiographic Technician
myocyte factors in, 1202 Cardiac myxoma, 1464–1475 cardiac plasmacytoma, 1490–1491, secondary, 1370 Carpentier, Alain, 580 (CCT), 754
of normal heart, 1185–1194 Cardiac output (CO), 230, 231f, 1280, 1281 1492f tachycardia-induced, 1388, 1395f Catheter-based LAA closure, 512 Cervical aortic arch, 1691
overview, 1176–1177 Cardiac Performance Analysis (CPA), 380 fibrosarcomas, 1488 Takotsubo cardiomyopathy, 1388, Catheter-based transcutaneous Cervical arteries Doppler spectral
pacing factors in, 1203–1204 Cardiac plasmacytoma, 1490–1491, 1492f hydatid cyst, 1491, 1493f–1498f 1394f interventional procedures, waveforms, 674f
right heart failure and, 1204 Cardiac resynchronization therapy (CRT), pericardial mesotheliomas, 1491 toxic, 1396–1397 531–532 CFR. See Coronary flow reserve (CFR)
right ventricle, 1198 233–234, 365, 1372 primary cardiac lymphoma, 1490 adriamycin-induced for device closure of cardiac shunts, CFVR. See Coronary flow velocity reserve
rotation, 1183 for heart failure, 369, 370f rhabdomyosarcoma, 1486–1488 cardiomyopathy, 1397f 548 (CFVR)
state-of-the-art, 1180–1181 LBBB and, 1377 sarcomas, 1484–1488 alcohol-induced cardiomyopathy, atrial septal defects (ASDs), 550–555 Chagas disease, 1875–1876, 1877f–1878f
composite of, 1181–1183 Cardiac rhabdomyoma, 1480–1481 mesothelial/macrophage incidental 1397 closure of PFOs, 555, 557 Chemotherapy cardiotoxicity, 2D STE
HVMB model, 1181 Cardiac sarcoidosis, 3D speckle tracking cardiac excrescences, 1511– chemotherapy-induced patent ductus arteriosus (PDA), and, 371–372
muscle contraction, asynchronous, and, 376t 1518 cardiomyopathy, 1396–1397 548–550 Chemotherapy-induced cardiomyopathy,
1181 Cardiac shunts, 171 cardiac calcified amorphous tumor, two-dimensional echo, 1407 ventricular septal defects (VSDs), 1396–1397
subendocardial muscle, 1198–1200, Cardiac situs, 1531f 1512–1514 Cardiopulmonary bypass, and HV 557–559 Chest trauma, 1969
1199f, 1200f Cardiac tamponade, 1438–1440, 1970 extracardiac masses, 1514, 1514f Doppler, 317, 318f fluoroscopy versus echocardiography cardiac injuries, 1969
torsion, 1183–1184 blunt force, 1970 intracardiac hardware, 1514–1517 Cardiopulmonary ultrasound, 1987 in, 532 penetrating injuries, 1969
twisting, 1183 cardiac involvement, 1970 mitral annular calcification, 1512, acute lung injury/ARDS, 1987 guidance of electrophysiology perioperative indications, 1970t
untwisting, 1183 clinical presentation, 1970 1512f B-lines, 1987 ablation procedures, 566, unintentional injury, 1969
mitral valve opening and, 1200–1201 and HV Doppler, 317, 317f moderator band, 1503 evaluation, 1987 568–569 Chiari network, 1503, 1776–1779
torsion and, 1184–1185, 1200 left ventricular outflow tract cardiogenic pulmonary congestion, Chordae rupture, 1015
perivalvular abscess, 1511 miscellaneous procedures, 569
ventricular septum, 1194–1198 diameter, 1974 1987
primary benign cardiac tumors, alcohol septal ablation, 571 2D transthoracic echocardiography,
ventricular structure, function of, myocardial contusion, 1970 echocardiography, 1987
1464–1484 left ventricular pseudoaneurysm 1012f, 1013f
1177f–1178f pericardial effusion, 1970 echography, 1987
benign cardiac fibromas, 1480 closure, 569, 571, 571f Chordal rupture, 857f
Cardiac MRI (CMR), 381 pulsed wave Doppler, 1443f lung ultrasound scan, 1987
cardiac hemangiomas, 1482–1484 right ventricular endomyocardial Chordoma, in right ventricle, 1502f
cardiac amyloidosis, 2008f and PV Doppler, 341 addition of, 1987
cardiac lipomas, 1481–1482, 1483f biopsy, 571 Chorionic villus biopsy (CVS), 1528
for cardiac hypertrophy, 2006–2008, right heart chamber collapses in, findings, 1987
cardiac myxoma, 1464–1475 occlusion of left atrial appendage Christian Doppler’s principle, 26, 26f
2007f 1442t helps in, 1987
cardiac rhabdomyoma, 1480–1481 (LAA), 559–561 Chronic aortic stenosis, myocardial
cardiomyopathies, 2008 right ventricular wall, 1971t pathological conditions, 1987
papillary fibroelastomas, 1475–1480 epicardial suturing of LAA, 563, 566 response to, 920
for left and right ventricle, 1998–1999, transmitral inflow velocities, 1970 pulmonary fibrosis, 1987
secondary cardiac tumors, 1492–1500 intracardiac device closure of LAA, Chronic effusive pericarditis, 1437
1999f transtricuspid velocities, 1970 three B-line scenarios, 1987, 1987t
carcinoid heart disease, 1500 561, 563 Chronic irreversible verus dynamics,
for left ventricular structure, 2000– tricuspid valve, 1971t valvular heart disease, 1987
malignant melanoma, 1500 transseptal puncture, 532–533 1356–1358
2004 Cardiac transplantation Cardiothoracic ratio, 1531f
thebesian valve, 1503 for valvular disease Chronic ischemic heart disease
LVNC, 2000–2002, 2001f, 2002f 2D STE and, 371 Cardiovascular Credentialing
Cardiomyopathies. See also specific aortic stenosis, 540–546 echocardiography role in, 1298–
tissue characterization, 2002–2003, velocity vector imaging in, 399–400, International (CCI), 754
2003t Cardiomyopathy Carditis, diagnosis of, 765–775 mitral regurgitation, 538–540, 541f, 1301
399f
limitations of, 2017–2019 Cardiac tumors and masses, 1462–1523 diabetic, 1407, 1408 autopsy, 771f 542f ventricular function in, 1300–1301
for mitral valve, 2012 atrial thrombus, 1504 dilated. See Dilated cardiomyopathy echo and Doppler studies, 768–770 mitral stenosis, 533–537, 537f–539f diastolic function assessment,
for myocardial infarction, 2003–2004, cardiac thrombi, 1504 (DCM) echocardiography paravalvular prosthetic leaks, closure 1300–1301
2004f cardiac vegetation, 1506–1511 2D STE and, 369–371 superiority of, 773–774 of, 546–548, 550f, 551f systolic function assessment, 1300
for myocarditis and sarcoidosis, Chiari network, 1503 hemochromatosis, 1405 two-dimensional, 770–772 CCAM. See Congenital cystic Chronic kidney disease (CKD). See Renal
2004–2006, 2005f, 2006f computed tomography for, 2051–2054 infectious, 1405–1407 vs. clinical examination, 767–768 adenomatoid malformation (CCAM) disease
for normal variants and masses, Coumadin ridge, 1503–1504 HIV-associated cardiomyopathy, echo interrogation, 767, 769t–770t CCB. See Calcium channel blockers Chronic obstructive pulmonary disease
2016–2017 crista terminalis, 1503 1407 M-mode in, 770 (CCB) (COPD)
overview, 1998 differential diagnosis of, 1462–1463 septic cardiomyopathy, 1405–1407 myocarditis, 766 CCTA. See Coronary computed and HV Doppler, 306, 307f
pericardial disease, 2014–2016, 2015f echocardiographic assessment of, infiltrative, 1405 pericarditis, 766 tomographic angiogram (CCTA) squatting stress echocardiography in,
of prosthetic valves, 2013–2014 1462–1464 left ventricular noncompaction, pitfalls in Jones criteria, 766–767 CCTGA. See Congenitally corrected 1323
strain assessment, 1999–2000, 2000f 3D TTE, 1464 1384–1388 regurgitation, 766 transposition of great arteries Chronic thromboembolic pulmonary
for valvular heart disease, 2009–2013 full-volume acquisitions, 1463 echocardiographic features of, rheumatic vs. nonrheumatic (CCTGA) hypertension (CTEPH), 1073t, 1074
aortic regurgitation, 2010–2011, 2011f limitation of, 1463 1385–1388, 1386f–1387f regurgitation, 768 CDG. See Congenital disorders of Circle of Willis, 669, 670f
aortic stenosis, 2010, 2011f papillary fibroelastomas, 1463, isolated, 1387f secondary prophylaxis, fidelity of, 775 glycosylation (CDG) Circulation, 13
mitral regurgitation, 2012–2013 1476f–1477f normal fetal ontogenesis, 1385 Carotid artery, 673f CDH. See Congenital diaphragmatic Circumferential strain, 362, 363f, 389
mitral stenosis, 2013 Eustachian valve, 1503 metabolic, 1407 Carotid bulb, 667, 668f hernia (CDH) Cleft mitral leaflet, 1613
I-X Comprehensive Textbook of Echocardiography Index I-XI
Clinical cardiac ultrasound, development postoperative adult, 1829 congenitally unguarded dextrocardia, 1570–1575, 1570f patient preparation, 1562–1563, 1562f echocardiographic findings in, 1445t
of, 4–7 stress echocardiography, 1828 tricuspid orifice, 1617–1618, 1617f Bernoulli equation, limitations of, pulmonary hypertension, 1901–1902 effusive, 1448f
Clipping, 89 surgical intervention for, 1829 Ebstein’s anomaly of, 1616–1617, 1574 RT 3DE approach to, 1170 hepatic vein and, 1447f
Clutter, 81 transesophageal echocardiography, 1616f color flow Doppler, 1570–1572 sequential chamber analysis, and HV Doppler, 315–317, 316f, 317f
CoA. See Coarctation of aorta (CoA) 1827–1828 tricuspid valve prolapse, 1617 color flow information, 1572 principle of, 1575–1582 M-mode and 2D echo, 1444, 1445f,
Coaptation depth, 1423, 1424 treadmill stress testing, 1828 Congenital aortic stenosis, 1766–1770 continuous wave Doppler, 1572 atrial arrangement, identification of, 1446f
Coarctation of aorta (CoA), 963, 964f, double outlet right ventricle, 1824, Congenital bicuspid aortic valve, Doppler gain and filter settings, 1575–1577 and PV Doppler, 340–341, 340f, 341f
1692–1694, 1770–1773 1825 1619–1620, 1619f inappropriate, 1571f, 1572f, atrioventricular connection, 1577– Continuity equation, 923
in adults, 1826–1829 D-transposition of great arteries, Congenital complete heart block (CCHB) 1573 1579 for aortic valve area, 906–907
cardiac catheterization, 1829 1835–1840, 1837t fetal, 1529, 1545 Doppler scales, inappropriate, 1573, ventricular arterial connection, for MVOA, 789
echocardiography, 1827–1828 cardiac catheterization, 1838 Congenital cystic adenomatoid 1573f 1579–1582 Continuous infusions, 421
echocardiography, 1836–1838 malformation (CCAM), 1530 echo gradients during cardiac shunt lesions/septal defects, 1733– Continuous wave Doppler, 63–64. See
MRI/CT for, 1828–1829
MRI/CT for, 1838 Congenital diaphragmatic hernia (CDH), catheterization, 1574, 1575f 1747 also Spectral Doppler
postoperative adult, 1829
postarterial switch repair, 1838 1530 nonimaging continuous wave atrial septal defects, 1734 disadvantage of, 68–69
surgical intervention for, 1829
postatrial switch repair, 1836 Congenital disorders of glycosylation Doppler probe, pitfalls of, 1573 atrioventricular septal defectsts methodology, 68–69, 69f
treadmill stress testing, 1828
reoperation for, 1838–1840 (CDG), 1407 poor echo windows, 1572 1746–1747 Continuous-wave jet intensity, MR and,
Doppler feature of, 1694
stress echocardiography, 1838 Congenital double orifice mitral valve, in pulsed Doppler, 1572 secundum ASD, 1734–1742 869
Coherent contrast imaging (CCI), 420
tetralogy of Fallot, 1829–1835, 1833t mitral stenosis, 844–845 signal alignment, inappropriate, sinus venosus ASD, 1742–1743 CONTISCAN transducer, 224–237
Collateral pathways, 669–671, 670f Congenital heart defects, simple, in
Color Doppler, 64, 113, 113ff, 114f cardiac catheterization, 1834–1835 1573, 1574f unroofed coronary sinus, 1743 use of, 224–225, 225f
echocardiography, 1830–1832 adults, 1798–1813 transducer frequency, inappropriate, ventricular septal defects, 1743–1746 acute coronary syndromes,
Color Doppler imaging, three- shunt lesions, 1798–1813
dimensional echocardiography and, MRI/CT for, 1835 1573 sinus of Valsalva aneurysm, 1784 evaluation of, 226
postoperative adult, 1835 atrial septal defects, 1799–1802, 1801t trivial lesions, pressure gradients hypoplastic left heart syndrome, 1784 ambulatory echocardiography,
248, 254–255, 266f atrioventricular septal defect,
postoperative adult, surgery in, 1835 across, 1574 right ventricular outflow obstruction 227–229
Color Doppler ultrasound 1809–1811
stress echocardiography, 1832–1834 double outlet right ventricle, 1779– and, 1784 balloon valvuloplasty, monitoring of,
history of development of, 11 coronary artery fistula, 1812–1813
truncus arteriosus, 1842–1844, 1843t 1783 Congenitally corrected transposition of 233
Color flow Doppler patent ductus arteriosus, 1805–1809
cardiac catheterization, 1844 left ventricular-RA communication, great arteries (CCTGA), 1840–1842 cannulation of coronary sinus,
advantages and disadvantages of, 71 patent foramen ovale, 1798–1799
echocardiography, 1843 1779–1782 cardiac catheterization, 1841 monitoring of, 233–236
methodology, 71, 71f persistent left superior vena cava,
MRI/CT for, 1844 right coronary artery fistula, 1782– echocardiography, 1840–1841 exercise echocardiography, 226–227
transthoracic echocardiogram, 137 1810–1811
postoperative adult, 1844 1783 exercise testing with stress intraoperative monitoring, 236–237
Color maps, 71. See also Color flow sinus of Valsalva aneurysm, 1811–
surgery for, 1844 echocardiography (imaging) of, echocardiography, 1841 noninvasive hemodynamic
Doppler 1812
univentricular hearts, 1845–1848 1563–1570 magnetic resonance imaging, 1841 monitoring, 229–230
Color processor, 71 ventricular septal defects, 1802–1805
cardiac catheterization, 1847–1848 apical view, 1566–1567, 1566f postoperative adult, 1842 pericardiocentesis, monitoring of,
Combined biventricular output (CVVO), Congenital heart disease (CHD), 1733–
echocardiography, 1846–1847 conventional views of, 1563t surgery for, 1841–1842 230–233
1529 1790, 1900–1902
MRI /CTA for, 1847 high parasternal or ductal view, 1569, velocity vector imaging in, 392 Contractile reserve, assessment of, 276
Comet tail artifact, 734 aortic arch anomalies, 1770–1773
transesophageal echocardiography, 1569f Congenitally corrected transposition of Contrast CMR (gadolinium), 2020
Common atrium, 1747–1751, 1751f–1752f aortic arch to pulmonary artery
1847 fistula, 1773 long-axis view, 1569, 1569f great vessels (CTGA), 1664, 1664f Contrast defect area (CDA), 423
atrial septum and, 1800 tricupsid atresia and post-fontan parasternal long-axis view, 1567, Congenitally stenotic tricuspid aortic Contrast defect length (CDL), 423–424
Common carotid artery (CCA), 665 coarctation of aorta, 1770–1773
adult, 1845, 1846f aortopulmonary window, 1751 1567f valve, 1620 Contrast echocardiography, 416–436,
Complex congenital heart defects, Computational fluid mechanics, 67 parasternal short-axis view, 1568– Congenitally unguarded tricuspid orifice, 737, 1308. See also Myocardial
1826–1848 atrial and atrioventricular valve
Computed tomography angiogram (CTA) abnormalities, 1773–1776 1569, 1568f, 1569f 1617–1618, 1617f contrast echocardiography (MCE)
Complex congenital heart defects, in flow limiting disease, accuracy in, pitfalls in, 1571–1572, 1571t Congenital mitral stenosis, 844 contrast administration
cor triatriatum sinister, 1773–1775
adults, 1826–1848 2044 short-axis view, 1570, 1570f Congenital parachute mitral valve, 845, bolus injection, 421
Ebstein’s anomaly, 1775–1776
CCTGA, 1840–1842, 1842t preoperative, 2049, 2050f subcostal window, 1563–1566, 846f continuous infusion, 421
isolated mitral valve cleft, 1775
cardiac catheterization, 1841 retrospective gated, 2043f 1564f–1565f Congenital subaortic membrane, 1359 contrast agents, 416–417, 417f
atrial septal defects, 1900–1901
echocardiography, 1840–1841 Congenital anomalies suprasternal views, 1569, 1569f, 1570f Conotruncal anomalies, 1754–1766 commercially available, 417, 418t
Chiari network, 1776–1779
exercise testing with stress of coronary arteries, 1343–1344 common atrium, 1747–1751, and HV Doppler, 317–318, 318f anomalous coronary artery, 1763– idle, properties of, 417
echocardiography, 1841 of mitral valve, 1610–1615 1751f–1752f other abnormalities, 1776–1779 1766 safety of, 434–435, 435t
magnetic resonance imaging, 1841 individual mitral lesions, evaluation computed tomography for, 2059–2063 outflow tract obstruction, 1766–1770 tetralogy of fallot, 1763 ultrasound and, interaction between,
postoperative adult, 1842 of, 1611–1615 conotruncal anomalies, 1754–1766 congenital aortic stenosis/bicuspid transposition of great arteries, 418–419, 419f
surgery for, 1841–1842 mitral valve lesions, anomalous coronary artery, 1763– aortic valve, 1766–1770 1754–1763 use of, 428–431
coarctation of aorta, 1826–1829, 1828t echocardiographic views, 1766 subaortic stenosis, 1770 Constrictive pericarditis (CP), 1444–1448 contrast imaging, physics of, 417–418,
cardiac catheterization, 1829 1610–1611 tetralogy of fallot, 1763 overview, 1733 Doppler flow velocity records, 418f
echocardiography, 1827 types of, 1611t transposition of great arteries, patent ductus arteriosus, 1751–1754, 1446–1448, 1447f history of development of, 11–13
MRI/CT for, 1828–1829 of tricuspid valve, 1616–1618 1754–1763 1755f–1757f 3D TTE vs. 2D TTE, 1456–1457 imaging modalities, 420, 420f
I-XII Comprehensive Textbook of Echocardiography Index I-XIII
intermittent/triggered imaging, Coronary artery anomalies, 1684–1688 Coronary sinus atrial septal defect, 1586 Decompressive venous channels, 1684 systolic function in, 1371 Dose length product (DLP), 2026
420–421 anomalous pulmonary origin Coronary sinus cannulation, monitoring Definity, 417, 418t. See also Contrast ventricular remodeling, 1379 Double-balloon catheter
real time imaging, 420 of coronary artery, 1685–1687 of, by CONTISCAN transducer, echocardiography wall motion abnormalities in, commissurotomy (CBC), 233
indications for, 426 of right coronary artery, 1687 233–236, 235f, 236f, 236t Degenerative disease, 882f 1371–1372 Double image artifacts, 736. See also
enhanced endocardial border tangential origin of coronary artery, Coronary stenosis, 1338, 1340f Degenerative mitral regurgitation, 863 Dilated pulmonary valve, 1555f Artifacts
delineation (EBD), 426, 426f 1687–1688 cut-off values for, 1341t Degenerative mitral valve stenosis, 844 Dipyridamole stress test, 1334 Double orifice mitral valve, 1614–1615,
left ventricular opacification (LVO), Coronary artery disease (CAD) in elderly, 1943–1946, 1944f, 1946f DENSE. See Displacement encoding with Discrete subaortic membrane, 1614f
426–427, 427f–429f ALVRM on squatting and, 1325 in elderly female, 1897f–1899f stimulated echoes (DENSE) 1771f–1772f Double outlet right ventricle, 1779–1783,
myocardial perfusion, evaluation of, 2D STE and, 367 Cor triatriatum sinister, 1773–1775 Depth, and frame rate, 59, 60f, 61 Displacement encoding with stimulated 1844–1845
427–428, 429f evaluation of, 226 Cosine correction, 67f Dextro Transposition of great arteries echoes (DENSE), 1144 cardiac catheterization, 1845
myocardial perfusion, principles of squatting SE vs. dobutamine SE for, Coumadin ridge, 1503–1504 (dTGA), 1528, 1549f, 1759f–1763f Distal coronary flow, 1340–1343 echocardiography, 1844
assessment of, 421–422, 422f 1325 Crab view, 184, 185f Dextrotransposition of great vessels, 6 D-malposition left ventricular-RA communication,
contrast modality during MCE, squatting stress echocardiography in, Crista terminalis, 188, 1503 DHF. See Diastolic heart failure (DHF) of aorta, 1548f 1779–1782
422–423 1323 Critical neonatal aortic stenosis, 1622– Diabetes, velocity vector imaging in, 398 of great arteries, 1548f MRI/CT for, 1845
qualitative MCE, 423, 424f, 425f three-dimensional vs. two- 1623 Diabetic cardiomyopathy, 1407, 1408 Dobutamine stress echocardiography postoperative adult, 1845
quantitative MCE, 424, 426 Cropping, 241 Diastolic dysfunction (DSE), 274, 1307 right coronary artery fistula, 1782–
dimensional in, 1332t
semiquantitative MCE, 423–424, 425f Cropping, 3D echocardiography and, cardiac motion and, 1201–1202 Dobutamine stress test, 1331–1333 1783
velocity vector imaging in, 396–398
and problems, 431 269–270, 269f grade I, 1128 “Dominance,” 1181 stress echocardiography, 1845
Coronary artery distribution, 1116f
attenuation artifacts, 431–433, 432f, CRT. See Cardiac resynchronization grade II, 1128 Doppler, 1993 surgery for, 1845
Coronary artery fistula, 2048–2049
433f HCM and, 1356 across-line Doppler shifts, 1993 Double outlet right ventricle (DORV),
in adults, 1812–1813 therapy (CRT)
blooming, 433, 434f 1580, 1644–1650
sequelae of, 2049 CT-based fractional flow reserve (FFR- identification of, 1126–1130 beam-to-vessel angle, 1993
swirling, 433, 434f definition, 1645
Coronary calcium scoringm, in computed CT), 2044 left ventricular hypertrophy patient color Doppler, 1993
thoracic cage artifacts, 433–434, 434f echocardiography of, 1647
tomography, 2044–2045, 2045f, CT dose index volume (CTDIvol), 2026 with, 1125f postacquisition, 1993
saline, 435, 436f planning surgical strategy, role in,
2046f CTEPH. See Chronic thromboembolic Diastolic heart failure (DHF), 1124–1125 Doppler, Christian, 9
training in, 757 1648–1650
Coronary computed tomography pulmonary hypertension (CTEPH) Diastolic pulmonary artery pressure Doppler angiography. See Power Doppler
Contrast microbubbles, 416–417 great arteries and, 1645–1646
angiogram (CCTA), 2035f. See also Curie, Pierre, 4 (DPAP), 1065t, 1066, 1270–1272, Doppler equation, 65, 66
Contrast score index, 441 preoperative assessment of, checklist
Cardiac computed tomography Curvilinear probe, 58, 58f 1272f Doppler hemodynamics, with stress
COPD. See Chronic obstructive for, 1648t
contraindications for, 2027 CVS. See Chorionic villus biopsy (CVS) DICOM format, 90, 95 echocardiography, 1318–1319
pulmonary disease (COPD) stenosis of pulmonary outflow,
coronary plaque, 2041–2042 CVVO. See Combined biventricular Diet drug-induced valvulopathy, 1006 aortic valve disease, 1318
CoreValve, self-expandable, 541–542, 543 1647–1648
diagnostic accuracy of, 2040–2041 output (CVVO) Digital Imaging and Communication dynamic pulmonary hypertension,
Coronary aneurysms, 1688–1689 aortic outflow obstruction, 1647
flow limiting disease, accuracy in, CWP. See Capillary wedge pressure (DICOM), 391 1318–1319, 1319f remote ventricular septal defect, 1648
atresia of left main coronary artery,
2044 (CWP) Dilated cardiomyopathy (DCM), 394–395, hypertrophic cardiomyopathy, 1318 ventricular septal defect, restriction
1689
in ICM/NICM, 1427 Cystic medial degeneration (CMD), 1936 445–446, 447f, 1202–1203, 1370– latent diastolic dysfunction, 1318 of, 1647f, 1648
Kawasaki disease, 1688–1689
optimization of, 2029t 1372, 1371f mitral valve disease, 1318 subaortic ventricular septal defect in,
Coronary anomaly, 1344f
cardiac MRI for, 2008f Doppler principle, 349
Coronary arteries, 1337–1347 optimization of anomalous, 2049t D chamber enlargement in, 1370 Doppler shift, 65
1647
anatomy and physiology, 1337 prognostic information from, 2042 subpulmonary ventricular septal
protocol on 64 detector scanner, Dark blood imaging (spin echo), 2020 decompensated, 1374 Doppler spectral display, 67–68 defect in, 1647
congenital abnormalities of, 1343–
2028t Data acquisition methods, 3D etiology of, 1370, 1379–1397 Doppler tissue imaging. See Tissue Taussig–Bing anomaly, 1647
1344
Coronary fistulas, 1343–1344 echocardiography familial, 1379–1381 Doppler ventricular septal defect, location of,
coronary flow reserve, 1340–1343
pediatric type, 1345f cropping, 269–270, 269f ischemic cardiomyopathy, 1388– Doppler ultrasound, 9–11, 63–64, 65–73 1645–1646
coronary ostia, 1338
Coronary flow reserve (CFR), 1340–1343 3DE color flow Doppler imaging, 269 1395, 1395f color Doppler, 64, 71 doubly committed, 1645
coronary stenosis, 1338
conditions decreasing, 1342t image display, 269–270 left ventricular contractility, 1370– continuous wave Doppler, 63–64, remote, 1645–1646, 1646f
distal coronary flow, 1340–1343
impairment of, 1343 multiplane mode, 268–269 1372 68–69 subaortic, 1645, 1645f
normal left main, 1339f
Coronary flow velocity reserve (CFVR), multiple-beat 3DE imaging, 269 mitral inflow filling pattern, 1376 Doppler velocity determination, 72–73 subpulmonic, 1645, 1646f
overview, 1337
446, 1337, 1341–1343, 1344 real time 3DE, 269 mitral regurgitation, 1376 history of development of, 9–11 Doubly committed ventricular septal
parasternal short-axis view for, 1569,
conditions impairing, 1342t tomographic slices, 270, 270f optimizing heart failure, information from, 73 defect, TGA and, 1659
1569f
noninvasive evaluation of, 1341–1342 3D beamformer, 77–78 echocardiography role in, instrumentation, 66–68 DPAP. See Diastolic pulmonary artery
proximal, 1338f, 1339f, 1341f, 1341t
visualization of, 1337–1340 normal, 1343f DDD pacing, for obstructive HCM, 1376–1379 physical principles, 63 pressure (DPAP)
transesophageal echocardiography, Coronary ostia, 1338 1363 secondary findings in, 1372–1376 power Doppler, 71–72 3DSTE. See Three-dimensional
1338–1340 Coronary sinus Debakey classification, 1974 atrial fibrillation, 1376 pulsed wave Doppler, 63–64, 69–70 speckle tracking
transthoracic echocardiography, defect in wall of, 1679–1680 aortic arch, 1974 cardiac dimensions, 1372–1374, 1373f tissue Doppler, 64, 72 echocardiography (3DSTE)
1337–1338 partial or completely unroofed, 1683 ascending aorta, 1974 Doppler echocardiography, 1375–1376 Doppler velocity determination, 72–73 dTGA. See dextro Transposition of great
Coronary arteriovenous fistulas, 1688, Coronary sinus aneurysm/diverticulum, proximally, 1974 pericardial and pleural effusion, 1374 errors in, 72 arteries (dTGA)
two-dimensional and M-mode DORV. See Double outlet right ventricle D-transposition of great arteries,
1689f 1683 Deceleration time (DT), 313
findings, 1375, 1375f (DORV) 1835–1840
I-XIV Comprehensive Textbook of Echocardiography Index I-XV
cardiac catheterization, 1838 EBCT. See Electron-beam computed pediatric, 6 pathophysiology of, 1923–1924 Elastography, 1994, 1995 measurement of flow-mediated
echocardiography, 1836–1838 tomography (EBCT) persistent left superior vena cava, prevalence of, 1923–1924 acoustic radiation force impulses, dilatation, 457, 457f
MRI/CT for, 1838 Ebstein’s anomaly, 986f, 1775–1776 1958 systolic murmur, 1923 1995 proximal and distal occlusions,
postarterial switch repair, 1838 associated with transposition of great progress in, 5f coronary stenosis, 1943–1946, 1944f, attraction of, 1995 456–457
postatrial switch repair, 1836 vessels, 1777f right atrium–right ventricle, 1958 1946f breast malignancies, 1995 quantification of shear stress, 457
stress echocardiography, 1838 2D transesophageal risk stratification, 1969 left ventricular mass, 1942–1943 human tissue, 1994 technique of FMD in brachial artery,
velocity vector imaging in, 392 echocardiography, 1025f–1028f superior vena cava, 1958f mitral annular calcification, 1946– mechanical properties, 1994 458t
Dual plane transesophageal echo (TEE) of mitral valve, 1615 supraventricular tachycardia, 1957 1948 shear wave, 1995, 1995f factors affecting, 451, 451t
probe, 99–100, 100f M-mode echocardiography, 986f supraventricular tachycardia, 1957 overview, 1921 advantage, 1995 future directions, 471, 474
Duchenne muscular dystrophy (DMD), RT 3DTTE for, 1777f–1779f Thebesian valve, 1958 penetrating aortic ulcer, 1921–1923 strain elastography, 1995 NO release and, 455, 455f
396 transthoracic echocardiography therapeutic, 1957 prosthetic valves, 1948–1949, 1949f tissues elastic properties other methods for endothelial
Ductal arch (DuAr), 1537f three-dimensional, 1029f thoracic aorta, 1958f Echocardiography, in women, 1886–1920 two ways to image, 1995 function assessment, 465
Ductus arteriosus, 548 two-dimensional, 1024f–1025f transesophageal echocardiogram, breast implant, 1887f tissue stiffness, 1995 laser Doppler flowmetry, 469–470
Ductus venosus (DV), 1532f, 1538f tricuspid valve, 1616–1617, 1616f 1957 congenital heart disease, 1900–1902 Electric safety, TEE probe equipment low flow-mediated vasoconstriction,
Duplex scanning protocol, carotid Echocardiography, 1957, 1969 transthoracic, 1969 atrial septal defects, 1900–1901, and, 110–111, 111f, 111t 470–471, 470f–474f
ultrasound, 672f ablation treatment, 1957 transthoracic echocardiogram, 1957 1900f–1901f Electromagnetic compatibility (EMC), peripheral arterial tonometry,
DuraHeart magnetically levitated accessory pathways, 1957 tricuspid valve, 1958 pulmonary hypertension, 1901–1092 110 467–469, 468f, 469f
centrifugal assist system, 1228 angiography, 1958 valve of Vieussens, 1958 echocardiographic measurements pulse wave velocity analysis, 466–467,
Electromagnetic interference (EMI), 112
466f, 467f
Dynamic pulmonary hypertension, stress coronary sinus contrast, 1958 Echocardiography, in elderly, 1921–1956 vs. technical considerations, 1886– Electron-beam computed tomography
shear stress and flow-mediated
echocardiography in, 1318–1319, arrhythmias, 1957 aortic aneurysm, 1934–1937 1888 (EBCT), 2023
dilatation, 452–454, 452f
1319f atrial fibrillation, 1957 cystic medial degeneration and, 1936 ischemic heart disease, 1889–1893 in ICM/NICM, 1427
shear stress and FMD response,
Dysplasia of mitral valve, 1613 atrioventricular, 1957 etiology of, 1936 overview, 1886 Emergency department setting, MDCT
analysis of, 457–458, 457f
Dysplastic pulmonary valve, 1554f body surface area, 1958 natural history of, 1937 polycystic ovarian syndrome, 1899 in, 2045–2047
vasoactive molecules in
cannulation, 1958 with rupture, 1936f stress echocardiography, 1894–1899 EMF. See Endomyocardial fibrosis (EMF)
vasoregulation, 454
cardiac ablation, 1958 aortic atherosclerosis, 1921–1923, structural heart disease, 1888–1889 End-diastolic volume (EDV), 1156–1157
E coronary sinus, 1957 1922f mitral valve calcification, 1889, 1895f Endocardial border delineation (EBD),
endothelins, 454
endothelium-dependent
Early cardiac flow Doppler era, 24–49 Doppler in, 65–66. See also Doppler aortic dissection, 1937–1942, mitral valve prolapse, 1888, 421, 426 hyperpolarizing factor, 454
color Doppler, development of, ultrasound 1937f–1938f, 1941f 1888f–1889f Endocardial cushion defect, 1535 nitric oxide, 454
37–38, 41f–44f echocardiogram, 1957 acute coronary syndrome and, 1940 mitral valve stenosis, 1888–1889 Endocardial trabecular contouring prostacyclin, 454
diastology, 47–48 transthoracic, 1962f color Doppler flow in, 1942 Takotsubo cardiomyopathy, 1899– algorithms, 1156–1157 Endothelial progenitor cells (EPCs), 450,
directional Doppler flowmetry, 29–30 echocardiographic modalities, 1957 descending thoracic aorta, 1942 1900 Endocarditis 450f
Doppler presentation, controversy electrophysiologist, 1957 perfusing lumen, 1942 Echocardiography training, 750–760. See pacemaker associated, 1214 Endothelin (ET), 454
on, 30–31 electrophysiology, 1957 rupture, 1938f–1940f also Training, in echocardiography of prosthetic valves, 1099–1100 Endothelium-dependent hyperpolarizing
flow concept, emergence of, 27–28 history of, 3–19 aortic stenosis, 1924–1934 Echocardiography transducer, 55 Endocarditis prophylaxis, HCM and, factor (EDHF), 454
heart cavities, exploring of, 33, 34f clinical cardiac ultrasound, 4–7 aortic valve area, estimation of, 1926 Echo-clear space, 1440f 1363–1364 Endovascular Valve Edge-to-Edge Repair
intracardiac Doppler flow velocity color Doppler ultrasound, 11 2D echocardiography, 1924–1928, Echo-guided pericardiocentesis, 1443– Endomyocardial fibrosis (EMF), 1402– Study (EVEREST II) trial, 539
traces changes, interpretation contrast echocardiography, 11–13 1925f, 1927f 1444 1404 End-stage renal disease (ESRD), 1871–
from, 33–34 2D echocardiography, 8–9 LFLG-AS with low EF, 1929f EchoPAC, 385 Endothelial cells, 449–450, 449f 1872, 1872f
mature flow Doppler era, 44, 46–47 3D echocardiography, 14–18 live/real time 3D TTE, 1930–1933 ECMO circuit. See Extracorporeal dysfunction, 451. See also End-systolic volume index (ESVI), 180,
nondirectional flow Doppler Doppler ultrasound, 9–11 mild, 1921f membrane oxygenator (ECMO) Endothelial dysfunction 1379
technique, 28–29 perspective on, 19 PLFLG-AS with preserved EF, circuit function of, 450, 450ff, 451t ICM/NICM and, 1421–1422
peripheral artery recordings, lessons tissue Doppler and speckle tracking 1929–1930 Edge-to-edge repair, 539 Endothelial dysfunction, 449–474. See Energy Doppler. See Power Doppler
from, 31, 33 imaging, 14 prevalence and pathophysiology, Edler, Inge, 4–5 also Flow-mediated dilatation Enhancement, 61
preflow Doppler era transesophageal echocardiography, 1924 Edwards Sapien Valve, 2057 (FMD) eNOS (type-III NO-synthase), 454
invasive procedures in, 25 13 severe, 1931f–1932f Effective orifice area (EOA) assessment of, methods for, 455–457, EOA. See Effective orifice area (EOA)
noninvasive procedures in, 25–26, ultrasound, 4 transcatheter aortic valve in calcific aortic stenosis, 900 456f, 456t Epiaortic ultrasonography (EAU),
26f, 27f immediate invasive intervention, replacement, 1934, 1935f patient prosthetic mismatch and, and carotid intimal medial thickness, 638–641
pulsed Doppler with 2D 1969 ventricular response to, 1933–1934 1091–1092 471, 474f in aortic pathology
echocardiography, intracardiac echocardiography, 1957 aortic valve sclerosis, 1923–1924, of prosthetic valves, 1084–1087 concept of FMD aortic atherosclerosis, 640–641, 641f
combination of, 36–37, 38f–40f midesophageal location, 1957 1923f Effective regurgitant orifice area (EROA), case studies on, 459–462 aortic dissection, 641
transcutaneous approaches, return nodal re-entrant tachycardia, 1957 bicuspid, 1931f 518–519, 992, 1038 limitations of, 458–459, 663 epiaortic probe and preparation,
to, 34–36 one-dimensional, 3f echocardiographic findings for, Effusive-constrictive pericarditis, 1448 conduit arteries, ultrasound imaging 638–639, 639f, 640f
Early gadolinium enhancement ostium, CS,CS-RA, 1958 1923–1924, 1930f Eisenmenger syndrome, 1597f of, 456 imaging views/planes, 639–640, 640f
(EGE), 2020 parasternal, 1958f normal hemodynamics in, 1924 Ejection fraction postamyocardial cuff inflation pressure, 457 indications for, 638
infarction, 1165t duration of occlusion, 457 three-dimensional, 641
I-XVI Comprehensive Textbook of Echocardiography Index I-XVII
Epicardial fat pad, 1518f fetal heart disease, 1529–1530 Flow image, 71 Gelatin-encapsulated nitrogen bubbles, Hematoma, 1517f tricuspid stenosis, 311, 312f
E-point septal separation (EPSS), 169, congenital cystic adenomatoid Flow-mediated dilatation (FMD), 13 after femoral artery access, 696, 697f heterotaxy syndrome and, 1706
1117, 1375, 1375f malformation, 1530 452–454, 452f–454f. See also Gender,effect of, on HV Doppler, 303 Hemochromatosis, 1405 HV Doppler flow patterns, schematic
EPSS. See E-point septal separation congenital diaphragmatic hernia, Endothelial dysfunction Geometric orifice area (GOA), 900 Hemodynamometer, 67 drawing of, 321f
(EPSS) 1530 clinical utility of, 465, 466t Gerbode defect, 1603, 1603f, 1604f Hepatic veins (Hep V), 1532f imaging of, 299–302
EROA. See Effective regurgitant orifice sacrococcygeal teratoma, 1530 factors affecting Geroulakos classification, of ultrasonic anatomy of, 299–300, 300f pulse Doppler across, 1539f
twin reverse arterial perfusion, 1530 ACE-inhibitors, 465 plaque, 678–679, 681f versus biliary ducts, 305, 305f spectral Doppler of, 319–321
area (EROA)
twin–twin transfusion syndrome, age and sex, 463 Ghost image, 61 blood flow in, 300, 301f versus superior vena cava, Doppler
Esophagus, 967
1529–1530 coronary risk factors, 463–464 Global circumferential peak systolic aging and, 303 pattern of, 304, 304f
ESVI. See End-systolic volume index
fundamentals of, 1530–1531 diet, antioxidants, and supplements, strain (GCS), 1728 Doppler V wave, 300, 302f transesophageal echocardiography,
(ESVI) 305
caval long-axis view, 1541 465 Global circumferential strain (GCS), exercise and, 303
Eulerian strain, 389 transthoracic echocardiography, 304,
core and cord Dopplers, 1542 exercise, 465, 465f 91–92 factors affecting, 302–303, 303f
European Association of Cardiovascular hyperglycemia, 464 305f
Imaging (EACI), 91, 750, 751, 757, ductal and aortic arch views, 1541 Global longitudinal peak systolic strain gender and, 303
infections, 465 visualization and recording of HV
fetal cardiac function, 1541–1542 (GLS), 1728 HV Doppler measurements, 302, 302f
758 smoking, 464, 464f Doppler, technical considerations in,
fetal ultrasound, safety of, 1546–1547 Global longitudinal strain (GLS), 91, 91f, normal, 300, 301f
European Association of sympathetic over activity, 465 305, 305f, 306t
four-chamber view, 1531–1541 273, 1165 physiology of, 300–302, 301f, 302f
Echocardiography (EAE), 132, 296, 907 unstable angina, 464–465, 465f Hepatic vein systolic filling fraction
rhythm assessment, 1542–1545 Global radial peak systolic strain (GRS), pregnancy and, 303
European Society of Cardiology (ESC), weight loss, 465 (HVFF), 1268
short-axis views, 1540–1541 1728 respiration and, 302–303, 303f Hertz, Hellmuth, 4–5
751 FMD response, 452
three-dimensional imaging, 1549– Global strain, 712–714, 712f–715f two components of systolic flow, 300, Heterotaxy syndrome, 1704–1709
European Union (EU), 110 Focus, and TEE image quality, 106, 106f
1553 GLS. See Global longitudinal strain (GLS) 301f anatomy, 1704
Eustachian valve, 1503 Fontan procedure, and HV Doppler, 318,
ventricular long-axis view, 1541 “Goose neck” deformity, 1746 challenge to use of HV Doppler, cardiac and extracardiac anomalies
EvaHeart left ventricular system, 1228– 318f
overview, 1527–1560 Gore-Helex atrial septal occluder, 553, 319–321, 320f in, 1705t
1129, 1228–1229 Food and Drug Administration (FDA),
physiology, 1528–1529 554f disease states, and HV flow pattern, initial echocardiogram of, 1704–1709
Exercise echocardiography, 226–227, 13, 110
prenatal counseling in, 1528 Fossa ovalis Gorlin equation, 923 305 abdominal situs and cardiac position,
227f, 228f, 1307 reasons and associations for, 1529– Great vessels, identification of, 1579–1580 atrial fibrillation, 309, 309f
anatomy of, 533, 535f 1704–1705
External carotid artery (ECA), 665–666 1530 apical view, 1579 atrioventricular dissociation, 308, aortic arch and branches, 1709
2D transesophageal examination,
Extracardiac masses, 1514, 1514f Fetal echocardiography, in women, connection of, 1579–1580 308f atria and appendages, 1707
988f
Extracorporeal membrane oxygenator 1906–1919, 1906f–1909f PLAX view, 1579 cardiac tamponade, 317, 317f atrial septum, 1707
Fossa ovalis atrial septal defect, 1586
(ECMO) circuit, 1127 Fetal heart abnormality, 1529 GSPECT. See 99mTc-sestamibi gated cardiopulmonary bypass, 317, 318f atrioventricular junction, 1707–1708
for percutaneous device closure, 1591
Extravascular lung water (EVLW), 1982 Fetal heart disease, fetal imaging, Four-chamber asymmetry, 1548f single-photon computed emission chronic obstructive lung disease, 306, branch pulmonary arteries, 1708–
1529–1530 Fourier, Joseph, 68 tomography (GSPECT) 307f 1709
pulmonary blood flow, sources of,
F congenital cystic adenomatoid Fractional area change (FAC), 1166 congenital heart disease, 317–318,
1708–1709
malformation, 1530 318f
Fabry disease, 352, 1401–1402, 1401f congenital diaphragmatic hernia,
Frame, 59 H constrictive pericarditis, 315–317, venoatrial connections, 1705–1707
Frame rate, 59
False lumen (FL), 1977f 1530 depth and, 59, 60f Haemophylus influenzae, 1043 316f, 317f ventricles and ventricular septum,
FAPS. See French Aortic Plaque in Stroke sacrococcygeal teratoma, 1530 image line density and, 59 Hand-carried ultrasound device (HCD), liver disease, 318–319, 319f 1708
(FAPS) study twin reverse arterial perfusion, 1530 sector angle and, 59, 60f 752 premature ventricular contractions, ventriculoarterial connections, 1708
Fast Fourier transform (FFT), 67, 113 twin–twin transfusion syndrome, Framingham Heart Study, 1161 Harmonic imaging, 57 309, 309f overview, 1704
Fat pad, 170 1529–1530 French Aortic Plaque in Stroke (FAPS) prolonged PR interval, 308, 308f venoatrial connections in, 1706t
apical four-chamber view, 57f
Fatty infiltration of liver, 318 Hewlett Packard SONOS 5500, 227
FFT analyzer, 67 study, 1921 HeartAssist system, 1228 pulmonary hypertension, 311, 313,
FDA 510 (K) Track 3 regulations, 111 HFnlEF. See Heart failure with preserved
Fibrinous adhesions, 1456–1457 Frequency, 55 Heart chamber segmentation algorithms, 313f
Feigenbaum, Harvey, 6 ejection fraction (HFnlEF)
Fibroma, 2051 in clinical use, 57 706–707, 707f restrictive cardiomyopathy, 315, 315f
High-flow, high-gradient aortic stenosis,
Feinstein, Steve, 13 Fibromuscular collar, 1625 and TEE image quality, 104–105, 105f Heart defects, in fetus, 1527–1528 right ventricular diastolic
920. See also Aortic stenosis
Fetal bradycardia, 1545 Fibrosarcomas, 1488 Fully sampled matrix-array transducers, Heart failure with preserved ejection dysfunction, 313–315, 315f
Highly focused ultrasound (HIFU), 1996
Fetal cardiac function, assessment of, by First pass imaging (perfusion), 2020 77, 268 fraction (HFnlEF), 1122 right ventricular end-diastolic High MI imaging, 420–421
velocity vector imaging, 390–391 Fixed anatomical obstruction, TGA and, Functional mitral regurgitation (FMR), HeartMate II, 694, 1228 pressure, assessment of, High-pass filtering, 66–67
Fetal cardiac imaging, 1527–1560 1660–1661 1372 HeartWare ventricular system, 1224f, 309–310, 310f High pulse repetition frequency (HPRF),
congenital heart disease, family Fixed subaortic obstruction, 1624–1625 DCM and, 1376, 1376f 1228 right ventricular systolic dysfunction, 64
history of, 1529 Flail tricuspid valve, 1007–1029 Fundamental imaging, 57 Heating safety, TEE probe equipment 313, 314f HIV-associated cardiomyopathy, 1407
fetal bradycardia, 1545 3d transthoracic echocardiography, apical four-chamber view, 57f and, 111 short PR interval, 307, 308f HLHS. See Hypoplastic left heart
fetal cardiac evaluation, indications 1012f Hemangioma sinus bradycardia, 306, 307f syndrome (HLHS)
for, 1528
fetal cardiology, scope of, 1527–1528
in male with dyspnea, 1012f
Flial posterior mitral valve leaflet, 853f
G involving mitral valve, 1485f

sinus tachycardia, 306, 307f
tricuspid regurgitation, 310–311, 311f,
HOCM. See Hypertrophic obstructive
left atrial, 1487f cardiomyopathy (HOCM)
fetal echocardiography, indications Flow convergence method, MR severity Gall stones, 220 right ventricular, 1486f 312f Hodgkin lymphoma, 1491
for, 1529 and, 870–871 Gating, 80
I-XVIII Comprehensive Textbook of Echocardiography Index I-XIX
Homograft aortic prosthesis, 1100f Hypertrophied heart, in HCM, 1363f Infectious cardiomyopathy, 1405–1407 abnormalities of, 1682–1683 clinical applications, 644 doppler echocardiography, 1421–
Human hearing, 55 Hypertrophy HIV-associated cardiomyopathy, bilateral, 1683 during electrophysiology intervention 1424
HVFF. See Hepatic vein systolic filling definition, 1349 1407 inferior vena cava interruption, atrial septal puncture, 644–645, coronary echocardiography, 1424
fraction (HVFF); HV systolic filling patterns in HCM, 1349f septic cardiomyopathy, 1405–1407 1682–1683 646f left atrial size, 1422
fraction (HVFF) Hypoplastic left heart syndrome (HLHS), Infective endocarditis inferior vena cava to left atrium, 1683 EP procedures and, 647–648 left ventricular diastolic dysfunction,
HV systolic filling fraction (HVFF), 1144 1540, 1549f, 1550f–1551f, 1701, aortic abscess cannula position in, 1252f pulmonary vein isolation for atrial 1422
Hydatid cyst, 1491, 1493f–1497f, 1784 in native valve, 1045f dilated, 1066f fibrillation ablation, 645, 647, left ventricular end-systolic volume
1493f–1498f velocity vector imaging in, 393–394 transesophageal approach, 1053f in dilated cardiomyopathy, 1373– 647f index, 1421–1422
pericardial, 1497f–1498f Hypoplastic mitral valve, 1613 aortic insufficiency, massive, 1046f 1374, 1374f right ventricular outflow tract left ventricular volumes, 1421
Hypereosinophilic cardiomyopathy, 1402 Hypovolemia, 1977, 1978f Candida albicans, 1044f hepatic vein, 1964 tachycardia, 647, 648f mitral regurgitation, mechanism,
Hypereosinophilic syndrome, 1868–1869, hyperkinetic, 1978 in childhood, 1856–1857 heterotaxy syndrome and, 1705–1706 ventricular tachycardia from left 1422–1424, 1423f
1870f hypotension, 1977 inspiratory collapse, 1964 ventricle, 647, 649f myocardial contrast
diagnosis of, 1059
Hypertension, three-dimensional speckle hypotensive, patient, 1978f plethora (dilation) of, 1441f VT foci from great vessels, 647, echocardiography, 1424
Duke criteria for, 1042, 1043t
tracking and, 376t hypo-volemia, 1977 pulse Doppler across, 1539f 648f–649f right ventricle dysfunction, 1422
false-positive results in, 1045t
Hyperthyroidism, 1879, 1880f for RAP, 1264–1266, 1266f equipment and catheters, 643 stress echocardiography, 1424
echocardiography in
Hypertrophic cardiomyopathy, 395 size, 1964 mechanical system, 643 tenting area, 1422, 1423f
three-dimensional speckle tracking
I findings, 1043–1047
subcostal window, 1964 phased array ultrasound system, 644, echocardiographic assessment, 1419
goals of, 1042–1043
and, 376t ICE. See Intracardiac echocardiography thrombi, 1966 644t echocardiographic differentiation of,
indications of, 1058–1059, 1059f
Hypertrophic cardiomyopathy (HCM), transgastric level, 1964 extracardiac use of, 651, 652f 1418–1434
(ICE) metallic aortic valve prosthesis,
1348–1368 ventricular assist devices and, 1233 imaging specifications, 644, 645f large mitral–septal separation, 1419
ICM. See Ischemic cardiomyopathy infected, 1057f
alcohol septal ablation, 1362–1363 Inferior vena cava interruption, 1682– limitations of, 652 M-mode echocardiography, 1419–
(ICM) prognostic stratification, role in, 1683 during structural intervention
apical, 1356 IEC 60601-1 document, on safety of 1050–1058 1421
Ingenious balloon, 535
definitions and types of, 1349–1356 medical electronic devices, 110 sequential transesophageal, Melody valves, 651 overview, 1418
Inkjet technology, 6
diastolic dysfunction, 1356 Image line density, and frame rate, 59 patent foramen ovale/atrial septal positron emission tomography, 1428
1051f–1052f Inlet VSD, 1659
differential diagnosis, 1359–1360 Image optimization and equipment, 60 defect closure, 648, 650, 650f severely dilated left ventricular cavity,
three-dimensional, 1049–1050, Instrumentation, Doppler, 66–68
athlete’s heart, 1359–1360, 1361f depth and, 61 periprosthetic valve, 650–651, 651f 1419
1050f–1058f Interagency Registry for Mechanically
LV noncompaction, 1359 focus and, 61 ventricular septal defect closure, 650, single-photon emission computed
overview, 1042–1043 Assisted Circulatory Support
dual chamber pacing for, 1363 gain and, 61 651f tomography, 1425–1427
periannular extension of, 1044–1045 (INTERMACS), 1224, 1226
endocarditis prophylaxis, 1363–1364 sector width and, 61 three-dimensional, 652, 653f Ischemic cardiomyopathy (ICM), 1388–
pericardial effusion, 1045–1047 Interatrial communication, TGA and,
hypertrophied heart in, 1363f 1657 Intracardiac hardware, 1514–1517 1395, 1395f, 1418, 1420ff–1421f
zoom and, 61 prosthetic valve, 1047
and latent obstruction, 1355f Interatrial membrane, color Doppler Intramural hematoma (IMH), 957–958, vs. NICM, 1425, 1426t
Image rendering, in 3D echocardiography abscess in patient with mechanical,
with mid–left ventricle (LV) across, 1532f 958f Ischemic cascade, 1307f
2D tomographic slices, 79–80 1047f
obstruction, 1357f INTERMACS. See Interagency Registry transesophageal imaging of, 978 Ischemic handgrip, 453
surface rendering, 79 dehiscence, 1047
mid-left ventricular, 1356–1358 for Mechanically Assisted Intrauterine growth retardation (IUGR), Ischemic heart disease, 1289–1305
volume rendering, 78–79, 79f “prosthetic pitch,” 1047
mitral apparatus and regurgitation, Circulatory Support 1528 central mitral regurgitation and,
Imaging artifact, 3D reconstruction pseudoaneurysm
1354–1356 (INTERMACS) Intravascular ultrasound (IVUS), 643, 1298f
imaging and, 17 in aortic annulus, 1046f
obstructive, 1355f, 1360f Internal carotid artery (ICA), 665–668 655–661 chronic. See Chronic ischemic heart
IMH. See Intramural hematoma (IMH) aortic valve, 1046f
overview, 1348 International Electrotechnical examination procedure, 656–657 disease detection of, 1289–1292
Impella catheter-based assist device, 1227 right-sided, 1047–1049
patterns of hypertrophy in, 1349f Commission (IEC), 110 future perspectives, 661 pharmacological stress testing, 1290
risk of SCD and, 1350 Impella device, 1250, 1251f transesophageal approach, 1048f image acquisition, 655–656 stress echocardiography for, 1289–
International Registry of Acute Aortic
stress echocardiography in, 1318 Individual mitral lesions, evaluation of, type of patient with, 1048–1049 electronically switched multielement 1291, 1290f
Dissection study, 980
sub-basal, 1349f 1611–1615 special considerations in, 1047–1050 array system, 656 vasodilator stress testing, 1290
International Society of Cardiovascular
systolic anterior motion of mitral abnormal mitral arcade, 1613–1614 tricuspid valve endocarditis, 1058f mechanically rotating transducer, echocardiography in
Ultrasound (ISCU), 753, 758
valve, 1350–1354, 1350f accessory mitral orifice, 1615 valve perforation, 1045, 1046f Interrupted aortic arch, 1694–1695, 1695f 655–656 myocardial contrast stress, 1291–1292
systolic dysfunction and, 1356– cleft mitral leaflet, 1613 bicuspid aortic, 1046f classification of, 1695t image interpretation, 657–659, 658f, speckle-tracking, 1301–1303, 1303f
1358 double orifice mitral valve, 1614– native mitral, 1046f, 1056f type B arch interruption, 1694 659f three-dimensional, 1301–1302
treatment of, 1361–1362 1615, 1614f vegetations, 1043–1044, 1044f Intersocietal Commission for the safety of, 661 overview, 1289
pharmacologic therapy, 1361 dysplasia of mitral valve, 1613 on bicuspid aortic valve, 1044f Accreditation of Echocardiography technology, 655 Ischemic LV dysfunction, 3D speckle
“resect-plicate-release” operation, Ebstein’s anomaly of mitral valve, 1615 fungus infection, 1054f Laboratories (ICAEL), 132 utility of, 659–660, 660f tracking and, 376t
1362 hypoplastic mitral valve, 1613 large aortic, 1044f, 1053f Interventricular septum (IVS), 1534f Ischemic and nonischemic Ischemic mitral regurgitation, 858–863
surgical myectomy, 1361–1362, 1362f mitral valve prolapse, 1615 on mitral native valve, 1046f, 1050f Intra-aortic balloon pump, and carotid cardiomyopathy Isolated carditis, 774–775
Hypertrophic cardiomyopathy (HCM) parachute mitral valve, 1613 transesophageal approach, 1053f ultrasound findings, 693 cardiac magnetic resonance imaging, Isolated mitral valve cleft, 1775
mutations, TD imaging in, 351 supramitral ring or membrane, Infective endocarditis prophylaxis, 1859 Intracardiac echocardiography (ICE), 1427–1428 Isomerism, echocardiography of, 1577
Hypertrophic obstructive 1611–1613 Inferior vena cava (IVC), 1063, 1143–1144, 643–653 cronary computed tomographic Isomerism syndromes. See also
cardiomyopathy (HOCM), 1260 Indocyanine green, 11 1532f, 1964 advantages of, 652 angiogram, 1427 Heterotaxy syndrome
I-XX Comprehensive Textbook of Echocardiography Index I-XXI
cardiac and extracardiac anomalies transthoracic echo, 1979 Left atrial function, 1255–1263 left upper pulmonary vein, 1962f Left ventricle, 1185–1194, 1246–1248 computed tomography and, 2063–
in, 1705f transthoracic images, 1979 anatomy of, 1255 M-mode echocardiography, 1960 apical velocity rotation and timing 2064
venoatrial connections in, 1706t ventricular apex, 1979 cardiac cycle and, 1256f pectinate muscles, 1962f for, 1202f left ventricular overfilling, 1240–1246
Isovolumic contraction (IVC), 1185–1186 Lariat procedure, 563, 566, 566f–568f functional assessment, 1257–1259 pulmonary veins, 1960, 1961f isovolumic contraction, 1185–1186 causes, 1245t
Isovolumic contraction time (IVCT), 1140 Laser Doppler flowmetry (LDF), 469–470 LA volume, 1257, 1258f rhythm analysis clues, 1960 lengthening, 1189 mitral flow prior to placement of,
Isovolumic relaxation time (IVRT), 313, Laser Doppler perfusion monitoring speckle tracking, 1259, 1259f spontaneous echo contrast, 1960 postejection isovolumic phase, 1243f
1069t, 1140 (LDPM). See Laser Doppler spectral Doppler, 1257–1259 swirling appearance, 1960 1188–1193, 1192f net forward cardiac output and,
IUGR. See Intrauterine growth flowmetry (LDF) left atrial size, 1257, 1258f thromboembolic, 1960 rapid filling, 1193–1194, 1193f 1247–1248
retardation (IUGR) Late gadolinium enhancement (LGE), overview, 1255 complications, 1960 recoiling, 1189 optimizing settings, 1248–1249, 1250f
IVC. See Inferior vena cava (IVC) 2003, 2020 overview of, 1255 event, 1960 torsion, 1186–1188 placement response of, factors affect,
IVCCI. See IVC collapsibility index Latent diastolic dysfunction, stress pathophysiology, 1259–1261, 1260f nonvalvular, 1960 ventricular assist devices and, 1235f
(IVCCI) echocardiography in, 1318 atrial fibrillation, 1259–1260 risk, 1960 1229–1230 power failure of, 1244f
IVC collapsibility index (IVCCI), 1265 Lateral resolution, 59, 59f, 733, 734f cardiac amyloidosis, 1261f valvular, 1960 Left ventricle, 3D quantitation of, structures in patient with, 1239f
IVCT. See Isovolumic contraction time Lavengin, Paul, 4 cardiomyopathies, 1260, 1260f transesophageal echocardiogram, 1149–1165 transesophageal echo, postoperative,
(IVCT) LBBB. See Left bundle branch block hypertension, 1259 1961f 2D echocardiography, limitations of, 1237f
IVRT. See Isovolumic relaxation time (LBBB) physiology of, 1256 volume, 1960 1149–1152 Left ventricular diastolic dysfunction
(IVRT) Lead infection, associated with phasic left atrial function, 1256 Simpson’s biplane method, 1960 apical foreshortening, 1152 grades of, 1127f
IVUS. See Intravascular ultrasound pacemaker, 1114–1115 physiological effects on, 1257 Left atrium myxoma, 2053f boundary recognition, 1152 ICM/NICM and, 1422
(IVUS) Lead perforation, 1215–1217 pressure-volume relationship of, Left bundle branch block (LBBB), 1217, 3D spatial coordinates, lack of, left atrial dilation in patient with,
IVUS with virtual histology (IVUS-VH), chest CT for, 1216 1256f 1377 1149–1151 1125f
657 echocardiography in, 1216f Left atrial hemangioma, 1487f Left heart hemodynamics, age groups for, geometric assumptions, 1151 Left ventricular diastolic function
reported cases of, 1217t Left atrial leiomyosarcoma, 1488f 1275t 3D reconstruction, 1152f 2D echocardiography, 1124–1125
Lead zirconate titrate (PZT), 1992 Left atrial myxomas, 845, 1467f, 1472, Left isomerism, 1577 limitations of, 1152
J Leakage current., 110–111, 111f, 111t 1474f, 1475f Left lower pulmonary vein (LLPV), 327, freehand scanning, 1150f
flow propagation velocity, 1127
color Doppler, 1129f
Jarvik-2000 Flowmaker, 1228, 1228f Left anterior descending (LAD), 1337 Left atrial pressure (LAP), 1279, 1281f 328f real time 3D ecocardiography,
future directions for, 1131–1132
Joyner, Claude, 6 artery disease, 1328 Left atrial septal myxoma, 1469f Left main coronary artery (LMCA) 1152–1156, 1152f
imaging techniques for, 1131–1132
Junctional rhythm, and PV Doppler, 333, distal post-stenotic diastolic-to- Left atrium, 1960 flow in distal, detection of, 1340 data acquisition, 1153–1155, 1154f,
integrating echocardiographic
334f systolic velocity ratio, 1340–1341 ablation therapies, 1960 transesophageal echocardiography, 1155f
variables, 1130–1131, 1130f–1131f
Left atrial appendage (LAA), 1960 anteroposterior dimension, 1960 1338–1340, 1339f first-generation matrix array
left atrial size, 1124–1125
abnormalities, 1960 arrhythmias patients, 1960 transthoracic echocardiography, transducer, 1153
K adequate visualization, 1960 atrial arrhythmias patients, 1960 1337–1338, 1339f left ventricular strain, 1157–1161,
left ventricular mass, 1124–1125
in nonsinus rhythm, 1129–1130
Kaul, Sanjiv, 13 clot detection 3D TEE for, 512, 619 atrial flutter, 1960 Left main coronary artery ostium, 647 1159f, 1160f
overview, 1124
Kawasaki disease (KD), 1688–1689 continuous wave, 1963f doppler, 1961 Left main coronary artery stenosis left volumes and EF, validation of,
pulmonary artery pressure, 1129
in childhood, 1861 3DE assessment of, 277, 277f color flow, 1961 (LMCAS), 1325 1156–1157
pulmonary artery systolic pressure
coronary arteries with, 1862t doppler interrogation, 1960 pulsed, 1961 Left parasternal approach, of three- peak filling rate from, 1158f
and, 1130f
velocity vector imaging in, 398 embolism, 1960 echocardiographer, 1960 dimensional echocardiography, for postmyocardial infarct risk
pulmonary venous flow, 1127–1128
endocardial device closure of, echodense mass, 1962f 244, 245f–254f stratification, 1165
561–563, 562f–565f Left-sided filling pressures, 1273–1279 tissue Doppler imaging for, 1127,
index, 1960 third-generation systems in, 1155–
L epicardial suturing of. See Lariat latrial tachycardia, 1960 color M-mode flow propagation 1156 1128f
procedure left atrial appendage, 1960 velocity, 1278 regional segments of, 1162f, 1163f Valsalva maneuver, 1126–1127
LAD. See Left anterior descending (LAD)
exclusion, 1961 abnormalities, 1960 echocardiographic methods for, serial evaluation of patients with wall function, 1124–1125
LaGrangian strain, 389
percutaneous, 561 adequate visualization, 1960 1273t–1274t 3DE, 1164–1165 Left ventricular dimensions, 1942–1943
Lambl’s excrescences, 1015, 1480f
surgical, 560–561 continuous wave, 1963f evaluation of, 1277f Left ventricle apical ballooning Left ventricular dysfunction, ultrasound
LAP. See Left atrial pressure (LAP)
mid-diastolic signals, 1960 doppler interrogation, 1960 variables used for, 1278t syndrome, 1388 stethoscope for screening of, 295
Large intracardiac thrombus, 1978
atrial appendage, 1978 mitral valve, surgery, 1960 embolism, 1960 left atrial dimensions, 1278–1279 Left ventricle twist, 276 Left ventricular dyssynchrony, 1218
atrial fibrillation, 1978 orthogonal mass abnormality, 1960 exclusion, 1961 mitral inflow parameters in, 1274– Left ventricle underfilling, 1246f three-dimensional speckle tracking
atrial flutter, 1978 pectinate lines, 1960 mid-diastolic signals, 1960 1276, 1275f, 1275t causes, 1247t and, 376t
autopsy, 1979 persistence, 1961 mitral valve, surgery, 1960 pulmonary venous flow and, 1276, evidence of, 1246–1248 Left ventricular ejection fraction (LVEF),
deep venous thrombosis, 1979 prominent ridges, 1960 orthogonal mass abnormality, 1960 1276f Left ventricular aneurysm, 1295f, 1298 381, 1116, 1117, 1124–1125
disystole, 1978 recanalized, 1963f pectinate lines, 1960 tissue Doppler annular diastolic Left ventricular apical thrombus, 1299f in LFLG-AS, 1929
echolucent, 1979 thrombus, 1960 persistence, 1961 velocities, 1277–1278 Left ventricular assist devices (LVAD), pacemaker and, 1218f
endocardium, 1978 traverse, 1962f prominent ridges, 1960 Left-sided superior vena cava (LSVC), 1204 systolic myocardial dysfunction and,
heart embolism, 1978 Left atrial appendage clot, embolization recanalized, 1963f 1535, 1545f and carotid ultrasound findings, 1131f
intracardiac devices, 1979 of, 1917f thrombus, 1960 Left upper pulmonary vein (LUPV), 327, 693–694 Left ventricular end-diastolic
systole, 1978 Left atrial appendage thrombus, 2058f traverse, 1962f 328f, 1962f complications of, 1240–1246 diameter (LVEDD), 1886
I-XXII Comprehensive Textbook of Echocardiography Index I-XXIII
Left ventricular end diastolic pressure Left ventricular outflow tract (LVOT) myocardial performance index, 1120 Lobster claw abnormality, 1352f, 1358, cardiogenic watery B-lines, 1982, LVIDs. See Left ventricular internal
(LVEDP), 1277, 1279, 1280f, 1375, obstruction, 1350–1354 overview, 1115 1358f 1983 dimension in systole (LVIDs)
1375f anomalous papillary muscle and, Quinones method, 1117 Loeffler endocarditis, 1403f–1404f differential diagnosis LV noncompaction, three-dimensional
Left ventricular end-diastolic pressure 1354f seventeen-segment model, 1116f Loeffler’s syndrome, 1006–1007, 2009f challenge, 1982 speckle tracking and, 376t
(LVEDP) aortic regurgitation, 1628–1630 sphericity index, calculation of, 1119 2d transthoracic echocardiography, extravascular lung water, 1982 LVOT. See Left ventricular outflow tract
assessment of, 334–335, 334f, 335f aortocameral communications, 1632 Tei's index, 1120, 1120f 1008f, 1009f human acute respiratory distress (LVOT)
Left ventricular end-diastolic volume conversely, dynamic, 1353–1354 tissue Doppler imaging, 1119 Loeys-Dietz syndrome, 958 syndrome, 1983 LVOT geometry, aging and, 1943
(LVEDV), 271 echocardiographic evaluation of, transthoracic echocardiography LOLIPOP. See London Life Sciences limitations, 1987 LVOT velocity time integral, in LFLG-AS,
Left ventricular end-systolic diameter 1351–1352, 1352f three-dimensional, 1122 Prospective Population B-line interpretation, 1987 1929
(LVESD), 1886 sinus of Valsalva aneurysm, 1630– two-dimensional, 1116–1119 (LOLIPOP) study obese patients, 1987 LV regional function, 3DE assessment of,
Left ventricular end-systolic volume 1632 velocity vector imaging, 1120 London Life Sciences Prospective patient-dependent, 1987 271–272, 276
(LVESV), 271 subvalvular aortic stenosis and, visual estimation of, 1115–1116 Population (LOLIPOP) study, 272, lung sliding, 1982 aortic stenosis, 279
Left ventricular filling pressure 1624–1626 Left ventricular thrombus, 1504–1506 1157 main approaches, 1983 aortic valve assessment, 278f, 279,
demonstration of elevated, 1126– supravalvular aortic stenosis and, Left ventricular volumes Longitudinal fiber shortening, 1119 methodology, 1983, 1984 279f
1130 1626–1628 assessment of, 81 Longitudinal peak systolic strain (LPSS), pulmonary parenchyma, 1983 contractile reserve, 276
restrictive transmitral filling pattern, systolic anterior motion and, 1350 in ICM/NICM, 1421 1069t thoracic scanning areas, B-lines left and right atria, 276–277, 277f
1126f TGA and, 1659–1661 Leiomyosarcoma, 627 Longitudinal strain, 362, 363f, 389 semiquantitative assessment,
valvular aortic stenosis and, 1618– left ventricle twist, 276
transmitral diastolic inflow for, 1126f aortic, 1489f–1490f Long-term axial flow devices, 1227–1228 1983, 1984f right ventricle, 277–278
Left ventricular free wall, rupture of, 1294 1624
left atrial, 1488f Berlin heart incor assist device, 1228 two-dimensional scanner, 1983 valvular assessment, 278, 278f
vs. mitral regurgitation, 1353f
Left ventricular geometry, partitions Levacor ventricular assist device, 1228 HeartAssist system, 1228 physical basis, 1982, 1983 LV systolic dysfunction, 3D speckle
Left ventricular overfilling, 1240–1246
value of, 1887f Levo-transposition of great arterie, 1759f HeartMate II continuous flow LV physical scatterer, 1982 tracking and, 376t
Left ventricular pseudoaneurysm, 569,
Left ventricular hypertrophy (LVH), 294, Levo transposition of great vessels, in assist device, 1228 physiological basis, 1982, 1983 LV volume assessment, 3D speckle
1294–1295
351, 352, 1125f, 1871, 1933–1934, female, 1916f Jarvik-2000 Flowmaker, 1228, 1228f pulmonary edema, 1983 tracking and, 376t
closure of, 571, 571f
1974 LFLG-AS. See Low-flow, low-gradient Long-term third generation centrifugal sonographic appearance, 1982, 1983f
Left ventricular quantification, 3D LV wall motion abnormalities
in female, 1911f aortic stenosis (LFLG-AS) flow systems, 1228–1229 consolidated lung, 1983f
echocardiography and, 82–85, 84f three-dimensional speckle tracking
Left ventricular internal dimension in Libman–Sacks endocarditis, 1867–1868, DuraHeart magnetically levitated multiple B-lines, 1982, 1983f
Left ventricular-RA communication, and, 376t
diastole (LVIDd), 1118 1868f centrifugal assist system, 1228 normal lung, 1983f
1779–1782 Lymphomas, 1500f, 2054
Left ventricular internal dimension in Libman-Sacks vegetations, 931 EvaHeart left ventricular system, LUPV. See Left upper pulmonary vein
Left ventricular remodeling, 3D primary cardiac, 1490
systole (LVIDs), 1118 echocardiography, 1162–1164 Lidocaine, 508 1228–1129, 1228–1229 (LUPV)
Left ventricular intracardiac metastasis, Left ventricular systolic function, Light and sound, 1995, 1996 HeartWare ventricular system, 1224f, LVAD. See Left ventricular assist device
1499f 1115–1123 acousto-optical imaging, 1996 1228 (LVAD) M
Left ventricular lipoma, 1482f arterial–ventricular coupling, complementary approach, 1996 Levacor ventricular assist device, LV concentric hypertrophy, 920
MAIN-COMPARE trial, 659
Left ventricular mass, 1124–1125, 1121–1122 endogenous absorbers, 1995 1228 LV contractility, reduction in, 920
Major adverse cardiovascular events
1942–1943 assessment of, 1116–1119 laser pulses, 1995, 1996 Lower bound velocity (LBV), 88 LV diastolic function, assessment of, by
(MACE), 2042
Bland–Altman analysis, 1162 biplane method of discs, 1118–1119 precision, 1995 Low-flow, low-gradient aortic stenosis TD imaging, 352–353, 355f, 356f
Malignant melanoma, 1500
3D echocardiography, 1161–1162 blood supply from particular photoacoustic imaging, 1995 (LFLG-AS) LV dyssynchrony, TD Imaging for, 353,
Malignant primary cardiac tumors,
epicardial and endocardial borders, coronary artery, 1116 Schlieren tank, 1996 with ejection fraction, 1929 354f
1161f classic implementation, 1996 1484–1511
contrast echocardiography of, 1121, with low ejection fraction, 920–921 LVEDD. See Left ventricular end-diastolic
partitions value of, 1887t Linear array system, 8 SAVR in, 927 diameter (LVEDD) angiosarcoma, 1485–1486
1121f
RT 3DE for, 1162 Linear/phase array transducers, 76, 76f with normal ejection fraction, LVEDP. See Left ventricular end diastolic cardiac plasmacytoma, 1490–1491,
Doppler echocardiography of, 1119
Left ventricular myxomas, 1474 Linear probe, 58, 58f 921–924 pressure (LVEDP) 1492f
2D speckle tracking
Left ventricular noncompaction (LVNC), Line density, 60 Low flow-mediated vasoconstriction LVEDV index, 1157 fibrosarcomas, 1488
echocardiography, 1120
395–396, 1384–1388, 1385f, fractional shortening at Lipoma, 2054f (LFMC), 470–471, 470f–474f LVEF. See Left ventricular ejection hydatid cyst, 1491, 1493f–1498f
1389f–1391f, 1394f endocardium, 1118 Lipomatous hypertrophy Low MI imaging, 420 fraction (LVEF) pericardial mesotheliomas, 1491
cardiac MRI for, 2000–2002, 2001f, longitudinal fiber shortening, 1119 of atrial septum, 1484f Low transvalvular gradients, aortic LV ejection fraction (LVEF), 270 primary cardiac lymphoma, 1490
2002f M-mode echocardiography, 1116– of interatrial septum, 182, 182f, 533, stenosis with, 913 in acute coronary syndromes, rhabdomyosarcoma, 1486–1488
echocardiographic features of, 1119 536f, 2054, 2056f LPSS. See Longitudinal peak systolic 1293–1294 sarcomas, 1484–1488
1385–1388, 1386f–1387f E point septal separation, 1117 Live 3DE, 269 strain (LPSS) LVESD. See Left ventricular end-systolic Manometers, 27
isolated, 1387f, 1393f left ventricular ejection fraction, Liver cirrhosis, and HV Doppler, 318, 319f LSVC. See Left-sided superior vena cava diameter (LVESD) Marfan syndrome (MFS), 958–959
normal fetal ontogenesis, 1385 1116, 1117 Liver disease, and HV Doppler, 318–319, (LSVC) LVESV index, 1157 TAA and, 1936
Left ventricular opacification (LVO), 421, parasternal long-axis, 1116 319f Lung artifact, 227 LV filling pressures, estimation of, by TD Matrix biplane transesophageal echo
426 modified cylinder-ellipse formula, LMCA. See Left main coronary artery Lung carcinoma, 1459f imaging, 352–353 (TEE) probe, 99–100, 100f
Left ventricular outflow tract (LVOT) 1118f (LMCA) Lung ultrasound scan (LUS), 1982, 1983 LVIDd. See Left ventricular internal MDCT. See Multidetector computed
forms of, 897t myocardial contractile velocity and, LMCAS. See Left main coronary artery aerated lung, 1982 dimension in diastole (LVIDd) tomography (MDCT)
in prosthetic valves, 1084 1119 stenosis (LMCAS)
I-XXIV Comprehensive Textbook of Echocardiography Index I-XXV
Mean left atrial pressure, assessment of, Mitral atresia, 1552f, 1701–1704 pap muscle dysfunction, 1972 continuity equation, 789 mitral valve area planimetry, 831 supramitral ring or membrane,
335, 335f associated systemic venous primary, 880–883 3DE assessment of, 280, 281f, 282 PISA method, 834 1611–1613
Mean pulmonary artery pressure (MPAP), anomalies, 1702–1703 leaflets, 880–883 echocardiography pressure half-time, 831–833, 832t, mitral valve lesions,
1063, 1064t, 1065t, 1066, 1272, 1272f, 1901 apical four-chamber view, 1703 mitral annulus, 883 exercise, 790 833f echocardiographic views,
Mechanical index (MI), 57, 111, 418–419 parasternal views, 1703 subvalvular apparatus, 883 pitfalls of using, 790–791 Wilkins Score, 830t 1610–1611
high, 422–423 subcostal view, 1702–1703 pulmonary venous flow, 886 recommendations for, 778t left atrium, 834 types of, 1611t
low, 423 suprasternal notch view, 1703 and PV Doppler, 336–338, 337f, 338f three-dimensional, 791 left atrium appendage, 834 Mitral valve area (MVA), 282
Mechanical safety, TEE probe echocardiography after stage 1 rupture, 1972 two-dimensional, 778–779, 779f long-term outcome, 842–843 Mitral valve calcification, 1889
examination and, 111–112 palliation, 1703–1704 secondary, 883–884 mitral valve, assessment of, 779–783 mitral regurgitation and, 834–836 in women, 1889
Mechanical valves, 1082 Mitral prosthetic regurgitation, 1106f, left ventricular remodeling, 883–884 abnormal motion of leaflets, 779–781 mitral valve morphology, 827–829 Mitral valve diseases, 775–776, 826–879,
bileaflet, 1083t, 1085f 1107f–1108f mitral annulus, 883 commissural fusion, 781–783 leaflet motion, 828 1318
single disc, 1083t paravalvular, 1102f–1104f mitral valve distortion, 883–884 PISA method, 789 subvalvular pathology, 828 anatomy of mitral valve, 775–776
Melody valves, 651 Mitral regurgitation (MR), 791–801, severity of, 885–889 planimetry, 785 valve calcification, 828–829 mitral regurgitation, 791–801
Membranous subaortic stenosis, 1625 880–895, 1972, 1974f grading of, 888 pressure gradient, 785–787 valve thickening, 827–828 assessment of, 793
Mesothelial/macrophage incidental acute, 1972 qualitative assessment of, 885–886 pressure half-time, 787–789 normal mitral valve area, 827 color Doppler flow, 793–796
cardiac excrescences, 1511–1518 acute vs. chronic, 885 quantitative assessment of, 886–888, and PV Doppler, 338–339, 339f pulmonary hypertension, 836–838 continuous wave Doppler, 796–797
cardiac calcified amorphous tumor, cardiac MRI for, 2012–2013 888f severity mitral valve echocardiographic exercise echocardiography, 800
1512–1514 chronic asymptomatic, sequential classification of, 784t follow-up in, 801
semiquantitative assessment of, 886 scoring system, 836
extracardiac masses, 1514, 1514f pulmonary vein flow, 798
evaluation of, 890–892 surgical correction of, 538–540, 541f, determination of, 784–785 Wilkins scoring system, 836–837
intracardiac hardware, 1514–1517 pulsed Doppler, 797–798
BNP levels, 891 542f indices of, 789–790 rheumatic, 827
mitral annular calcification, 1512, severity of, 793
Doppler echocardiography, 891–892 transesophageal echocardiography, methods for quantification of, 785t RT 3DE scoring system, 838t
1512f integrative approach for, 800–801
follow-up in, 890–891 1972 Mitral stenosis, echocardiographic Mitral valve (MV), 577–579, 578f, 579f
Metabolic cardiomyopathy, 1407 organic, 801t
chronic functional, 884f vena contracta, 886 assessment, 826–847, 827f in adults, 1818–1820
Metastasis, 2053–2054 role of TEE in assessing, 788–800,
classifications, 1973t Mitral regurgitation, echocardiographic anatomic considerations, 826–827 cleft, 1818–1819
from melanoma, 2055f 799t
color Doppler, 1973t, 1974f, 1975f, assessment of, 847–863 atrial septal defects, 842 cor triatriatum, 1818
Metastatic left pleural effusion, in female, supportive signs, 798
1975t causes of, 848t balloon mitral valvotomy, 839t, 842f double orifice mitral valve, 1819–1820
1913f three-dimensional
Doppler vena contracta, 1973t, 1975t echocardiography, role of, 848t complications of, 840–841 echocardiography, 1818
Metastatic melanoma, 1499f echocardiography, 800
regurgitant fraction, 1973t, 1975t etiology and mechanism of, 848–849 echocardiography in patients for, mitral regurgitation, 1820
involving right ventricle, 1499f two-dimensional echocardiography,
Micro-beam forming, 78 regurgitant orifice area, 1973t hemodynamic consequences, 838–840 parachute, 1820 792–793
Microbubbles, 1993, 1994 regurgitant volume, 1973t, 1975t 874–876 evaluation of patient, 838–840, 839t anatomy, assessment of mitral stenosis, 776–791
first human studies, 1993 color flow imaging, 885 left atrial size and pulmonary mitral regurgitation in, 840–841 Cormier score, 782t overview of, 826
future potential, 1993 continuous wave Doppler, 886 pressures, 876 post, 841f, 843f, 844f Wilkins score, 782t role of 3D TEE in operating room in,
liver metastasis, 1993, 1994f continuous wave Doppler signal of, left ventricle size and function, cardiac perforation, 841–842 anatomy of, Cormier Score, 837t 577–582, 578f–593f
macro/microvascular systems, 1993 1279 874–876 causes of, 846–847 3DE assessment of, 278, 278f, 280, stress echocardiography in, 1318
mechanical index, 1993 3DE assessment of, 282–283, 282f, key notes, 847–849 conditions clinically mimicking 281f Mitral valve insufficiency, 1860f
microbubble lymphangiography 283f mitral valve prolapse, 849 rheumatic, 844–846 3D echo of, 516–520, 517f, 518f Mitral valve lesions, echocardiographic
sentinel lymph node, 1993, 1994f echocardiographic criteria for, 889t severity of, 863–876 atrial ball valve thrombus, 845 pulsed wave Doppler across, 1533f views, 1610–1611
preclinical work, 1993 endocarditis, 1972 antegrade velocity of mitral inflow, congenital double orifice mitral real time 3D echocardiographic score Mitral valve morphology, 827–829
ultrasound contrast, 1993 etiology of, 880–884 869 valve, 844–845 of, 783t leaflet motion, 828
VEG-F2 study, 1993 exercise echocardiography, role of, color Doppler jet area, 863–868 congenital mitral stenosis, 844 short-axis, 1533f subvalvular pathology, 828
Mid-left ventricular hypertrophic 892–893 continuous-wave jet intensity, 869 congenital parachute mitral valve, Mitral valve, congenital anomalies of, valve calcification, 828–829
cardiomyopathy, 1356–1358 hemodynamically stable, 1972 echocardiographic assessment of, 845, 846f 1610–1615 valve thickening, 827–828
Midmuscular ventricular septal defect, hemodynamic consequences of, 864t degenerative mitral valve stenosis, individual mitral lesions, evaluation Mitral valve opening, and untwisting,
1544f 889–890 PISA method, 870–871 844 of, 1611–1615 1200–1201
Midwall fractional shortening (MWFS), left atrium, 890 pulmonary venous flow, 869 left atrial myxoma, 845 abnormal mitral arcade, 1613–1614 Mitral valve orifice area (MVOA), 777,
1118 left ventricle, 889–890 quantitative echo/Doppler methods, diseases of other valves, 834–836 accessory mitral orifice, 1615 790–791
Miniaturization technology, 102–103, pulmonary arterial pressure, 890 869–874 echocardiography cleft mitral leaflet, 1613 continuity equation, 789
103f, 104f right ventricle, 890 semiquantitative echo/Doppler in balloon mitral valvotomy, 838–840, double orifice mitral valve, 1614– mitral PHT and, 787–789
MinivisorTM, 291, 292f ICM/NICM and, 1422–1424, 1423f methods, 863–869 839t 1615, 1614f Mitral valve performance, 1249
Mirror image artifacts, 735–736. See also mechanism of, 884–885 vena contracta width, 868–869 exercise, 844 dysplasia of mitral valve, 1613 Mitral valve prolapse, 169
Artifacts functional classification, 884 surgical considerations in, 876 role of three-dimensional, 844 Ebstein’s anomaly of mitral valve, 3D TTE and, 282, 282f
MitraClip® device, 539 jet direction, 884–885 Mitral stenosis, 776–791 two-dimensional, parameters of, 827 1615 ultrasound stethoscope for screening
Mitral annular calcification (MAC), 1512, mitral valve repair, feasibility of, 892 anterior mitral valve leaflet, 777, 780f indices of severity, 829–834 hypoplastic mitral valve, 1613 of, 295
1512f, 1895f, 1946–1948 overview, 880 associated lesions, 783–784 continuity equation, 833 mitral valve prolapse, 1615 Mitral valve prolapse (MVP), 849–863,
in elderly, 1946–1948 papillary muscle, 1972 cardiac MRI for, 2013 diastolic pressure gradient, 829–831 parachute mitral valve, 1613 1615
I-XXVI Comprehensive Textbook of Echocardiography Index I-XXVII
echocardiographic assessment of, 99mTc-sestamibi gated single- for myocardial viability detection, 431 Myofiber structural orientation, 1183f right and left supraclavicular PAH. See Pulmonary arterial
850–855 photon computed emission Myocardial contrast stress Myxomas, 622–623, 1474f, 2051 examination, 189–190, hypertension (PAH)
M-mode, 850 tomography (GSPECT), 1157, echocardiography, 1291–1292 atrial, 1468 205f–212f Papillary fibroelastoma, 624,1023f–1024f,
three-dimensional, 851–855 1159f Myocardial deformation imaging, 360. attached to atrial septum, 1471 live/real-time three-dimensional 1476f–1477f
two-dimensional transesophageal, Mucopolysaccharidosis, 931 See also Strain imaging biatrial, 1470f transthoracic echocardio- on tricuspid valve, 2019f
851, 855f, 856f MullinsTM catheter, 533 Myocardial disease, detection of, by TD left atrial, 1467f graphy, 209f–212f Papillary muscle rupture, 862f, 1297,
two-dimensional transthoracic, 851, Multi-beat acquisition, in 3D imaging, 350–352 left atrial septal, 1469f two-dimensional transthoracic 1297f
852f–855f echocardiography, 74–75, 75f Myocardial fiber organization, 1180f right atrial, 1470f echocardiography, 205f–209f PAPS. See Pulmonary hypertension
surgical methods and indicators in, Multicenter Aneurysm Screen Study Myocardial infarction right ventricular, 1471f–1472f, 1473f right parasternal examination planes, (PAPS)
855–863 (MASS), 294 mechanical complications of, ventricular, 1468–1470 188–189, 189f–204f PAPVC. See Partial anomalous pulmonary
degenerative mitral regurgitation, 863 Multidetector computed tomography 1294–1298 live/real-time, three-dimensional venous connection (PAPVC)
functional mitral regurgitation, (MDCT), 2023 left ventricular aneurysm, 1298–1300 transthoracic echocardio- Parachute mitral valve, 1613
857–858 acute chest pain evaluation with, left ventricular free wall, rupture of,
N graphy, 196f–204f Paradoxical low-flow, low-gradient aortic
infective endocarditis, 863 2045–2047 1294 Narrow-angled display, 241 two-dimensional transthoracic stenosis (PLFLG-AS), 921–922
ischemic mitral regurgitation, for coronary artery anomalies, left ventricular pseudoaneurysm, National Board of Echocardiography echocardiography, 189f–196f cardiac catheterization and, 923
858–863 2047–2049 1294–1295 (NBE), 754 Non-ST elevation myocardial infarction diagnosis of, 924
rheumatic mitral regurgitation, 863 techniques of, 2047 papillary muscle rupture, 1297, 1297f National Electrical Manufacturers (NSTEMI), evaluation of, 226 invasive vs. noninvasive evaluation of
tricuspid and pulmonary valve Multigate pulsed Doppler, 70 ventricular septal rupture, 1295– Association (NEMA), 110 No-reflow phenomenon, 443 AS, 922–924
prolapse with, 855f Multipath reflection, 81 1297, 1296f Normal heart, 1185–1194
National Institutes of Health, 8 LVEF and, 921–922
Multiplane mode, 268–269
in women, 1888, 1888f–1889f speckle-tracking echocardiography, Native valve endocarditis, role of 3D TEE left ventricle, 1185–1194 mechanisms for, 924–925
Multiple-beat 3DE imaging, 269
Mitral valve quantification 1302 in operating room in, 597–604, isovolumic contraction, 1185–1186 cardiac output, 924
Multiple thrombi, with poor ventricular
three-dimensional echocardiography vs. cardiac death, 1311–1315 609f–612f lengthening, 1189 impaired myocardial function, 924
function, 1377f
and, 81–82, 82f, 83f Myocardial ischemia, speckle-tracking Nitric oxide (NO), 454 postejection isovolumic phase, pseudostenosis, 925
Multislice computed tomography
Mitral valve segmentation analysis, 881f echocardiography, 1302 and endothelial function, 450, 451 1188–1193, 1192f with preserved ejection fraction,
(MSCT), 1427
Mitral valve stenosis, in female, 1888– Myocardial perforation, pacemaker Nodal re-entrant tachycardia (NRT), 1957 rapid filling, 1193–1194, 1193f 1929–1930
Muscular inlet defect, 1593
1889 associated, 1215–1217 Noninvasive hemodynamic monitoring, recoiling, 1189 SAVR in, 927
Muscular outlet defect, 1593
Mitral valve vegetation, in female, 1911f 2D transesophageal 229–230, 230f, 231f torsion, 1186–1188 Parametric display, 708–711, 709f–711f
Muscular trabecular defect, 1594
Mitral valvular verrucous nodules, 773f echocardiography in, 1216, Nonischemic dilated cardiomyopathy sonomicrometer crystal tracings, Parasternal long-axis (PLAX) view, 1116,
Muscular ventricular septal defects,
M-mode echocardiography, 3, 3f, 58, 58f, 1216f (NICM), 1418 1203f 1117
557–558, 1593–1594, 1593f, 1594f.
119–130 See also Ventricular septal defects reported cases of, 1217t mitral–septal separation in, 1419f ventricular narrowing, 1187f of aortic root, 1797f
development of, 4–7 (VSDs), closure of RT3DE in, 1216f vs. ICM, 1425, 1426t Nutritional deficiency, 1880 Parasternal window, TTE
in left ventricular systolic function, muscular inlet defect, 1593 Myocardial performance index (MPI), Nonsinus rhythm, 1129–1130 Nyquist, Harry, 70 linear measurements, 139–141,
1116–1119 muscular outlet defect, 1593 1120 Nonstandard echocardiographic Nyquist limit, 69, 70, 736 139f–141f
E point septal separation, 1117 muscular trabecular defect, 1594 ICM/NICM and, 1419 examination, 188–223 ascending aorta, 140, 141f
left ventricular ejection fraction, in rheumatoid arthritis, 1868 abdominal examination, 220 Doppler imaging, 141, 141f
1116, 1117
MV clipping, 538–540, 541f, 542f
Myocardial perfusion examination from back, 216, 218, 220
O parasternal long-axis (PLAX) plane,
3D TEE guidance of, 540
parasternal long-axis, 1116 MitraClip device for, 539 echocardiography, 441–447 two-dimensional transthoracic Obesity, and HV Doppler, 318–319, 319f 137–139, 138f, 139f, 139t
in right ventricle, 1136–1138 patient selection for, 540 acute coronary syndromes, 443 echocardiography, 221f Off-axis imaging, 164 M-mode imaging, 138–139, 139f
transthoracic echocardiogram, 136, MVOA. See Mitral valve orifice area chronic coronary artery disease, left atrial appendage examination, One-dimensional (1D) technique, 137–138
136f (MVOA) 443–445 212, 214, 216, 217f–219f echocardiography, 3f, 25, 25f two-dimensional anatomic imaging,
of tricuspid valve, 984–986 MVP syndrome (MVPS), 1888 myocardial perfusion evaluation, by live/real-time three-dimensional Optigo, 291, 292f 138, 138f, 139f, 139t
Ebstein’s anomaly, 986f MWFS. See Midwall fractional shortening CE, 441–443 transthoracic echocardiography, Optison, 417, 418t. See also Contrast parasternal short-axis plane, 144–146,
functional events, demonstrating, (MWFS) nonischemic dilated 217f–219f echocardiography 145f–147f, 145t
984f Myocardial blood flow (MBF), 442 cardiomyopathy, assessment two-dimensional transesophageal Ostium primum atrial septal defect, 1586 aortic valve level, 145, 146f
septal leaflet, identification, 985f Myocardial blood volume (MBV), 422 of, 445–446, 447f echocardiogram, 219f Outflow tract obstruction, 1766–1770 coronary artery imaging, 145
systolic abnormalities, 986f Myocardial contractile motion, 1159– and stress echocardiography, 1319 two-dimensional transthoracic congenital aortic stenosis/bicuspid Doppler imaging, 146, 147f
using contrast injections, 1160 Myocardial tagging, 405 echocardiography, 217f aortic valve, 1766–1770 left ventricular apex level, 146, 147f
identification, 985f Myocardial contractile velocity, 1119 Myocardial viability, speckle-tracking left parasternal and apical planes for subaortic stenosis, 1770 linear measurements, 146
Moderator band, 1503 Myocardial contrast echocardiography echocardiography, 1302–1303 coronary arteries, 190, 204, 207, mitral valve level, 145, 146f
MPAP. See Mean pulmonary artery (MCE), 416, 422, 430. See also Myocardial viability assessment, contrast 213f–216f papillary muscle level, 145, 146f
pressure (MPAP) Contrast echocardiography echocardiography for, 443 live/real-time three-dimensional P technique, 144–145, 145f, 145t
mPAP. See mean pulmonary artery for CAD detection, 430–431 Myocardial wall motion abnormality, transthoracic echocardio Paced rhythm, and PV Doppler, 334, 334f right parasternal long axis view,
pressure (mPAP) coronary flow reserve by, 431 1311 graphy, 214f–216f PAcT. See Pulmonary flow acceleration 141–142
MSCT. See Multislice computed for myocardial perfusion assessment, Myocarditis, velocity vector imaging in, two-dimensional transthoracic time (PAcT) Doppler imaging, 142
tomography (MSCT) 427–428, 429f 398–399 echocardiography, 213f–214f PA diastolic pressure (PADP), 230, 231f technique, 141–142
I-XXVIII Comprehensive Textbook of Echocardiography Index I-XXIX
two-dimensional anatomic imaging, MRI/CTA, 1809 congenital anomalies, 1448–1449 Pericardial tumors, computed cerebrovascular anatomy, 664 PET. See Positron emission tomography
142 postoperative adult, 1809 constrictive pericarditis, 1444 tomography for, 2065–2066 collateral pathways, 669–671, 670f (PET)
right ventricle inflow view, 142, 142f, pulmonary arterial pressure, 1601 Doppler flow velocity records, Pericardiocentesis, monitoring of, by grading carotid stenosis, 679, PFRa. See PFR difference (PFRa)
142t, 143f stepwise evaluation for, 1599t 1444–1448 CONTISCAN transducer, 230–233, 682–683, 683f PFR difference (PFRa), 1158
Doppler imaging, 142, 143f TGA and, 1659 M-mode and 2D echo, 1444 232f grading internal carotid artery Phantom tumors, 737
technique, 142, 142f transesophageal echocardiography, 3D echocardiography, 1452–1461 Pericarditis. See also Pericardial disease stenosis, 683–684, 684t, Phased array technology, 58
two-dimensional anatomic imaging, 1808 echocardiographic appearance, acute, 1436 685f–688f Phase sensitive inversion recovery (PSIR),
142, 143f Patent foramen ovale, 1585–1586 1435–1436 chronic effusive, 1437 intima-media thickness and carotid 2020
right ventricular outflow tract view, Patent foramen ovale (PFO), 7 effusive-constrictive pericarditis, constrictive. See Constrictive plaque assessment, 676–679, Phonocardiography, 25, 26f
142–144, 143f, 143t, 144f closure of, 555, 557, 558f 1448 pericarditis (CP) 678f–681f Physio ring, 582, 591f–593f
Doppler imaging, 143–144, 144f ICE imaging during, 648, 650, 650f fibrin deposits and, 1456 effusive-constrictive, 1448 near occlusion and total occlusion of Piezoelectric crystal, 55–56
technique, 142, 143f, 143t color contrast in, 1552f M-mode and 2D echocardiography, etiology of, 1437t ICA, 685–688, 690f PISA. See Proximal isovelocity surface
two-dimensional anatomic imaging, Patient prosthetic mismatch (PPM), 1437–1438 Pericardium. See also Pericardial disease posterior circulation, 668–669, 670f area (PISA); Proximal isovelocity
143, 144f 1091–1092 multimodality imaging of functions of, 1436t scanning protocol, 671–676, surface area (PISA) method
Paravalvular aortic prosthetic Pattern correlation (PC), 88 pericardium, 1450 multimodality imaging of, 1450 672f–675f PISA method, 282–283
regurgitation, 1105f PAU. See Penetrating aortic ulceration overview, 1435 normal, 1435 technical aspects of carotid studies, PISA (proximal isovelocity surface area)
Paravalvular mitral prosthetic (PAU) pathophysiology of, 1436 Perimembranous, 1592–1593, 1592f, 671 method, 70
regurgitation, 1102f–1104f PA wedge pressure (PAW), 230, 231f pericardial effusion, 1436–1437 1595f, 1596f vertebral arteries, assessment of, PLAATO device, 561–563
Paravalvular mitral regurgitation, in PCWP. See Pulmonary capillary wedge pericardial tamponade, 1438–1440 inlet defect, 1592–1593 691–693, 694f Plane wave ultrafast imaging, 1990, 1990f,
female, 1918f–1919f pressure (PCWP) Doppler flow velocity recordings, outlet defect, 1592 femoral access complications by, 1991
Paravalvular prosthetic leaks Peak filling rate (PFR), 1157–1158 1442–1443 trabecular defect, 1593 694–701 elastography, 1991
closure of, 546–548, 549f, 550f RT 3DE in, 1158f echo-guided pericardiocentesis, Perimembranous inlet defect, 1592–1593 arterial dissection, 700 fast graphic chips, 1991
3D TEE monitoring of, 548, 550f, 551f Pediatric echocardiography, birth and 1443–1444 Perimembranous outlet defect, 1592 AV fistula, 699–700, 701f radio frequency data, 1991
3D TEE and, 512, 513f development of, 6 two-dimensional echo, 1440–1442 Perimembranous trabecular defect, 1593 bleeding and hematoma, 696, 697f software-driven systems, 1990
Paravalvular regurgitation (PVR), 608–612 Pediatric hearts, imaging of, 285–286 physiology of, 1436 Perimembranous ventricular septal patient-related risk factors, 696 spatial resolution, 1990
of prosthetic valves, 1099 Penetrating aortic ulcer (PAU), 961, 962f Pericardial duplication cyst, computed defect, 1747f procedure-related risk factors, 696 potential penalty, 1990
vs. transvalvular regurgitation, 1106 in elderly, 1921–1923 tomography for, 2064 Perimembranous VSDs, 557. See also pseudoaneurysm, 696–699, 698f ultrafast doppler, 1990, 1991, 1991f
Partial anomalous pulmonary venous Penetrating chest trauma, 1972 Pericardial effusion, 1045–1047, 1436– Ventricular septal defects (VSDs), retroperitoneal bleeding, 696 ultrasound imaging, 1990
connection (PAPVC), 1672 close evaluation, 1972 1437 closure of Perivalvular abscess, 1511 Plaques, 677–678, 680f, 681f
PASP. See Pulmonary artery systolic hemothorax, 1972 circumferential, 1500f Peripartum cardiomyopathy, 1381–1384, Permanent pacemakers/implantable Plasmacytoma, 1492f
pressure (PASP) impaling object, 1972 computed tomography for, 2065, 1381f–1384f cardioverter-defibrillators cardiac, 1490–1491, 1492f
Patches, 78 transesophageal echocardiogram 2065f definition, 1381 complications in, 1212 PLAX. See Parasternal long-axis (PLAX)
Patent ductus arteriosus (PDA), 1599– (TEE), 1972 3D transthoracic echocardiography dobutamine echocardiography in, deleterious effects of, 1217–1218 Pleural effusion (PE), 1985
1602, 1751–1754, 1755f–1757f, wounds, 1972 advantages of, 1453t–1454t 1381–1384 echocardiographic findings in, chest X-ray, 1985
1805–1809, 1808t lower left parasternal, 1972 live/real-time, 1454f, 1455f–1456f TAPSE and, 1381 1210–1212 computed tomography, 1985
anatomy, 1599 lower right parasternal, 1972 vs. 2D transthoracic vs. DCM, 1381 endocarditis and, 1214 detection, 1985
aortic runoff, 1601 Percutaneous continuous flow devices, echocardiography, 1453–1456 in women, 1904–1905, 1904f–1905f myocardial perforation, 1215–1217 effusion, 1985
cardiac catheterization, 1808 1250–1252 echocardiography of Peripheral arterial tonometry (PAT), 2D transesophageal etiologies, 1985
chamber dimensions, 1601 Impella device, 1250, 1251f M-mode, 1439f 467–469, 468f, 469f echocardiography in, 1216, 1216f lung appearance, 1985
closure of, 548–550, 551f–552f, 1809 TandemHeart, 1250–1251 two-dimensional, 1438f Peripheral artery/intra-coronary infusion reported cases of, 1217t minimal effusions
direction of shunt, 1601 Percutaneous mitral balloon epicardial fat and, 1438 of Ach, 453 RT3DE in, 1216f detection, 1985
duct morphology, 1600–1601 valvuloplasty (PMBV), 533–537, exudative, 1439f Peripheral hand warming, 453 overview, 1210 ultrasound, 1985
subclavian origin, 1601 537f–539f tuberculous, 1458f Peripheral vascular ultrasound, 663–701 RV apical pacing with LV ultrasound images, 1985
usual ductus, 1600 Percutaneous transvenous mitral Pericardial hydatid cyst, 1497f–1498f carotid artery diseases and, 663–664 dyssynchrony, 1217–1218 PLFLG-AS. See Paradoxical low-flow,
vertical duct, 1600–1601 commissurotomy (PTMC), 777, Pericardial masses, 1458–1461 anterior circulation, 665–668, thrombosis and stenosis associated low-gradient aortic stenosis
ductus, characteristics of, 1600 790 Pericardial mesotheliomas, 1491 667f–669f with, 1215 (PLFLG-AS)
echocardiography, 1599–1600 Perfluorocarbon gases, 13 Pericardial metastasis, 1500f aortic arch, 664–665, 665f, 666f tricuspid regurgitation, 1212–1214 Pneumothorax (PTX), 1986
ductal view, 1599 Perfusion Score Index (PSI), 423 from malignant thymoma, 1459f assessment after carotid artery lead infection associated with, diagnosis, 1986
echocardiographic views, 1599–1600 Pericardial cysts, 1460f, 2054, 2055f Pericardial tamponade, 1438–1440 endarterectomy and stenting, 1114–1115 conditions needed, 1986
objectives, 1599 Pericardial diseases, 1435–1451 Doppler flow velocity records, 688–691, 692f time course for, 1213 lung sliding
suprasternal view, 1599–1600 acute pericarditis, 1436 1442–1443 cardiac pathology and ultrasound transthoracic echocardiography for, abolition, 1986
echocardiography for, 1805–1807 anatomy of, 1435–1436 echo-guided pericardiocentesis, findings, 693–694 1213–1214, 1213f absence, 1986
hemodynamic significance of, 1601 computed tomography for, 2064– 1443–1444 2003 carotid duplex SRU consensus Persistent left superior vena cava presence, 1986
with left-to-right shunt, 1807 2066 two-dimensional echo, 1440–1442 criteria, 684–685 (PLSVC), 1958 nondependent condition, 1986
I-XXX Comprehensive Textbook of Echocardiography Index I-XXXI
pathognomonic LUS sign, 1986 benign cardiac fibromas, 1480 color Doppler reconstruction, 1106f, Pseudosevere aortic valve stenosis, 910f characteristics, 1985 B-lines significance, 1985
radiography, 1986 cardiac hemangiomas, 1482–1484 1107f–1108f PTMC. See Percutaneous transvenous ultrasonic window, 1985 heart failure hospitalization, 1985
Point-of-care diagnosis, 291–296 cardiac lipomas, 1481–1482, 1483f paravalvular aortic prosthetic mitral commissurotomy (PTMC) Pulmonary flow acceleration time (PAcT), persistent hemodynamic congestion,
acute care environment and, 294 cardiac myxoma, 1464–1475 regurgitation, 1105f Pulmonary arterial hypertension (PAH), 1270 1985
battery-powered ultrasound imagers cardiac rhabdomyoma, 1480–1481 paravalvular mitral prosthetic 1063, 1129 Pulmonary hypertension (PH), 171, 890, treatment, 1984, 1985
and, 291–292, 292f Primary cardiac lymphoma, 1490 regurgitation, 1102f–1104f 2D STE and, 372 1063–1079 B-lines, 1985
cardiac disorders, screening and Private tags, 95 prosthetic dehiscence, 1108f–1109f echocardiographic prognostic clinical classification of, 1064t–1065t degree of dyspnea, related to, 1985
identification of, 294 Processing technology, TEE, 103, 104f, right atrial lipoma, 1096f predictors in, 1073t–1074t conventional echocardiography, pharmacological therapy, 1985
abdominal aortic aneurysm, 294–295 105f 3D transthoracic echocardiography, idiopathic, 1068f–1069f, 1070f 1063–1070 tailoring, 1985
left ventricular dysfunction, 295 Prolonged PR interval 1095–1100 pressure response to exercise, 2d echocardiographic features, 1067 pulmonary congestion, reducing of,
mitral valve prolapse, 295 and HV Doppler, 308, 308f of homograft aortic prosthesis, 1100f, 1071t–1072t pulmonary hemodynamics, 1063– 1984
and future directions, 296 and PV Doppler, 331, 331f 1101f Pulmonary artery (PA) 1067 medicines effectiveness assessment,
new physical examination Propagation velocity, 55, 63 of St. Jude aortic prosthesis, 1097f, bifurcation, 1537f stress echocardiography, 1067–1070 1984
follow-up echocardiography, 293–294 in different materials, 56 1101f bifurcation and smaller, 1547f transesophageal echocardiography, monitoring body weight, 1984
ultrasound stethoscope, applications PROSPECT trial, 369 of St. Jude mitral prosthesis, 1097f, catheterization, 229–230 1067 ventricular dysfunction, 1985
of, 293, 293f Prostacyclin (PGI-2), 454 1098f–1099f Pulmonary artery aneurysm, in women, 2d echocardiographic signs, indirect, Pulmonary regurgitation, 1036, 1038f,
preparticipation screening of Prosthetic aortic valve abscess, of tissue mitral prosthesis, 1100f 1903 1069t 1039f
athletes, 295 1109f–1110f, 1111f 3D visualization, 1094–1095 Pulmonary artery hemodynamics, diagnosis of causes of, 1037f
remote areas and developing Prosthetic dehiscence, 1108f–1109f 2D visualization, 1111t 1269–1273 echocardiography role in, 1075f severe, 1037f
countries, imaging in, 295 “Prosthetic pitch,” 1047 echocardiographic evaluation of, diastolic pulmonary artery pressure, ESC guidelines for, 1067 Pulmonary stenosis, 171, 1032–1036
traditional physical examination, Prosthetic regurgitation 1084t 1270–1272, 1272f diagnostic algorithm in, 1073–1074 acquired causes of, 1033f
292–293, 292f paravalvular aortic, 1105f effective orifice area of, 1084–1087 mean pulmonary artery pressure, Doppler echocardiographic indices congenital causes of, 1032f
training requirements, 295–296 paravalvular mitral, 1102f–1104f in elderly, 1948–1949, 1949f 1272, 1272f of, 1065t echocardiographic assessment of,
Poiseuille, Jean, 67 Prosthetic valve dysfunction, 1087–1092 endocarditis of, 1099–1100 pulmonary vascular resistance, hemodynamic definitions of, 1064t 1034–1036, 1034f, 1035f–1036f
Poiseuille’s law, 66, 67 endocarditis, 1088–1090 identification of thrombus, 1098 1272–1273 and HV Doppler, 311, 313, 313f continuous wave Doppler signal in,
Polycystic ovarian syndrome, in women, obstruction, 1090–1092 mitral, 1095 systolic pulmonary artery pressure, mitral stenosis and, 836–838 1034f
1899 patient prosthetic mismatch, overview, 1080, 1094 1270, 1270f, 1271f mitral valve echocardiographic enlarged systolic frame, 1036f
Positron emission tomography (PET), 1091–1092 paravalvular regurgitation, 1099 Pulmonary artery pressure (PAP), 1129 scoring system, 836 midtransesophageal view, 1036f
1429 role of 3D TEE in operating room in, prosthetic valve dysfunction, echocardiographic methods for, M-mode echo for, 7, 7f patient with tetralogy of fallout, 1035f
Posterior circulation, 668–669, 670f 605–617, 613f–624f 1087–1092 1266t–1267t nonconventional echocardiography, transpulmonary valve velocity, 1035f
Posterior displacement of outlet septum, regurgitation, 1087 endocarditis, 1088–1090, 1089f Pulmonary artery sling, 1696 1070–1073 etiology, 1032
1625 Prosthetic valve infective endocarditis, obstruction, 1090–1092 Pulmonary artery systolic pressure real time, 3d echocardiography, 1073 grades of, 1034
Posterior wall thickness (PWTd), 1118 1047 patient prosthetic mismatch, (PASP), 1034, 1129, 1130f, 1987t strain imaging, 1072–1073 normal pulmonary valve area in,
Postsystolic contraction (PSC), 367 abscess in patient with mechanical, 1091–1092 Pulmonary atresia, 1033, 1581f tissue Doppler imaging, 1070–1072 1034
Posttreadmill exercise echocardiography, 1047f regurgitation, 1087, 1088f Pulmonary capillary wedge pressure overview, 1063 pathology, 1032
8 dehiscence, 1047 Ross procedure, 1086f, 1090f (PCWP), 1064t, 1065t, 1984 RT 3DE of, 1170 pulmonary atresia, 1033
Power Doppler, 113, 114f “prosthetic pitch,” 1047 surgical mitral and aortic clocks, 1096 Pulmonary embolism (PE), 1067, 1985, RV systolic function in, impaired, secondary anatomic changes in, 1033
advantages and disadvantages of, 72 Prosthetic valves, 1015, 1021f–1022f, types of, 1080–1082 1986 1069t, 1070f types of, 1033
methodology, 71–72 1080–1093, 1092–1093, 1094–1112, bioprosthetic valves, 1081–1082, anticoagulation treatment, 1986 velocity vector imaging in, 399 Pulmonary thrombo disease, 1976
Power Doppler harmonic imaging 1948–1949 1081t, 1082t, 1088f color Doppler imaging, 1986 Wilkins scoring system, 836–837 arotic dissection, 1976f
(PDHI), 421 aortic position, failed homograft in, mechanical valves, 1082, 1083t consolidated lung tissue, 1986 in women, 1901–1092 dilatation, 1976
Power modulation, 420 1087f true biological valves, 1080–1081 associated mechanical alterations, severe, 1903f echocardiographic evaluation, 1976
Power pulse inversion, 421 aortic prosthetic valve stenosis, 1100 valved canduit, 1085f–1086f 1986 Pulmonary interstitial edema, 1984, 1985 Echogenic structure, 1977f
PPM. See Patient prosthetic mismatch aortic valve and ascending aorta, 1096 Proximal isovelocity surface area (PISA) disgnosis, 1985 diagnosis, 1984 false lumen, 1977f
(PPM) assessment of, 1082–1087 method, 82, 785, 992 localization, 1985 American College of Cardiology post diagnosis, 1976
Pregnancy cardiac catheterization, 1092–1093 for AR severity, 938–939 localized multiple B-lines, 1986 guidelines, 1984 pulmonary hypertension, 1977f
echocardiography in, 1902–1904 cardiac magnetic resonance imaging, in mitral regurgitation, 887–888 lung ultrasound scan B-lines, 1984 pulmonary thrombo-embolic, 1976
effect of, on HV Doppler, 303 1093, 1093f MR severity and, 870–871 accuracy, 1985 chest X-ray, 1984 pulmonary thromboembolism, 1976,
Pre-left ventricular assist device cinefluoroscopy, 1092 Proximal transverse aortic arch, pulse echocardiography, 1986 chronic heart failure (HF), 1984 1976f
echocardiogram, 1236t clinical data for, 1083t Doppler across, 1536f leg vein compression sonography, gray zone, 1984 right ventricle/left ventricle end-
Premature ventricular contractions, and computed tomography scan, 1092, Pseudoaneurysms 1986 limitations, 1984 diastolic dimension, 1976
HV Doppler, 309, 309f 1092f in aortic annulus, 1046f pleuritic chest pain, 1985 pulmonary capillary wedge pressure, true lumen, 1977f
Primary benign cardiac tumors, 1464– 3D transesophageal aortic valve, 1046f pulmonary arterial flow area, 1986 1984 ventricular dysfunction, 1976
1484 echocardiography, 1100–1107 femoral, 696–699, 698f sonographic findings, 1985 prognosis, 1985 Pulmonary valve, 1031–1041
I-XXXII Comprehensive Textbook of Echocardiography Index I-XXXIII
in adults, 1816–1818 anatomy, 1963 flow pattern, 1964 pulmonary vein stenosis and, 1678 left ventricle mass, 722, 722f Real time three-dimensional
cardiac catheterization, 1817 delineating, 1963 atrial reversal, 1964 pulmonary venous confluence, left ventricle shape analysis, 722–723, echocardiography (RT3DE)
MRI/CTA for, 1817 anatomy of, 325 diastolic dysfunction, 1964 1673–1675 723f future perspectives of, 1728
postoperative adult, 1818 anomalies of, 1672–1673 diastolic forward flow, 1964 septum primum malposition defect fluid mechanics algorithms and left ventricular ejection fraction,
surgical treatment for, 1817–1818 abnormal number of pulmonary mitral regurgitation, 1964 and, 1676–1678, 1677f particle imaging, 720–721, 721f 1722–1723
cardiac CT scans, 1040 veins, 1672 systolic forward flow, 1964 Pulmonary vein stenosis future outlook, 728–729, 729f analysis in children, 1723, 1724t
cardiac MRI, 1040 anomalous pulmonary venous A Flutter, 1963 and PV Doppler, 342, 342f global strain, 712–714, 712f–715f meta-analysis, 1723
catheterization, 1040 return, 1672–1673 imaging, 1963 with TAPVC, 1678 goal of, 706 for left ventricular mass, 1723
congenital diseases in newborns, blood flow in imaging of, 325–329 Pulmonary venous confluence (PVC), heart chamber segmentation left ventricular volumes, 1722–1723
incidence of, 1032t aging and, 329–330, 330f technical considerations in, 327, 329, 1673–1675, 1674f algorithms, 706–707, 707f in adults with congenital heart
echocardiographic evaluation, factors affecting, 329–331 329t orientation and site of drainage of, limitations of speckle tracking disease, 1722
1037–1038 loading conditions and, 330–331 transesophageal echocardiography, 1674–1675 echocardiography, 720 analysis in children, 1723, 1724t
effective regurgitant orifice area, 1038 physiology of, 325–327, 326, 328f 327 Pulmonary venous flow, MR severity and, mitral valve assessment, 725–727, correlation with CMR, 1722
epidemiology, 1031–1032 respiration and, 329 transthoracic echocardiography, 327 869 726f–728f meta-analysis, 1723
parasternal short-axis view of, 1034f cardiac MR, 1963 isolation, 1963 Pulmonic valve parametric display, 708–711, overview, 1721–1722
postpulmonary valve surgery, color contrast in, 1552f left inferior, 1963 3D echo of, 522–523, 522f, 523f 709f–711f regional wall motion and synchrony,
1039–1040 color Doppler of, 1533f left upper, 1963f ventricular assist devices and, 1232 regional strain, 714–715, 715f, 716f 1726–1727
allograft replacement, 1040 CT angiography, 1963 limitations and technical pitfalls, 342, Pulmonic valve disease, 3DE assessment right ventricular quantification, right ventricular ejection fraction,
pulmonary regurgitation, 1036, 1038f disease states, and PV blood flow 342f of, 284, 284f, 285f 723–725, 723f–726f 1723–1725
causes of, 1037f atrial fibrillation, 333, 333f localization, 1964 Pulsed Doppler, 63–64. See also Spectral rotation, twist, and torsion, 717–720, analysis in children, 1724–1725
severe, 1037f atrial flutter, 333, 333f lower, 1963f Doppler 719f meta-analysis of, 1725
pulmonary stenosis, 1032–1036 atrial septal defect (ASD), 342, 342f macro-reentrant tachycardia, 1963 Pulsed wave Doppler segmental/regional analysis of left right ventricular volumes, 1723–1725
acquired causes of, 1033f atrioventricular dissociation, midesophageal, 1963 advantages and disadvantages of, 69 ventricle, 707–708, 708f, 709f analysis in children, 1724–1725
congenital causes of, 1032f 331–332, 332f level, 1963f and aliasing, 70, 70f strain quantification and speckle meta-analysis of, 1725
echocardiographic assessment of, cardiac tamponade, 341 probe, 1963 methodology, 69–70, 69f tracking algorithms, 711–712 single ventricular ejection fraction,
1034–1036, 1034f, 1035f–1036f conduction disorders, 331 morphological features, 1963 Pulse inversion, 420 three-dimensional speckle tracking 1725–1726
etiology, 1032 constrictive pericarditis, 340–341, normal flow pattern of, 1670–1672 Pulse pressure (PP), 467 and strain, 715, 717, 717f–719f single ventricular mass, 1725–1726
grades of, 1034 340f, 341f ostium, 1964 Pulse repetition frequency (PRF), 63, 69, Quantitative gated single-photon single ventricular volumes, 1725–
normal pulmonary valve area in, junctional rhythm, 333, 334f peak diastolic velocity, 1965f 736 emission computed tomography 1726
1034 left ventricular end-diastolic pulmonary hypertension, 1964 Pulse wave velocity (PWV) analysis, (QGSPECT), 271 strain analysis by, 1727–1728
pathology, 1032 pressure, assessment of, pulse Doppler of, 1533f 466–467, 466f, 467f Receiver, 60
pulmonary atresia, 1033 334–335, 334f, 335f right middle, 1965f PVR. See Pulmonary vascular resistance Red blood cells (RBCs), 416
secondary anatomic changes in, 1033 mean left atrial pressure, assessment to right-sided left atrium, 1546f (PVR)
R Reflection artifacts, 62, 62f
types of, 1033 of, 335, 335f right upper, 1963f PWTd. See Posterior wall thickness RABT (red away blue toward), 64 Regional strain, 714–715, 715f, 716f
pulse Doppler across, 1536f mitral regurgitation, 336–338, 337f, caval view, 1964 (PWTd) Radial strain, 362, 363f, 389 Regional wall motion and synchrony,
Ross procedure, 1038–1039 338f spectral Doppler of, 325–345 Pyramidal imaging, 241 Range ambiguity, 736. See also Artifacts RT3DE of, 1726–1727
artificial conduits in, 1039 mitral stenosis, 338–339, 339f artifacts, 343–345, 344f–345f PZT (lead zirconate titanate) ceramics, RAP. See Right atrial pressure (RAP) Region of interest, 71
pulmonary homograft, 1038, 1039f myocardial relaxation pattern, differential diagnosis for abnormal 102, 103f Rashkind procedure, 531 Registered Cardiac Sonographer (RCS),
right ventricular outflow tract, 1038 impaired, 335–336 PV flow patterns, 345, 345f RBBB. See Right bundle branch block 754
Pulmonary valve fibroelastoma, 1478f paced rhythm, 334, 334f limitations and technical pitfalls, Registered Cardiovascular Invasive
Pulmonary vascular resistance (PVR), premature ventricular contractions, 342–343, 344f
Q (RBBB)
RCM. See Restrictive cardiomyopathy Specialist (RCIS), 754
1063, 1065t, 1066, 1272–1273 332, 332f stenosis, 1965f QLab, 385,514 (RCM) Registered Diagnostic Cardiac
Pulmonary vein ablation prolonged PR interval, 331, 331f development, 1964 Quadrature detector, 66 Reactive hyperemic index (RHI), 467–468 Sonographer (RDCS), 754
cardiac computed tomography for, pseudonormal filling pattern, 336 systemic venous anomalies, 1678– Quadricuspid aortic valve, 1620, 1620f Reactive oxygen species (ROS), 454 Registered Vascular Specialist (RVS), 754
2054–2057 pulmonary vein stenosis, 342, 342f 1684 in female, 1915f Real time, three-dimensional TEE Registered Vascular Technologist (RVT),
computed tomography for, 2054– rate and rhythm disorders, 332–334 systolic forward flow, 1964 Quantification techniques in (RT3DTEE) probe, 101–102, 101f, 754
2057 restrictive cardiomyopathy, 339–340, thoracic aorta,descending, 1963 echocardiography, noninvasive, 102f Regurgitant jet, spectral strength of, 811t
Pulmonary vein isolation, for atrial 340f total anomalous pulmonary 705–729 Real time 3D echo (RT3DE), 1142–1143 Regurgitant volume (RV), 1376
fibrillation, 566, 568–569, 569f, restrictive filling pattern, 336, 337f venous connection, 1673–1678, aortic valve assessment, 727–728, Real time (RT) 3D mode, 269 Reid, John, 6
570f short PR interval, 331, 331f 1673f, 1674f–1675f 728f Real time (RT) 3D TTE, 74 Remote ventricular septal defect, 1648
Pulmonary veins (PV), 1670–1684, 1963 sinus bradycardia, 332, 333f anomalous drainage of, 1676 3DE quantification tools, clinical Real time/live 3D echocardiography, 241 Renal disease, 1870–1872
ablative therapies, 1963 sinus tachycardia, 332, 332f diagnosis steps in, 1673–1676 applications of, 721–723 Real time myocardial perfusion Reperfusion, speckle-tracking
abnormalities, 1963 flaring degree, 1964 echocardiographic goals, 1673 left ventricle global and regional echocardiography (RTMPE), echocardiography, 1302
additional ablation, 1963 floating thrombus, 1965f features of, 1673 function, 722 444–445 Resolution, 58, 655
I-XXXIV Comprehensive Textbook of Echocardiography Index I-XXXV
axial, 59, 59f Right atrial pressure (RAP), 1063, 1065t, disc summation method, 1169f Right ventricular ejection fraction RV fractional area change (RV FAC), 1139 software-driven scanners, 1991
frame rate, 59, 60f 1143–1144, 1264–1269 of pulmonary hypertension, 1170 (RVEF), 381, 1137–1138 RVFS. See RVOT fractional shortening symmetrical effect, 1992
lateral, 59, 59f Doppler and tissue Doppler imaging, second-generation, 1167–1168 global function of RV, 1166 (RVFS) tissue characterization conference,
line density, 60 1268–1269, 1269f transverse shortening and, 1166 Right ventricular end-diastolic pressure RV index of myocardial performance 1992
Respiration, effect of echocardiographic methods for, Right ventricle, evaluation of, 1134–1148 (RVEDP), 309–310, 310f (RIMP), 1141 transesophageal echocardiography
on hepatic venous flow, 302–303, 1265t “apex-forming,” 1136 Right ventricular end-diastolic volume RVOT. See Right ventricular outflow tract live/real time three/four-
303f, 306, 307f estimation of, 1066t cardiac magnetic resonance imaging, (RVEDV), 1168 (RVOT) dimensional, 1992, 1992f
on pulmonary venous flow, 329 IVC parameters, 1264–1266, 1266f 1136f, 1144–1145, 1144f Right ventricular endomyocardial biopsy, RVOT fractional shortening (RVFS), 1138 ultrasound system, 1991
Resting myocardial perfusion size and collapsibility, 1266 cardiovascular computed 571 RV regional isovolumic relaxation time SCD. See Sudden cardiac death (SCD)
abnormality, 2044f RA dimensions, 1269, 1269f tomography, 1145 Right ventricular fibroma, 1481f (RVrIVRT), 1268 Sclerosing mediastinitis, 1515f–1517f
Restrictive cardiomyopathy (RCM), 395, systemic venous flow, 1268, 1268f contractile function, 1140–1141 Right ventricular function, TD RVrIVRT. See RV regional isovolumic Screen, 60
1369, 1397–1404 Right atrial thrombus, 1504f Doppler echocardiography, 1139– assessment of, 354, 356f–357f relaxation time (RVrIVRT) Secondary cardiac tumors, 1492–1500
amyloid cardiomyopathy, 1398–1401, Right atrium–right ventricle (RA–RV), 1141 Right ventricular hemangioma, 1486f RVSV. See Right ventricular stroke volume carcinoid heart disease, 1500
1399f–1400f 1958 continuous wave Doppler spectral Right ventricular lipoma, 1483f (RVSV) malignant melanoma, 1500
contrast echocardiography, 1402 Right atrium thrombus, 1505f display, 1139 Right ventricular myxomas, 1471f–1472f, Sector angle, and frame rate, 59, 60f
Doppler echocardiography, 1398 Right bundle branch block (RBBB), 1726 1473–1474, 1473f Sector probe, 58, 58f
endomyocardial fibrosis, 1402–1404, Right coronary artery fistula, 1782–1783
conventional doppler, 1139–1140
Right ventricular noncompaction,
S Secundum ASD, 1734–1742
pulsed wave Doppler spectral
1404f Right heart failure, and cardiac motion, display, 1140, 1142f 1389f–1391f Sacrococcygeal teratoma, 1530 device embolization in, 1739
Fabry disease, 1401–1402, 1401f 1204 tissue doppler, 1140–1141, 1141f isolated, 1392f Saline contrast, 12–13 3D TEE in, 1738–1739, 1739f,
and HV Doppler, 315, 315f Right isomerism, 1577 Right ventricular outflow tract (RVOT), Saline contrast chocardiography, 435, 1740f–1742f
hypereosinophilic cardiomyopathy, Right parasternal approach, of three- 1135, 1138 436f repair of, 1734–1736
M-mode, 1136–1138
1402 dimensional echocardiography, flow velocity envelope, 1068f SAM. See Systolic anterior motion (SAM) Semilunar valves, short axis of, 1536f
three-dimensional, 1142–1143
and PV Doppler, 339–340, 340f 246, 248, 259f–266f outcomes of allograft conduits for, Sapien valve, balloon expandable, Septal aneurysms, 182
transesophageal, 1143 541–543
transesophageal echocardiography, Right pulmonary artery (RPA), short axis 1039 Septal wall thickness at diastole (SWTd),
two-dimensional, 1138–1139, 1139f, Sarcoidosis, 1405, 1405f, 1876–1879
1402 of, 1536f pulmonary hypertension and, 1118
1140f, 1141f Sarcomas, 1484–1488, 2054
transthoracic echocardiography, Right-sided infective endocarditis, 1066–1067, 1067f Septic cardiomyopathy, 1405–1407, 1406f
echo windows for, 1137f right ventricular, 1487f
1402 1047–1049 Ross procedure and, 1038 echocardiographic features of,
gadolinium contrast in, 1145 SAVR. See Surgical aortic valve
Retroaortic innominate vein, 1682, 1682f transesophageal approach, 1048f Right ventricular quantification, 723–725, 11406–407
geometry of, 1135f replacement (SAVR)
Retrograde aortic arch flow, 1551f type of patient with, 1048–1049 723f–726f Septum (ventricular), 1194–1198, 1205f
hemodynamics, 1143–1144 Scan line, 77
Retrograde ductal arch (DuAr), 1554f Right upper pulmonary vein (RUPV), 327, Right ventricular sarcoma, 1487f echocardiographic pattern of, 1196f
HV systolic filling fraction, 1144 Scanners, trends, 1991–1993
Retroperitoneal bleeding, 696 328f Right ventricular stroke volume (RVSV), endocardial and epicardial fibers,
right atrial pressure, 1143–1144 acoustic structure quantification,
Reverberation artifacts, 62, 62f, 734–735, Right ventricle, 1198 1167 1195f
morphology, 1135–1136 liver, 1992, 1992f
735f. See also Artifacts cardiac motion and, 1198 Right ventricular systolic dysfunction, fiber orientation of, 1195f
regional RV wall motion, 1136 advantage, 1992
transesophageal echocardiography double outlet, 1547f 313, 314f “septal line,” 1194
right ventricular outflow tract, 1135 B-mode signal
and, 107, 109–110, 109f evaluation of, 1235t Right ventricular thrombus, 1506f analysis, 1992 ultrasonic crystal tracings, 1197f
Reynolds, Osborne, 67 failure, 1247f RV systolic function, 1135–1136 Right ventricular wall (RVW), 1971t Septum primum malposition defect
myofibrillar arrangement of, 1136– cable-free transducer systems, 1992
Reynold’s number, 66, 67 ventricular assist devices and, RIMP. See RV index of myocardial development of, 1992 TAPVC and, 1676–1678, 1677f
Rhabdomyosarcomas, 1486–1488 1233–1234 1137 performance (RIMP) Serial evaluation, of patients with 3DE,
capacitance micro-machined
Rheumatic heart disease (RHD), 1859 Right ventricle, 3D quantitation of, overview, 1134–1135 Ring dehiscence, 605–606 1164–1165
ultrasound transducer, 1992
ARF and, 774 1165–1170 evaluation of, 1134–1148 Rotatable transesophageal echo (TEE) Shah, Pravin, 11
complete cross-sectional display
chronic, 775 anatomic considerations, 1165–1166 real time 3D echo, 1142–1143 probe, 100, 100f, 101f computed tomography, 1993 Shear rate, 452
morphological features of, 777t conventional approaches, 1165–1166 Simpson’s method of discs for, 1140f Rotation (cardiac motion), 1183 magnetic resonance, advantages of, Shear stress, 452–454, 452f–454f, 455. See
tricuspid stenosis and, 1006f 2D echocardiography, 1166f “triangle of dysplasia,” 1135, 1135f RSOV. See Ruptured sinus of Valsalva 1993 also Endothelial dysfunction
WHF echocardiographic criteria for, 3D reconstruction approaches, two-dimensional strain (speckle (RSOV) contrast-enhanced ultrasound, 1991, Short PR interval
775, 776t previous, 1167 tracking), 1141–1142, 1142f RT3DE. See Real time 3D echo (RT3DE) 1992 and HV Doppler, 307, 308f
Rheumatic mitral regurgitation, 863 global function, 1166 volume/body surface area, 1143t Ruptured sinus of Valsalva (RSOV), 1632 crystal piezoelectric materials, 1991 and PV Doppler, 331, 331f
Rheumatic mitral stenosis, 533–537, longitudinal contraction, 1166 Right ventricle fractional area change RVEDV. See Right ventricular end- electrostatic loudspeaker, 1992 Short-tau-inversion-recovery (STIR),
537f–539f retrosternal location of, 1166 (RVFAC), 1071f diastolic volume (RVEDV) fusion imaging, 1993f 2020
in female, 1890f–1891f, 1893f–1984f RT 3DE approach to, 1167–1170 Right ventricular diastolic dysfunction, RVEF. See Right ventricular ejection Lead zirconate titrate, 1992 Short-term circulatory support devices,
Rheumatic valvular heart disease, 898 apical rotation method, 1168f 1644f fraction (RVEF) miniaturization, 1991 1226–1227
Rheumatoid arthritis (RA), 1868 automatic boundary tracking and HV Doppler, 313–315, 315f RVFAC. See Right ventricle fractional area scanner user interfaces, 1993 Abiomed AB5000, 1227
Right aortic arch, 1690–1691 algorithm, 1169f Right ventricular dysfunction change (RVFAC) semiconductors, 1992 Impella catheter-based assist device,
Right atrial lipoma, 1096f of congenital heart disease, 1170 ICM/NICM and, 1422 RV FAC. See RV fractional area change smartphone ultrasound system, 1991, 1227
Right atrial myxomas, 1470f, 1473 data acquisition, 1167f mild residual, 1242f (RV FAC) 1991f TandemHeart system, 1227
I-XXXVI Comprehensive Textbook of Echocardiography Index I-XXXVII
Thoratec CentriMag system, 1227 muscular, 1804 Sonar system, development of, 4 high frame rate, 89 limitations of, 1326 Strain
Thoratec paracorporeal ventricular postoperative adult, 1805 Sonic reflector, good, properties of, 65 imaging mode, 90, 90f LV function and, 1324f calculation of, 362
assist device, 1227 supracristal, 1803 SonoHeart, 292f lateral gain, 89–90 mechanism of, 1325 imaging, 360
Shunt lesions “venturi effect,” 1803f Sonovue, 417, 418t. See also Contrast raw data format, 90 overview, 1323 normal, 385, 389
aortopulmonary window, 1602–1603 Shunt lesions/septal defects, 1733–1747 echocardiography scan range, 89 protocol, 1324 normal rotation and torsion values,
atrial septal defects, 1585–1591 atrial septal defects, 1734 Sound velocity, effect of, 107, 107f, 107t, two-dimensional frequency, 90 results/observations, 1324–1325 364t
features of, 1582–1585 atrioventricular septal defectsts, 108t Speckle-tracking echocardiography vs. dobutamine stress normal values for, in normal adults,
Gerbode defect, 1603, 1603f, 1604f 1746–1747 Sound waves, 55–56, 56f (STE), 360–376, 382, 1302–1304. echocardiography, 1325, 1326t 364t
ventricular septal defect, 1591–1599 secundum ASD, 1734–1742 SPAMM. See Spatial modulation of See also Velocity vector imaging wall motion abnormalities, 1323 peak, 365
Shunt lesions, in adults, 1798–1813 sinus venosus ASD, 1742–1743 magnetization (SPAMM) (VVI) left ventricular, 1324–1325 physics of, 385, 389, 389f
atrial septal defects, 1799–1802, 1801t unroofed coronary sinus, 1743 SPAP. See Systolic pulmonary arterial cardiac muscular anatomy, 360–361, rate, 362
mechanism of, 1325
cardiac catheterization, 1802 ventricular septal defects, 1743–1746 pressure (SPAP); Systolic 361f shear, 385, 389
St. Jude aortic valve, 1085f
closure of, 1802 Sickle cell disease, three-dimensional pulmonary artery pressure (SPAP) image acquisition and processing, types of, 362, 362t, 363f
St. Jude bileaflet mitral valve, 1092f
contrast echocardiography, 1801– speckle tracking and, 376t SPARC. See Stroke Prevention: 367 Streptococcus viridans, 1054
Stanfard classification, 1974
1802 Side lobe artifacts, 62–63, 63f, 736. See Assessment of Risk in a limitations of, 374– 375 Stress (Takotsubo) cardiomyopathy
apical, 1976t
echocardiography, 1799 also Artifacts Community (SPARC) study myocardial infarction, 1302 three-dimensional speckle tracking
ascending aorta, 1974
exercise testing, 1802 transesophageal echocardiography Spark gap position-locating approach, 14 myocardial ischemia, 1302 and, 376t
and, 107, 108f diastolic collapse, 1976
MRI/CTA for, 1802 Spatial and temporal resolution, 3D myocardial viability, 1302–1303 dissections, 1974 Stress echocardiography, 226–227,
postoperative adult in, 1802 Sigmoid septum, of elderly, 167 imaging and, 80, 80f reperfusion, 1302 1306–1322
Single element transducers, 76, 76f esophagus, 1976
transesophageal echocardiography, Spatial modulation of magnetization strain and, 362–365, 362t, 363f cardiac event rate as function of,
Single-photon emission computed left parasternal, 1976t
1800–1801, 1800f (SPAMM), 2000 three-dimensional, 372–373, 373f, 1315f
tomography (SPECT), 1425–1427 lumen, 1976
types of, 1799–1800, 1799f Spatial resolution, 55 374f cost-effectiveness of, 1317–1318
Single plane transesophageal echo (TEE) mitral valve, 1975f
atrioventricular septal defect, Spatiotemporal image correlation (STIC), area strain measurement and, 373, Doppler hemodynamics with,
probe, 99, 99f pressure Half-time, 1975t
1809–1810 285–286, 1550 375, 375f, 375t 1318–1319
Single ventricle, velocity vector imaging systolic expansion, 1976
echocardiography, 1809–1810 Speckle tracking acquisition, 87–98 clinical applications of, 373–374, 376t aortic valve disease, 1318
in, 392–394 transesophageal probe, 1976
postoperative adult, 1810 arrhythmias and, 98 and tissue Doppler imaging, 360, 361t dynamic pulmonary hypertension,
Sinotubular (ST) junction, 168, 1621 transthoracic views, 1976t
coronary artery fistula, 1812–1813 2D speckle tracking, limitation of, 92 two-dimensional, 365–367, 365t, 366t 1318–1319, 1319f
Sinus bradycardia visualization, 1974
patent ductus arteriosus, 1805–1809, gain setting, 97 cardiac transplantation and, 371 hypertrophic cardiomyopathy, 1318
and HV Doppler, 306, 307f Stanford type A aortic dissection, 935,
1808t M-mode, 87–88, 88f cardiomyopathies and, 369–371 latent diastolic dysfunction, 1318
and PV Doppler, 332, 333f 936f
cardiac catheterization, 1808 multiview monitoring during live chemotherapy cardiotoxicity and, mitral valve disease, 1318
Sinus of Valsalva aneurysm, 1630–1632, Staphylococcus aureus, 1048
closure of, 1809 acquisition, 97, 97f 371–372 fundamentals of, 1306–1307
1631f, 1784 STARFlex occluder, 557
echocardiography for, 1805–1807 multiview orientation, 97, 98f clinical application of, 367–372, 368t future directions for, 1319
in adults, 1811–1812 Starr–Edward valve, 1085f
with left-to-right shunt, 1807 patient breath-hold, 98 congenital heart disease and, 372 impact on patient outcome, 1317
aortic regurgitation and, 1631 State Food and Drug Administration
MRI/CTA, 1809 R–R interval, 91 coronary artery disease and, 367 interpretation of, 1309–1319
associated anomalies, 1631–1632 (SFDA),China, 110
postoperative adult, 1809 standardization, 91–92 CRT for heart failure and, 369 diagnostic accuracy to detect CAD,
echocardiographic evaluation of, State-of-the-art, 1180–1181
transesophageal echocardiography, 1630–1631 standard views, 91 right ventricular function and, 372 1310, 1313t
1808 three-dimensional acquisition, 92, rotational dynamics, role of, 372, 372f composite of, 1181–1183 transient ischemic LV cavity
hypoplastic left heart syndrome, 1784
patent foramen ovale, 1798–1799 94f–95f valvular heart diseases and, 371 HVMB model, 1181 dilatation, 1315
right ventricular outflow obstruction
persistent left superior vena cava, frequency, 95 SPECT. See Single-photon emission muscle contraction, asynchronous, “warranty time,” 1317
and, 1784
1810–1811 modes, 96, 97f computed tomography (SPECT) 1181 myocardial infarction vs. cardiac
ruptured right, 1784f–1785f
echocardiography, 1810 one-beat acquisition, 96 Spectral broadening, 68 Statins, 450 death by, prediction of,
VSD and, 1631
MRI/CTA, 1810–1811 raw data storage, 95 Spectral Doppler, 66, 112–113, 113f Steady-state free precession (SSFP), 2020 1311–1315
Sinus tachycardia
postoperative adult, 1811 scan range/volume width, 96 transthoracic echocardiogram, for aortic regurgitation, 2010 myocardial perfusion and, 1319
and HV Doppler, 306, 307f
sinus of Valsalva aneurysm, 1811– and PV Doppler, 332, 332f triggered full volume, 96, 97f 136–137, 137f Stenosis of pulmonary outflow, 1647– overview, 1306
1812 Sinus venosus ASD (SVASD), 1586, volume rate, 96 “Splenic syndromes,” 1704 1648 predictors of risk, 1314t
ventricular septal defects, 1802–1805, 1742–1743, 1743f wall motion tracking, 92, 95 Squatting stress echocardiography, aortic outflow obstruction, 1647 prognostic value of, 1316t
1806t Sinus venous defect, 1800 versus tissue doppler imaging, 92 1323–1327, 1324–1326 remote ventricular septal defect, 1648 risk stratification and prognosis,
cardiac catheterization, 1805 Sjogren’s syndrome, 1545 two-dimensional speckle tracking, acute LV remodeling on, 1325 ventricular septal defect, restriction 1310–1315, 1314f
closure of, 1805 SLE. See Systemic lupus erythematosus 88, 88f advantages of, 1326 of, 1647f, 1648 left atrial size in, role of, 1317
echocardiography, 1804–1805 (SLE) acquisition considerations, 88 chronic obstructive pulmonary Stenotic aortic valve, morphology, myocardial wall motion abnormality,
inlet, 1804 Slicing methods, 79 depth, 89 disease, 1323 1619–1620 1311
locations of, 1804f Smearing, 59 dynamic range, 90 coronary artery disease, 1323 STIC. See Spatiotemporal image RV wall motion abnormalities in, role
membranous, 1803 Society of Cardiovascular frame rate, 88–89, 89f electrocardiography (ECG), 1323 correlation (STIC) of, 1315
MRI/CTA for, 1805 Anesthesiologists (SCA), 638 gain level, 89, 89f end-systolic frames in, 1324f Stiffness index (SI), 465, 467 safety of, 1308–1309
I-XXXVIII Comprehensive Textbook of Echocardiography Index I-XXXIX
three dimensional, 1319 membranous subaortic stenosis, Swirling, 433, 434f total anomalous systemic venous postoperative adult, 1835 multibeat acquisition in, 74–75, 75f
training in, 757 1625 Swiss cheese defects, 1594 drainage, 1684 postoperative adult, surgery in, 1835 in operating room, 577–634
types of, 1307–1309 posterior displacement of outlet SWTd. See Septal wall thickness at venous valves, 1683–1684 stress echocardiography, 1832–1834 aortic valve disease, 582–588
contrast echocardiography, 1308 septum in, 1625 diastole (SWTd) Systolic abnormalities, of tricuspid valve, aortic regurgitation in, 1641, 1641f cardiac masses, 617–627
dobutamine stress echocardiography, Subvalvular infundibular stenosis, 1033, Systemic diseases, 1867–1885 986 cardiac catheterization, indications limitations of 3D TEE and future
1307, 1308f 1035f, 1036f amyloidosis, 1872–1874, 1873f–1874f Systolic anterior motion (SAM), 1348 for, 1641 directions, 628–629
exercise echocardiography, 1307, Sudden cardiac death (SCD), 1348 carcinoid tumors, 1874–1875 echocardiographic evaluation of, computed tomography for, 2062, mitral valve disease, 577–582
1308f Summed difference score (SDS), 1158 Chagas disease, 1875–1876, 1351–1352, 1352f 2063f native valve endocarditis, 597–604
vasodilator stress echocardiography, vs. WMS difference, 1159f, 1159t 1877f–1878f mitral-septal contact, 1359f echocardiographic measurements in, prosthetic valve dysfunction, 605–617
1307–1308 Summing, 77 echocardiography in, 1867–1885 of mitral valve, 1350–1354, 1350f, 1640–1641, 1640f tricuspid valve disease, 589–590,
vs. nuclear SPECT imaging, 1313t Superior vena cava (SVC), 1140 hypereosinophilic syndrome, 1359f Hoffman's variant, 1635f, 1636f 593–597
Stress testing, three-dimensional anomalies of, 1678–1682 1868–1869, 1870 pathophysiology of, 1353 postoperative evaluation of, 1641– quantification, 81–85
echocardiography for, 274, 274f absent right, 1680 nutritional deficiency, 1880 Systolic blood pressure (BP), 1644, 1642f left ventricular, 82–85, 84f
Stroke distance (SD), 1280, 1281f aneurysm of, 1682 overview, 1867 pseudonormalization of, 911 with pulmonary stenosis, 1633 mitral valve, 81–82, 82f, 83f
Stroke Prevention: Assessment of Risk in clinical significance of, 1680–1681 renal disease, 1870–1872 Systolic dysfunction, and HCM, 1356– velocity vector imaging in, 391–392 rendering in, 78, 79f
a Community (SPARC) study, 1921 defect in wall of coronary sinus, rheumatoid arthritis, 1868 1358 Thebesian valve, 1503 2D tomographic slices, 79–80
Stroke volume (SV), 1280, 1281f 1679–1680 sarcoidosis, 1876–1879 Systolic dyssynchrony index (SDI), 373, Thermal index (TI), 111 surface rendering, 79
in DCM, 1375 left superior vena cava to coronary systemic lupus erythematosus, 1726 Thoracic aortic aneurysm (TAA), volume rendering, 78–79, 79f
in dilated cardiomyopathy, 1375 sinus, 1679–1680 1867–1868 Systolic function 1934–1937 right parasternal approach, 246, 248,
Structural heart disease, in women, left superior vena cava to left atrium, systemic sclerosis, 1869–1870 in dilated cardiomyopathy, 1371 in Marfan syndrome, 1936 259f–266f
1888–1889 thyroid disorders, 1879–1880 natural history of, factors impacts,
1680 in ICM/NICM, 1419 strengths of, 81
Systemic hypertension, in female, 1910f 1937
mitral valve calcification, 1889 persistent left, 1678–1679 Systolic myocardial dysfunction, 1131f subcostal approach, 244, 256f–255f
Systemic lupus erythematosus (SLE), Thoracic cage artifacts, 433–434, 434f
mitral valve prolapse, 1888, right superior vena cava to left Systolic pulmonary artery pressure supraclavicular approach, 244,
1545, 1867–1868 Thoratec CentriMag system, 1127
1888f–1889f atrium, 1681, 1681f (SPAP), 1063, 1065t, 1066, 1067, 258f–259f
in female, 1912f Thoratec HeartMate II, 1223f
mitral valve stenosis, 1888–1889 heterotaxy syndrome and, 1705 1270, 1270f, 1271f suprasternal approach, 244, 257f
Systemic pulmonary shunts (QP/QS), Thoratec paracorporeal ventricular assist
Subaortic stenosis, 1770 normal spectral Doppler of, 304, 304f technology related to, 240–241,
1280 device, 1227
in adults, 1824–1825 Supraclavicular approach, of three- 240f–241f
Subcostal approach, of three- dimensional echocardiography,
Systemic sclerosis, 1869–1870 T Three-dimensional echocardiographic
transducer technology and, 76
Systemic veins, anomalies of, 1678–1684 guidance of percutaneous
dimensional echocardiography, 244, 258f–259f TAA. See Thoracic aortic aneurysm (TAA) 3D matrix array transducers, 76–77,
classification of, 1678 procedures, 531–571. See
244, 256f–255f Suprasternal approach, of three- Tachycardia-induced cardiomyopathy, 77f–78f
coronary sinus, abnormalities of, also Catheter-based
Subcostal window, TTE dimensional echocardiography, 1388, 1395f linear/phase array transducers, 76,
1683 transcutaneous interventional
color and spectral Doppler imaging, 244, 257f Takotsubo cardiomyopathy, 1388, 1394f procedures 76f
decompressive venous channels,
158–159 Supravalvular aortic stenosis, 1626–1628, in women, 1899–1900 Three-dimensional echocardiography single element transducers, 76, 76f
1684
four-chamber view, 155, 156f, 156t, 1626f vector velocity imaging, 1900 (3DE), 14–18, 240–267, 705 for valvular heart disease, 515–528
inferior vena cava, abnormalities of,
157f in adults, 1825–1826 1682–1683 TandemHeart device, 1225f, 1227, advantages/disadvantages of, 262, aortic valve, 520–522, 520f
great vessel imaging, 157–158, 158f, aortic valve anomalies, 1627 bilateral, 1683 1250–1251 267 case examples of, 525–528
159f branch pulmonary arteries in, 1628, inferior vena cava interruption, TAPSE. See Tricuspid annular plane apical approach, 244, 254f, 255f data acquisition, 515–516, 516f, 517f
abdominal aorta, 158, 159f 1628f 1682–1683 systolic excursion (TAPSE) artifacts in, 736–737. See also image optimization, 516
hepatic vein, 158, 158f coronary artery abnormalities, 1628 inferior vena cava to left atrium, 1683 in peripartum cardiomyopathy, 1381 Artifacts mitral valve, 516–520, 517f, 518f
inferior vena cava, 158, 158f morphology, 1627 retroaortic innominate vein, 1682, TAPVC. See Total anomalous pulmonary basics of, 74–85 pulmonic valve, 522–523, 522f, 523f
short-axis views, 157, 157f Supravalvular stenosis, 1033 1682f venous connection (TAPVC) beam forming, 77–78 tricuspid valve, 523–525, 524f
Subendocardial muscle, 1198–1200, Supraventricular tachycardia (SVT), 1542, superior vena cava, 1678–1682 TAPVR. See Total anomalous pulmonary color Doppler imaging, 248, 254–255, Three-dimensional epiaortic
1199f, 1200f 1957 absent right, 1680 venous return (TAPVR) 266f ultrasonography, 641
Subvalvular aortic stenosis, 1624–1626, Surface rendering, 79, 79f aneurysm of, 1682 Taussig–Bing anomaly, 1647 evolution of, 74–75, 75f Three-dimensional intracardiac
1624f Surgical aortic valve replacement (SAVR), clinical significance of, 1680–1681 Tei's index, 1120, 1120f, 1419 examination protocol, 241–244, echocardiography, 652, 653f
aortic insufficiency and, 1626 540–541 defect in wall of coronary sinus, Temporal tap, 674, 675f 242f–243f Three-dimensional matrix array
cause of, 1624 in aortic stenosis, 926–927 1679–1680 Tenting area, 1422, 1423f image quality, limitations in transducers, 76–77
diffuse tunnel obstruction and, 1626 in LFLG-AS with low ejection left superior vena cava to coronary Tetralogy of fallot (TOF), 1763 aperture, 80 Three-dimensional (3D) speckle tracking,
discrete subvalvular, 1626 fraction, 927 sinus, 1679–1680 with absent pulmonary valve, 1637, artifacts, 80–81 92, 94f–95f
echocardiographic evaluation of, PLFLG-AS and, 922 left superior vena cava to left atrium, 1637f gating, 80 frequency, 95
1624 in PLFLG-AS with normal ejection 1680 in adults, 1829–1835 spatial and temporal resolution, 80, modes, 96, 97f
fibromuscular collar in, 1625 fraction, 927 persistent left, 1678–1679 cardiac catheterization, 1834–1835 80f for morphological study, 94f
fixed subaortic obstruction, 1624– Suture-less valves, 606 right superior vena cava to left echocardiography, 1830–1832 left parasternal approach, 244, one-beat acquisition, 96
1625 SVC. See Superior vena cava (SVC) atrium, 1681, 1681f MRI/CT for, 1835 245f–254f raw data storage, 95
I-XL Comprehensive Textbook of Echocardiography Index I-XLI
scan range/volume width, 96 real time (RT) 3D, 508 aortic stenosis, 279 longitudinal shortening, 1188 training requirements, 751t acoustic energy safety, 111
triggered full volume, 96, 97f simultaneous biplane mode, 509 aortic valve assessment, 278f, 279, structural reasons for, 1184f transesophageal echocardiography electric safety, 110–111
volume rate, 96 zoom view, 508 279f and untwisting, 1200 and, 755, 756t electromagnetic compatibility, 112
wall motion tracking, 92, 95, 97f imaging protocol for, 510t contractile reserve, 276 Total anomalous pulmonary venous Transcatheter aortic valve implantation heating safety, 111
Three-dimensional speckle tracking and indications for, 508t left and right atria, 276–277, 277f connection (TAPVC), 1577, 1672, (TAVI). See Transcatheter aortic mechanical safety, 111–112
strain, 715, 717, 717f–719f procedure for left ventricle twist, 276 1673–1678, 1673f, 1674f–1675f. See valve replacement (TAVR) three-chamber view, 60f
Three-dimensional speckle tracking image acquisition, 508–509 right ventricle, 277–278 also Pulmonary veins Transcatheter aortic valve replacement training in, 755, 756t
echocardiography (3DSTE), imaging sequence, 509–510 valvular assessment, 278, 278f anomalous drainage of, 1676 (TAVR), 512–513, 540–546 Transesophageal echocardiography,
372–373, 373f, 374f, 1727–1728, preprocedural planning, 508 reproducibility, 272–273 diagnosis steps in, 1673–1676 AVA calculation, 543 tricuspid valve
1727f RT3D TEE imaging, advances in, 3D speckle-tracking applications, echocardiographic goals, 1673 CTA for, 2058f in inferior myocardial infarction,
area strain measurement and, 373, 511–512 275, 275f features of, 1673 CT and, 2029t, 2057–2059 1014f
375, 375f, 375T technology for, 507 3D stress echocardiography, 274–275, infracardiac, 1674 in elderly, 1935f three-dimensional, 988–990
clinical applications of, 373–374, 376t uses of, 512 274f pulmonary vein stenosis and, 1678 intra- and postprocedural monitoring two-dimensional, 988, 988f, 989f–990f
use of, 404–405, 405f catheter-based LAA closure, 512 global LV function, evaluation of, 276 pulmonary venous confluence, by 2D/3D TEE, 543, 546, 546f bicaval view, 988f
Three-dimensional stress congenital heart disease, 513 LV mass, 273–274, 273f 1673–1675 patient seletion for, 543 longitudinal plane examination, 989f
echocardiography, 1328–1336 LAA clot, detection of, 512 methods of validation, 275–276 septum primum malposition defect TAVR valves, 541–543, 543f mid-esophageal four-chamber view,
advantages of, 1329–1330, 1330t left ventricular function and tricuspid valve disease, 283–284, 283f, and, 1676–1678, 1677f three-dimensional speckle tracking and, 988f
contraction front mapping in, role of, dyssynchrony assessment, 514 284f Total anomalous pulmonary venous 376t normal tricuspid valve anatomy,
1334–1335 mitral prosthesis paravalvular leak Three-dimensional TTE, 159–161, 161f return (TAPVR), 1743 Transducer probe, 60 989f–990f
contrast in, 1335 closure, 512 Thyroid disorders, 1879–1880 Toxic cardiomyopathies, 1396–1397 Transducers, 58 Transjugular intrahepatic portosystemic
current standards vs., 1331–1334 mitral stenosis and balloon T2* (star) imaging, 2020 adriamycin-induced Transducer technology, 102, 103f shunt (TIPS), 319
adenosine stress test, 1333–1334 valvotomy, 513–514 Time–acoustic intensity curve, 442 cardiomyopathy, 1397f Transesophageal echocardiography Transmitral diastolic inflow, 1126f
dipyridamole stress test, 1334 mitral valve assessment, 513 Time gain compensation (TGC), 61 alcohol-induced cardiomyopathy, (TEE), 13, 99–116, 487 Transmitter, 60
dobutamine stress test, 1331–1333 transcatheter aortic valve Tissue Doppler (TD) imaging, 14, 64, 72, 1397 acoustic shadowing, 61, 62f Transposition of great arteries (TGA),
treadmill exercise stress test, 1333 replacement, 512–513 349–357, 360, 382 chemotherapy-induced artifacts 1653–1663, 1754–1763
future directions, 1335 trans-septal puncture, 512 in atrial fibrillation, 353 cardiomyopathy, 1396–1397 reverberation, 107, 109–110, 109f anatomy, 1653–1654, 1653f
image acquisition in, 1330–1331 Three-dimensional transthoracic color TD imaging, 349 Training, in echocardiography, 750–751, side lobes, 107, 108f associated defects, 1657–1661
overview, 1328 echocardiography examination, development of, 350 759f, 760f sound velocity, effect of, 107, 107f, coronary arteries, 1661
parametric imaging in, 1334 268–286 diastolic function, assessment of, appropriate use criteria, 758 107t, 108t fixed anatomical obstruction,
postacquisition analysis, 1331 aortic annulus, 280 352–353, 355f, 356f cardiac sonographers, training of, of coronary arteries, 1338–1340 1660–1661
stress protocol in, 1331 aortic regurgitation, 280 for LV dyssynchrony, 353, 354f 753–754 current/future technologies, 112–116 inlet VSD, 1659
three-dimensional transducers, 1329, data acquisition, methods for LV filling pressures, estimation of, certification and maintenance of and artifacts, 113–116, 114f left ventricular outflow obstruction,
1329f cropping, 269–270 352–353 proficiency, 758, 759f, 760f CMUT technique, 115f, 116, 116f 1659–1661
vs. 2DSE, in wall visualization, 1334 3DE color flow Doppler imaging, 269 myocardial disease and, 350–352 contrast echocardiography and, 757 color Doppler, 113, 113f, 114f septal defects, 1657–1659
Three-dimensional transducers, 1329 image display, 269–270 and prognosis, 354–355 CT and MRI, training in, 755 power Doppler, 113, 114f VSD, 1658–1659
Three-dimensional transesophageal multiplane mode, 268–269 RV function, assessment of, 354, duration and sites, 755 spectral Doppler, 112–113, 113f associated lesions, 1653
echocardiography (3D TEE), 18, multiple-beat 3DE imaging, 269 356f–357f fellowship training, 751–752, 752t image quality computed tomography for, 2059–
507–514 real time 3DE, 269 spectral TD imaging, 349 content of, 754–755 aperture and, 104, 106f 2062, 2061f
aortic valve display in short-axis view tomographic slices, 270, 270f and technical considerations, level 1 training, 751 focus and, 106, 106f coronary artery patterns in, 1661–
by, 511f–512f left ventricular assessment 349–350, 350f level 2 training, 751 frequency and, 104–105, 105f 1663
biplane (X-plane) image of left atrial image acquisition methods, 270, 270f transthoracic echocardiogram, 137 level 3 training, 752 kinds of intramural coronary, 1662–1663
appendage, 509f LV regional function, 271–272 uses of, 350–357 intraoperative TEE and, 756–757 dual plane probe, 99–100, 100f inverted origin of coronaries, 1662
image display recommendations, normal values, 272 Tissue plasminogen activator (tPA), 1996 intravascular and intracardiac matrix array probe, 101–102, 101f single left coronary artery, 1662,
511, 511t volume and systolic function Tissue velocity imaging. See Tissue ultrasound and, 757 miniaturization technology, 102–103, 1662f–1663f
image optimization, 510 assessment, 271 Doppler noncardiologists, training of, 752–753 103f, 104f single right coronary artery, 1661
colors, 510 mitral regurgitation, 282–283, 282f, TomTec Cardiac Performance Analysis, in pediatric echocardiography and processing technology, 103, 104f, 105f echocardiographic evaluation,
compression, 510 283f 380 congenital heart disease, 753 real time, three-dimensional TEE, 1654–1656
cropping and rotation, 510 mitral stenosis, 280–282, 281f Torsion (cardiac motion) special echocardiographic 102 chamber size, 1655–1656, 1656f
gain, 510 mitral valve assessment, 280, 281f with co-contraction of base and helix, procedures and, 755 rotary plane probe, 100, 100f, 101f two-dimensional, 1654–1655, 1654f,
smoothing, 510 pediatric and fetal cardiac 1190f stress echocardiography and, 757 single plane probe, 99, 99f 1655f
imaging modalities in, 509t pathologies and, 285–286 compression, 1188 three- and four-dimensional TTE and transducer technology, 102, 103f Transseptal cardiac catheterization, 233
full volume, 509 pulmonic valve disease, 284, 284f, definitions, 1183–1184 TEE and, 757 variable plane probe, 100, 100f Transseptal puncture, 512, 532–533, 534f
full-volume and live 3D color flow, 285f during ejection, 1191f tissue Doppler and speckle tracking pulmonary veins, imaging of, 327 Transthoracic echocardiography (TTE),
509 regional LV function, 276 left ventricle, 1186–1188, 1186f echocardiography, 757–758 safety considerations, 110 132–162
I-XLII Comprehensive Textbook of Echocardiography Index I-XLIII
apical window, 146, 148 Transthoracic examination, 164–186 Treadmill exercise stress test, 1333 torrential, 996f pulsed wave Doppler across, 1533f Tricuspid valve prosthesis
five-chamber plane, 153–154, 153f, apical views Treadmill stress echocardiography transesophageal echocardiography, three leaflets of, 991f bovine pericardial, 1022f
154f, 154t color and spectral Doppler, 176–177, equipment and set-up for, 1308f 1000f–1002f transesophageal examination normal functioning porcine, 1021f
four-chamber plane, 148–153, 176f hyperdynamic response to stress, before and after annuloplasty, 1001f three-dimensional, 988–990 Tricuspid valve vegetation
148f–153f, 149t five-chamber view, 178–179, 178f, 1310f coronary sinus, 1000f two-dimensional, 988, 988f, 989f–990f 2d transthoracic echocardiography,
long-axis or three-chamber plane, 179f in ischemia, 1311f two jets of, 1001f transthoracic examination 1016f, 1018f–1019f
155, 156f four-chamber view, 175, 175f–178f vs. coronary angiography, 1312t Tricuspid regurgitation velocity (TRV), three-dimensional, 988–990 in intravenous drug abuser
two-chamber plane, 154–155, 154f, left ventricle, 175 “Triangle of dysplasia,” 1135, 1135f 1063, 1066, 1066f, 1067 two-dimensional, 986–988, 987f adult female, 1017f–1018f
155f long-axis view, 180, 181f, 182 Tricuspid annular plane systolic Tricuspid stenosis, 812–813, 1004–1007 ventricular assist devices and, 1232, adult male, 1020f–1021f
beginning of, 132, 135 LV and atrium quantitation, 180, 180f, excursion (TAPSE), 177, 178f, carcinoid heart disease and, 1006 1233f transesophageal echocardiography
cardiac structures assessed in, 181f 381–382, 1069t diet drug-induced valvulopathy, 1006 three-dimensional, 1019f
Tricuspid valve annulus, 1474f
133t–134t mitral and tricuspid valves, 175–176 longitudinal contraction of RV, 1166 in elderly female with rheumatic two-dimensional, 1017f–1018f, 1019f
Tricuspid valve diseases
apical window, 133t–134t two-chamber view, 179, 179f right ventricular ejection fraction heart disease, 1006f Trivial lesions, pressure gradients across,
anatomy of tricuspid valve, 812
parasternal window, 133t variations on four-chamber view, 176 and, 1137–1138, 1138f grading scales, 1004t 1574
3DE assessment of, 283–284, 283f,
of coronary arteries, 1337–1338 parasternal long-axis view, 166–171, RV systolic function and, 1137–1138 hemodynamically significant, True biological valves, 1080–1081
284f
four-chamber view, 60f 167f Tricuspid atresia, 1700–1701 findings, 1004t True lumen (TL), 1977f
tricuspid regurgitation, 813–816
imaging modalities aortic valve, aortic root and classification of, 1701t and HV Doppler, 311, 312f Truncus arteriosus, 1650–1653,
color flow Doppler, 137 ascending aorta, 167–168, 168f color flow imaging, 814
type I, 1700 Loeffler’s syndrome, 1006–1007 continuous wave Doppler, 815, 815f 1650f–1652f
M-mode echocardiography, 136, 136f calcification, 170 type II, 1701 severity of, 813 in adults, 1842–1844
spectral Doppler, 136–137, 137f chamber size and function, 167, 168f 3D echocardiography, 816
Tricuspid regurgitation (TR), 813–816, transthoracic echocardiography cardiac catheterization, 1844
tissue Doppler imaging, 137 Doppler imaging, 170 flow convergence method, 814–815
990–1004 three-dimensional, 1006f echocardiography, 1843
two-dimensional echocardiography, extracardiac structures, 170 measurement of VC, 814
cardiac MRI for, 2013, 2014f two-dimensional, 1005f–1006f MRI/CT for, 1844
135–136, 135f mitral valve, 168–169, 169f pulmonary artery pressure, 815
color flow imaging, 814 Tricuspid valve (TV), 589–590, 1233 postoperative adult, 1844
imaging windows and planes, 135 M-mode measurements, 169–170, pulsed wave Doppler, 815
continuous wave Doppler, 815, 815f in adults surgery for, 1844
left ventricle and wall motion 169f, 170f role of 3D TEE in operating room in,
2d transthoracic echocardiography, cardiac catheterization, 1815 classification of, 1650–1652
determination, 161–162, 162f pulmonary artery long axis, 171 589–590, 593–597, 601f–608f
992f, 993f–996f, 1002f Ebstein’s anomaly, 1813–1814, 1815t Doppler imaging, 1653
parasternal window right ventricular inflow view, 171, RV dimensions and function,
effect of Nyquist limit, 997f echocardiography, 1814 echocardiography in, 1651f–1652f,
linear measurements, 139–141, 171f 815–816
echocardiography in MRI/CT for, 1814–1815 1652
139f–141f parasternal short axis, 172–175 severity of, 816, 816t
three-dimensional, 816 postoperative adult, 1815–1816 two-dimensional, 1653
parasternal long-axis plane, 137–139, aortic valve, 172, 172f transesophageal echocardiography
transesophageal, 816 stress echocardiography, 1814 infant with, 1582f
138f, 139f, 139t atria, 172–173 in, 816
two-dimensional, 814 tricuspid valve surgery, 1815 TRV. See Tricuspid regurgitation velocity
parasternal short-axis plane, 144–146, left ventricle, 174–175, 174f two-dimensional echocardiography,
flow convergence method, 814–815 anatomy of, 984, 989f–990f (TRV)
145f–147f, 145t mitral valve, 174, 174f 814
hepatic veins in, 999f congenital anomalies of, 1022,1616– Trypanosoma cruzi, 1875
right parasternal long axis view, right ventricular outflow tract, tricuspid stenosis, 812–813
and HV Doppler, 310–311, 311f, 312f 1618 TTTS. See Twin–twin transfusion
141–142 173–174, 173f, 174f severity of, 813
measurement of VC, 814 congenital lesions of, 1616t syndrome (TTTS)
right ventricle inflow view, 142, 142f, set-up and patient positioning, 164, Tricuspid valve endocarditis, 1008–1015,
142t, 143f pacemaker associated, 1212–1214 congenitally unguarded tricuspid TUPLE maneuver, 551, 553f
166 1058f
right ventricular outflow tract view, standard recommended protocol, lead infection associated with, orifice, 1617–1618, 1617f Turbulence, detection of, 66
1114–1115 Ebstein’s anomaly of, 1616–1617, in adult female, 1017f Twin reverse arterial perfusion (TRAP),
142–144, 143f, 143t, 144f 165t–166t MRSA positive, 1020f
patient positioning for, 135 subcostal views time course for, 1213 1616f 1530
transthoracic echocardiography for, tricuspid valve prolapse, 1617 Tricuspid valve fibroelastoma, 1023f, Twin–twin transfusion syndrome (TTTS),
pulmonary veins, imaging of, 327, abdominal aorta and inferior vena
1213–1214, 1213f 3DE assessment of, 278, 278f 1477f, 1479f 1529–1530
328f cava, 183, 183f, 184f
parasternal approach for, 999f–1000f 3D echo of, 523–525, 524f Tricuspid valve myxoma, 1465f Twisting (cardiac motion), 1183
subcostal window four-chamber view, 182, 182f
PISA method for, 992 and dilated cardiomyopathy, 1376 Tricuspid valve prolapse, 1007–1029, 1617 Two-dimensional echocardiography
color and spectral Doppler imaging, short-axis views, 182, 183f
pulmonary artery pressure, 815 dilated cardiomyopathy and, 1376 2d transesophageal (2DE), 8–9, 705
158–159 suprasternal views, 184–185, 185f,
pulmonary hypertension, systemic flail, 1007–1029 echocardiography, 1010f–1011f transthoracic echocardiogram,
four-chamber view, 155, 156f, 156t, 186f
157f level, 995f M-mode echocardiography, 984–986 in elderly male with dyspnea, 1011f 135–136, 135f
Transvalvular pressure gradients, 1622
great vessel imaging, 157–158, 158f, Transvalvular regurgitation, 1106 pulsed wave Doppler, 815 Ebstein’s anomaly, 986f myxomatous degeneration, 1011f weaknesses of, 81
159f Transverse aortic arch, 1537f rheumatic, 1002f functional events, demonstrating, prosthetic valves and, 1015, Two-dimensional (2D) scanning concept,
short-axis views, 157, 157f Transverse strain, 362, 363f right heart failure and, 1204 984f 1021f–1022f 4f
suprasternal notch window, 159, 160f TRAP. See Twin reverse arterial perfusion RV dimensions and function, septal leaflet, identification, 985f transthoracic echocardiography Two-dimensional (2D) speckle tracking,
three-dimensional, 159–161, 161f (TRAP) 815–816 systolic abnormalities, 986f three-dimensional, 1011f 88, 88f
of tricuspid valve Traumatic tricuspid papillary muscle severity, echocardiography criteria, using contrast injections, two-dimensional, 1009f–1010f acquisition considerations, 88
three-dimensional, 988–990 2d transthoracic echocardiography, 993t identification, 985f TV endocarditis, 1008–1015 depth, 89
two-dimensional, 986–988, 987f 1012f, 1013f severity of, 816, 816t pacer and, 1215f TV tumors, 1015 dynamic range, 90
I-XLIV Comprehensive Textbook of Echocardiography Index I-XLV
frame rate, 88–89, 89f left atrial appendage, 481f problems, 1996 bilateral ostial vertebral artery Valvular heart disease, cardiac MRI, future directions
frequency, 90 left ventricular outflow tract view and therapeutic applications, 1996 stenosis, 501f–502f 2009–2013 LV twist, evaluation of, 406
gain level, 89, 89f aortic valve, 483f diagnostic ultrasound, 1996 carotid body paraganglioma, aortic regurgitation, 2010–2011, 2011f MRI tagging versus MRI velocity
high frame rate, 89 lower esophageal four-chamber view, tissue plasminogen activator, 1996 detection of, 500f aortic stenosis, 2010, 2011f vector imaging, 405–406
imaging mode, 90, 90f 484f Ultrasound contrast agents (UCAs), Doppler signal of vessels on mitral regurgitation, 2012–2013 three-dimensional STE, 404–405, 405f
lateral gain, 89–90 mid-esophagus four-chamber view at 416–417, 417f, 441. See also transpharyngeal ultrasound, 488t mitral stenosis, 2013 physics of strain and, 385, 389
raw data format, 90 0°, 481f Contrast echocardiography internal carotid artery stent, tricuspid regurgitation, 2013, 2014f and reproducibility and correlation
scan range, 89 mitral valve view at 0° and 90°, 483f commercially available, 417, 418t detection of, 499f Valvular heart disease, 3D echo in, between vendors, 401–404
Two-dimensional speckle tracking mitral valve view at 45° and 135°, 483f idle, properties of, 417 left- and right-sided carotid arteries, 515–528 Vena contracta area (VCA), 278
echocardiography (2D STE), pulmonic valve and right ventricular safety of, 434–435, 435t 494f aortic valve, 520–522, 520f MR severity and, 869–870
365–367, 365f, 366f outflow tract, 484f ultrasound and, interaction between, left carotid artery stent, detection of, case examples of, 525–528 Vena contracta technique, 82
clinical application of, 367, 368t pulsed wave Doppler interrogation of 418–419, 419f 498f data acquisition, 515–516, 516f, 517f Vena contracta width, MR and, 868–869
cardiac transplantation, 371 LAA, 482f left carotid bulb and internal carotid image optimization, 516 Ventricular arterial connection,
use of, 428–431
cardiomyopathies, 369–371 pulsed wave Doppler interrogation of artery stenosis, 496f–497f mitral valve, 516–520, 517f, 518f identification of, 1579–1582
Ultrasound frequency, and Doppler
chemotherapy cardiotoxicity, pulmonary vein, 482f left internal mammary artery, 493f concordant, 1580–1581
signal, 66 pulmonic valve, 522–523, 522f, 523f
371–372 short-axis view of aortic valve, 483f left-sided carotid arteries, 489f–490f
Ultrasound-guided compression therapy tricuspid valve, 523–525, 524f spatial relationship, 1582
congenital heart disease, 372 transgastric long-axis view of left left subclavian artery, 491f–492f
of pseudoaneurysms, 699, 700f Valvular heart diseases, 2D STE and, 371 Ventricular assist devices, 1222–1254
coronary artery disease, 367 ventricle, 485f left subclavian artery stenosis and
Ultrasound-guided thrombin injection, Valvular regurgitation, 278 apical thrombi, 1230f
CRT for heart failure, 369 transgastric short-axis view of left steal syndrome, 502f–503f
699 Valvular stenosis, 1033 cardiac structure and function,
right ventricular function, 372 ventricle, 485f left vertebral artery, 490f–491f
Ultrasound image, 87 “Valvuloarterial impedance” (Zva), 1934 changes in, 1234–1240
rotational dynamics, role of, 372, 372f transgastric short-axis view of right left vertebral artery origin stenosis,
Ultrasound stethoscope, 291. See also Valvuloarterial impedance formula, 911 clinical uses of, 1224–1226
valvular heart diseases, 371 ventricle, 485f 501f
Point-of-care diagnosis Variable plane probe, 100, 100f dilated cardiomyopathy
Two-dimensional stress Two-dimensional tomographic slices, pan-diastolic backflow in aortic arch
Umbilical artery, pulse Doppler across, branches and neck vessels, Vasa vasorum, 450 baseline study, 1230f
echocardiography, 1328–1329 79–80 Vascular rings, 1691 with small secundum atrial septal
1539f 504f–505f
vs. 2DSE, in wall visualization, 1334 Two-dimensional transthoracic Vasoconstrictors, 451 defect, 1232f
Umbilical cord vessels, 1539f right-sided carotid arteries, 495f, 496f
Two-dimensional subcostal echocardiography Vasodilators, 451 echocardiographic evaluation of,
Umbilical vein, pulse Doppler across, right subclavian artery, 494f
echocardiography, abdominal subcostal approach Vasodilator stress echocardiography, 1222–1254
1539f
aorta, 222f abdominal aorta, 222f–223f 1307–1308 explantation, 1249–1250
Unicuspid aortic valve, 1619
Two-dimensional (2D) TEE examination, Type B arch interruption, 1694
Univentricular atrioventricular
V Velocity vector imaging (VVI), 365, 365f, implantation by device strategy,
480–485 Type II tricuspid atresia, 1701 380–406 1226t
connections, 1697–1700 Valsalva aneurysm, sinus of, 1630–1632
esophageal intubation, 481 Type I tricuspid atresia, 1700 analysis, 400 left ventricular over-filling, evidence
Univentricular heart, in adults, 1845–1848 Valsalva maneuver, 1126–1127
informed consent for, 480 of, 1240–1246
cardiac catheterization, 1847–1848 Valve perforation, 1045, 1046f for cardiac resynchronization therapy
patient selection for, 480
preparation and conscious sedation,
U echocardiography, 1846–1847 bicuspid aortic, 1046f response, 400–401 levels of severity, 1225t
transesophageal, 1847 native mitral, 1046f, 1056f dyssynchrony in pediatric and list of, 1229t
480 UCAs. See Ultrasound contrast agents overview, 1222–1224
MRI /CTA for, 1847 Valvular aortic stenosis, 1618–1624 congenital heart disease and, 401
procedure, 481–485, 481f–485f (UCAs) percutaneous continuous flow
tricupsid atresia and post-fontan aortic root, 1621 application of
bicaval view, 482f Uhl’s anomaly, 1618, 1618f
adult, 1845, 1846f aortic valve annulus, 1621 amyloidosis, 399 devices, 1250–1252
color Doppler interrogation of aortic Ultraharmonics, 421
Univentricular heart post-fontan aortic valve area, 1622 cardiomyopathy, 394–396 Impella device, 1250, 1251f
valve in long axis, 483f Ultrasound
tricuspid atresia, 1848t associated anomalies in, 1624 congenital heart disease, 391–394 TandemHeart, 1250–1251
color Doppler interrogation of aortic artifacts, 61–63
Unroofed coronary sinus, 1743 critical neonatal aortic stenosis, congenitally corrected transposition postsurgical evaluation, immediate,
valve in short axis, 484f basics of, 55–64
Unscrolled myocardial band model, 1182f 1622–1623 of great arteries, 392 1234
color Doppler interrogation of atrial definition of, 4
Untwisting (cardiac motion), 1183 hemodynamics, 1623 coronary artery disease, 396–398 preoperative echocardiographic
septum, 482f highly focused ultrasound, 1996
during elongation, 1193 severity of, 1621–1622 D transposition of great arteries, 392 evaluation, 1229–1234
color Doppler interrogation of LAA, history of, 4
mitral valve opening and, 1200–1201 sinotubular junction, 1621 exercise and, 400 aorta, 1232
482f imaging by, 57–60
during postejection isovolumic phase stenotic aortic valve, morphology, fetal cardiac function, 390–391 aortic valve, 1231, 1231f
color Doppler interrogation of mitral A-mode, 57, 58f
mirrors torsion, 1189 1619–1620 heart transplantation, 399–400, 399f atrial septum, 1231–1232
valve, 483f B-mode, 57, 58f
prominent left-sided vectors in, transvalvular pressure gradients, Kawasaki disease (KD), 398 inferior vena cava, 1233
color Doppler interrogation of M-mode, 58, 58f
1191–1193 1622 myocarditis and, 398–399 left atrium, 1231
tricuspid valve, 484f resolution, 58–60, 60f
torsion and, 1184–1185, 1200 vs. hypoplastic left ventricle, 1623 in patients with diabetes, 398 left ventricle, 1229–1230
continuous wave Doppler transducers and probes, 58, 58f
Upper transesophageal and Valvular disease, in adults, 1813–1826 pulmonary artery hypertension, 399 mitral valve, 1231
interrogation of tricuspid valve, 2D ultrasound, 58, 58f
transpharyngeal examination, aortic valve, 1821–1826 single ventricle, 392–394 pericardium, 1231
484f low-frequency ultrasound, 1996
487–506 mitral valve, 1818–1820 systemic right ventricle and, 392 pulmonic valve, 1232
descending aorta (DA) imaged at 0° microbubbles, 1996
arch vessels, identifications of, 488f pulmonary valve, 1816–1818 tetralogy of fallot, 391–392 right ventricle, 1233–1234
and 90°, 485f nanodroplets, 1996
tricuspid valve, 1813–1816 cardiac anatomy and, 380–381 tricuspid valve, 1232, 1233f
I-XLVI Comprehensive Textbook of Echocardiography
reverse remodeling, 1226 pressure gradient across, 1597–1598 ventricular ectopy, 1966
septal contour preleft, 1230f Qp/Qs ratios in, 1598 common conditions, 1966t
Thoratec HeartMate II, 1223f regurgitation and stenosis, 1598 Ventriculoarterial discordance, 1580f
types of, 1226–1229 restriction of, 1647f, 1648 Vertebral arteries, assessment of,
long-term axial flow devices, 1227– stepwise evaluation for, 1591t 691–693, 694f
1228 suitability for device closure, 1598 Vijaya's echo criteria, 771, 771t
long-term third generation TGA and, 1658 Visualization ,of received ultrasound
centrifugal flow systems, 1228–1229 transesophageal echocardiography energy, 56, 56f
short-term circulatory support, in, 1599 Volume rendering, of 3DE data set, 78–79,
1226–1227 79f
Ventricular septal defect closure, ICE
ventricular size and function, V-ScanTM, 292, 292f
imaging during, 650, 651f
1234–1240 VSD. See Ventricular septal defect (VSD)
Ventricular septal defects, in adults,
diastolic performance of ventricle, VVI. See Velocity vector imaging (VVI)
1802–1805, 1806t
1238–1240
cardiac catheterization, 1805
inlet cannula, 1237, 1240f
left ventricle size and function, closure of, 1805 W
1234–1237 echocardiography, 1804–1805
inlet, 1804 Wall motion abnormalities (WMA)
motion of aortic valve, 1238
locations of, 1804f in DCM, 1371–1372
right ventricle size and function,
membranous, 1803 detection of, on echocardiogram, 226
1237f, 1238
MRI/CTA for, 1805 in dilated cardiomyopathy, 1371–
Ventricular deptal defect, 1581f
muscular, 1804 1372
Ventricular morphology,
echocardiography of, 1579 postoperative adult, 1805 ischemic, 1291–1292, 1292f–1293f
Ventricular myxomas, 1468–1470 supracristal, 1803 squatting stress echocardiography in
Ventricular papillary muscle rupture, “venturi effect,” 1803f left ventricular, 1324–1325
right Ventricular septal dropout, 1543f mechanism of, 1325
in female with dyspnea, 1014f–1015f Ventricular septal muscle rupture, Wall motion score index (WMSI), 1313f,
transesophageal echocardiogram, 1295–1297 1343
1013f–1014f postmyocardial infarction, 1296f Wall motion scores (WMS), 1158
Ventricular septal contour position, 1248 transesophageal echocardiogram, Wall visualization, 3DSE vs. 2DSE in, 1334
Ventricular septal defect (VSD), 1361, 1013f–1014f Watchman, 563
1543f, 1591–1599, 1743–1746, Ventricular systolic function, assessment Wavelength, 55
1802–1805 of, traditional echocardiographic Wide-angled display, 241
closure of, 557–559, 560f limitations in, 381–382, 381f Wilkins’s score, 536
direction of shunt, 1597 William's syndrome, 1628f
Ventricular tachycardia (VT), 1966
Doppler evaluation of, 1596–1597 WMA. See Wall motion abnormalities
apical thrombi, 1966
color Doppler, 1596–1597 (WMA)
coronary artery disease, 1966t
pulsed and continuous wave Doppler WMS. See Wall motion scores (WMS)
dilated cardiomyopathy, 1966t
examination, 1597 WMSa. See WMS difference (WMSa)
3D TEE for, 513 WMS difference (WMSa), 1158
midmuscular, 1544f hypertrophic cardiomyopathy, 1966t
left ventricle (LV), 1966 vs. summed difference score, 1159f,
M-mode echocardiography, 1598
noncompaction, 1966t 1159t
morphological location, 1591–1596
sarcoid heart disease, 1966t WMSI. See Wall motion score index
doubly committed subarterial
ventricular arrhythmias, 1966t (WMSI)
defects, 1594–1595, 1594f, 1596f
arrhythmogenic RV dysplasia World Health Organization–International
inlet ventricular septal defect,
(ARVD), 1966 Society of Hypertension (WHO-
1595–1596, 1595f
muscular ventricular septal defects, coronary artery disease, 1966 ISH), 294
1593–1594, 1593f, 1594f dilated cardiomyopathy, 1966
perimembranous, 1592–1593, 1592f, hypertrophic cardiomyopathy, 1966 Z
1595f, 1596f infiltrative diseases, 1966
objectives, 1591 substrates, 1966 Zoom mode, 269

Comprehensive Textbook of Echocardiography Volume 2 PDF

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Comprehensive Vol.

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Comprehensive Textbook of Echocardiography (Vol. 2)

Neeraj Awasthy FNB Aditya Bharadwaj MD Gerald Buckberg MD

Leon H Charney Michele D’ Alto MD PhD Daniel Forsha MD

Gopal Ghimire MD DM MRCP Donald Hagler MD Rachel Hughes-Doichev MD FASE

Martin G Keane MD FACC FAHA FASE Arthur J Labovitz MD Gerald R Marx MD

Dilbahar S Mohar MD Ryozo Omoto MD Eugenio Picano MD PhD

Sharath Subramanian MD George Thomas MD Isidre Vilacosta MD PhD FESCC

Section 1: History and Basics

2. Early Cardiac Flow Doppler Era: A Key for a New Clinical

4. Doppler Echocardiography—Methodology, Application and Pitfalls 65

7. Instrumentation for Transesophageal Echocardiography Including

Section 2: Echocardiography/Ultrasound Examination and Training

• Color M-Mode 120

9. The Complete Transthoracic Echocardiography 132

10. The Standard Transthoracic Examination: A Different Perspective 164

11. Nonstandard Echocardiographic Examination 188

12. Technique and Applications of Continuous Transthoracic Cardiac Imaging 224

13. The Basics of Performing Three-Dimensional Echocardiography 240

14. How to do Three-Dimensional Transthoracic Echocardiography Examination 268

15. Point-of-Care Diagnosis with Ultrasound Stethoscopy 291

16. Spectral Doppler of the Hepatic Veins 299

17. Spectral Doppler of the Pulmonary Veins 325

18. Tissue Doppler Imaging 349

19. Speckle Tracking Echocardiography: Clinical Usefulness 360

20. Echocardiographic Assessment of Global and Segmental

21. Contrast Echocardiography 416

22. Myocardial Perfusion Echocardiography 441

23. Endothelial Dysfunction 449

• Role of Acetylcholine 451

24. How to do a Two-Dimensional Transesophageal Examination 480

25. Upper Transesophageal and Transpharyngeal Examination 487

26. How to Perform a Three-Dimensional Transesophageal Echocardiogram 507

27. Three-Dimensional Examination to Evaluate Valvular Heart Disease:

• Case Study 8: S/P Balloon Aortic Valvuloplasty 527

28. Three-Dimensional Echocardiographic Guidance of Percutaneous Procedures 531

29. Three-Dimensional Echocardiography in the Operating Room 577

30. Epiaortic Ultrasonography 638

31. Intracardiac Echocardiography 643

32. Intravascular Ultrasound Imaging 655

33. Peripheral Vascular Ultrasound 663

34. Advanced Noninvasive Quantification Techniques in Echocardiography 705

35. Artifacts in Echocardiography 732

36. Echocardiography Training 750

Section 3: Valvular Heart Disease

• Aortic Stenosis 802

38. Echocardiographic Assessment of Mitral Valve Disease 826

39. Mitral Regurgitation 880

40. Aortic Stenosis 896

41. Low-Gradient, Severe Aortic Stenosis with Depressed and

42. Aortic Regurgitation 930

43. Echocardiographic Evaluation of Aortic Disease 945

44. Transesophageal Echocardiography in the Diagnosis of Aortic Disease 967

45. Echocardiographic Examination of the Tricuspid Valve 984

46. Echocardiographic Assessment of Pulmonary Valve 1031

Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders,

INTRODUCTION by using a comprehensive diastolic assessment—incor