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Eisenmenger Syndrome
Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD more...
Updated: Nov 23, 2014
Background
This radiograph reveals an enlarged right heart and pulmonary artery dilatation in a
24-year-old woman with an unrestricted patent ductus arteriosus (PDA) and Eisenmenger
syndrome.
Pulmonary hypertension
Intracardiac communication
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This computed tomography (CT) chest scan shows a large, unrestricted patent ductus
arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome.
Examples of congenital heart disease subtypes that may cause pulmonary vascular
disease and proceed to Eisenmenger syndrome include the following:
Increased pulmonary arterial flow - Atrial septal defect (ASD), systemic
arteriovenous fistulae, total anomalous pulmonary venous return
Increased pulmonary arterial pressure and flow - Large VSD, large PDA,
truncus arteriosus, single ventricle with unobstructed pulmonary blood flow
Elevated pulmonary venous pressure - Mitral stenosis, cor triatriatum,
obstructed pulmonary venous return
Epidemiology
Etiology
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remain complex.
Natural history
Failure to reduce pulmonary pressures in the first 2 years of life may result in the
failure of the normal regression of the intimal smooth muscle. This is followed by the
progressive changes described by Heath and Edwards.[9] The condition then
advances to irreversible pulmonary hypertension, defined as unresponsiveness to
inhalation of 100% oxygen or nitric oxide. This point usually correlates to a PVR of
more than 12 Woods units.
Clinically, patients gradually develop the following complications of advanced
pulmonary vascular disease:
Dyspnea upon exertion
Syncope
Chest pain
Stroke
Brain abscess
Cyanosis
Congestive heart failure
Dysrhythmia
Hyperviscosity complications
Pulmonary hemorrhage/hemoptysis
Endocarditis
The time frame for this process depends on the anatomic nature of the lesion and
whether conditions, such as trisomy 21 (Down syndrome), that are known to
accelerate the development of PVOD are present.[10] Without intervention, reversal
of flow may happen in early childhood or around puberty, and progression of
symptoms may lead to death by the second or third decade of life.[11, 12]
Interestingly, adult patients with Eisenmenger syndrome may have a better
prognosis compared with those with other causes of pulmonary hypertension.[13]
Causes
Large, uncorrected cardiac shunt or palliative, surgically created systemicto-pulmonary shunt for congenital heart disease
Large, nonrestrictive VSD
Nonrestrictive PDA
Atrioventricular septal defect, including a large ostium primum ASD without a
ventricular component
Aortopulmonary window
Palliative, surgically created systemic-to-pulmonary anastomosis for
treatment of congenital heart disease
Prognosis
Eisenmenger syndrome is uniformly fatal; however, some patients survive into the
sixth decade of life. The usual life expectancy of a patient with Eisenmenger
syndrome is 20-50 years if the syndrome is diagnosed promptly and treated with
vigilance. The onset of pulmonary hemorrhage is usually the hallmark of a rapid
progression of the disease.[14]
The complications of chronic cyanotic heart disease affect multiple organ systems,
including the hematologic, skeletal, renal, and neurologic systems, causing
significant morbidity and mortality.
The quality of life is poor in patients with Eisenmenger syndrome because exercise
tolerance is extremely limited (due to limited oxygen uptake resulting from an
inability to increase pulmonary blood flow) and complications are profound. Poor
prognosis is predicted by syncope, elevated-right sided pressures, and hypoxemia.
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Occurrence
The frequency of pulmonary hypertension and the subsequent development of
reversed shunting vary depending on the specific heart defect and operative
intervention. Such variations include the following:
Morbidity
Mortality
Patients with Eisenmenger syndrome usually do not survive beyond the second or
third decade. Long-term survival depends on the patients age at the onset of
pulmonary hypertension and the coexistence of additional adverse features, such as
Down syndrome. Survival predominantly depends on right ventricular function. The
mortality rate in pregnant patients with Eisenmenger syndrome is reported to be
approximately 50%, although it may be higher.
The investigators stated that almost none of the studies appropriately accounted for
immortal time bias and therefore overestimated patients survival chances by as
much as 20 years. When Diller and colleagues took immortal time bias into account,
they determined that the 10-year mortality rate among untreated patients
approached 30-40%. They also found no indication that the chances of survival for
these patients were better than they would have been in the 1970s, 1980s, or
1990s, although survival prospects were found to be better than for patients in the
1950s and 1960s.[17]
Patient Education
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Additional resources for patients with pulmonary hypertension can be found at the
Pulmonary Hypertension Association Web site.
Clinical Presentation
Author
Mikhael F El-Chami, MD Assistant Professor, Department of Cardiology, Division of Electrophysiology, Emory
University School of Medicine
Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha, American College
of Cardiology, Heart Rhythm Society
Disclosure: Received grant/research funds from Medtronic Inc for principle investigator.
Coauthor(s)
Charles D Searles, Jr, MD Assistant Professor of Medicine, Division of Cardiology, Emory University School of
Medicine; Consulting Staff, Division of Cardiology, Director of Stress Echo Laboratory, Grady Memorial Hospital
Charles D Searles, Jr, MD is a member of the following medical societies: American Heart Association, Sigma Xi
Disclosure: Nothing to disclose.
Chief Editor
Park W Willis IV, MD Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North
Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.
Acknowledgements
Stuart Berger, MD Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of
Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart
Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American
College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for
Cardiac Angiography and Interventions
Disclosure: Nothing to disclose
Brian M Cummings, MD Pediatric Critical Care; Director Pediatric Transport, Medical Director PALS,
MassGeneral Hospital for Children, Instructor in Pediatrics, Harvard Medical School
Brian M Cummings, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.
Elyse Foster, MD Director of Adult Echocardiography Laboratory and Adult Congenital Heart Disease Service,
Department of Internal Medicine, Division of Cardiology, Moffitt Hospital; Assistant Professor of Cardiology,
University of California, San Francisco, School of Medicine
Elyse Foster, MD is a member of the following medical societies American College of Cardiology, American
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Lisa A Hourigan, MBBS, FRACP Consulting Staff, Department of Cardiology, University of California, San
Francisco School of Medicine
Disclosure: Nothing to disclose.
Christopher Johnsrude, MD, MS Chief, Division of Pediatric Cardiology, University of Louisville School of
Medicine; Director, Congenital Heart Center, Kosair Children's Hospital
Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics
and American College of Cardiology
Disclosure: St Jude Medical Honoraria Speaking and teaching
John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of
Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics,
American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.
Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts
General Hospital; Associate Professor of Surgery, Harvard Medical School
Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American
Heart Association, and International Society for Heart and Lung Transplantation
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College
of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of
Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Nothing to disclose.
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