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MEDICAL MASTERCLASS
EDITOR-IN-CHIEF

JOHN D FIRTH DM FRCP


Consultant Physician and Nephrologist
Addenbrooke’s Hospital
Cambridge

CARDIOLOGY AND RESPIRATORY


MEDICINE
EDITORS

PAUL R ROBERTS MD FRCP


Consultant Cardiologist
Southampton General Hospital
Southampton

STEPHEN J FOWLER MD MRCP (UK )


Lecturer and Honorary Consultant in Respiratory Medicine
University of Manchester and Lancashire Teaching Hospitals NHS Trust
Royal Preston Hospital
Preston

Second Edition
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Disclaimer
Although every effort has been made to ensure that drug doses
and other information are presented accurately in this publication, the
ultimate responsibility rests with the prescribing physician. Neither the
publishers nor the authors can be held responsible for any consequences
arising from the use of information contained herein. Any product
mentioned in this publication should be used in accordance with the
prescribing information prepared by the manufacturers.

The information presented in this publication reflects the opinions of its


contributors and should not be taken to represent the policy and views of the
Royal College of Physicians of London, unless this is specifically stated.

Every effort has been made by the contributors to contact holders of


copyright to obtain permission to reproduce copyrighted material. However,
if any have been inadvertently overlooked, the publisher will be pleased to
make the necessary arrangements at the first opportunity.
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LIST OF CONTRIBUTORS

Dr P Bhatia MBBS MRCP(Ireland) Dr DKC Lee MRCP(UK)


Consultant Physician Respiratory and Specialist Registrar
Internal Medicine Department of Respiratory Medicine
Tameside General Hospital Papworth Hospital
Ashton-Under-Lyne Cambridge

Dr B Chandrasekaran MRCP(UK) Dr N Melikian BSc (Hons) MBBS MRCP(UK)


Clinical Research Fellow British Cardiac Society
The Royal Brompton Hospital John Parker Research Fellow
London Cardiology Department
King’s College Hospital
Dr PWX Foley MRCP(UK) London
Specialist Registrar in Cardiology
and Honorary Research Fellow Dr A Pawlowicz PhD FRCP
(University of Birmingham) Consultant Physician in General
Cardiology Department and Respiractory Medicine
Portsmouth Hospitals NHS Trust Department of Respiratory Medicine
St Mary’s Hospital The Queen Elizabeth Hospital
Portsmouth King’s Lynn

Dr SJ Fowler MD MRCP(UK) Dr PR Roberts MD FRCP


Lecturer and Honorary Consultant Consultant Cardiologist
in Respiratory Medicine Southampton General Hospital
University of Manchester and Lancashire Southampton
Teaching Hospitals NHS Trust
Royal Preston Hospital Dr R Sharma MRCP(UK)
Preston Consultant Cardiologist
Ealing Hospital NHS Trust
Dr PR Kalra MRCP(UK) London
Consultant Cardiologist
Cardiology Department
Portsmouth Hospitals NHS Trust
St Mary’s Hospital
Portsmouth

Dr S Kaul MRCP(UK)
Specialist Registrar in Respiratory
and Intensive Care Medicine
Department of Respiratory Medicine
King’s College Hospital
London
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© 2008, 2010 Royal College of Physicians of London

Published by:
Royal College of Physicians of London
11 St. Andrews Place
Regent’s Park
London NW1 4LE
United Kingdom

Set and printed by Graphicraft Limited, Hong Kong

All rights reserved. No part of this publication may be reproduced, stored


in a retrieval system, or transmitted, in any form or by any means,
electronic, mechanical, photocopying, recording or otherwise, except as
permitted by the UK Copyright, Designs and Patents Act 1988, without the
prior permission of the copyright owner.

First edition published 2001


Reprinted 2004
Second edition published 2008
This module updated and reprinted 2010

ISBN: 978-1-86016-270-1 (this book)


ISBN: 978-1-86016-260-2 (set)

Distribution Information:
Jerwood Medical Education Resource Centre
Royal College of Physicians of London
11 St. Andrews Place
Regent’s Park
London NW1 4LE
United Kingdom
Tel: +44 (0)207 935 1174 ext 422/490
Fax: +44 (0)207 486 6653
Email: merc@rcplondon.ac.uk
Web: http://www.rcplondon.ac.uk/
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CONTENTS

List of contributors iii 1.3 Communication skills and 2.2 Cardiac arrhythmia 76
Foreword viii ethics 37 2.2.1 Bradycardia 76
Preface ix 1.3.1 Advising a patient against 2.2.2 Tachycardia 78
Acknowledgements xi unnecessary 2.3 Cardiac failure 82
Key features xii investigations 37 2.4 Diseases of heart muscle 86
1.3.2 Explanation of 2.4.1 Hypertrophic
uncertainty of diagnosis cardiomyopathy 86
38 2.4.2 Dilated
CARDIOLOGY 1.3.3 Discussion of the need cardiomyopathy 89
to screen relatives for 2.4.3 Restrictive
an inherited condition cardiomyopathy 89
PACES Stations and Acute
38 2.4.4 Arrhythmogenic right
Scenarios 3
1.3.4 Communicating news ventricular
1.1 History-taking 3 of a patient’s death to a cardiomyopathy 90
1.1.1 Paroxysmal palpitations spouse 39 2.4.5 Left ventricular non-
3 1.3.5 Explanation to a patient compaction 90
1.1.2 Palpitations with of the need for 2.5 Valvular heart disease 90
dizziness 6 investigations 40 2.5.1 Aortic stenosis 90
1.1.3 Breathlessness and ankle 1.3.6 Explanation to a patient 2.5.2 Aortic regurgitation 92
swelling 9 who is reluctant to receive 2.5.3 Mitral stenosis 93
1.1.4 Breathlessness and treatment 41 2.5.4 Mitral regurgitation 95
exertional presyncope 1.4 Acute scenarios 42 2.5.5 Tricuspid valve
12 1.4.1 Syncope 42 disease 97
1.1.5 Dyspnoea, ankle 1.4.2 Stroke and a murmur 46 2.5.6 Pulmonary valve
oedema and cyanosis 1.4.3 Acute chest pain 49 disease 98
14 1.4.4 Hypotension following 2.6 Pericardial disease 98
1.1.6 Chest pain and recurrent acute myocardial 2.6.1 Acute pericarditis 98
syncope 16 infarction 52 2.6.2 Pericardial effusion 100
1.1.7 Hypertension found at 1.4.5 Breathlessness and 2.6.3 Constrictive
routine screening 19 collapse 54 pericarditis 102
1.1.8 Murmur in pregnancy 1.4.6 Pleuritic chest pain 57 2.7 Congenital heart disease 104
23 1.4.7 Fever, weight loss and a 2.7.1 Acyanotic congenital
1.2 Clinical examination 25 murmur 60 heart disease 105
1.2.1 Irregular pulse 25 1.4.8 Chest pain following a 2.7.1.1 Atrial septal
1.2.2 Congestive heart failure ’flu-like illness 64 defect 105
27 2.7.1.2 Isolated
1.2.3 Hypertension 29 ventricular
Diseases and Treatments 69
1.2.4 Mechanical valve 29 septal defect
1.2.5 Pansystolic murmur 30 2.1 Coronary artery disease 69 107
1.2.6 Mitral stenosis 31 2.1.1 Stable angina 69 2.7.1.3 Patent ductus
1.2.7 Aortic stenosis 32 2.1.2 Unstable angina and arteriosus 107
1.2.8 Aortic regurgitation 33 non-ST-elevation 2.7.1.4 Coarctation of
1.2.9 Tricuspid regurgitation myocardial infarction the aorta 108
34 71 2.7.2 Cyanotic congenital
1.2.10 Eisenmenger’s 2.1.3 ST-elevation heart disease 109
syndrome 35 myocardial infarction 2.7.2.1 Tetralogy of
1.2.11 Dextrocardia 36 72 Fallot 109

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CONTENTS

2.7.2.2 Complete 3.3 Ambulatory monitoring 154 1.1.2 Solitary pulmonary


transposition of 3.4 Radiofrequency ablation and nodule 193
great arteries implantable cardioverter 1.1.3 Exertional dyspnoea with
111 defibrillators 156 daily sputum 195
2.7.2.3 Ebstein’s 3.4.1 Radiofrequency 1.1.4 Dyspnoea and fine
anomaly 112 ablation 156 inspiratory crackles 197
2.7.3 Eisenmenger’s 3.4.2 Implantable 1.1.5 Nocturnal cough 199
syndrome 113 cardioverter 1.1.6 Daytime sleepiness and
2.8 Infective diseases of the defibrillator 157 morning headache 202
heart 114 3.4.3 Cardiac 1.1.7 Lung cancer with
2.8.1 Infective endocarditis resynchronisation asbestos exposure 204
114 therapy 158 1.1.8 Breathlessness with a
2.8.2 Rheumatic fever 119 3.5 Pacemakers 159 normal chest radiograph
2.9 Cardiac tumours 120 3.6 Chest radiograph in cardiac 206
2.10 Traumatic heart disease 122 disease 161 1.2 Clinical examination 209
2.11 Disease of systemic arteries 3.7 Cardiac biochemical 1.2.1 Coarse crackles:
124 markers 163 bronchiectasis 209
2.11.1 Aortic dissection 124 3.8 CT and MRI 164 1.2.2 Fine crackles:
2.12 Diseases of pulmonary 3.8.1 Multislice spiral CT 164 interstitial lung
arteries 126
3.8.2 MRI 165 disease 210
2.12.1 Primary pulmonary
3.9 Ventilation–perfusion 1.2.3 Stridor 212
hypertension 126
imaging 166 1.2.4 Pleural effusion 213
2.12.2 Secondary pulmonary
3.10 Echocardiography 167 1.2.5 Wheeze and crackles:
hypertension 129
3.11 Nuclear cardiology 170 chronic obstructive
2.13 Cardiac complications of
3.11.1 Myocardial perfusion pulmonary disease 215
systemic disease 130
imaging 170 1.2.6 Cor pulmonale 216
2.13.1 Thyroid disease 130
3.11.2 Radionuclide 1.2.7 Pneumonectomy/
2.13.2 Diabetes 131
ventriculography 170 lobectomy 217
2.13.3 Autoimmune
3.11.3 Positron emission 1.2.8 Apical signs: old
rheumatic diseases 131
tomography 171 tuberculosis 218
2.13.4 Renal disease 132
3.12 Cardiac catheterisation 171 1.2.9 Cystic fibrosis 219
2.14 Systemic complications of
3.12.1 Percutaneous coronary 1.3 Communication skills and
cardiac disease 133
intervention 172 ethics 220
2.14.1 Stroke 133
3.12.2 Percutaneous 1.3.1 Lifestyle modification
2.15 Pregnancy and the heart 134
2.16 General anaesthesia in heart valvuloplasty 173 220
disease 136 1.3.2 Possible cancer 221
2.17 Hypertension 136 1.3.3 Potentially life-
Self-assessment 176
2.17.1 Hypertensive threatening illness 222
4.1 Self-assessment questions 176 1.3.4 Sudden unexplained
emergencies 140
4.2 Self-assessment answers 183 death 224
2.18 Venous thromboembolism 141
2.18.1 Pulmonary embolism 1.3.5 Intubation for ventilation
141 225
2.19 Driving restrictions in 1.3.6 Patient refusing
cardiology 145 RESPIRATORY ventilation 226
1.4 Acute scenarios 228
MEDICINE 1.4.1 Pleuritic chest pain 228
Investigations and Practical 1.4.2 Unexplained hypoxia
Procedures 147 232
PACES Stations and Acute
3.1 ECG 147 1.4.3 Haemoptysis and weight
Scenarios 191
3.1.1 Exercise ECGs 151 loss 234
3.2 Basic electrophysiology 1.1 History-taking 191 1.4.4 Pleural effusion and fever
studies 152 1.1.1 New breathlessness 191 237

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CONTENTS

1.4.5 Lobar collapse in non- 2.8.4 Pulmonary vasculitis


Investigations and Practical
smoker 239 269
Procedures 297
1.4.6 Upper airway obstruction 2.8.5 Pulmonary eosinophilia
241 270 3.1 Arterial blood gas sampling
2.8.6 Iatrogenic lung disease 297
272 3.2 Aspiration of pleural
Diseases and Treatments 243 effusion or pneumothorax
2.8.7 Smoke inhalation 274
2.1 Upper airway 243 2.8.8 Sickle cell disease and 298
2.1.1 Sleep apnoea 243 the lung 276 3.3 Pleural biopsy 298
2.2 Atopy and asthma 245 2.8.9 Human 3.4 Intercostal tube insertion
2.2.1 Allergic rhinitis 245 immunodeficiency virus 300
2.2.2 Asthma 246 and the lung 3.5 Fibreoptic bronchoscopy and
2.3 Chronic obstructive 278 transbronchial biopsy 302
pulmonary disease 251 2.9 Malignancy 279 3.5.1 Fibreoptic bronchoscopy
2.4 Bronchiectasis 253 2.9.1 Lung cancer 279 302
2.5 Cystic fibrosis 256 2.9.2 Mesothelioma 283 3.5.2 Transbronchial biopsy
2.6 Occupational lung disease 258 2.9.3 Mediastinal tumours 302
2.6.1 Asbestosis and the 285 3.6 Interpretation of clinical
pneumoconioses 258 2.10 Disorders of the chest wall data 302
2.7 Diffuse parenchymal lung and diaphragm 287 3.6.1 Arterial blood gases 302
disease 261 2.11 Complications of respiratory 3.6.2 Lung function tests 304
2.7.1 Usual interstitial disease 288 3.6.3 Overnight oximetry 306
pneumonia 261 2.11.1 Chronic respiratory 3.6.4 Chest radiograph 306
2.7.2 Cryptogenic organising failure 288 3.6.5 Computed tomography
pneumonia 262 2.11.2 Cor pulmonale 289 scan of the thorax 307
2.7.3 Bronchiolitis obliterans 2.12 Treatments in respiratory
263 disease 290
Self-assessment 312
2.8 Miscellaneous conditions 264 2.12.1 Domiciliary oxygen
2.8.1 Extrinsic allergic therapy 290 4.1 Self-assessment questions
alveolitis 264 2.12.2 Continuous positive 312
2.8.2 Sarcoidosis 265 airways pressure 292 4.2 Self-assessment answers 324
2.8.3 Respiratory 2.12.3 Non-invasive
complications of ventilation 292 The Medical Masterclass Series 329
rheumatoid arthritis 267 2.13 Lung transplantation 294 Index 345

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FOREWORD

Since its initial publication in 2001, Medical Masterclass has been regarded
as a key learning and teaching resource for physicians around the world.
The resource was produced in part to meet the vision of the Royal College of
Physicians: ‘Doctors of the highest quality, serving patients well’. This vision
continues and, along with advances in clinical practice and changes in
the format of the MRCP(UK) exam, has justified the publication of this
second edition.

The MRCP(UK) is an international examination that seeks to advance the


learning of and enhance the training process for physicians worldwide. On
passing the exam physicians are recognised as having attained the required
knowledge, skills and manner appropriate for training at a specialist level.
However, passing the exam is a challenge. The pass rate at each sitting of
the written papers is about 40%. Even the most prominent consultants
have had to sit each part of the exam more than once in order to pass.
With this challenge in mind, the College has produced Medical Masterclass,
a comprehensive learning resource to help candidates with the preparation
that is key to making the grade.

Medical Masterclass has been produced by the Education Department of


the College. A work of this size represents a formidable amount of effort
by the Editor-in-Chief – Dr John Firth – and his team of editors and authors.
I would like to thank our colleagues for this wonderful educational product
and wholeheartedly recommend it as an invaluable learning resource for all
physicians preparing for their MRCP(UK) examination.

Professor Ian Gilmore MD PRCP


President of the Royal College of Physicians

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PREFACE

The second edition of Medical Masterclass is produced and published by


the Education Department of the Royal College of Physicians of London.
It comprises 12 textbooks, a companion interactive website and two
CD-ROMs. Its aim is to help doctors in their first few years of training to
improve their medical knowledge and skills; and in particular to (a) learn
how to deal with patients who are acutely ill, and (b) pass postgraduate
examinations, such as the MRCP(UK) or European Diploma in Internal
Medicine.

The 12 textbooks are divided as follows: two cover the scientific background
to medicine, one is devoted to general clinical skills [including specific
guidance on exam technique for PACES, the practical assessment of clinical
examination skills that is the final part of the MRCP(UK) exam], one deals
with acute medicine and the other eight cover the range of medical
specialties.

The core material of each of the medical specialties is dealt with in seven
sections:

• Case histories – you are presented with letters of referral commonly


received in each specialty and led through the ways in which the patients’
histories should be explored, and what should then follow in the way of
investigation and/or treatment.

• Physical examination scenarios – these emphasise the logical analysis of


physical signs and sensible clinical reasoning: ‘having found this, what
would you do?’

• Communication and ethical scenarios – what are the difficult issues that
commonly arise in each specialty? What do you actually say to the
‘frequently asked (but still very difficult) questions?’

• Acute presentations – what are the priorities if you are the doctor seeing
the patient in the Emergency Department or the Medical Admissions
Unit?

• Diseases and treatments – structured concise notes.

• Investigations and practical procedures – more short and to-the-point notes.

• Self assessment questions – in the form used in the MRCP(UK) Part 1 and
Part 2 exams.

The companion website – which is continually updated – enables you to


take mock MRCP(UK) Part 1 or Part 2 exams, or to be selective in the
questions you tackle (if you want to do ten questions on cardiology, or any
other specialty, you can do). For every question you complete you can see
how your score compares with that of others who have logged onto the site
and attempted it. The two CD-ROMs each contain 30 interactive cases
requiring diagnosis and treatment.

ix
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PREFACE

I hope that you enjoy using Medical Masterclass to learn more about
medicine, which – whatever is happening politically to primary care,
hospitals and medical career structures – remains a wonderful occupation.
It is sometimes intellectually and/or emotionally very challenging, and also
sometimes extremely rewarding, particularly when reduced to the essential
of a doctor trying to provide best care for a patient.

John Firth DM FRCP


Editor-in-Chief

x
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CONTENTS
ACKNOWLEDGEMENTS

Medical Masterclass has been produced by a team. The names of those who
have written or edited material are clearly indicated elsewhere, but without
the support of many other people it would not exist. Naming names is risky,
but those worthy of particular note include: Sir Richard Thompson (College
Treasurer) and Mrs Winnie Wade (Director of Education), who steered the
project through committees that are traditionally described as labyrinthine,
and which certainly seem so to me; and also Arthur Wadsworth (Project
Co-ordinator) and Don Liu in the College Education Department office. Don
is a veteran of the first edition of Medical Masterclass, and it would be fair to
say that without his great efforts a second edition might not have seen the
light of day.

John Firth DM FRCP


Editor-in-Chief

xi
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CONTENTS
KEY FEATURES

We have created a range of icon boxes that sit among the text of the
various Medical Masterclass modules. They are there to help you identify key
information and to make learning easier and more enjoyable. Here is a brief
explanation:

Iron-deficiency anaemia with


a change in bowel habit in a
middle-aged or older patient means
colonic malignancy until proved
otherwise.

This icon is used to highlight points of particular importance.

Dietary deficiency is very


rarely, if ever, the sole cause of
iron-deficiency anaemia.

This icon is used to indicate common or important drug interactions, pitfalls


of practical procedures, or when to take symptoms or signs particularly
seriously.

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CARDIOLOGY
Authors:
B Chandrasekaran, PWX Foley, PR Kalra, N Melikian, PR Roberts
and R Sharma

Editor:
PR Roberts

Editor-in-Chief:
JD Firth
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CARDIOLOGY: SECTION 1
PACES STATIONS AND ACUTE
SCENARIOS

from life-threatening ventricular History of the presenting problem


1.1 History-taking arrhythmias. You should always
have in mind a list of the possible What are the palpitations like?
causes of palpitations (Table 1). In The characteristics of the palpitations
1.1.1 Paroxysmal palpitations
most situations it will be essential can provide valuable clues in making
to perform investigations during a the diagnosis. Question the patient
Letter of referral
symptomatic episode. Remember about the following.
to cardiology
outpatient clinic that the severity of symptoms does • Ask her to ‘tap out’ the rhythm
not always reflect the seriousness of of her palpitations. Note how
Dear Doctor, the underlying problem: some patients fast this is and whether it is
in sinus rhythm may experience regular or irregular. Irregular
Re: Miss Jenny Pinto, aged severe palpitations, whereas others means that AF or frequent
28 years may be asymptomatic when in extrasystoles (ectopics) are most
ventricular tachycardia (VT). In this likely. A ‘missed beat’ is typically
Thank you for seeing this case the family history should make caused by an extrasystole: after
secondary-school teacher with a you particularly keen to exclude the compensatory pause the next
5-year history of intermittent significant inherited conditions sinus beat is felt with extra force.
palpitations. Episodes are that may predispose to arrhythmias, These missed beats are almost
short-lived. She has no other eg hypertrophic cardiomyopathy. always of no pathological
symptoms. She is, however, significance. However, they can
concerned about her symptoms cause worry that is likely to be
as she has discovered recently Severe symptoms do not reinforced because anxiety is the
necessarily mean a dangerous most common cause of awareness
that two relatives (one cousin
arrhythmia and minor symptoms do
and one more distant) have died of extrasystoles. They are likely
not necessarily mean a benign
following sudden collapses. I arrhythmia. to have been long-standing and
would be grateful if you would previously asymptomatic.
investigate her symptoms.
TABLE 1 POTENTIAL CAUSES OF PALPITATIONS
Yours sincerely,
Type of palpitation Cause

Introduction No arrhythmia Anaemia


Anxiety
The symptom of palpitations Panic attacks
(abnormal awareness of the heart Depression
beat) can be caused by a range of Extrasystoles Atrial
clinical conditions, from the very Ventricular
benign to the potentially life- Bradyarrhythmia Atrioventricular block
Sinus node disease
threatening. Approach the patient
Tachyarrhythmia VT
with this in mind. It is unusual to Atrial fibrillation (AF)/flutter
see someone during a symptomatic Atrioventricular nodal re-entry tachycardia
episode, so as much information as Atrioventricular re-entry tachycardia
Sinus tachycardia
possible should be gained from the
history, with the main aim being to VT, ventricular tachycardia.
assess the patient’s potential risk

Station 2: History Taking 3


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CARDIOLOGY: PACES STATIONS AND ACUTE SCENARIOS

• Does she feel the palpitations in • Drugs (prescribed and non- • look for sinus bradycardia or
the neck? These are suggestive prescribed): a range of these tachycardia;
of cannon waves, indicating can cause arrhythmia. Always
• check if there are features
simultaneous atrial and consult the drug datasheet or
suggestive of a cardiac structural
ventricular contraction. This can the British National Formulary.
abnormality, eg P mitrale or left
occur in atrioventricular (AV)
• Family history is clearly an ventricular hypertrophy (LVH)
block, AV dissociation associated
important element in this case, (Fig. 1);
with VT or atrioventricular nodal
so it is important to obtain as
re-entry tachycardia (AVNRT). • measure the PR interval;
much information as possible,
• How do the palpitations start, eg which particular relatives • check if there is AV block;
what brings them on and how were involved, what were the
do they stop? Does she get surroundings of their deaths • check for delta waves (Fig. 2);
any warning at all? Do attacks and at what ages did they die?
• ask the patient if she has had a
come on gradually or suddenly? It is often helpful to draw a
previous myocardial infarction
Palpitations that come on and go simple family tree. A patient is
(Q-wave or T-wave changes);
away gradually are most likely due much more likely to be concerned
to sinus tachycardia. about palpitations, even if of • measure the QT interval and
benign cause, if a relative has calculate the QT correction
• Are there accompanying
died at a young age of heart (QTc, QT adjusted for rate);
symptoms? Does she feel faint
disease. Check if there is any
or dizzy when these occur? Has • check for any atrial or ventricular
post-mortem information
she ever collapsed? Arrhythmias extrasystoles.
available for any members of the
causing these symptoms are
patient’s family who have died However, if an ECG has been
more likely to be serious
from heart disease. recorded during symptoms and
(potentially life-threatening)
and clearly mandate thorough • Is the patient prone to anxiety? documents an arrhythmia, it may
investigation. Some patients with Does she ever have anxiety not be necessary to investigate
supraventricular tachycardia attacks? Has she a history of further because this alone may
(SVT) develop polyuria as a recurrent presentation to doctors enable a precise diagnosis to be
result of atrial stretch causing with medically unexplained made.
the release of atrial natriuretic symptoms. This aspect of the
peptide. history needs to be explored Chest radiograph
sensitively. It is unhelpful if the This is likely to be normal, although
• How frequent are the an increased cardiothoracic ratio or
patient thinks that this line of
palpitations? Palpitations that abnormal cardiac outline may
questioning infers that you do
occur infrequently are likely to suggest significant pathology.
not believe her. If there is a high
be difficult to catch on simple
degree of anxiety, it may be the
ambulatory monitoring.
cause of her symptoms, although Ambulatory monitoring
• What treatments have been other pathological substrates may See Section 3.3. An example
tried already? An SVT may be present. of an arrhythmia captured on an
be terminated by a Valsalva ambulatory monitor record is
manoeuvre. Plan for investigation and shown in Fig. 3.
management
Other relevant history After examining the patient and Echocardiography
confirming no abnormalities, you This is an important test that
• General health: is there anything
would plan as follows. helps stratify the patient’s risk.
to suggest thyrotoxicosis? (See
If this shows the patient to have a
Endocrinology, Section 1.1.3.)
ECG structurally and functionally normal
• Smoking, alcohol, tea and coffee In most situations only a 12-lead heart, it puts her into a very low-risk
consumption: acute excess of ECG in sinus rhythm is available. group. However, it is important not
these can trigger arrhythmia When assessing the 12-lead ECG to discount the possibility of
in those predisposed to it. of a patient with palpitations: significant arrhythmia just because

4 Station 2: History Taking


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CARDIOLOGY: PACES STATIONS AND ACUTE SCENARIOS

›Fig. 1 ECG showing LVH with strain (lateral ST/T changes) in a patient with previously undiagnosed aortic stenosis.

›Fig. 2 Twelve-lead ECG of patient with Wolff–Parkinson–White syndrome. Note the short PR interval and delta waves.

›Fig. 3 Ambulatory monitor of a patient with SVT. Sinus tachycardia is followed by ventricular bigeminy before the sudden onset of SVT.

Station 2: History Taking 5


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CARDIOLOGY: PACES STATIONS AND ACUTE SCENARIOS

the echocardiogram is normal, antiarrhythmic medication which would be consistent with the
particularly in a patient with a or referral to a specialist diagnosis of ventricular tachycardia
potentially significant family electrophysiologist for (VT).
history. consideration of catheter
It is vital to ensure that the patient
ablation (see Section 3.4).
is safe while a diagnosis is being
established. He should thus
1.1.2 Palpitations with
be admitted from clinic for
dizziness
A patient with a normal investigation and monitoring.
physical examination, normal It is essential to document his
ECG and normal echocardiogram is at Letter of referral for heart rhythm during an episode.
very low risk of life-threatening urgent assessment in the Some patients with VT are
arrhythmia.
cardiology clinic asymptomatic, whereas others are
extremely symptomatic from only
Dear Doctor,
short runs of VT. Both groups are at
Blood tests risk of cardiac arrest as a result of
Re: Mr Matthew Carney, aged
VT or the VT degenerating into
• Abnormal electrolytes, particularly 57 years
ventricular fibrillation.
hypokalaemia, may predispose to
arrhythmias. Please assess this retired
History of the presenting problem
• Hyperthyroidism may cause policeman who has a 2-month

AF or sinus tachycardia and history of rapid palpitations.


What is the relationship of the
hypothyroidism sinus bradycardia. Initially he was well during
presyncope and palpitations?
the episodes, but more recently
Review in clinic with results of It is important to determine the
he has noticed that he is dizzy
investigations when available. order of symptoms; many patients
when they go on for more than
with presyncope or syncope will
20 seconds. He came to see me
Further discussion have a reactive sinus tachycardia
today as he nearly blacked out
In many cases a benign arrhythmia after the event that might cause a
this morning. He has previously
is detected, such as ventricular or feeling of palpitation. In this case
been very well and this is the
atrial ectopy, and occasionally it is clear that the presyncope is
first time he has asked to see a
symptoms are clearly associated occurring with more prolonged
doctor. Examination today was
with sinus rhythm. In most cases episodes of palpitation.
unremarkable but I am quite
explanation and positive reassurance concerned about the presyncopal Aside from ventricular arrhythmia,
to the patient are all that is required. episode today and would value consider other causes of palpitations
Only in rare instances, where the your opinion. Does he require and syncope:
patient is very debilitated, should detailed cardiac investigation?
a beta-blocker be prescribed. • bradyarrhythmias;

Significant symptoms can Yours sincerely, • atrial flutter with 1:1 conduction;
occasionally be associated with sinus • atrial fibrillation (AF) and Wolff–
tachycardia. In these circumstances Parkinson–White syndrome;
it is important to exclude causes of
Introduction • aortic stenosis.
sinus tachycardia, the most common
Your main concern is that this
being anxiety, before attributing the
patient gives a history of presyncope, And do not forget the following.
arrhythmia to inappropriate sinus
which places him in a higher risk
node function. • Vasovagal syncope: the most
category for life-threatening
common cause of presyncope
If an arrhythmia has been found arrhythmia. The main objective
and syncope.
to be associated with symptoms, must be to exclude a significant
management will be tailored to ventricular arrhythmia. With the • Epilepsy: a common cause of
the individual and the specific little information available, it is syncope, but there seem to be
arrhythmia. In different situations apparent that the palpitations are no features here to support this
this may require reassurance, directly related to the presyncope, diagnosis.

6 Station 2: History Taking


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• Acute blood loss: this will usually Family history ECG


be obvious, but it is a mistake to This is particularly pertinent in Obtaining an ECG during an
miss the fact that the patient has young patients presenting with episode is a key objective in
had melaena. arrhythmias. Always enquire if establishing a diagnosis (Fig. 4).
anyone in the family has had a Beware of confusing VT and
similar problem, or if anyone has supraventricular tachycardia
died suddenly and unexpectedly. with aberrant conduction
Cardiac arrhythmias that can
VT may be part of a primary (see Section 3.1).
cause syncope:
electrophysiological disturbance
• VT/ventricular fibrillation; or secondary to any pathology that
• bradyarrhythmias;
produces structural changes in the
• atrial flutter with 1:1 conduction; A broad-complex tachycardia
• AF and Wolff–Parkinson–White ventricles. Any ‘cardiac history’ should always be treated as VT
syndrome. could therefore be relevant, eg until proven otherwise
valvular heart disease, congenital
heart disease, right ventricular
Other relevant history dysplasia or previous cardiac Ambulatory monitoring
surgery. If the diagnosis is not apparent,
Ischaemic heart disease monitoring for longer periods
This is a common cause of VT. In Epilepsy may be necessary (see Section 3.3).
this situation there appears to be no The history in this case points very
previous history, but it is important clearly to a cardiac arrhythmia, Electrophysiology study
to clarify whether there is a previous but it would be sensible to enquire If symptoms are infrequent or
history of angina or myocardial briefly to ensure that the patient doubt exists as to the diagnosis,
infarction. If not, then specific does not have epilepsy and confirm then provocation of the rhythm
symptons of angina should be that there are no features to suggest during an electrophysiological study
sought: ‘What is the most exercise that this might be responsible will provide definitive evidence
that you do? Have you had any for the current episodes (eg aura, (see Section 3.2).
tightness in your chest when you’ve tongue biting or urinary
been doing that recently?’ It will also incontinence). Other
be appropriate to ask about risk If VT is suspected, investigations
factors for ischaemic heart disease. Plan for investigation and to identify possible causes
management should be considered. Specifically,
Cardiomyopathy In this case examination of the investigations should be concerned
Most forms can cause VT. It is patient was normal. You would with identifying any structural
important to find out whether plan as follows. cardiac abnormality (Table 2).
there is a history of breathlessness,
lethargy or recent viral illness.
An alcohol history should be
taken, both for the current time
TABLE 2 INVESTIGATIONS FOR IDENTIFYING A CARDIAC
and for the past.
ABNORMALITY THAT MAY PROVOKE VENTRICULAR ARRHYTHMIAS

Drugs (prescribed and Investigation Looking for:


non-prescribed)
Drug toxicity can provoke CXR Cardiomegaly, cardiac silhouette and pulmonary oedema (Fig. 5)
VT, eg digoxin, quinidine and Electrolytes Abnormalities of potassium or magnesium can be associated with
catecholamines. Check the datasheet arrhythmia
or British National Formulary for Echocardiogram Cardiac function, valve structure/function and intracardiac
details of any drug that the patient masses (Fig. 6)
is taking. Is arrhythmia reported Exercise ECG Ischaemia and exercise-induced arrhythmias
as a side effect? Recreational drugs Coronary angiography Coronary atherosclerosis and valvular function
such as cocaine and ecstasy are CT or MRI Mediastinal pathology and pericardial/myocardial disease
associated with arrhythmias.

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›Fig. 4 Twelve-lead ECG of VT. Note broad complexes and concordance across chest leads. Right bundle-branch block morphology suggests left ventricular
origin.

Further discussion either catheter ablation or thus making it better tolerated


The patient is likely to be having pharmacological therapy may or unnoticed. Monitoring will
presyncope associated with VT. be considered. It is essential to usually be by ambulatory ECG
Management will consist of treating monitor the patient to ensure recording, but exercise testing
any immediate episodes of VT suppression of the arrhythmia; if the arrhythmia is induced by
(DC shock/pharmacological symptomatology is not always exercise or provocation during
cardioversion) and identification adequate because the drugs may electrophysiological study may
of the cause of the arrhythmia slow but not prevent the VT, be appropriate in some cases.
(see Section 2.2.2). If possible,
the underlying cause should be
corrected and the risk of arrhythmia
then reassessed. In this case it
may turn out that the patient has
significant coronary artery disease
that warrants revascularisation,
either with percutaneous
intervention or coronary artery
bypass grafting. Following this it
would be important to reassess left
ventricular function and consider
an electrophysiological study
to determine whether recurrent
VT was likely (see Section 3.2). All
patients with VT should be assessed
as to whether they would benefit
from an implantable cardioverter
defibrillator (ICD) (see Section 3.4).

In those patients who do not


›Fig. 5 Chest radiograph of patient with dilated cardiomyopathy. The cardiothoracic ratio is increased.
have an indication for an ICD, There is a pleural effusion at the right base.

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differential diagnoses are given in


Table 3. Consider the main causes of
chronic heart failure when taking
the history. It is important to assess
the impact of symptoms on general
daily activities, including work.

History of the presenting problem


If the following do not emerge
spontaneously, make specific
enquiry about them.

• Chest pain: if present, does this


sound like ischaemic cardiac pain
or like pleurisy?

• Cough/sputum: has it been present


and has there been haemoptysis?
›Fig. 6 Echocardiogram demonstrating dilated cardiomyopathy. This is a ‘four-chamber’ view with both • Wheeze: note that this is not
ventricles dilated, particularly the left ventricle (seen in the centre at the top).
synonymous with airway disease.
It may occur in pulmonary
oedema when it is known as
‘cardiac asthma’.
1.1.3 Breathlessness and ankle Introduction
swelling These symptoms are most
commonly caused by cardiac or
Asthma is not the only cause
pulmonary disease. The cause
Letter of referral of wheezing.
usually becomes apparent early in
to cardiology
the history: subsequent questions,
outpatient clinic
examination and investigation Cardiovascular system
should be directed to providing Progressive breathlessness
Dear Doctor,
confirmatory details. The common associated with orthopnoea,

Re: Professor Freddie Walsh,


aged 48 years
TABLE 3 DIFFERENTIAL DIAGNOSIS OF ANKLE SWELLING
AND BREATHLESSNESS
Thank you for seeing this
professor of mathematics who
System Condition
has a 3-month history of
progressive exertional dyspnoea, Cardiac Left ventricular dysfunction
fatigue and peripheral oedema.
Valvular heart disease
Pericardial effusion/constriction
He has generally been fit and Cyanotic congenital heart disease
well without prior history. His High-output cardiac failure secondary to anaemia
father died in his forties of a Pulmonary Chronic airway or parenchymal lung disease (cor pulmonale)
‘large heart’. He is not taking Chronic, repeated pulmonary embolism (PE)
routine medication, although I
Primary pulmonary hypertension
have started him today on Gastrointestinal Liver failure
Protein-losing enteropathy
furosemide 40 mg once a day.
Please assess the cause of his Renal Nephrotic syndrome
Chronic renal failure
symptoms.
Endocrine Hypothyroidism
Yours sincerely, Notes – most common causes in bold.

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paroxysmal nocturnal dyspnoea • Smoking, which is obviously a dilated cardiomyopathy or


and cough productive of clear frothy substantial risk factor for both premature coronary artery disease.
sputum would suggest a cardiac chronic airway disease and
cause. The ankle swelling in cardiac ischaemic heart disease. Plan for investigation and
failure is usually bilateral and management
• Alcohol intake, which is a risk
symmetrical, but it is not uncommon After explaining to the patient that
factor for cardiomyopathy. Ask:
for one ankle to swell initially. you would normally complete a full
‘How much alcohol do you drink
examination, plan the following
A preceding episode of severe now? Have you ever been a heavy
baseline investigations.
central chest pain at rest, drinker in the past?’
particularly if occurring against a
• Previous BP measurements: ECG
background of stable angina, would
untreated hypertension can A routine ECG is very helpful.
suggest a precipitating myocardial
lead to left ventricular failure. If it is completely normal, then a
infarction (MI). A ‘really bad episode
diagnosis of chronic heart failure is
of indigestion’ may have been • Previous cardiac surgery might
unlikely. Other abnormalities may
something different. be suggestive of impaired left
help elucidate the aetiology of the
ventricular function or
Has the patient been started on patient’s symptoms.
constrictive pericarditis.
diuretic therapy? Has this helped?
• A dominantly negative P wave
A good response would support but Whilst the GP’s letter has stated that
in lead V1, reflecting left atrial
not prove a cardiac cause for the the patient is not taking any regular
hypertrophy, is an indirect sign
symptoms. medication, it is essential to confirm
of left heart dysfunction (Fig. 7).
this and ensure that he has not been
Respiratory system taking non-prescribed treatment that • Right ventricular hypertrophy
The development of increasing may be causing or exacerbating his (right bundle-branch block
breathlessness and ankle swelling symptoms, eg NSAIDs. with dominant R waves in V1)
may indicate the development of secondary to any cause of
cor pulmonale in a man with long- A detailed family history is
pulmonary hypertension.
standing respiratory disorder. His particularly important in this case.
symptoms are said to have started His father died at a young age of • Low voltages and electrical
only 3 months ago, but what was presumed cardiomyopathy (‘a large alternans, which occur with a
he like before then? What is the heart’). This might reflect a familial large pericardial effusion.
most vigorous exercise he ever took?
Three months ago was his breathing
more laboured than that of his wife,
family or friends?

Stepwise progression (sudden


deterioration followed by periods of
stability) should raise the suspicion
of multiple recurrent PE, even in the
absence of pleuritic chest pain or
haemoptysis.

Other relevant history


It is clearly important to establish
any history of cardiovascular or
respiratory disease. Ask about the
following.

• Rheumatic fever or history of a


cardiac murmur.

• Recurrent asthma/bronchitis
›Fig. 7 ECG showing left atrial strain (inverted P wave in V1) and partial left bundle-branch block in a
or any other respiratory problem. patient with severe congestive cardiac failure secondary to alcoholic cardiomyopathy.

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requires cardiac catheterisation for


confirmation.

Other tests
Other more specialist investigations
may be required and will be directed
by the clinical features and initial
investigations. These include cardiac
catheterisation (for coronary artery
anatomy and valvular dysfunction)
and spiral CT (for PE).

Management
If chronic heart failure is
suspected and supported by initial
investigations, eg abnormal ECG
and CXR, then initial management
might include the adjustment of
diuretic dose and commencement
of an angiotensin-converting enzyme
›Fig. 8 Chest radiograph showing cardiomegaly and pulmonary oedema in a patient with congestive
cardiac failure caused by severe mitral regurgitation. Note cardiomegaly and enlarged left atrium.
(ACE) inhibitor. The patient should
be reviewed with results in due
course, but remember that renal
function should be monitored in
• Atrial arrhythmias: common in Urinalysis the interim. This should occur
both cardiac and pulmonary Do not forget this simple test. approximatey 1–2 weeks after
disease. If there is significant proteinuria starting ACE inhibitor treatment
on dipstick testing (>2+), then (see Section 2.3), with advice given
• Previous MI, left bundle-branch
nephrotic syndrome is possible. to the GP to stop the ACE inhibitor
block or poor R-wave progression
In this case check serum albumin if serum creatinine rises by more
indicating left ventricular disease.
and urinary albumin/creatinine than 20%.
ratio or 24-hour urinary protein
Chest radiograph excretion. Remember that
In the context of an elevated JVP: Further discussion
proteinuria of up to 1 g/day
The impact of symptoms on
• a large heart should prompt (occasionally more) can be
daily living are very important.
echocardiography (Fig. 8); caused by severe cardiac failure.
Recommendations regarding work
and exercise should all be discussed.
• check for signs of pulmonary Echocardiography Education, with particular emphasis
oedema; This is most useful for excluding on the rationale for treatment, may
• if heart size is normal, inspect significant valvular or left ventricular help compliance. Involvement of a
the lung fields closely for evidence disease. If a pericardial effusion is specialist heart failure nurse is
of chronic obstructive airway found, then careful clinical and extremely helpful.
disease or parenchymal lung echocardiographic assessment is
required to judge whether this is If the patient were to deteriorate
disease;
contributing to his symptoms. despite full medical therapy,
• if the heart size and lung fields Assessment of right heart function then cardiac resynchronisation
are both normal, consider PE or is largely subjective, but reasonably therapy, with or without
pericardial constriction. accurate indirect measurements of implantable cardioverter
pulmonary artery systolic pressure defibrillator (see Section 3.4),
Blood tests can be obtained. Echocardiography or referral for transplant
Check FBC, electrolytes and renal, may suggest pericardial constriction assessment might be required
liver and thyroid function tests. or restrictive cardiomyopathy, which (see Section 2.3).

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1.1.4 Breathlessness and Introduction History of the presenting problem


exertional presyncope There are suggestions in this
• Is the patient limited by fatigue
history that stress or anxiety
(may indicate low cardiac output),
may be contributing to this
Letter of referral breathlessness or by something
patient’s symptoms, but your
to cardiology else? If so, what?
primary concern should be to
outpatient clinic exclude the significant organic • How far can she walk/run?
conditions that can present How many flights of stairs can
Dear Doctor, insidiously in this way (Table 4). she climb? Be specific about this,
and try to get a feeling for the
Re: Miss Susan Ward, aged
pace of progression by asking:
38 years
‘How does this compare with
Exertional syncope or
presyncope is a symptom to be last Christmas or during your
Thank you for seeing this
taken seriously. It usually indicates an summer holidays?’
accountant who is currently inability to increase the cardiac output
out of work. Over the last few appropriately as a result of a fixed • Have there been any other
months she has complained of obstruction or ventricular dysfunction. instances of syncope/presyncope,
gradually worsening fatigue and exactly what were the
and exertional dyspnoea. A year circumstances? It is important
Although not usually associated to establish the environment in
ago she was fit and active but is
with exertional dyspnoea, other which these episodes occurred
now unable to jog or attend her
causes of syncope and sudden (eg warm and not having eaten,
usual exercise classes. There
cardiac death in young subjects such or following alcohol consumption
has been no improvement with
as arrhythmogenic RV dysplasia might suggest a vagal component)
bronchodilator therapy. She is
and QT prolongation should also and if there was any warning.
extremely anxious about an
be considered. An accurate and
episode last week when she
detailed family history is imperative. Ask specifically about the following
nearly fainted while hurrying for
associated symptoms.
a train to a job interview. Please Fatigue is a non-specific symptom
advise on further management. of multifactorial aetiology, but is • Chest pain: if present, is this
a common limiting symptom in pleuritic or anginal? This
Yours sincerely, patients with heart failure and woman is young for ischaemic
valvular disease. heart disease, but anginal pain
can be associated with pulmonary
hypertension. This is thought to
TABLE 4 DIFFERENTIAL DIAGNOSIS OF EXERTIONAL DYSPNOEA originate from the hypertrophied
AND PRESYNCOPE (and therefore relatively hypoxic)
RV. Rare other causes include
Pathophysiology Specific conditions anomolous origins of coronary
Left ventricular outflow tract Hypertrophic cardiomyopathy (HCM) arteries.
obstruction Aortic subvalvular/valvular/supravalvular stenosis
• Haemoptysis: a feature of
Pulmonary hypertension Primary pulmonary hypertension
Secondary, eg to respiratory disease, pulmonary pulmonary hypertension, but
thromboembolism or mitral valve disease could also indicate PE.
(see Section 2.12.2)
• Cough/wheeze/sputum: features
Right ventricular (RV) outflow Infundibular/pulmonary stenosis
tract obstruction that would suggest chronic lung
Left ventricular dysfunction disease.
See Section 2.3
Pericardial compromise of Effusion • Orthopnoea/paroxysmal nocturnal
cardiac filling Constriction dyspnoea: suggests incipient
Anaemia – pulmonary oedema.
Sustained arrhythmia Atrial fibrillation
Complete heart block • Palpitations (see Sections 1.1.1
and 1.1.2).

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• Ankle oedema/calf swelling or • Appetite suppressants: these (see Section 3.1). Left bundle-branch
tenderness: unilateral problems have been implicated in valve block is commonly associated with a
raise the possibility of venous disease and pulmonary dilated left ventricle (LV).
thromboembolism; bilateral hypertension.
swelling suggests RV failure. Chest radiograph
• Cardiotoxic chemotherapy.
Note heart size and shape,
• Raynaud’s phenomenon: this
• Cocaine: this can cause left pulmonary arteries, lung fields and
may be present in autoimmune
ventricular dysfunction and any valve calcification or pleural
rheumatic disease and also
pulmonary hypertension. effusions.
in 10% of women with primary
pulmonary hypertension (PPH).
Family history Echocardiography
• Any features that would suggest A detailed family history is Enables visualisation of ventricular
autoimmune rheumatic disease, important. Ask broad questions dimensions, hypertrophy and
eg joint pains and rashes. such as ‘Has anyone in your family function, together with outflow
died suddenly at a young age?’ tracts and valves (with gradients)
Other relevant history Specifically consider: and any intracardiac shunt or
Enquire specifically about a history pericardial effusion. If there is
• premature ischaemic heart
of the following: significant pulmonary hypertension,
disease;
a dilated hypertrophied RV that
• venous thromboembolism;
• PE; compresses the LV into a ‘D’ shape
• rheumatic fever or ‘heart murmur’; (Fig. 9) can usually be seen, and the
• HCM;
presence of tricuspid regurgitation
• any problems during a previous
• pulmonary hypertension. enables the estimation of pulmonary
pregnancy (if relevant, see below);
artery pressure.
• chest trauma or tuberculosis (may Social history
lead to pericardial problems); Stress may be a contributary Ambulatory monitoring
factor. Ask questions regarding To exclude tachyarrhythmias,
• respiratory disease.
work (financial consequences of particularly if structural
Also ask about the following. currently being unemployed), abnormalities are found on
the implications of looking for echocardiography.
• Smoking.
a new job and her general home
• Alcohol. circumstances. Oxygen saturation
Check pulse oximetry. Perform
• Pregnancy: many previously
Plan for investigation and arterial blood gases if oxygen
silent cardiorespiratory conditions
management saturation is below 95% or the
manifest themselves in pregnancy
patient looks cyanosed.
because of the physiological
changes it engenders.
Blood tests
• Other risk factors for ischaemic The echocardiogram is the key Check FBC, electrolytes, renal and
heart disease (eg hypertension, investigation in the patient liver function, glucose, cholesterol
smoking, hypercholesterolaemia, with dyspnoea and syncope on and inflammatory markers
exertion.
family history and diabetes). (C-reactive protein and erythrocyte
sedimentation rate). Other tests
• Other risk factors for PE
for autoimmune rheumatic disease
(eg immobility and clotting
Explain that you will carry out may be indicated (see Rheumatology
abnormalities).
a full clinical examination before and Clinical Immunology,
conducting the following Section 3.2).
Drug history
investigations.
Ask directly about the use of the
Urinalysis
following.
ECG Look specifically for protein,
• Oral contraceptive: this carries a Note the rhythm, axis, and any blood and glucose. Could there be a
risk factor for thromboembolism. atrial or ventricular hypertrophy multisystem inflammatory condition?

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generally best performed via a


specialist service with trained
counsellors.

Do not forget that a diagnosis


of HCM or other genetic
condition has implications for the rest
of the family, not just the patient in
front of you.

1.1.5 Dyspnoea, ankle oedema


and cyanosis

Letter of referral
to cardiology
›Fig. 9 Short-axis echocardiographic view of a patient with PPH showing the high-pressure, dilated right
ventricle (RV) compressing the left ventricle (LV) into a characteristic ‘D’ shape. (Courtesy of Dr L.M. Shapiro.) outpatient clinic

Dear Doctor,
Other tests occasion. If exercise limitation
If the echocardiogram suggests persists, then an exercise test
Re: Mr Rob Owen, aged 45 years
pulmonary hypertension but (with monitoring of arterial oxygen
no cause is apparent, further saturation) can be valuable in
Many thanks for assessing
investigations are needed (see providing reassurrance that it
this reclusive 45-year-old man.
Section 2.12.1). These should is safe to resume previous levels
Despite having been registered at
initially be directed towards of activity.
the practice for over 10 years he
excluding secondary causes of
has recently presented for the
pulmonary hypertension. If a cause Further discussion first time. His major complaint
is not discovered and the diagnosis Exertional breathlessness is a
was ankle swelling that has
of PPH is made, other investigations common reason for referral to
prevented him from putting
(eg right heart catheterisation) are cardiology clinics. Identifying
on his shoes. I was, however,
used to determine prognosis and patients with significant pathology
surprised to find that he
optimise treatment. Consider can sometimes be difficult, and
was centrally cyanosed and
pulmonary function tests. even if patients do have genuine
moderately dyspnoeic. Many
pathology, anxiety may influence
thanks for your urgent help in
how their symptoms are manifested.
Management investigating his symptoms.
Exertional syncope should be taken
Further management will depend
seriously even in a young, apparently
on the specific diagnosis. Urgent Yours sincerely,
fit individual; in rare cases it can
referral for specialist care is required
precede sudden cardiac death. Initial
if a structural cardiac abnormality
investigations should be ordered on
is found, eg HCM or pulmonary
an urgent basis, particularly if the
hypertension. In contrast, if Introduction
ECG is abnormal.
examination and investigations are Cyanosis can be of cardiac
normal, provide reassurance and If HCM is diagnosed, it will be (right-to-left shunting) or respiratory
encourage the patient to maintain important to ask about children origin or (very rarely) associated
activity. Make sure you retain an and other family members because with abnormal haemoglobin. If
open mind and keep her under issues of screening will need to be cyanosis develops over a long period
review on at least one further considered and discussed. This is it can be reasonably well tolerated,

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but may lead to other complications • How long has his ankle swelling Other relevant history
(see Section 2.7). The list of been going on for? Ask specifically about the following.
differential diagnoses for this
• Has he noticed that he has • Does he know if he was a
patient includes:
become blue and, if so, when? blue baby? Did he have a
• respiratory failure and cor Has he been a ‘funny colour’ for heart murmur? Did he have
pulmonale secondary to chronic as long as he can remember? If rheumatic fever (try St Vitus’
obstructive pulmonary disease this has been very long-standing, dance) as a child or as a
(COPD), but also bronchiectasis, it suggests a cardiac rather than a young man?
pulmonary fibrosis or respiratory explanation.
• Tuberculosis and whooping cough:
hypoventilation syndromes;
• Is he limited by breathlessness? these would put him at risk of
• Eisenmenger’s syndrome Quantify his functional status. bronchiectasis.
(see Section 2.7.3); How far can he go on the flat?
• Did any siblings die young?
Can he go up stairs? How many
• primary pulmonary hypertension If so, consider cystic fibrosis.
times does he have to stop?
(not likely in a middle-aged man,
Also ask about the following:
see Section 1.1.4); Also ask about the following:

• Has he suffered from dizziness, • Symptoms of autoimmune


• secondary pulmonary
headache, visual disturbance or rheumatic disease, which can be
hypertension of another cause
paraesthesiae? These could be associated with interstitial lung
(see Section 2.12);
symptoms of hyperviscosity, disease.
• other congenital heart disease probably indicating secondary • Use of prescribed medications and
(patients with Ebstein’s anomaly polycythaemia in this case. other drugs: are any associated
or mild cases of tetralogy of Fallot
• Has he had any episodes of with chronic lung disease or
may survive to middle age).
syncope or presyncope? These pulmonary hypertension?
You need to determine the cause of are worrying signs in patients • History of stroke or transient
his cyanosis by looking for evidence with pulmonary hypertension. ischaemic attack. These would
of respiratory disease, pulmonary
• Does he find it difficult to stay be uncommon in a patient aged
hypertension and intracardiac
awake sometimes? Has he ever 45 years, but may be attributable
shunts.
fallen asleep during the day when to paradoxical embolism from
he was not trying to, eg when right-to-left shunting in this
driving a car? Has anyone ever case.
Causes of cyanosis in an adult: complained that he snores
• respiratory failure and cor excessively when he sleeps? Plan for investigation and
pulmonale; Does he wake up with headaches management
• Eisenmenger’s syndrome; in the mornings? Any of these
• pulmonary hypertension (primary or
features would suggest obstructive
secondary);
• other congenital heart disease;
sleep apnoea.
• abnormal haemoglobin (very rare). Cardiac cyanosis will not
• Does he smoke? Does he have
improve with maximal inspired
chronic cough, sputum or
oxygen, whereas respiratory cyanosis
wheeze? Is there any history of generally will.
asbestos exposure? These may
History of the presenting problem suggest chronic lung disease.
Enquire about the duration and
Ask about features that would
severity of the presenting symptoms, Explain that after examining him
suggest thromboembolic disease:
remembering that some patients, you would want to organise the
perhaps such as this man, may not • asymmetrical calf swelling or following tests.
be reliable witnesses, tending to tenderness;
deny ill-health and generally playing Oxygen saturation
• pleuritic chest pain;
down the issues. Ask specifically Check pulse oximetry. Also check
about the following. • haemoptysis. arterial blood gases for PO2 and PCO2

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when he is breathing air and transthoracic echocardiography 1.1.6 Chest pain and recurrent
(monitoring him continuously (see Section 3.10); syncope
in case he retains CO2 and is
• ventilation–perfusion scan or
dependent on hypoxic drive) Letter of referral
spiral CT scan if pulmonary
after 10 minutes on high-flow to cardiology
thromboembolism is considered
oxygen (see Respiratory Medicine, outpatient clinic
possible (see Section 3.11);
Section 2.11.1).
• MRI of the heart to define Dear Doctor,
ECG anatomy more clearly
Look for evidence of right (see Section 3.8). Re: Mr John Morris, aged
ventricular hypertrophy (RVH; 65 years
see Section 3.1). Management
Management depends on the This man works as a farmer and
Chest radiograph underlying condition. Note that presents with a 3-month history
Look for signs of pulmonary in all patients with pulmonary of syncopal episodes and
hypertension and chronic hypertension, great care must exertional chest pain. He has
lung disease, particularly the be taken with the use of diuretics been seen in the practice over
hyperexpansion of COPD and for oedema: the risk is that the last few years with a number
the interstitial shadowing of overzealous fluid removal can of minor ailments, but has no
parenchymal lung disease. lead to reduction in RV filling significant past medical history.
pressure, thereby causing I would be grateful if you would
Blood tests circulatory collapse. see him and advise on further
Check his FBC: is the patient investigation and management.
polycythaemic? Check electrolytes,
and renal and liver function. Other Yours sincerely,
tests, eg for autoimmune rheumatic
• Swollen ankles do not mean
disorders, may be indicated in some
diuretic deficiency.
cases. • Be very careful with diuretics in
patients with pulmonary
Echocardiography hypertension: overzealous fluid
Introduction
removal can cause circulatory The history of syncope and
This is a key investigation for
collapse. exertional chest pain strongly
assessing right ventricular (RV)
suggests a cardiac problem,
function, RVH and pulmonary
with syncope due to outflow
pressures. Examine the heart valves
tract obstruction or arrhythmia
and look for septal defects and
and pain due to cardiac ischaemia.
shunts. Further discussion
Severe pain can sometimes cause
The optimal management
vasovagal syncope, and patients
Pulmonary function tests of severe pulmonary hypertension
on vasodilatory medications can
Does the patient have severe requires early referral to a
develop orthostatic syncope.
obstructive or restrictive lung specialist clinic. Patients with
However, neither of these would
disease? Check spirometry, lung Eisenmenger’s syndrome should
seem likely from the history given.
volumes and gas transfer. Depending be referred to a cardiologist with
on the initial results consider the an interest in adult congenital
following: heart disease; other patients
may benefit from referral to a
• high-resolution CT scan of
specialist pulmonary hypertension
the chest (see Section 3.8); Cardiac causes of syncope
service. If anticoagulation is
• transoesophageal required, it will be important • Vasovagal .
• Arrhythmia.
echocardiography for atrial to ensure that the patient will
• Aortic stenosis.
septal defect if there is pulmonary be compliant with monitoring
• Hypertrophic cardiomyopathy.
hypertension and a shunt is and understand the key importance • Orthostatic hypotension.
suspected but not seen on of this.

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History of the presenting problem Other relevant history cardiomyopathy or dilated


Does the patient have a history cardiomyopathy, or a previous
Syncope of ischaemic heart disease, valvular myocardial infarct, which
Ask the patient to provide as detailed heart disease or arrhythmia? Has may predispose to ventricular
an account of the syncopal episodes he ever had a heart attack? Has arrhythmia (Fig. 10b). Are there
as they can. he ever seen a specialist for his other abnormalities such as
heart? Has he ever been aware delta waves or long QT interval
• Outflow tract obstruction resulting
of his heart beating fast or (Fig. 10c)?
from vasodilatation and reflex
abnormally?
bradycardia usually occurs on
Chest radiograph
exertion. Does he have any risk factors for
Look for LVH and for evidence of
ischaemic heart disease? Does he
• Arrhythmias can occur at any aortic valve calcification.
smoke? Is there a family history
time, although some may be
of this condition? Does he have
provoked by exertion due to Echocardiography
diabetes or high blood cholesterol?
ischaemia or increased release This will determine the presence
of catecholamines. Was the Ask specifically about rheumatic of aortic valve stenosis and left
patient aware of his heart fever. This usually occurs in ventricular outflow obstruction
beating in an unusual way childhood and may have involved a and allow measurement of left
at any time? sore throat, prolonged bed-rest and ventricular function, which if poor
aching joints. Also, although clearly may predispose to ventricular
• Orthostatic hypotension can occur not relevant in this case, note if a arrhythmias (Fig. 11).
after variable periods of standing patient has congenital heart disease
and may be prominent after such as tetralogy of Fallot, which Ambulatory ECG monitoring
exertion. can be associated with exertional Prolonged monitoring, initially
• Vasovagal syncope is typically syncope. with a 24-hour tape, may pick up
preceded by a definite prodrome At the age of 65 years and with the ventricular or other arrhythmias,
of worsening nausea and history of chest pain, the following which can sometimes be revealed
sweating. conditions are not likely but check on standard 12-lead recordings
if there is any family history of (Fig. 12).
Chest pain sudden death, especially at a
Confirm the nature of the pain: young age. If there is, this Blood tests
it is important to be clear that may suggest hypertrophic These are not likely to be critical
he is experiencing angina rather cardiomyopathy, long QT investigations in this case, but
than any other pain. Establish syndrome with associated anaemia may worsen the symptoms
quickly its character, radiation, arrhythmias, Brugada syndrome of ischaemic heart disease or aortic
etc. or arrhythmogenic right ventricular valve disease, and assessment
dysplasia. of cardiovascular risk factors
Other features (glucose and cholesterol) would
Ask directly if any witnesses Plan for investigation and be appropriate.
have told the patient what management If doubt about the diagnosis
happened when he collapsed. The diagnosis will be aided by the remains, then further tests to be
Did he change colour? If so, this examination and confirmed by the considered would including tilt-table
suggests a cardiac cause. Did anyone following investigations. testing (vasovagal syncope may be
check his pulse? This may firmly
provoked, with either bradycardia or
establish the diagnosis if done by a ECG
hypotension initiating the event) and
reliable witness. And were there any Is it normal? Is there any evidence
coronary angiography.
features to suggest epilepsy (aura, of heart block or other arrhythmia?
limb shaking, tongue biting and Measure the PR interval and QRS If you consider that the patient may
urinary incontinence)? However, duration. Is there evidence of left be at risk of ventricular arrhythmias,
remember that seizures can ventricular hypertrophy (LVH) then consider admitting him for
occasionally be secondary to (Fig. 10a)? This could suggest further investigation and management
a cardiac cause of collapse. aortic stenosis, hypertrophic (see Section 1.1.2).

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›Fig. 10 Twelve-lead ECGs: (a) LVH; (b) old anterior myocardial infarction.

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›Fig. 10 Twelve-lead ECGs: (c) long QT.

diagnosis is made and treatment


established.

1.1.7 Hypertension found at


routine screening

Letter of referral to
medical outpatient clinic

Dear Doctor,

Re: Mrs Joy King, aged 30 years

This Afro-Caribbean woman


attended the family planning
clinic to obtain a prescription for
the oral contraceptive pill (OCP).
›Fig. 11 Stenotic aortic valve seen at operation prior to replacement.
However, her BP on numerous
occasions is typically around
Further discussion (DVLA) that recurrence is unlikely 180/100 mmHg. She smokes
(see Section 2.19). between 10 and 20 cigarettes a
Driving day but drinks minimal amounts
Patients with syncope cannot General advice of alcohol. She has no other
drive until they satisfy the Driver Advise the patient to avoid heavy known medical problems, but
and Vehicle Licensing Agency lifting and vigorous exercise until a

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›Fig. 12 Twelve-lead ECG showing ventricular tachycardia.

is no element of ‘white coat’ smoking) and a decision regarding


there is strong family history of hypertension) and, if hypertension prescription of the OCP.
high BP with her mother, older is confirmed, treating the blood The differential diagnosis of
sister and one maternal aunt all presssure. This should then be this woman’s high BP reading is
being treated for the condition. followed by an assessment of summarised in Table 5.
Her father died from a whether there are secondary causes
haemorrhagic stroke when she for her hypertension, followed by History of the presenting problem
was a child. On examination she advice and management of her other Hypertension is usually
is overweight with a BMI of 28. vascular risk factors (her weight and asymptomatic until there is
Please can you advise on further
investigation and management?

Yours sincerely, TABLE 5 DIFFERENTIAL DIAGNOSIS OF HYPERTENSION

Comment Diagnosis

Common Essential hypertension


Introduction False elevation as a result of inadequate BP cuff size
Hypertension is a common Isolated clinic (‘white coat’) hypertension
problem, especially in black people. Must consider Renal hypertension
Although there may be multiple Renovascular hypertension
Primary hyperaldosteronism (Conn’s syndrome)1
causes for her father’s death from a
Phaeochromocytoma1
haemorrhagic stroke, uncontrolled Coarctation of the aorta1
and unrecognised hypertension Other causes2 Cushing’s syndrome
is certainly a possibility. The Acromegaly
initial approach in managing Polycystic ovarian syndrome
this patient should be directed
1. Rare or very rare.
towards confirming the diagnosis 2. Hypertension not likely to be the dominant feature of these conditions.
of hypertension (ie to ensure there

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progression to end-organ damage.


Some patients, on being given a TABLE 6 POSSIBLE SECONDARY CAUSES OF HYPERTENSION AND
diagnosis of hypertension, will RELEVANT QUESTIONS TO BE ASKED IN THE HISTORY
ascribe many different and varied
complaints to it. A multitude of Cause of hypertension History to be elicited
symptoms, eg headache, epistaxis,
Atheromatous The presence of other atheromatous complications such as
tinnitus, dizziness and fainting, renovascular disease peripheral vascular disease and cerebrovascular disease
are often blamed on an elevated increase the likelihood of renovascular hypertension
BP, but probably occur with similar Does she get pain in the legs on walking?
Has she had any neurological symptoms?
frequency in those whose BP is
Renal parenchymal Has she ever had tests on her urine or kidneys (eg during
normal. It is important to elicit any
disease pregnancy or for insurance purposes)?
potential complications of untreated Has she ever been told that she had a problem with her kidneys?
hypertension, as detailed below. Is there any family history of kidney disease?
Has she noticed any blood in her urine, or ever had bad swelling
Cardiovascular system in her legs?
Hypertension initially results in Phaeochromcytoma Ask about intermittent episodes of panic attacks (anxiety),
left ventricular hypertrophy (LVH), sweating, tremors, palpitations and chest pain
followed by diastolic and finally Primary There are no specific symptoms, but a history of muscle cramps
hyperaldosteronism secondary to low potassium levels, and possibly polyuria and
systolic left ventricular dysfunction. (Conn’s syndrome) polydipsia, may be relevant
Pursue a history of shortness of
Cushing’s syndrome Has she gained weight?
breath on exertion or at rest, as Has she noticed striae, or thinning of her hair or skin?
well as one of pulmonary oedema. Does she bruise easily?
Enquire about swelling of ankles. Any changes in menstrual cycle?
Has she been prescribed steroids?
Other non-specific symptoms can
include palpitations and potentially Acromegaly Any change in her hand or foot size?
Has anyone commented on changes in her facial appearance?
chest pain (if associated with Has she noticed any change in vision?
ischaemic heart disease, although Does she have any joint problems?
this is unlikely in this patient). Drugs Has she taken any prescribed or non-prescribed medication?
Has she been eating lots of liquorice?
Neurological system
Note: it is very rare for a secondary cause of hypertension to be diagnosed from history
Ask about any transient or
alone, but the history can provide useful clues to follow.
prolonged episodes of weakness in
any of her limbs, or problems with
her speech or eyesight. The answers
to these questions can rule out a secondary cause of high BP • diabetes;
transient ischaemic attacks or a (Table 6). In most cases, however,
• hyperlipidaemia;
stroke. Ask whether the patient has a secondary cause is not found and
had any episodes of loss of vision or hypertension is classified as primary • family history of cardiovascular
blurred vision that may have been or ‘essential’. A strong family history events;
caused by retinal bleeds. of hypertension would support the • alcohol consumption.
diagnosis of essential hypertension
Peripheral vascular system and hence a detailed family history Plan for investigation and
Ask about symptoms that might must always be pursued, or confirmed management
indicate intermittent claudication, when it is stated (as in this case).
although this would be extremely
unlikely in a 30-year-old woman. Cardiovascular risk factors
In any patient presenting with When investigating the patient
Other relevant history hypertension it is very important with hypertension consider the
following:
to assess other cardiovascular
Could there be a secondary cause
risk factors, including the • Is there a secondary cause?
of hypertension? • Is there evidence of end-organ
following:
It is important to address specific damage?
symptoms that may point towards • smoking;

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After examining her your strategy


for investigation should be directed
towards detecting secondary
causes and identifying evidence
of end-organ complications
(see Section 2.17).

ECG
Look particularly for evidence of
LVH (see Fig. 10a).

Urine
Check for proteinuria and
haematuria using dipsticks. If
positive for protein, quantification
of albumin /creatinine ratio with a
spot urine or 24-hour collection is
required. The presence of proteinuria ›Fig. 13 CXR of patient with coarctation of the aorta, showing (a) rib notching, (b) site of coarctation and
(c) prestenotic and poststenotic dilatation. (Reproduced with permission from Ray KK, Ryder REJ and
and /or haematuria would be Wellings RM, An Aid to Radiology for the MRCP. Oxford: Blackwell Science, 1999).
consistent with the patient having
a renal disorder with secondary
hypertension, or with renal damage Other tests where BP recorded in clinic is very
caused by hypertension. Other tests may be appropriate high but there seems to be no
depending on the findings of those evidence of end-organ damage.
Blood tests detailed above. Ambulatory blood
Check FBC, electrolytes, renal and pressure monitoring may be needed Other specific tests may be required
liver function, uric acid, fasting to confirm the diagnosis and exclude as dictated by the clinical setting to
glucose and lipid profile. The most ‘white coat’ hypertension, the latter diagnose primary renal disease
common cause of hypokalaemia is being suspected particularly in cases (serological tests or renal biopsy),
diuretic treatment, but low values
are often found in untreated
accelerated-phase hypertension
and in primary hyperaldosteronism
(suspect only if patients are not on
diurectic treatment). Is her renal
function normal? Does she have
glucose intolerance or diabetes?
Is her cholesterol elevated?

Chest radiograph
Assess heart size and look for
pulmonary oedema and possible
(but very unlikely) radiographic
signs of coarctation (Fig. 13).

Echocardiography
This is more sensitive than ECG
at detecting LVH, especially if
patients are of Afro-Caribbean
descent (Fig. 14). Look for evidence
of diastolic and systolic left
›Fig. 14 LVH: compared with the normal parasternal M-mode (see Fig. 121), it is evident that the
ventricular impairment. interventricular septum is grossly thickened in this patient. (Courtesy of Dr J. Chambers.)

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renovascular disease other method of contraception structural cardiac lesion, can you
(renal ultrasound and Doppler should be made on the basis of predict and prevent problems that
examination, or MRI angiography), overall cardiovascular risk and might arise during the pregnancy?
Conn’s syndrome (plasma renin benefits. The most common diagnosis will
and aldosterone levels) or be an innocent systolic murmur
phaeochromocytoma (24-hour 1.1.8 Murmur in pregnancy due to the hyperdynamic circulation
urinary and blood catecholamine of pregnancy, requiring no further
levels). (See Section 2.17.) Letter of referral intervention. Mitral or aortic
to cardiology valve disease, hypertrophic
Management outpatient clinic cardiomyopathy (HCM) and
Management consists of treating congenital abnormalities such as a
any underlying secondary cause ventricular septal defect may require
Dear Doctor,
of hypertension if present. careful monitoring; at the very least
Otherwise, a stepwise approach they require antibiotic prophylaxis
Re: Mrs Rose Berry, aged 23
to antihypertensive medication is during vaginal delivery. Occasionally,
most likely. rare and severe conditions such as
Thank you for seeing this woman
who is 29 weeks pregnant and cardiomyopathy of pregnancy can
Further discussion present in the third trimester.
was noted to have a systolic
Do not forget to offer advice
murmur at one of her routine
and treatment (where possible) to History of the presenting problem
antenatal visits. This is her first
reduce other cardiovascular risk Most patients who present in this
pregnancy and there have been
factors. Decisions regarding the way will be asymptomatic. The
no other problems. The rest of
treatment of hypertension (or presence of symptoms should
her history and examination are
hypercholesterolaemia) should never raise the suspicion of significant
unremarkable and I would be
be taken in isolation. For example, pathology. However, bear in mind
grateful for your opinion as to
in this patient it is important that that a degree of weakness, exertional
the significance of her murmur.
any treatment for high BP is dyspnoea, dizziness and peripheral
combined with general lifestyle oedema are quite common during
Yours sincerely,
measures (stop smoking, increase pregnancy and are a result of
physical activity and try to lose physiological adaptation rather
weight). Should she have an than intrinsic cardiac disease. Be
abnormal lipid profile this should Introduction sure to gauge the precise severity of
also be actively managed with Your major concern will be to the symptoms and relate it to the
dietary advice (and possibly review differentiate an innocent murmur stage of the pregnancy.
by a dietitian) and statin therapy. from one that suggests underlying
It is only after addressing these pathology (Table 7). Can you Ask some general questions.
issues that the choice of OCP or reassure the patient or, if there is a • Have you been getting out of
breath more easily?
TABLE 7 DIFFERENTIAL DIAGNOSIS OF A SYSTOLIC MURMUR
• How far can you walk?
DURING PREGNANCY
• Have you woken up breathless at
Comment Diagnosis night?

Common Innocent systolic murmur • Have you had any chest pains?
Mitral valve prolapse
• Have you had any palpitations,
Must consider Mitral valve disease (regurgitation/mixed/stenosis with tricuspid
regurgitation) when your heart seems to beat
Aortic valve disease (stenosis/mixed/regurgitation with flow murmur) with an unusual rhythm?
HCM
Atrial or ventricular septal defect • When do you get them?
Other causes Peripartum cardiomyopathy
• Have you experienced any
HCM, hypertrophic cardiomyopathy. blackouts? What were you doing
at the time?

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›Fig. 15 ECG: axis shift in pregnant woman.

• Do you ever feel as if you are After explaining to the patient that an innocent murmur: they, and
going to pass out? under normal clinical circumstances other doctors involved in their care,
you would examine her to confirm can be reassured. For information
Other relevant history the clinical findings as stated on the regarding other conditions, see the
Enquire about any previous referral letter, you would plan as relevant sections in Part 2 of this
cardiac history. Occasionally, follows. module.
a minor abnormality will have
Further review for innocent
been documented in infancy or ECG
murmurs is not usually required.
childhood, and the patient may Minor flattening of T waves or
Patients with structural heart
have been told that she has a axis shift (Fig. 15) are common in
disease will need further input in
murmur, ‘hole’ or ‘sound’ in her normal pregnancy, as are sinus
liaison with the obstetric team. The
heart. Ask about any difficulties tachycardia and ectopic beats. Look
timing and frequency of follow-up
before the pregnancy that might also for evidence of atrial enlargement,
will depend on the nature and
suggest a congenital abnormality. left or right ventricular hypertrophy,
severity of the structural
Also ask about any heart problems or conduction abnormalities.
abnormality.
in other family members: a history
of sudden death at a young age Chest radiograph
Further discussion
might raise the possibility of a This is best avoided (because of the
Congenital heart disease in
hereditary cardiomyopathy or radiation dose) unless there are clear
pregnancy is the third commonest
Marfan’s syndrome. clinical indications.
cause of maternal death. It will
Plan for investigation and become an increasing problem as
Echocardiography
management more patients survive with complex
This is the investigation of choice
congenital cardiac abnormalities:
to confirm or rule out cardiac
the recurrence rate for most
pathology (Fig. 16).
(non-syndromic) congenital
Avoid radiation exposure in abnormalities in the offspring is 5%.
pregnancy unless there is a
Management
pressing clinical need. Management will depend on the Mild lesions may get worse or
diagnosis. Most patients will have ventricular function may deteriorate

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prevent endocarditis. Warfarin


is teratogenic; hence special
arrangements (usually conversion
to low-molecular-weight heparin)
are required for those who need
anticoagulation during pregnancy.

1.2 Clinical examination

1.2.1 Irregular pulse

Instruction

This woman has had palpitations.


Please examine her heart.

General features
Your approach to this case will
be aimed at identifying any
systemic condition that may cause
palpitations and identifying whether
there is any specific cardiovascular
abnormality present.

Look for thyrotoxicosis or anaemia.


Consider the conditions associated
with atrial fibrillation (AF) (Table 9).
Are there any general features
that would support one of these
›Fig. 16 (a) Mitral valve prolapse: in the two-dimensional image on the left, the anterior mitral valve
leaflet is seen to bow into the left atrium. The effect of this can be seen in the colour-flow mapping in the diagnoses? Look for surgical
image on the right: a broad regurgitant jet can be seen. (b) Ventricular septal defect: in these parasternal
long- and short-axis views, a small jet of orange colour represents the abnormal blood flow across the
scars (eg thoracotomy or
septum from the left to right ventricle. LA, left atrium; LV, left ventricle; RV, right ventricle; Ao, aorta. pacemaker/implantable
(Courtesy of Dr J. Chambers.)

TABLE 8 RISKS OF PRE-EXISTING HEART DISEASE IN PREGNANCY


as pregnancy progresses, and hence
careful monitoring with specialist Level of Risk Condition
input is necessary. In some patients
careful consideration will need to High Pulmonary hypertension
Mitral stenosis
be given to advising termination Aortic and pulmonary stenosis
of pregnancy on medical grounds; Marfan’s syndrome
when the current pregnancy is Intermediate Coarctation of aorta
over, patients with high-risk HCM
and intermediate cardiac lesions Cyanotic congenital heart disease without pulmonary hypertension
should be offered advice about Low Well-tolerated valvular regurgitation
Septal defects without pulmonary hypertension
contraception (Table 8).
Totally corrected congenital heart disease
Prosthetic valves
Patients with significant cardiac
lesions undergoing vaginal delivery HCM, hypertrophic cardiomyopathy.
require antibiotic prophylaxis to

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to confirm the diagnosis of an


TABLE 9 CAUSES OF AF arrhythmia an ECG will clearly be
the first investigation to perform.
System Cause If the pulse is rapid and regular,
Cardiac Hypertension then it suggests sinus tachycardia,
Ischaemic heart disease supraventricular tachycardia (SVT)
Non-ischaemic cardiomyopathy or ventricular tachycardia (VT).
MV disease
In a formal examination such as
Pericardial disease
Endocarditis PACES it is highly improbable that
Atrial myxoma the patient will be in VT, although
Respiratory Chest infection1 SVT is possible, eg atrial flutter with
Pulmonary infarction a pulse of 150 bpm. If the pulse is
Bronchial carcinoma
irregularly irregular, then it is either
Other Hyperthyroidism AF or sinus rhythm with frequent
Alcohol
Haemochromatosis ectopics. A regularly irregular
Sarcoidosis rhythm will either be sinus rhythm
Recreational drug use with regular ectopic beats (eg
ventricular trigeminy) or heart
Notes: common causes in bold; 1common in routine clinical practice but not in PACES.
AF, atrial fibrillation; MV, mitral value. block (eg Wenckebach).
Patients with structural cardiac
abnormalities are more likely
cardioverter defibrillator), Heart sounds to have arrhythmias: specific
evidence of hypercholesterolaemia Careful auscultation should reveal conditions to look for are impaired
(xanthelasma or tendon xanthomata) any valve abnormalities. Particular LV function, valvular abnormalities
and the characteristic skin colour of focus should be on the mitral valve and cardiomyopathies. Many
chronic amiodarone use (Fig. 17). as there is an increased incidence cardiomyopathies may just have
of AF with mitral stenosis or features of impaired left or right
Cardiovascular examination regurgitation. Mitral regurgitation ventricular function, but some
Key points to look for include the is common in patients with impaired may have very specific findings,
following. dilated left ventricles. Is there eg HCM (Table 10). A CXR and
an ejection systolic murmur echocardiogram would clearly be
Pulse that might suggest hypertrophic the first-line investigations to look
Check rate and rhythm. Is there an cardiomyopathy (HCM) (Table 10)? for structural cardiac lesions.
arrhythmia now? Is the pulse small
volume suggestive of low cardiac
Further discussion Remember that in routine
An irregular pulse may obviously clinical practice many patients
output? Are there alternate large-
be the cause of her symptoms, and who complain of palpitations do
and small-amplitude beats
(alternans) suggestive of impaired
left ventricular (LV) function? Is the
TABLE 10 CLINICAL FEATURES OF HCM
radial pulse absent? This may be the
Pulse ‘Jerky’: brisk rising and declines in mid-systole (as gradient develops)
case with congenital abnormalities,
arterial embolism, and Blalock shunt JVP Prominent a wave (reduced right ventricular compliance due to massive
or those used as surgical conduits. hypertrophy of septum)
Apex beat May be displaced laterally and often forceful. Presystolic impulse may
produce a ‘double’ apical beat
Signs of heart failure
Heart sounds Fourth heart sound precedes first sound (corresponds to presystolic impulse)
Look for evidence of congestive
Murmurs Harsh crescendo–decrescendo murmur heard after the first heart sound at
heart failure or tricuspid the left sternal edge (flow over obstructed outflow tract). Tends to radiate to
regurgitation by examining the JVP. axilla rather than the carotids.
Is the apex beat displaced? What is With severe gradients there may be an associated murmur of mitral
regurgitation. Approximately 10% of cases will also have aortic regurgitation
the character of the apex beat? Is
there evidence of pulmonary oedema HCM, hypertrophic cardiomyopathy
or right-sided heart failure?

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not have a pathological arrhythmia:


anxiety can lead to an increased
awareness of sinus rhythm or
normal ectopic beats.

1.2.2 Congestive heart failure

Instruction

This 78-year-old man has a


6-week history of progressive
breathlessness, orthopnoea and
swollen ankles. Please examine
his cardiovascular system.

General features
Look for cyanosis, anaemia,
stigmata of chronic liver disease
or nicotine-stained fingers.

Are there any features to suggest


previous cardiac interventions, such
as a sternotomy scar or presence of
(a) pacemaker?

Although this is not a respiratory


station, note the shape of the chest
and whether it seems to expand
normally as the patient breathes.
Do appearances suggest chronic
airways disease? If they do, and
particularly if you find signs other
than basal crackles when you
listen to the patient’s lungs, then a
respiratory cause of breathlessness
and oedema is likely (consider right
heart failure of cor pulmonale).

Expose both arms fully to ensure


that there are no fistulae present.

Oedema of the hands and face


is a feature of hypoalbuminaemia
and very rarely the result of congestive
cardiac failure (Fig. 18).

Cardiovascular examination
(b)
Key points to look for during the
cardiovascular examination include
›Fig. 17 Typical slate-grey skin coloration associated with long-term amiodarone treatment. the following.

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finding a constellation of signs


makes left ventricular impairment
much more likely. The diagnosis
should always be confirmed by an
objective assessment of cardiac
function, generally in the form of an
echocardiogram. Along with an ECG
and CXR, this would be the first-line
investigations to request in this case.

The following are important factors


when considering the case of a
patient with possible congestive
cardiac failure.

• The presence of bilateral basal


›Fig. 18 Hand oedema in a patient with hypoalbuminaemia.
crackles on auscultation of the
chest has a very poor positive
Signs of cardiac dysfunction Signs of pulmonary hypertension predictive value for the presence
This is suggested by the following. of pulmonary oedema; a CXR is
• Pulse: rate, rhythm, volume and
character. A sinus tachycardia may • Raised JVP. much more accurate.
be caused by anxiety or cardiac • Left parasternal heave (palpable • Remember that marked abnormality
failure; consider specifically atrial right ventricle). of renal and liver function tests
fibrillation (AF). A low-volume commonly occurs in cases of
• Loud pulmonary component of
pulse might indicate cardiac congestive cardiac failure, and
the second heart sound.
failure or alternatively severe does not necessarily indicate
mitral regurgitation, but is there Also note whether one leg is much primary disease in these organs.
anything about the character to more swollen than the other, which
• The severity of left ventricular
suggest either aortic stenosis or might indicate deep venous
dysfunction on echocardiography
incompetence? thrombosis.
correlates poorly with the severity
• BP, including pulsus paradoxus, of the clinical syndrome of heart
which suggests pericardial failure, but normal systolic
Examination of the JVP
effusion/tamponade. left ventricular function on
• Do not finish your
echocardiography should prompt
• JVP: is this raised? Check for the examination until you have found
out where the JVP is. Make sure that a review of a diagnosis of heart
features of JVP, including the
you correctly position the patient, failure.
effect of respiration (eg increasing ensuring that he or she is at 45° with
with inspiration in constrictive the head well supported by a pillow,
thereby relaxing the neck muscles.
pericarditis and cv waves in
Good lighting will help. Look for
tricupsid regurgitation). When the cause of
position, waveform characteristics (if
breathlessness is not obvious,
in AF, then large waves must be v
consider chronic repeated
• Apex beat: is this displaced? Is it waves) and the effect of respiration.
pulmonary thromboembolism, which
hyperdynamic, in keeping with a • Remember that it is possible to miss
is a commonly missed diagnosis.
a markedly elevated JVP: if you
volume-overloaded left ventricle Multiple small pulmonary emboli lead
cannot see it and the neck appears
(eg mitral or aortic incompetence)? to progressive occlusion of the
‘full’, then look again when the
pulmonary arteriolar bed, classically
patient is sitting up at 90°.
presenting with breathlessness that
• Heart sounds: is there an S3
becomes more severe in a stepwise
gallop? Are there murmurs manner. Pulmonary hypertension
(especially diastolic)?
Further discussion eventually leads to right ventricular
Clinical diagnosis of heart failure failure and ankle swelling. Prominent
• Lungs: are there basal crepitations? can be difficult. Although any pulmonary arteries may be the only
finding on a CXR. Diagnosis is made by
Remember that these are not individual sign (eg elevated JVP)
lung ventilation–perfusion scan or
specific for pulmonary oedema has a relatively low positive spiral CT scan of the chest.
and cardiac failure. predictive value for the diagnosis,

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1.2.3 Hypertension for any evidence of left ventricular may be placed on the subsequent
hypertrophy or heart failure. treatment of the patient’s
Instruction • Peripheral pulses: examine
hypertension, including lifestyle
changes, risk factor modification
carefully, in particular for the
Please examine the and medical management
radiofemoral delay of coarctation
cardiovascular system of this (see Section 2.17).
of the aorta. Also assess the
woman with hypertension.
presence and volume of all pulses
1.2.4 Mechanical valve
as the patient may have peripheral
vascular disease.
Instruction
General features
• Abdomen: feel for an abdominal
The main objectives of the
aortic aneursym and listen over This man has had cardiac
examination are to assess for
the renal arteries for bruits. surgery. Please examine his
evidence of organ damage secondary
cardiovascular system.
to hypertension and to uncover a • Fundi: offer to examine for
secondary cause of hypertension. evidence of hypertensive
retinopathy (again this will
Comment on the patient’s general
almost certainly by declined by General features
appearance and particularly on
the examiners in Station 3 as From the foot of the bed, can you
any obvious appearances that
fundoscopy is frequently hear the characteristic clicking
might indicate a secondary cause
performed in Station 5). sound of a ball-and-cage valve?
of hypertension, eg obvious
Look for pallor or jaundice, which
cushingoid appearance or features
may be caused by haemolysis from a
of acromegaly (unlikely to be in the
failing valve. Also check for bruising,
cardiac station of PACES; more When examining a patient
which could suggest problems with
likely to appear in Station 5). with hypertension, consider
the following. anticoagulation use. Aside from the
scar of cardiac surgery, look for
Cardiovascular examination • Essential hypertension: if the patient
scars on the upper chest suggestive
Pay particular attention to the has elevated BP, evidence of left
ventricular hypertrophy or of a pacemaker (atrioventricular
following.
hypertensive retinopathy and block is more common following
• BP: in a scenario such as this you evidence of secondary causes is aortic valve surgery) and on the
should obviously offer to measure absent. legs suggestive of vein harvest for
• Isolated clinic hypertension: look for
the patient’s BP, but it is unlikely coronary artery bypass grafting.
anxiety, eg tachycardia. Is the BP
that the examiners will actually lower when measured by the clinic
Remember also to look for a mitral
allow you to do so because of the nurse or the GP, or at place of work valvotomy under the left breast.
time constraints of the station. or at home? There must be no Does the patient have any
However, if they do, make sure evidence of target organ damage. phenotypic features of conditions
you do it properly. The patient • Renovascular disease: are there associated with aortic valve
abdominal bruits, abdominal aortic
is likely to have been lying on pathology, eg Marfan’s syndrome?
aneurysm or other evidence of
a couch for some time, but atherosclerosis?
remember that they should be • Coarctation: look for absent femoral Cardiovascular examination
recumbent for at least 3 minutes pulses and radiofemoral delay, The following are the key points
before taking a reading and that collaterals in the back muscles and a to look for in the cardiovascular
widespread systolic murmur heard
the arm should be supported examination.
best over the back.
at the level of the heart when
• Pulse: check rate and rhythm.
making the measurement. Make
Is the patient in sinus rhythm?
sure you use an appropriately
Further discussion Atrial fibrillation is very common
sized cuff and offer to measure
You should be able to discuss the after cardiac surgery. A collapsing
the BP in both arms (which will
secondary causes of hypertension pulse suggests significant aortic
almost certainly be declined).
and in particular any obvious cause regurgitation and valve failure.
• JVP, apex beat, heaves, heart that you may have elicited from the A slow rising pulse suggests
sounds and lungs: examine these examination. Particular emphasis valvular stenosis.

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and antibiotic prophylaxis as


TABLE 11 TYPES OF PROSTHETIC HEART VALVE detailed in the British National
Formulary is mandatory.
Mechanical Biological
Haemolysis from mechanical
Ball and cage (Starr–Edwards) Porcine (Carpentier–Edwards) valves is more common than from
Single disc (Björk–Shiley or Medtronic Hall) Pericardial bioprosthetic valves. It may be acute
Bileaflet (St Jude or Carbomedics) Homograft or chronic, and may be related
Autologous (pulmonary autograft) to valve failure. Presentation is
typically with anaemia and mild
jaundice. Blood films show a
• BP: a wide pulse pressure suggests Further discussion
microangiopathic haemolytic picture
aortic regurgitation and a narrow Prosthetic aortic or mitral valves
(see Haematology, Section 2.1.7).
pulse pressure outflow tract can be mechanical or biological
Infective endocarditis must be
obstruction (offer to check this (Table 11).
excluded. Management may vary
at the end of your examination).
from regular transfusions to repeat
• Signs of congestive cardiac valve surgery.
failure: an elevated JVP, All patients with mechanical
Replacement of a prosthetic
displaced or prominent apex beat, valves require:
valve should only be considered in
parasternal heave, added heart • lifelong anticoagulation; patients who are symptomatic with
sounds, basal crackles and ankle • prophylaxis against endocarditis.
objective evidence of valve failure.
swelling may be a result of
It is a high-risk procedure and may
prosthetic valve failure.
not provide a better outcome than
All mechanical heart valves need
• Mechanical aortic valve: if an expectant approach in both the
anticoagulation to prevent valve
present the second heart sound elderly and those with significant
thrombosis and the resulting
will be prosthetic and loud. There comorbidity.
complications. However, despite the
will always be abnormal forward
best anticoagulation control, the
flow with a mechanical valve 1.2.5 Pansystolic murmur
incidence of systemic emboli is 1%
and therefore an ejection systolic
per patient-year. The recommended
murmur will be present, the Instruction
INR is 2.0 –3.0 for bileaflet valves
intensity of which has no bearing
and 2.5–3.5 for other disc and
on the function (or dysfunction) This man has a murmur. Please
Starr–Edwards valves.
of the valve. Listen carefully for an examine his cardiovascular
early diastolic murmur suggestive Endocarditis is a feared system.
of valve failure and remember that complication of all prosthetic
a shorter duration of the diastolic valves. The greatest risk of infection
murmur indicates severe is immediately following surgery: General features
regurgitation. from 12 months onwards the annual Comment on the patient’s general
incidence is 0.4%. The causative appearance and in particular
• Mechanical mitral valve: if
organisms are most likely to be if he appears short of breath at
present the first heart sound will
coagulase-negative staphylococci rest, cyanosed or has a phenotype
be prosthetic and a diastolic flow
and Staphylococcus aureus in suggesting a particular valvular
murmur may be heard. However,
the early period (see Section 2.8, abnormality, eg Marfan’s syndrome
these are not typically as loud as the
Table 34). The mortality from (this can be associated with mitral
prosthetic sound and flow murmur
prosthetic valve endocarditis regurgitation as well as aortic
associated with a mechanical aortic
is 60% and the condition is difficult regurgitation). Look carefully
valve. A systolic mitral regurgitant
to treat with medical therapy for surgical scars, remembering
murmur may be due to a
alone; hence urgent referral to a especially that the left thoracotomy
prosthetic or paraprosthetic leak.
cardiothoracic surgical centre is scar of mitral valvotomy is easy to
• Biological valves: these do not required. To prevent endocarditis, miss (especially in women, when
produce the harsh metallic sounds dental care must be meticulous in it can be hidden under the fold of
of mechanical valves. patients with prosthetic heart valves the breast).

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Cardiovascular examination Further discussion 1.2.6 Mitral stenosis


Check for stigmata of endocarditis. The commonest causes of mitral
Pay attention to dental hygiene. regurgitation are mitral valve Instruction
Check if the patient is in atrial prolapse and ischaemic heart
fibrillation. Look for signs of disease. Other causes are rheumatic This woman has had increasing
heart failure, particularly heart disease, previous mitral shortness of breath over the past
elevation of JVP and displacement valvotomy and dilated 6 months. Please examine her
of the apex. Do not forget to cardiomyopathy. cardiovascular system.
examine for a parasternal heave
suggesting pulmonary hypertension. Features of the examination
Remember the following when that would suggest severe mitral
regurgitation include a third General features
trying to decide the cause of the
heart sound, displaced apex Comment on the patient’s general
pansystolic murmur.
beat, signs of heart failure and well-being and in particular if she is
• Mitral regurgitation: a thrusting signs of pulmonary hypertension. short of breath at rest or cyanosed.
displaced apex beat suggests An echocardiogram will confirm Look for surgical scars, particularly
volume overload of the left the diagnosis and (probably) a mitral valvotomy under the left
ventricle, which means that the aetiology, as well as the severity, breast. Does the patient have a
murmur is probably mitral. The by giving information about the malar flush?
murmur will typically be loudest haemodynamic consequences of the
in expiration, most prominent at mitral leak. This is particularly the Cardiovascular examination
the apex and radiate to the axilla. case for left ventricular dilatation, Check for stigmata of endocarditis.
A soft first heart sound and loud impaired left ventricular systolic Pay attention to dental hygiene. In
third sound would support the function and pulmonary mitral stenosis (MS) the following
diagnosis. hypertension. may be seen.

• Ventricular septal defect (VSD): Patients with mild or moderate • Pulse: atrial fibrillation (AF) is
you should suspect this condition disease should be reviewed annually very common in MS.
if the murmur is loudest in in a cardiac clinic. All patients • Signs of heart failure: elevated
inspiration and best heard should be given advice regarding JVP and giant v waves due to
over the lower left sternal edge. antibiotic prophylaxis against secondary tricuspid incompetence
In cases of VSD the apex is endocarditis. Many patients with (also hepatomegaly, ascites and
undisplaced, the first sound mitral regurgitation will require ankle oedema).
normal and no third heart lifelong anticoagulation with
sound is heard. warfarin. • Apex beat: tapping (palpable first
heart sound) that is not displaced.
• Tricuspid regurgitation:
Surgery is indicated in those
typically the murmur is loudest • Parasternal heave: suggests
with severe mitral regurgitation
over the lower left sternal edge pulmonary hypertension.
and symptoms. For asymptomatic
during inspiration. Giant v
cases, surgery is recommended if • Heart sounds: the first is loud,
waves will be present and a
there is evidence of left ventricular then there is a loud pulmonary
pulsatile liver edge. The apex is
dilatation, impaired left ventricular second sound and an opening
not displaced and a third sound
systolic function or pulmonary snap followed by a mid-diastolic
not heard.
hypertension. The outcome is rumbling murmur (with
generally better if the mitral presystolic accentuation if
valve can be repaired rather the patient is in sinus rhythm)
than replaced, but suitability localised to the apex and heard
for repair will depend on the loudest in expiration with the
Differential diagnosis of a
pansystolic murmur: complexity of the valvular patient in the left lateral position.
disease. Coronary angiography A Graham Steell early diastolic
• mitral regurgitation;
is required prior to surgery to murmur due to secondary
• VSD;
• tricuspid regurgitation. look for coexistent coronary pulmonary regurgitation
artery disease. may be heard.

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Further discussion general, mechanical and not tissue ejection systolic murmur in
By far the commonest cause of MV prostheses are required (see the aortic region, radiating to
MS is rheumatic heart disease. Table 11). Coronary angiography is the neck and loudest in expiration.
The murmur of MS may be difficult required prior to surgery to look for Typically the murmur quietens
to hear, so be alert for clues prior coexistent coronary artery disease. across the precordium and
to auscultation. If a patient in becomes loud again at the apex
PACES is in AF and their face looks 1.2.7 Aortic stenosis (Galliverdin’s sign). First heart
as though it has a malar flush, then sound may be normal or soft.
MS is much more likely to be the Instruction Second heart sound may be
diagnosis than it might be in routine single (there is no aortic
clinical practice. Note that the This woman has chest tightness component from a calcified
murmur of MS is accentuated on effort. Please examine her aortic valve that does not move)
with exercise, but tachycardia cardiovascular system. or with reversed splitting due
may make it more difficult to hear. to delayed aortic valve closure
The presence of an opening snap (if the aortic valve cusps are still
suggests the mitral valve (MV) is still mobile). Fourth heart sound may
General features
pliant. The closer the murmur is to be present.
Comment on the patient’s general
the second heart sound, the more
well-being and in particular if In late presentation, classic signs
severe the stenosis.
she is short of breath at rest or may lessen and left ventricular
Transthoracic echocardiography cyanosed. Look carefully for failure and secondary pulmonary
confirms the diagnosis and allows surgical scars. hypertension dominate.
an assessment of severity
(see Section 2.5.3). Cardiovascular examination
Check for stigmata of endocarditis.
Patients with mild or moderate In aortic stenosis the murmur
Pay attention to dental hygiene.
is not a guide to severity – look
disease should be reviewed annually Check for signs of heart failure, for clinical signs that reflect the
in a cardiac clinic. All patients noting particularly that in cases of haemodynamic significance.
should be given advice regarding aortic stenosis the following may be
antibiotic prophylaxis against observed.
endocarditis. All patients with MS
require lifelong anticoagulation • Pulse: this will be regular, slow
Further discussion
with warfarin unless there are rising and small volume due to
The differential diagnosis of
very pressing contraindications. narrow pulse pressure. Reduced
aortic stenosis include the
arterial compliance in older
following.
Surgery is indicated for severe patients may negate these
MS with limiting symptoms, findings. Atrial fibrillation is • Innocent systolic murmur, eg
embolic events or an episode less common than with mitral aortic sclerosis.
of pulmonary oedema. If this valve disease.
• Pulmonary stenosis: dominant
is planned, a transoesophageal
• BP: narrow pulse pressure. indications are a murmur loudest
echocardiogram should be
in inspiration, palpable right
performed to assess the degree • JVP: prominent a wave.
ventricular heave and signs
of valve calcification, to check for
• Apex: this is usually undisplaced of right heart failure. Usually
the presence of mitral incompetence
and heaving; it may have a double congenital in origin, eg tetralogy
and to examine for thrombus in
beat due to additional left atrial of Fallot. Always consider in a
the left atrial appendage. Patients
impulse. Displacement suggests cardiac patient who looks
with minimal MV calcification
left ventricular dilatation. A cyanosed.
(no more than mild mitral
systolic thrill may be palpable
regurgitation and no left atrial • Hypertrophic cardiomyopathy
over the aortic region and
appendage thrombus) should be (HCM): if you suspect this look
carotids.
considered for percutaneous mitral for jerky impulse and a double
valvotomy. Otherwise, the patient • Heart sounds: the dominant apex beat. Patients with HCM
requires MV replacement. In feature is likely to be a harsh are often young.

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pregnant in the future. In the latter • Pulse: this would be regular,


situation patients will often elect to collapsing in nature and large
Features present in aortic have a tissue valve: this eliminates volume. Atrial fibrillation is less
stenosis that would not be
the need for teratogenic warfarin common than with mitral valve
expected in aortic sclerosis include:
during their childbearing years but disease.
• low pulse pressure;
accepts that valve replacement will
• slow rising pulse; • BP: wide pulse pressure.
need to be performed again at a
• carotid thrill; This may be associated with a
• radiation of murmur to neck; later date.
number of eponymous signs
• abnormal heart sounds;
(Table 12).
• forceful/displaced apex (unless there 1.2.8 Aortic regurgitation
is another possible explanation, eg
hypertension). • Apex: thrusting and displaced
Instruction (volume overload). A systolic
thrill may be palpable over
This woman is short of breath. the aortic region and
Symptoms of aortic stenosis Please examine her carotids.
include angina, shortness of cardiovascular system.
breath and syncope. Transthoracic • Heart sounds: the dominant
echocardiography confirms finding is an early diastolic
the diagnosis and enables an General features murmur best heard over the
assessment of severity (see Comment on the patient’s general lower left sternal edge during
Section 2.5.1). Note, however, well-being and in particular if she is expiration while the patient is
that the aortic valve gradient short of breath at rest or cyanosed. sitting forward. There is almost
will be underestimated in patients Look for previous surgical scars. always an accompanying ejection
with heart failure, so a dynamic Look for a marfanoid habitus or systolic murmur. An Austin
assessment with dobutamine stress features of arthropathy, especially Flint murmur, which needs to be
may be required in this situation. ankylosing spondylitis. distinguished from the murmur
Coronary angiography is required of mitral stenosis, is a rumbling
prior to surgery to look for Cardiovascular examination mid-diastolic murmur caused by
coexistent coronary artery Check for stigmata of endocarditis. the aortic regurgitant jet hitting
disease. Pay attention to dental hygiene. the mitral valve leaflets. The
Check for signs of heart failure, second heart sound is single
Patients with mild or moderate
noting particularly that in aortic (no aortic component), but P2
disease should be reviewed annually
regurgitation (AR) the following may be loud. The third heart
in a cardiac clinic. All patients
may be observed. sound may be heard.
should be given advice regarding
antibiotic prophylaxis against
endocarditis.

All patients with symptoms due to TABLE 12 CLINICAL SIGNS OF THE WIDE PULSE PRESSURE
aortic stenosis require aortic valve SEEN IN AORTIC REGURGITATION
replacement because the prognosis
is poor for sufferers who remain Sign Clinical observation
untreated. In particular, the onset
of heart failure is a very poor De Musset’s Head nods with each pulsation
prognostic sign and such patients Quincke’s Capillary pulsation visible in nail beds
should be considered for urgent Duroziez’s Double ‘to-fro’ (systolic and diastolic) murmur heard over femoral
valve replacement. Mechanical arteries when auscultation with firm pressure from stethoscope
valve prostheses (see Table 11) Corrigan’s Visible carotid neck pulsations
are generally preferred unless the Müller’s Pulsating uvula
patient is elderly, increased risks Hill’s Pistol shot sounds over femoral arteries when auscultation with
of bleeding on anticoagulation are light pressure from stethoscope
present, or the patient is a young Traube’s Pistol shot sounds over femoral arteries
woman who wishes to become

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Further discussion wishes to become pregnant in inspiration and typically most


the future. In the latter situation prominent at the lower left
patients will often elect to have a sternal edge. A loud pulmonary
tissue valve: this eliminates the need component of the second heart
Causes and associations of AR
for teratogenic warfarin during their sound would suggest pulmonary
Chronic: childbearing years but accepts that a hypertension.
valve replacement will need to be
• Bicuspid aortic valve. • Other signs: palpable pulsatile
performed again at a later date.
• Marfan’s syndrome. liver, sacral and peripheral
• Infective endocarditis. In acute AR there is often no oedema. Look for evidence of
• Arthritides – ankylosing spondylitis,
murmur due to very rapid chronic lung disease as TR may
rheumatoid and Reiter’s syndrome.
• Hypertension. equalisation of pressures between be caused by cor pulmonale.
• Syphilis. the aorta and the left ventricle in
early diastole. The only murmur Further discussion
Acute: that sounds like AR is pulmonary
• Dissection of aorta. regurgitation, which can be
• Infective endocarditis. distinguished because it is louder in
• Failure of prosthetic valve. inspiration and usually associated Causes of TR
• Acute rheumatic fever.
with signs of right heart • Right ventricular (RV)
compromise. dilatation due to RV failure:
(a) Mitral valve disease.
Symptoms of AR include angina, (b) Pulmonary hypertension.
1.2.9 Tricuspid regurgitation
shortness of breath and lethargy. (c) Intracardiac shunt.
Transthoracic echocardiography (d) RV infarction.
Instruction • Infective endocarditis (intravenous
confirms the diagnosis (see
drug abuse).
Section 2.5.2), may reveal the
This man has a murmur. Please • Carcinoid syndrome.
aetiology and enables assessment • Congenital, eg Ebstein’s anomaly.
examine his cardiovascular
of severity. If there is a suggestion • Trauma.
system.
of significant dilatation of the • Myxomatous change.
proximal aorta from CXR or
echocardiography, a cardiac MRI
General features
scan should be considered. Coronary The character of the JVP establishes
Comment on the patient’s general
angiography is required prior to the presence of TR, but the examiner
well-being and in particular if short
surgery to look for coexistent will expect you to be able to discuss
of breath at rest or cyanosed. Look
coronary artery disease. the differential diagnosis of a
for previous scars on the chest and
pansystolic murmur: mitral
Patients with mild or moderate more widely over the skin for
regurgitation, ventricular septal
disease should be reviewed annually evidence of intravenous drug abuse.
defect and TR (see Section 1.2.5).
in a cardiac clinic. All patients should
be given advice regarding antibiotic Cardiovascular examination An echocardiogram will confirm the
prophylaxis against endocarditis. Check for stigmata of endocarditis. diagnosis and severity of TR. It is
Pay attention to dental hygiene. possible to estimate the pulmonary
Asymptomatic patients with severe
Check if the patient is in atrial artery pressure from the velocity
AR should be considered for surgery
fibrillation. Note that in tricuspid of the tricuspid regurgitant jet.
if there is evidence of declining left
regurgitation (TR), the following If pulmonary hypertension is
ventricular systolic function or left
may be observed. suspected to be the cause of TR,
ventricular dilatation. The onset of
then CXR, lung function tests and
heart failure is a poor prognostic • Signs of heart failure: JVP
lung ventilation–perfusion scanning
sign. Mechanical valve prostheses elevated with giant v waves.
(or spiral CT) should be pursued.
(see Table 11) are generally preferred
• Apex: undisplaced and with
unless the patient is elderly, Surgery is rarely indicated, even
parasternal heave.
increased risks of bleeding on for severe TR. However, when the
anticoagulation are present, or the • Heart sounds: a pansystolic cause is endocarditis it should be
patient is a young woman who murmur that is loudest in considered if there is a large

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vegetation (>1 cm), persistent • JVP: this will always be significantly • Counselling: this can include
sepsis despite the patient taking raised. Are there flutter waves? advice regarding pregnancy and
antibiotics, or evidence of delivery risks for both mother and
• RV heave.
embolisation. In patients undergoing fetus. Contraceptive advice is also
mitral valve surgery, tricuspid • Listen for RV gallop rhythm and important. In a case where the
annuloplasty is sometimes loud P2. patient is pregnant, the early
performed in the presence of opinion of a fetal medicine
• Pulmonary or tricuspid
severe TR and dilatation of the obstetrician and congenital heart
regurgitation.
annulus (>5.0 cm). All patients disease specialist are vital.
should be given advice concerning • Ankle oedema.
• Antibiotic prophylaxis: patients
antibiotic prophylaxis against
Once Eisenmenger’s syndrome with shunts need antibiotic
endocarditis.
has developed, the murmur of the prophylaxis prior to dental
original shunt will have disappeared. procedures or other
1.2.10 Eisenmenger’s
instrumentation.
syndrome
Further discussion
Early detection and closure of
Eisenmenger’s syndrome is a
Instruction haemodynamically significant left-to-
clinical diagnosis aided by ECG
right shunts is important in order
(particularly for RV hypertrophy),
This woman has become to prevent Eisenmenger’s syndrome
CXR (for cases of prominent
breathless on minimal exertion. from developing. Other options
pulmonary arteries and peripheral
A doctor noted that she had a include pulmonary artery banding to
pruning), echocardiography
murmur as a child. Please limit the flow to the lungs and prevent
and cardiac catheterisation.
examine her heart. the development of pulmonary
Echocardiography enables the
hypertension. When Eisenmenger’s
shunt to be visualised and an
syndrome is established, the 10-year
assessment of RV pressure to be
General features survival rate is 80% and the 25-year
made.
A large left-to-right shunt causes survival rate 40%. Poor prognostic
increased pulmonary blood flow, Optimal treatment of patients features include syncope, low cardiac
which in turn causes increased with Eisenmenger’s syndrome output, hypoxaemia and RV failure.
pulmonary vascular resistance and is provided by a congenital heart
right ventricular (RV) hypertrophy. disease specialist service, and may
Eventually the pulmonary resistance involve the following.
Patients with Eisenmenger’s
exceeds the systemic resistance, and syndrome should be told to
• Continuous oxygen, which acts as
the blood flow is reversed causing a avoid volume depletion, systemic
a vasodilator.
right-to-left shunt with resulting vasodilators, altitude, heavy exertion
cyanosis. • Aspirin for patients with and pregnancy. They should also be
polycythaemia to reduce advised to take antibiotics before
Look for cyanosis and evidence of dental or other procedures.
the risk of stroke.
stroke in a young person. Is there a
sputum pot? Look specifically for • Venesection for symptomatic
haemoptysis. polycythaemia.

• Atrial arrhythmias: these are Who was Eisenmenger?


Cardiovascular examination In 1897 Dr Victor Eisenmenger
common but may be lethal and
Look specifically for the following. reported the case of a 32-year-old man
can often be treated with catheter
who had showed exercise intolerance,
• Cyanosis. ablation (see Section 3.4). cyanosis, heart failure and haemoptysis
prior to death. At post-mortem a
• Clubbing: seen more dramatically • Ventricular arrhythmias: patients
large ventricular septal defect and
in cyanotic congenital heart at high risk may require an an overriding aorta were found.
disease than in any other implantable cardioverter Eisenmenger described the link between
context. defribrillator (see Section 3.4). a large congenital cardiac shunt defect
and the development of pulmonary
• Pulse: atrial fibrillation or flutter • Transplantation is an option in hypertension for the first time.
are common. selected cases.

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1.2.11 Dextrocardia • Precordium: examine for thrills of TR? This is not an uncommon
and heaves. finding with complex congenital
Instruction conditions.
• Apex beat: identify the location
and nature of the apex beat. If
This patient has a congenital Further discussion
you cannot feel it in the normal
heart condition. Please examine This patient had the relatively
position, percuss the area of
his heart. rare condition of dextrocardia
cardiac dullness and remember
with no other associated cardiac
to feel the right side of the chest
abnormalities. Keeping an open
to identify dextrocardia.
mind would have led to a successful
General features • Heart sounds: careful outcome in this instance when the
This instruction raises many auscultation will reveal any added apex beat was difficult to feel and
possibilities, making general sounds/murmurs (Table 13). If you the heart sounds very quiet. This
inspection from the end of the bed suspect dextrocardia, auscultate was the only abnormality on
particularly important in this case. over the right side of the chest as examination and so the case would
Are there any obvious dysmorphic well as the left. easily confuse if the abnormal apex
features that may indicate a well- beat had been missed. There are
known congenital condition? Is • Signs of congestive heart increasing numbers of patients
the patient cyanosed, which may failure: is there any evidence with surgically corrected complex
indicate a cyanotic congenital heart of pulmonary oedema? Is there congenital conditions surviving to
lesion or Eisenmenger’s syndrome peripheral oedema or hepatic adulthood. With a methodical
(see Section 1.2.10)? Are there any enlargement, or a pulsatile liver approach to the examination it
obvious surgical scars? Look
carefully all over the torso.
TABLE 13 KEY CLINICAL SIGNS WITH CONGENITAL HEART DISEASE
Cardiovascular examination
Congenital condition Clinical signs
You will need to keep an open mind
as you approach this case and may Dextrocardia Quiet/absent sounds on left side of chest. Area of cardiac
need to focus on particular aspects dullness shifted. Apex felt on the right side
of the examination depending on Ventricular septal defect (VSD) Palpable thrill at left sternal edge. Loud pansystolic murmur
what you discover. Key points to Atrial septal defect (ASD) Wide fixed splitting of second heart sound (does not vary
look for include the following. with respiration) and soft ejection systolic murmur over
pulmonary area
• Pulse: check all peripheral pulses
Pulmonary stenosis (PS) Right ventricular (RV) heave and thrill in second right
to ensure that they are present space. Split second heart sound (not fixed) and systolic
and equal. Previous surgery may click may be heard
cause absent pulses, and Coarctation of the aorta Systemic hypertension, reduced lower limb or left arm
coarctation of the aorta or pulses and radiofemoral delay
stenoses may cause delayed or Surgically corrected Mustard or Senning operations are indicated by RV heave,
weakened pulses. Is the pulse transposition of the great single second heart sound and pansystolic murmur of TR.
arteries Switch-operation patients may have ejection systolic
irregular and/or tachycardic? murmurs of supravalvular PS or aortic stenosis
Atrial arrhythmias are very
Congenitally corrected Raised JVP and pansystolic murmur of TR. Signs of
common in patients with transposition systemic (right in this situation) ventricular dysfunction
congenital cardiac conditions,
Ebstein’s anomaly JVP often normal even with severe TR. First and second
especially if they have been heart sounds widely split. Often third and fourth heart
surgically corrected. sounds present
Eisenmenger’s syndrome Cyanosed and clubbed. Will have clinical features of
• JVP: this may be significantly
underlying shunt, ie ASD, VSD or patent ductus arteriosus,
elevated with right heart although these may not be apparent if the shunt has
conditions or pulmonary reversed
hypertension. Tricuspid
Note: some patients will have extensive surgical scars.
regurgitation (TR) may be TR, tricuspid regurgitation.
evident (giant v wave).

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should be possible to identify many Key issues to explore find relaxation tricks such as taking
of the clinical signs. It is not always What is the patient’s main a few deep breaths or lying down
necessary to obtain the exact concern? Why does she want can be helpful.
diagnosis, as the complexity of some further investigation? Does her
Patient: are there any tablets
of these cardiac conditions can be desire stem from the actual
that you can give me to help
exceptional. symptoms or the perceived risk
with them?
from the condition in view of her
family history? Doctor: there are drugs that can
help suppress the symptoms, but
Key points to establish these ectopic beats are, essentially,
1.3 Communication Reassure the patient that the a normal heart rhythm. We would
skills and ethics diagnosis of ectopics is certain, not generally advise patients to take
as her symptoms have been any medication unless absolutely
clearly correlated with ectopics necessary, because you can end up
1.3.1 Advising a patient
on the 24-hour ECG. Additional with more symptoms from the side
against unnecessary
reassurance is often provided effects of the medication than the
investigations
when patients understand that actual palpitations themselves. If
most people have ectopic beats you are desperate to take something
Scenario at some stage every day, the for these then beta-blockers may
majority of whom are unaware help. I can explain how they work
Role: you are a junior doctor in of them. Some people have a and what side effects they might
a cardiology outpatient clinic. lot more ectopics than others, cause.
but this does not signify anything
Patient: am I likely to die suddenly
Miss Jenny Pinto, aged 28 years, if the heart is normal. In this
like my relatives?
has been referred to the clinic case we know from investigations
for investigation of palpitations. that her heart is normal and Doctor: it is difficult to answer
She had previously not been further tests will add nothing this question without further
worried about these symptoms, to this. knowledge of exactly what was
but recent knowledge of the responsible for the deaths of your
It is important that the patient
deaths of two relatives following two relatives. However, we have
understands her symptoms are
sudden collapses has made her very carefully assessed your heart
not being dismissed. An explanation
very concerned. At her first and can find no problems that
that ectopic beats can be very
appointment it became clear would give us cause for concern
debilitating in some people can
from her history that the at all. I can certainly reassure you
reassure. Further, knowing the
palpitations were consistent that the palpitations will not cause
symptom is benign often leads to
with ventricular ectopic beats. you to die.
a significant improvement in the
Examination was normal,
degree of intensity and awareness Patient: I am really worried
as was a routine 12-lead ECG.
the patient feels. about these symptoms. Would
Echocardiography showed her
it be possible to have a second
heart to be normal and a
Appropriate responses to likely opinion?
24-hour ECG demonstrated
questions
ectopic beats when she was Doctor: of course you can.
Patient: what can I do to make them
symptomatic. She is keen to Either your GP or I can
go away?
have further investigations, but organise this for you, but
these would not be appropriate. Doctor: in many cases they will I would emphasise that all of
just settle down without needing to the investigations have been
Your task: to reassure Miss do anything. Some people find that reassuring and we know that
Pinto that her condition is they are worse after alcohol or after these ectopic beats, whilst
benign and explain that drinks containing caffeine. It might unpleasant, are not in any way
further investigations are be worthwhile trying to reduce your life-threatening, but if you’d like
not necessary. intake of these to see whether the to have a second opinion, then
symptoms improve. Other people I can help arrange this.

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1.3.2 Explanation of Reassure her that this is a common Doctor: I’m afraid that we can’t do
uncertainty of diagnosis presentation and that the vast them right away. Your husband
majority of syncopal episodes seems well now and when the
Scenario have a benign cause. Explain that consultant saw him earlier on we
sometimes an exact diagnosis is not agreed that we didn’t need to keep
Role: you are a junior doctor determined, and the importance of him in hospital and would do the
working on a general medical investigations is to rule out the more tests as an outpatient.
ward. serious causes for which there are
Wife: can he drive?
effective treatments rather than to
A 65-year-old man is admitted to pinpoint the specific cause. Doctor: not at the moment.
your ward from the Emergency However, if he has no recurrence
Department following an Appropriate responses to likely of his symptoms then he can return
unexplained syncope while questions to driving in 4 weeks (see
shopping. There have been Wife: what caused my husband to Section 2.19). However, if there
no previous episodes and since collapse? are any further symptoms then
his arrival on the ward he has he should await the results of his
Doctor: at the moment it is not
been alert and orientated with remaining investigations and clinic
possible to give an exact cause, but
normal observations. Physical review before recommencing
the most common cause of collapse
examination and investigations driving.
is a simple faint. We will make a
including ECG, CXR and blood
plan to do further tests, mainly to Wife: will a pacemaker help?
tests (including troponin at
rule out other causes.
12 hours after the collapse) Doctor: at this stage there is no
have been normal. His telemetry Wife: does this mean that this will evidence that a pacemaker would be
up to this point has shown no never happen again? helpful. The results of his tests will
abnormalities. The plan agreed help decide whether this needs to
Doctor: unfortunately there is
after consultant review on the be considered in the future.
no guarantee that the symptoms
ward round is to discharge him
will not reoccur, but the fact that Wife: what happens if he collapses
home, with arrangements for an
he is well now, that there are no again at home?
outpatient 24-hour tape and
abnormalities when we examine
echocardiogram. Doctor: as I’ve explained, we
him, and that the initial tests, the
don’t think that this is likely or
EGG (an electrical tracing of his
Your task: to explain to his wife we wouldn’t be suggesting that he
heart), a CXR (a chest X-ray) and
the uncertainty of the diagnosis goes home. If he does collapse, then
blood tests, are all normal, makes
and what the management plan – the same as if you or I were to
it less likely that there is a serious
is likely to be. collapse – you would need to call
underlying cause.
the doctor or an ambulance.
Wife: you said he needed more tests:
Key issues to explore what are these? 1.3.3 Discussion of the need
What is the wife’s current level to screen relatives for an
Doctor: it’s very unlikely that
of understanding of events? What inherited condition
they will show anything worrying,
are her concerns and expectations
but to be on the safe side we plan
regarding her husband’s condition Scenario
to organise for a 24-hour tape
and treatment?
recording of his heart beat to check
Role: you are a junior doctor in
that it doesn’t go too fast or too slow
Key points to establish a cardiology outpatient clinic.
at any time, and an echocardiogram
Firstly, establish that you have the
– that’s a special scan – to look at the
patient’s consent to talk to his wife Mr Patrick McDonagh is a 37-
heart in more detail than you can
about his condition. Explain that the year-old builder and father of
see on the CXR. We plan to do these
cause of the collapse is uncertain, three who was admitted on the
with your husband as an outpatient.
but initial assessment has so far medical ward with a syncopal
been reassuringly normal, as have Wife: can’t these tests be done before episode 2 months ago. He has
the appropriate investigations. he goes home?

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Key points to establish Patient: what are the chances that


been previously fit and well. Establish that there are two main my children have it?
Examination on admission issues to be explored: firstly, the
Doctor: because of the way it runs
revealed a normal pulse rate, impact of HCM on the patient and
in the family the chances for each
but his BP was elevated the potential need for him to have
child are about 50/50. So at some
persistently at 160/95 mmHg. further investigations; secondly, the
stage it will be important for you
There was a soft ejection systolic hereditary nature of the condition. It
to have your children seen by a
murmur over the left sternal is important to understand precisely
specialist, when a simple test like
edge. His ECG was normal apart why the patient is concerned about
an ultrasound scan of the heart
from large-voltage complexes the impact of the diagnosis on his
may allow the diagnosis to be made.
consistent with left ventricular family. Is his main concern the
However, it’s not always possible to
hypertrophy. He was discharged impact of his health (or ill-health)
say that a child definitely does not
and prescribed atenolol for his on the family? Has he understood
have the condition.
hypertension, and arrangements the genetic aspect of the condition?
were also made for him to Or are both issues of concern to Patient: is there a blood test that will
have a 24-hour ECG and an him? Both are very important, but enable a diagnosis to be made?
echocardiogram as an outpatient. an understanding of the patient’s Doctor: at the moment there is no
The 24-hour ECG was normal but main concern will allow a more single test that will give a definite
the echocardiogram demonstrated productive consultation. diagnosis. There have been a lot of
severe hypertrophic
advances in the genetic testing of
cardiomyopathy (HCM) with Appropriate responses to likely blood samples that may allow us to
an outflow tract gradient of questions get this answer in the future, and we
50 mmHg, following which an Patient: I feel great now. Does this can refer you to a clinical geneticist
urgent appointment for the mean I don’t need any further tests? who will be able to give you more
cardiac clinic has been made.
information on the inherited aspect
His GP has told him that the Doctor: that is really good news and
of the condition.
condition can affect the family, an excellent sign, but it is important
that we do further tests of your heart
and he is concerned about this.
as some patients with this condition
1.3.4 Communicating news of
can have very serious problems
a patient’s death to a spouse
HCM is typically an autosomal
later on.
dominant disorder with very Scenario
variable manifestations: some Patient: does this mean I am going
people with the condition have to die? Role: you are a junior doctor on
no problems, but others die a coronary care unit.
suddenly. Further investigation, Doctor: that’s not what I said, but a
eg electrophysiological studies, small number of patients with this Mr Smith, a 40-year-old man,
will be advised. condition are at risk of dangerous is admitted from work with
heart rhythm problems and sudden a large anterior myocardial
Your task: to explain the blackouts. The further tests will help infarct, which is treated with
diagnosis of HCM and the us assess whether you are at risk of thrombolysis. Unfortunately he
potential genetic implications this. If you are, then there are a arrests and, despite prolonged
of the condition. number of ways that we can attempts at resuscitation, he
reduce this risk. dies. His wife arrives 5 minutes

Patient: have I given this to my after he dies.

children?
Key issues to explore Your task: to inform Mrs Smith
Has the patient had any further Doctor: I assume that none of that Mr Smith is dead.
symptoms since discharge? your children have had any heart
What does he understand problems so far? [Patient confirms
about his condition and what that they have not.] But yes, this Key issues to explore
are his main concerns regarding condition can be passed on to your What does the patient’s wife know
his family? children. already? She will be more prepared

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for bad news if she knows he is brought into hospital as an attack that this wasn’t possible.
gravely ill than if she doesn’t know emergency, but not that he has died; We tried everything we could to
why he is in hospital. Explaining an and speaking quietly, slowly and resuscitate him, but I’m afraid
unexpected death is one of the most deliberately to let the information that it didn’t work.
difficult communication tasks that a sink in.] Your husband was brought
Wife: did he suffer?
medical professional has to perform: to the hospital as an emergency.
if it is done with compassion and He was very unwell: he had suffered Doctor: no – it was very quick
sensitivity it can ease the inevitable a big heart attack. We gave him and he was unconscious throughout,
distress that family and friends will the best treatment we could – an so he wasn’t aware of what was
go through. injection of a drug designed to open going on and he would not have
up the artery that had blocked off – suffered.
Key points to establish but I’m afraid that things did not go
Wife: will he have a post-mortem?
Find a quiet room, if possible well. The damage to his heart was
a relatives’ room, and ask the too great, it couldn’t beat properly Doctor: it is unlikely that he will
nurse looking after the patient to and, despite us doing everything we have to have a post-mortem. We will
accompany you. Leave your pager could, he passed away. need to inform the coroner, which
with someone else so that you are is something that we have to do
Wife: you mean he’s dead?
free of interruptions. There is no after any unexpected death, and
hiding from the fact that you must Doctor: yes. I’m very sorry, but very occasionally they will insist on
inform Mrs Smith that her husband I’m afraid he’s dead. a post-mortem. However, I think this
has had a heart attack and is very unlikely in this case, because
Wife: why did this happen?
unfortunately has not survived. State we know why your husband died. If
that you and the team did what you Doctor: I don’t know why it you would like further information
could, and say how sorry the whole happened, but he had a big heart about his health and how he died
team is. Demonstrate empathy: if it attack. This must mean that the then we can request a hospital
feels appropriate hold her hand or blood vessels going to his heart post-mortem, but it may be difficult
touch a non-threatening area, such muscle were narrowed, and that for you to discuss this now. We can
as the arm or the shoulder. Wait one of them blocked off and gave talk about this again later if you
until asked to explain details, but him a heart attack. want to.
keep it simple. Allow her to cry with
Wife: but people can survive heart
dignity, such as by handing her some 1.3.5 Explanation to a
attacks, why didn’t he?
tissues. Do not be afraid of silence, patient of the need for
but if this becomes uncomfortable Doctor: you’re right, many people investigations
it is often helpful to make an open do survive heart attacks, but
statement, such as ‘This must have I’m afraid that many also don’t. Scenario
come as a shock’. Sometimes the heart attack is so
big that it damages too much of the Role: you are a junior doctor
In finishing the discussion, explain
heart muscle for the heart to work at working on a cardiology ward.
that should further questions arise
all; and sometimes the heart attack
you will be happy to answer them.
affects the wiring mechanism that Mr Hugh Jones, aged 23 years,
Also say that you will have to notify
makes the heart beat in a regular has congenital heart disease.
the coroner, which is routine
manner, so that instead of pumping He was admitted from clinic
following any unexpected death, and
in a normal way the heart can’t for further investigations into
that the nursing staff will provide
pump at all. I’m sorry to say that the cause of his breathlessness.
her with information about practical
both of these things happened in The view of the cardiac team is
matters such as death certification.
your husband’s case. that it will not be possible to give
best advice about prognosis and
Appropriate responses to likely Wife: why couldn’t you bring him
treatment without information
questions back to life?
from a cardiac catheterisation
Wife: what’s happened?
Doctor: we did absolutely everything study, but he is refusing to
Doctor: [After ascertaining that she we could to restart his heart, but he consent.
knows that her husband was had suffered such a large heart

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Doctor: I know that things aren’t 1.3.6 Explanation to a patient


Your task: to determine what terrible at the moment, but we have who is reluctant to receive
concerns Mr Smith has and found a problem with the heart that treatment
explain the purpose of further could be serious and which may get
investigation. worse. It may be that treatment now Scenario
can improve things so that they
don’t get any worse, or the rate of Role: you are a junior doctor in
Key issues to explore any deterioration can be slowed a medical outpatient clinic.
First find out what the patient down so that you will feel well for
knows about his condition: he longer. Mrs Jessica Yelland, aged
may be concerned that nothing can 30 years, has been found to be
Mr Smith: can you guarantee that
be done or be in denial about the significantly hypertensive when
the problem can be sorted?
seriousness of the problem. Then she came to her GP’s family
establish what he knows about Doctor: no, I’m afraid that I can’t. planning clinic. Her BP has been
cardiac catheterisation and his Until we know exactly what the measured on several occasions
fears about the procedure: some problem is, we won’t be able to and found to be consistently in
patients are worried about pain and tell you. the region of 180/100 mmHg. It
discomfort, whereas others worry has been explained to her that
Mr Smith: I still don’t like the idea of
about complications. Try and put she has high BP that requires
a cardiac catheter. Is there an
any such fears in perspective. investigation and treatment,
alternative?
Explain any alternative investigative but she feels well and only
strategies that are available, but also Doctor: yes, we can and will do wants a prescription for the oral
why a cardiac catheterisation study scans that will give us some contraceptive pill, not any tests
is needed to give him best advice information. However, cardiac or medication.
about his condition. If possible offer catheterisation gives us the most
him information booklets and if important information, such as the Your task: to inform Mrs Yelland
there is a specialist nurse available, amount of oxygen in the chambers why investigations and treatment
ask him or her to speak with the of the heart, which we cannot get in are required.
patient. any other way. We wouldn’t
recommend this if there were better
Key points to establish alternatives. Key issues to explore
Mr Smith does not have to undergo The key to a successful outpatient
Mr Smith: will it hurt?
any investigation or treatment unless consultation will be to understand
he agrees to it. He will still receive Doctor: the procedure may be the reason why the patient does
care even if he does not undergo the uncomfortable while the local not want further investigation or
investigations recommended, but a anaesthetic is being given. This treatment. Does she feel that
proper investigation may improve lasts a few minutes and after this investigation and treatment are
the care that can be given to him it should not be uncomfortable. unnecessary because she feels well?
and thus alleviate some of his It’s a bit like going to the dentist: Is she afraid of what may be found?
symptoms. the injection is unpleasant, but then Is she concerned about the effects of
things go numb. treatment?
Appropriate responses to likely
Mr Smith: could I die during the
questions Key points to establish
procedure?
Mr Smith: I feel fine. It is very important to establish
Doctor: that’s very unlikely indeed. a rapport with this woman so
Doctor: I hear what you say, but
This is a routine procedure, although that she will trust you and thus
you went to the doctor because your
as you can imagine any procedure hopefully follow the recommended
breathing isn’t as good as it should
involving the heart carries a small management plan. Explain to her
be and it looks as though this is due
risk, but it is very small. The risk that hypertension is a common and
to a problem with your heart.
of death is 1 in 4,000, which means often asymptomatic condition that
Mr Smith: but the problem isn’t that 3,999 survive out of 4,000 is frequently detected on routine
very bad. people undergoing the procedure. screening, or incidentally as part of

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investigations for other medical Patient: what will happen if I have Patient: am I always going to have
problems. It is important that she nothing done? high blood pressure?
understands what hypertension
Doctor: over a period of many years Doctor: not everyone who is started
is and why it should be taken
high blood pressure can result in on medication for blood pressure
seriously, even in the absence of
serious damage to many important continues with high blood pressure
any complaints or limitations: the
organs in the body. For example, if for the rest of their life. In some
potential harmful effects of long-
untreated it can lead to major heart situations the changes to their
term high BP must be explained.
problems and strokes, and very lifestyle may mean that they do
She will need reassurance and an rarely it can result in problems with not need to continue taking
explanation that investigations are the eyes that can affect normal vision medication long term. The treatment
necessary to exclude a secondary and in extreme cases may result in is something that your doctor will
cause of high BP, which might mean blindness. However, all these problems want to review on a regular basis.
that the hypertension can be cured can be avoided by achieving good
Patient: will one tablet cure me?
and that she would not need long- blood pressure control.
term treatment. If no specific cause Doctor: it might do, but a significant
Patient: what causes high blood
for hypertension is found, then number of patients actually require
pressure?
simple changes to her lifestyle may a combination of tablets. We will
be adequate to treat her BP. But in Doctor: a good question, and I wish start you off on one tablet and then
some situations this is not enough I could give you a good answer. For review your blood pressure, and only
and she may require medication. most patients we don’t know, but in add in additional tablets if required.
some cases it can be caused by
Patient: what if I get side effects from
Your advice should be accompanied problems with the kidneys or glands
the pills?
by provision of reading material and so we will recommend some tests –
help with associated programmes blood tests and urine tests – to see Doctor: there are lots of different
for smoking cessation, weight loss if this might be the case for you. sorts of blood pressure pills, and
and dietary advice. But remember we want to make sure that we get
Patient: how can you tell if high
that most patients diagnosed wih one that suits you. If you do get
blood pressure is causing damage to
hypertension perceive themselves side effects from the first one that
the body?
as being healthy and leading a we try, I’d like you to tell me so that
normal lifestyle with no day-to-day Doctor: by examining you and doing we can try and find one that suits
limitations; hence starting treatment tests. For instance, we can look in you better.
and addressing lifestyle issues can your eyes to see if it is having an
Patient: can I still take the oral
be difficult and in some cases effect on the blood vessels at the
contraceptive pill?
unacceptable. back of the eye; we can do an ECG –
an electrical tracing of the heart – or Doctor: yes, as long as we can get
Appropriate responses to likely an echocardiogram – a special scan your blood pressure under control.
questions of the heart – and see if it is having
Patient: I feel very well and only an effect there; and we can do urine
went to the doctor for a prescription, and blood tests to check kidney
function.
so I can’t have much of a problem, 1.4 Acute scenarios
can I? Patient: what is the treatment likely
to consist of ?
Doctor: high blood pressure is a 1.4.1 Syncope
very common condition that can Doctor: the first thing is for us to
affect up to 20% of people. As in look at your lifestyle to see whether Scenario
your case, high blood pressure is we can help you make it more
often discovered when someone has healthy to bring your blood pressure A 75-year-old woman presents
their blood pressure measured for down. Examples of things that can in the Accident and Emergency
an entirely unrelated problem. The help are ensuring you take regular Department with a history of
fact that it was discovered for that exercise, stopping smoking and sudden collapse. This occurred
reason does not mean that having looking at your diet. But it is likely unexpectedly while she was
high blood pressure is unimportant. that tablets will also be needed.

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meticulous examination may elicit episode itself and of the recovery


shopping and there have been the cause. A history from a witness phase may provide information to
no previous similar episodes. should be obtained if at all possible establish the cause. Be very
When the paramedic team – it might be invaluable. particular in your enquiries: ‘Can
arrived at the scene she was you remember exactly what you
alert and orientated, and all History of the presenting problem were doing before you collapsed?’
observations were normal and Did the woman really have a Do not accept ‘I was out shopping’.
have remained so. syncopal episode, or did she just Ask: ‘Were you sitting down . . .
trip up? If syncope is likely, direct standing up . . . had you just turned
questions should be targeted towards your head?’ This history should be
Introduction the causes listed in Table 14. One obtained from the patient, any
of the most important of these is witness and preferably both.
How common are unexplained seizures. It is essential therefore to
collapses? Specific questions about symptoms
differentiate between seizures and
These are a very common that would suggest a cardiac cause
cardiac syncope. Seizures are
clinical problem, and account include the following.
associated with the following:
for up to 3% of attendances • Did you feel sweaty or nauseous
at Accident and Emergency • blue face (not pale);
before the episodes?
departments and 1% of hospital • convulsive movements
admissions. A difficult aspect • Did you get palpitations, chest
(usually, but not always);
of managing patients such pain or breathlessness beforehand?
as this is that there are many • tongue biting;
And if someone has had more than
causes of syncope, both cardiac • incontinence; one episode.
and non-cardiac (Table 14).
• unconsciousness for less than • How long have these episodes
Who are the high-risk patients? 5 minutes; being going on for and how many
Untreated cardiac-related syncope have you had?
• drowsiness and disorientation
has a 1-year mortality rate of
for a variable length of time on • Are the episodes becoming more
20 –30%, making it of paramount
recovery. frequent?
importance to identify this group
of patients as early as possible. A A detailed description of the events • Have you had fits of any sort
carefully taken history may exclude leading up to the syncopal episode, before?
a large proportion of these and a and a description of the syncopal
• Is there anything that brings on
the symptoms?

Which conditions make the patient


TABLE 14 CAUSES OF SYNCOPE at high risk of recurrent syncope or
death?
Type Cause
• Aortic dissection: was syncope
Non-cardiac Seizures* associated with chest pain? Is
Postural hypotension* there a history of hypertension?
Situational (micturition, defecation, cough and swallow)*
Cerebrovascular Has the patient had any previous
Psychogenic (anxiety, panic, somatisation and depression) vascular conditions?
Cardiac Bradyarrhythmias (including vasovagal)* • Pulmonary embolism: has
Tachyarrhythmias*
Left ventricular outflow obstruction (aortic stenosis and hypertrophic the patient had a period of
cardiomyopathy) immobility, or had any operations
Pulmonary obstruction (pulmonary embolism and pulmonary hypertension) recently? Has there been
Cardiac tamponade
a previous history of
Aortic dissection
Other rarities, eg atrial myxoma thromboembolism?

* commonest causes. • Aortic stenosis: has a murmur


been noticed in previous

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examinations? Is there a history • BP: is there a postural drop and is What would you do next for this
of dyspnoea? Is there a history of it the same in both arms? woman?
exertional presyncope? She will need to be admitted
• JVP.
for further investigations and
Specific treatments are available
• Cardiac apex. management, the nature of which
for all these conditions, making it
will be determined by your initial
important to consider them when • Are there carotid bruits (or bruits
history and examination. If you feel
taking a history, even though they elsewhere)?
that her syncope is most likely to
are unlikely.
• Heart sounds and murmurs. be neurological in origin, then
investigation should be pursued as
Other relevant history • Lung bases.
described in Neurology, Section 1.1.3.
Identification of any underlying
• Liver edge. However, in the absence of a firm
cardiac disease places the patient
diagnosis, a cardiac cause of the
in a high-risk group for recurrent • Peripheral oedema.
syncope can never be totally
syncope. Establish whether there
What is the significance of any excluded without thorough
are other symptoms suggestive of
abnormal cardiac signs? They investigation.
cardiac abnormality.
increase the chances of a cardiac
• Is there a history of angina / cause of syncope. What are the immediate
myocardial infarction? investigations required for a
What other bedside examinations
patient with probable cardiac
• Is there any cardiac family history? may help reach a diagnosis?
syncope?
Ask the patient to move her neck
• Is there a history of rheumatic Of patients with cardiac syncope,
through its full range of movement:
fever? 10% will have an identifiable
if this provokes feelings of
abnormality on their 12-lead ECG
• Are there any risk factors for presyncope or dizziness, then this
that suggests a cause. Look for the
ischaemic heart disease? suggests vertebrobasilar ischaemia
following:
as a likely cause for her syncope.
Also ask if she is taking any
Consider carotid sinus massage • sinus rate;
medication and, if so, what? Are
whilst recording an ECG rhythm
there any that might predispose her • PR interval;
strip to investigate carotid sinus
to syncope, eg diuretics that could
sensitivity, but be extremely cautious • QRS axis;
cause postural hypotension, or
in those patients who are at risk of
agents that might predispose her to • QRS width;
atheromatous carotid disease.
arrhythmia (check all drugs in the
• QT interval (long QT syndromes);
British National Formulary)?
Neurological system
• left ventricular hypertrophy;
The presence of any focal
Examination
neurological signs would raise the • right ventricular hypertrophy;
possibility that syncope was caused
General features • P-wave morphology;
by a cerebrovascular event, but does
Look for evidence of injury caused
not prove that this is the case. A • evidence of pre-excitation
by the syncope, and concentrate
cardiac cause of syncope could have (Wolff–Parkinson–White
specifically on cardiovascular and
led to cerebrovascular ischaemia. syndrome);
neurological assessment.
• evidence of acute (or old)
Investigation
Cardiovascular system myocardial infarction;
Take careful note of the following:
• Brugada syndrome (partial right
• Peripheral perfusion. bundle-branch block and ST
Remember that patients with
syncope are likely to be in a elevation V1–V3).
• Pulse rate and rhythm.
high state of anxiety and it is essential
On the CXR, look for the following:
• Peripheral pulses, including left that all other explanations are
radial and femorals. carefully considered before attributing • cardiac size and shape;
the problem to psychogenic causes.
• Pulse character: is it slow rising? • prominent pulmonary vasculature;

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• Echocardiography: this may


indicate structural or functional
cardiac abnormality. Transthoracic
echocardiography (Fig. 20) is
usually adequate, but in some
instances transoesophageal
echocardiography may be
required, eg when aortic
dissection is being considered.

• If pulmonary embolism is
›Fig. 19 Complete heart block demonstrated on a Holter monitor.
plausible, check the patient’s
blood gases and organise lung
• pulmonary oedema; Further investigations ventilation–perfusion scanning
• aortic outline (is the mediastinum or CT angiography (see
of normal width?). What further investigations should Section 3.8).
you consider during hospital
Blood tests are rarely useful admission? • If aortic dissection is possible,
in the diagnosis of syncope, It is more than likely that the ECG, request a CT scan of the chest.
but cardiac enzymes, FBC, CXR and screening blood tests will
electrolytes, renal and liver not demonstrate any clear cause of What should you do for those with
function tests, and inflammatory this patient’s syncope, in which case recurrent unexplained syncope?
markers will usually be consider the following. Consider referral for specialist
requested as a ‘screen’. investigations such as tilt-table
Electrolyte disturbance • Ambulatory monitoring: 24-hour testing and electrophysiological
(particularly hypokalaemia) Holter monitoring and patient- tests (see Section 3.2).
might predispose to arrhythmia activated devices may be useful
and syncope. Raised inflammatory for excluding tachyarrhythmias Management
markers may indicate a systemic and bradyarrhythmias (Fig. 19)
problem. (see Section 3.3). If a cardiac cause of syncope is
established, what management is
indicated?

• Bradyarrhythmias: consider
permanent pacemaker
(see Section 3.5).

• Tachyarrhythmias:
pharmacological therapy, ablation
or implantable cardioverter
defibrillator (ICD) (see
Sections 2.2.2 and 3.4).

• Valvular disease: consider


surgical intervention (see
Section 2.5).

• Pulmonary embolism: will


need anticoagulation (see
Section 2.18 and Haematology,
Section 3.6).

• Hypertrophic cardiomyopathy:
›Fig. 20 Transthoracic echocardiogram of an atrial myxoma. The myxoma (MYX) is seen to occupy most of give advice on lifestyle changes
the left atrium and is almost prolapsing through the mitral valve (MV) into the left ventricle (LV). The aorta
(AO) is seen above the left atrium. This would be an exceedingly rare cause of syncope. and pharmacological therapy, and

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consider an ICD if there are any 1.4.2 Stroke and a murmur


features suggestive of a high risk • dilated cardiomyopathy;
• infective endocarditis;
of sudden death (see Section Scenario • paradoxical embolism;
2.4.1). If there is significant
• left atrial myxoma;
left ventricular outflow tract A 58-year-old woman who is • calcific aortic stenosis;
obstruction, then consider referral married with two sons and • aortic arch atheroma.
for surgical myomectomy or works as a part–time teacher
alcohol septal ablation presents with a left-sided
(see Section 2.4.1). hemiparesis of sudden onset. History of the presenting problem
She had previously been fit and The first priority will be to confirm
What should you do if no cause for well. However, a murmur had the diagnosis of stroke, then to focus
syncope is established and the been noted, but not investigated, on possible causes. With regard to
patient is asking to go home? when she was 45 years old identification of cardiac causes of
If after 24 – 48 hours there has and undergoing a minor stroke, establish whether there have
been no recurrence of presyncope gynaecological procedure. been any previous thromboembolic
or syncope, the patient has There is no past medical events. Is the patient known to have
‘mobilised’ satisfactorily on the history or family history any cardiac condition? Does she
ward and serial 12-lead ECGs of note. She is on no medication. have any cardiac symptoms?
show no change, then the patient Specifically, is there a history of
should be discharged home with palpitations suggestive of AF?
the following: Introduction
A stroke can be a devastating Other relevant history
• reassurance that nothing terrible
condition with high morbidity In most cases it will be obvious if
has been found, but also a clear
and mortality. It is not possible at there is any pre-existing cardiac
statement that no firm diagnosis
presentation to predict accurately condition, but ask the patient the
has been made, meaning that
the degree of recovery that this following.
patient and doctors must remain
woman will make. However, it is
alert; • Are you known to have an
important to identify whether she is
irregular pulse?
• a letter for the patient’s GP; at high risk of further events, and in
particular whether she is at risk of • Have you had angina or a heart
• instructions to report recurrence
cardiac embolic stroke. Most strokes attack?
of presyncope or syncope
are related to cerebrovascular
immediately; • Have you had rheumatic fever?
atheromatous disease and not to
• arrangements for 24-hour cardiac disease, but most cardiac • Have you had any heart
ambulatory monitoring (and causes of embolic stroke are operations?
perhaps echocardiography) if it treatable, indicating that further
• Has a murmur ever been heard?
has not been possible to obtain events in this group are potentially
this during the patient’s brief preventable. History or examination • Did you have a ‘hole in the heart’
admission. will detect most cases where there is as a child?
a cardiac cause for stroke.
Examination

General features
Consider the causes of cardiac In the absence of previous cardiac
Most patients will be aware of
embolic stroke when taking a conditions and with an entirely
the serious nature of most
history and examining a patient who normal clinical examination, the
cardiac causes of syncope. It is
has had a stroke:
essential to be aware of the likelihood of stroke being cardiac
psychological needs of such patients. • ‘non-valvular’ atrial fibrillation (AF); in origin is small. It is therefore
Reassurance and appropriate • acute myocardial infarction (MI)
vital that examination of the
information at an early stage may with mural thrombus;
cardiovascular system is thorough.
prevent problems at a later stage in • mechanical prosthetic valves;
their management. • rheumatic heart disease; A patient with a recent stroke may
have difficulty in cooperating with

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›Fig. 21 ECG demonstrating anterior Q waves and poor R-wave progression across the chest leads. This is most probably secondary to a previous large anterior MI.
Left ventricular mural thrombus is possible in this scenario.

you during the examination, eg particularly if the patient is unable carotid arteries or vasculitis of the
rolling the patient on to the side to give a history. Do not forget to cerebral vessels (very rare).
to listen for mitral stenosis may examine the back and the breast
be difficult if he or she has a crease where there may be scars Investigation
hemiparesis. However, it is from previous mitral surgery In most cases, simple non-invasive
important not to compromise the (valvuloplasty). investigations will provide the
quality of your examination – seek information needed to establish
help in moving the patient if Cardiovascular system a cardiac cause of a stroke.
necessary. Take careful note of the following.
ECG and CXR
It is unlikely that there will be many • Pulses: check the rhythm and
Every patient who has a stroke
signs from a general examination character. In particular, is there
should have a 12-lead ECG and a
that will help in establishing AF? Is it possible that this woman
CXR. The ECG may provide valuable
whether the cause was cardiac. has had an aortic dissection?
clues to the aetiology of thrombus
• The patient with a previous • BP: this is often elevated in (Fig. 21).
MI or dilated cardiomyopathy someone who has just had a stroke.
might be dyspnoeic as a result Echocardiography
• Heart sounds: this woman is
of cardiac failure, but there This should be requested if
said to have a murmur – listen
are many other causes of there is a clinical, ECG or chest
carefully for both mitral stenosis
breathlessness, including radiographic indication of cardiac
and aortic incompetence, which
aspiration pneumonia, in someone abnormality (Fig. 22). If the heart is
are easy murmurs to miss.
who has just suffered a stroke. structurally normal on transthoracic
You are most unlikely ever to
echocardiography and no other
• The patient with infective hear the ‘tumour plop’ of an atrial
cause of stroke is found, a
endocarditis or atrial myxoma myxoma, but it is absolutely
transoesophageal echocardiogram
may have fever and peripheral certain that you will not if you
should be requested to look for
stigmata, but these are uncommon never listen!
aortic arch atheroma, patent
conditions (see Section 1.4.7).
Consider non-thromboembolic foramen ovale (PFO) (Fig. 23)
• Look carefully for any signs cardiovascular causes for her stroke, or left atrial appendage thrombus
of previous cardiac surgery, eg aortic dissection involving the (Fig. 24). CT or MRI of the chest

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(a) (b)

›Fig. 22 Transthoracic echocardiogram of an apical thrombus after MI. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; Ao, aorta.

tumour. Evidence of multiple


cortical infarcts in different
vascular territories and a
structurally normal heart on
transthoracic echocardiography
should raise the possibibility
of PFO if carotid artery disease
is not found.

Management
The patient will require care
appropriate to the disability
produced by their stroke (see
Medicine for the Elderly, Section 2.8;
Acute Medicine, Section 1.2.30;
and Neurology, Sections 1.4.2 and
›Fig. 23 Transoesophageal echocardiogram of a PFO. LA, left atrium; RA, right atrium; SVC, superior vena
cava. 2.8.1). Particular attention to
anticoagulation, restoration of
may rarely be required to define a mundane explanation, consider sinus rhythm (in some cases) and
structural abnormality. endocarditis, myxoma and surgical correction of cardiac lesions
vasculitis, and perform (in rare cases) will be required in
Blood tests appropriate specialist patients with a cardiac cause for
blood tests. stroke.
• FBC (if polycythaemia or
thrombocytosis is possible). • Blood cultures (if any suspicion of Anticoagulation
• Electrolytes. endocarditis). Consider if the stroke is confirmed
as ischaemic on brain CT scan and
• Renal and liver function tests.
Brain CT scan a cardiac cause has been identified.
• Inflammatory markers: if these This is required to exclude The balance of benefit versus risk in
are raised and there is no other haemorrhage, aneurysm or the acute setting is difficult: leaving

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1.4.3 Acute chest pain

Scenario

A 55-year-old Glaswegian
merchant seaman presents with
chest tightness after unloading
his ship of heavy goods.

Introduction
If the patient is still in pain,
the first concern is to rule out
life-threatening emergencies such
as myocardial infarction (MI) or
aortic dissection where treatment
›Fig. 24 Transoesophageal echocardiogram of thrombus in left atrial appendage.
can be life-saving. Quickly assess
the patient’s haemodynamic status,
including checking for bradycardia
the patient without anticoagulation Antiarrhythmics
and tachycardia, and measuring the
keeps her at risk of further After anticoagulation, restoration
BP. Obtain an initial brief history
thromboembolism, but of sinus rhythm should be the
and conduct a quick examination
anticoagulation puts her at primary target in those with AF.
whilst the ECG is being recorded.
increased risk of haemorrhagic Pharmacological cardioversion is
transformation of a cerebral preferable to DC cardioversion in
History of presenting problem
infarct. There are no good data this context so as to avoid general
This patient’s pain was associated
to determine when anticoagulation anaesthetic after a recent stroke
with heavy exertion, but it is
should be started. Assuming that (see Section 2.2.2).
important to determine answers to
the patient is recovering from a
the following questions if they do
stroke, most physicians would Closure of PFO
not emerge spontaneously.
begin with heparin (intravenous Percutaneous closure is
or low-molecular-weight) at some recommended if no other cause of • When did it start?
time between 7 and 14 days, stroke is found, there are recurrent
• What were you doing when it
with a view to long-term warfarin episodes despite anticoagulation and
started?
therapy if the thromboembolic a large PFO is demonstrated by
risk persists. echocardiography. • What was it like?
Characteristically ischaemic
Surgical correction of cause pain is described as tight,
Repair of an aortic dissection, squeezing or crushing. It may
• Strokes may be replacement of an infected valve or be helpful to offer suggestions,
haemorrhagic, even in those removal of an atrial myxoma may be but it is important to realise that
at risk of thromboembolism. necessary immediately. There is high patients may find it difficult to
• Some features are more likely with risk of further cerebral insult when describe pain, especially if it is
cerebral haemorrhage than with
the patient goes on cardiac bypass, severe.
infarction, such as nausea/vomiting,
cerebral irritation and depressed but this has to be balanced against
• How bad was the pain? Severity
conscious level. the potential risk of not treating the
should be recorded on a scale
• Ischaemic and haemorrhagic strokes underlying condition. In those with
cannot be distinguished with
of 1 to 10.
valvular pathology, it is usual to
certainty on clinical grounds and a
allow time for the patient to make as • How long did it last? Pain that
brain CT scan should always be
complete a recovery as possible from last for only a few seconds or
performed before commencing
anticoagulation. the stroke before considering constantly for days is unlikely to
surgery. be cardiac.

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• Did it start suddenly? If so, any previous history of indigestion, Examination


consider aortic dissection, use of treatments for indigestion (eg
although this is also typical antacids) or previous investigations General features
of non-cardiac pain related to for indigestion (barium meal or All patients with chest pain need
anxiety. Or did it build up rapidly endoscopy test). Remember that prompt rapid assessment. How does
over minutes? This is more typical severe epigastric pain can be caused the patient look: well, ill, very ill or
of cardiac ischaemia. by myocardial ischaemia as well as about to die? Patients with MI
the abdominal conditions usually usually look very ill (or worse).
• Did the pain go anywhere else?
considered (peptic ulceration, biliary
In particular, did it radiate in a After checking vital signs,
pain, pancreatitis and intestinal
manner typical of ischaemic examine particularly for epigastric
ischaemia).
cardiac pain? tenderness and guarding: patients
Musculoskeletal This tends to with perforations or pancreatitis
• Was it sharp and stabbing in
be very localised and affected by may present in this way and be
nature? If yes, consider causes
the position of the sufferer, unlike misdiagnosed. Where the patient
of pleuritic chest pain.
ischaemic pain, which is not. It does not have a life-threatening
• Has the patient experienced may occur after strenuous exertion, condition, palpation for tenderness
unusual breathlessness or chest which often leads to anxiety that that reproduces the pain is useful.
tightness on exertion previously? this has ‘brought on a heart attack’.

• Beware of pain which comes on at Cardiovascular system


Pericarditis This is unlikely in this
rest or during sleep: both unstable This is frequently normal in
case, but ask whether the patient
angina and acute coronary patients with acute central chest
has had any ‘flu-like’ symptoms.
syndromes may occur at rest. pain. However, a full examination
The pain tends to be sharper than
is essential, looking in particular
Also ask about associated symptoms, ischaemic cardiac pain and is
for features that would be
such as breathlessness, sweating, usually eased by sitting forward.
diagnostically useful.
nausea, faintness and whether the Anxiety This can manifest itself as
pain was frightening, all of which • Pulses: unequal radial pulses and
pain, but severe pain may cause
are features of cardiac ischaemia. unequal BP in the arms may
patients to become anxious, so
indicate aortic dissection.
beware of dismissing the patient’s
symptoms. Patients may develop • Heart sounds: an early diastolic
A patient with stable angina tingling in the fingers due to murmur of aortic regurgitation
who then presents with hyperventilation in response may indicate dissection; a
increasing frequency and severity of to chest pain. pericardial rub would suggest
angina (‘crescendo angina’) is likely to pericarditis.
have an unstable plaque and should be
admitted, even if pain-free when seen. Other relevant history
Does the patient have known Investigation
coronary artery disease? Ask about Appropriate investigations are the
history of angina, MI and cardiac key to proving a diagnosis of an
Differential diagnoses
catherisation/revascularisation acute coronary syndrome.
Aortic dissection A sudden onset of
procedures (percutaneous coronary
severe pain radiating to the back,
intervention or coronary artery ECG
which may be described as ‘tearing’,
bypass surgery). If not, are they An entirely normal ECG at
should ring alarm bells and the
at high risk of coronary disease? presentation does not exclude
patient should be evaluated for
Note previous vascular events: does serious underlying coronary disease.
dissection. This is more likely in
the patient have a history of stroke Look in particular for:
patients with known hypertension.
or peripheral vascular disease?
• ST-segment elevation/ left bundle-
Digestive pain (reflux oesophagitis Consider risk factors for coronary
branch block (MI) (Fig. 25);
or peptic ulceration) Ask whether disease, including family history,
the pain was associated with the smoking, diabetes mellitus, • ST-segment depression
bringing up of wind or an acid taste hypercholesterolaemia and [non-ST elevation MI (NSTEMI)
in the mouth. Also enquire about hypertension. or unstable angina] (Fig. 26);

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Persisting pain without ECG


abnormalities and no other clear
diagnosis
Reassure the patient, give analgesia,
and admit for overnight observation
with repeat ECG and troponin at
12 hours.

Do not discharge patients who


present with undiagnosed chest
pain without a further ECG and
troponin test at 12 hours – unstable
›Fig. 25 Acute inferior MI. The ECG shows ST-segment deviation in the inferior leads (II, III and aVF).
ST-segment depression in leads V2 and V3 may indicate posterior extension. ischaemic heart disease can be difficult
to diagnose clinically and is easily
missed.

Pain resolved but diagnosis


uncertain
Patients who attend hospital
with chest pain that is not clearly
musculoskeletal should stay for
further assessment, with a troponin
test and ECG at 12 hours after the
pain. A negative troponin test will
not rule out important coronary
disease, so an investigation to look
›Fig. 26 ECG showing anterior ST depression in a patient presenting with chest pain. Thrombolysis is not
beneficial and may do harm. Antithrombotics should be commenced. for ischaemia (eg exercise test)
should be arranged as an outpatient.
The patient should be advised to
seek medical help immediately
• T-wave inversion (NSTEMI or Chest radiograph if the pain recurs after discharge.
unstable angina); Is the mediastinum widened
(aortic dissection) or is there
• concave ST-segment elevation
pulmonary oedema?
in multiple leads that do not
conform to a single coronary Difficult cases of chest pain
artery territory (pericarditis).
Management may be due to coronary
vasospasm (Fig. 27). Features of
Ischaemic cardiac pain spasm include:
Biochemical markers
Troponins are markers of Patients will usually already • normal coronary angiogram or
myocyte necrosis and should have received aspirin 300 mg. ‘minor irregularities’;
Give oxygen and analgesia if • paroxysms of crushing central chest
be measured at 12 hours in
the patient is still in pain. Further pain at rest;
order to diagnose MI (see • affects postmenopausal women the
Section 3.7). management will depend on the
most;
diagnosis: • relieved by glyceryl trinitrate;
Other blood tests • does not limit exercise;
• unstable angina/NSTEMI • may be associated with syncope;
FBC, creatinine and electrolytes, (see Section 2.1.2); • usually benign unless associated
glucose, cholesterol and, in patients
with ECG changes or ventricular
with epigastric pain, amylase should • ST-elevation MI (see arrhythmias.
be measured. Section 2.1.3).

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(a) (b)

›Fig. 27 Left coronary angiogram demonstrating coronary vasospasm. This 34-year-old woman presented with chest pain and anterior ST-segment elevation
on the ECG. Shortly afterwards, she had a ventricular fibrillation arrest. After resuscitation, the ECG returned to normal. (a) Initial angiography showed a normal left
coronary artery. (b) Intracoronary ergometrine induced localised spasm of the proximal left anterior descending coronary artery and reproduced the chest pain with
ECG changes.

History of the presenting problem


coronary intervention (PCI) Pay particular attention to the
Oesophageal pain is difficult to the right coronary artery via following.
to distinguish from cardiac
the right femoral artery. He was
pain. Cardiac pain may be relieved by • How quickly did the
reviewed the following morning
belching. It is inadvisable to discharge
once the coronary care team hypotension arise? Sudden onset
a high-risk patient with a clinical
diagnosis of oesophageal pain. were happy with his progress. of hypotension usually indicates a
His creatine kinase (CK) was catastrophic event, whereas pump
4,010 IU/mL and troponin I failure is more gradual.
>50 IU/mL (both grossly
1.4.4 Hypotension following • Does the patient have chest pain?
elevated) and an angiotensin-
acute myocardial infarction This may indicate reinfarction or
converting enzyme (ACE)
cardiac rupture.
inhibitor was prescribed.
Scenario However, the coronary care • Drugs: streptokinase is a cause
nurses ask you to review him of hypotension, which usually
Mr Jones is a 60-year-old man
before giving the first dose of the occurs in the first 20 minutes
who was admitted with an acute
ACE inhibitor because his systolic of administration. Other than
inferior myocardial infarction
BP has fallen to 70 mmHg. this, drugs are rarely the cause
(MI), which was treated
of severe hypotension unless
with aspirin, clopidogrel and
the patient is fluid depleted,
thrombolysis. Ninety minutes
but look for a temporal
after thrombolysis he was still Introduction relationship.
in pain and there was no change Hypotension after MI requires
in the elevation of ST segments rapid assessment and intervention. • Breathlessness: this may
in the inferior leads. He The combination of clinical indicate pulmonary oedema or,
underwent percutaneous examination, ECG and CXR will more rarely in this situation,
usually determine the diagnosis. pulmonary embolism.

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• Volume depletion: has the patient • ECG: arrhythmia or • Attempt to chemically cardiovert
received diuretics? Examine the reinfarction? new-onset atrial fibrillation
fluid charts. by correcting potassium
• CXR: look for pulmonary oedema,
and magnesium levels, and
and also for widening of the
Examination administering intravenous
mediastinum.
amiodarone. These patients
General features • Arterial blood gases: these will should receive therapeutic
Assess the general condition. confirm oxygenation and heparin.
If the patient appears on the verge ventilation. Hypoxia in the
• Most patients are not fluid
of arresting, call the resuscitation presence of a normal CXR
depleted after MI, but in a
team immediately: do not wait until suggests PE.
hypotensive patient with clear
the heart has definitely stopped.
• Other blood tests: creatinine lung fields who is not hypoxic
Check the Glasgow Coma Scale to
and electrolytes will document it is reasonable to give a fluid
confirm the baseline. Assess pain
renal function; FBC will be bolus of 250 mL 0.9% saline
level (if any).
used to assess for blood loss intravenously and then reassess.
(could the patient be having a This can be repeated if necessary.
Cardiovascular system
gastrointestinal bleed following However, if there is no response
The following are important.
thrombolysis?) or sepsis (unlikely it is important to seek further
• Peripheral circulation: does the in this context). advice and consider inserting
patient have cold feet and hands? a PA catheter to guide fluid
• Echocardiography: this is essential
How far up the arms and legs management.
to assess left and right ventricular
is perfusion impaired? Severe
function, mitral regurgitation, • The combination of pulmonary
peripheral shutdown indicates
VSD and tamponade. oedema and hypotension indicates
a poor prognosis.
a poor prognosis. If there is
When contemplating the results of
• BP: recheck the measurement, and evidence of pulmonary oedema,
these investigations, consider the
record in the left arm as well as you must not administer fluid
diagnoses listed in Table 15.
the right. Could the patient have as this could be fatal. Give
an aortic dissection presenting furosemide 80 mg as an
Management
with an inferior MI? intravenous bolus. Central
Rapid assessment and diagnosis are
venous access should be
• JVP: if it is grossly elevated, vital: delay can be disastrous. Early
obtained, inotropes started
consider right ventricular consultant advice is important.
and consultant advice sought.
infarction, ventricular septal Continuous monitoring of rhythm
Intra-aortic balloon pumping
defect (VSD), tamponade or and BP and of hourly urine output
may be helpful, particularly if
pulmonary embolism (PE). is essential. Serum creatinine and
revascularisation may be feasible
electrolytes should be checked at
• Heart sounds: a gallop rhythm in the future. Notify the intensive
least daily.
suggests left ventricular failure. A care unit, as ventilation may be
new pansystolic murmur suggests • Stop drugs which cause required.
VSD or mitral regurgitation from hypotension, except beta-blockers
chordal or papillary muscle in patients with heart failure who Further comments
rupture. have been taking them long term, Hypotension after MI is a
as there is a risk of mortality on serious problem with a high
• The groin: could the patient be
rapid cessation. mortality. Remember that
bleeding into the leg or abdomen
the patient must be kept as
from the femoral puncture? • Monitor pulse oximetry and give
comfortable as possible:
high-flow oxygen.
• Urine output: an essential aid for explain what needs to be done
assessment of end-organ perfusion. • Assess and treat the underlying and why, and give analgesia in
condition if possible: patients small doses (eg diamorphine
Investigations with VSD or mitral regurgitation 2.5 mg iv) as often as needed.
Routine investigations will help require urgent cardiothoracic The patient’s relatives should
establish the diagnosis. surgical referral. be contacted.

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TABLE 15 DIFFERENTIAL DIAGNOSIS OF SUDDEN HYPOTENSION FOLLOWING MI

Differential diagnosis Features

Right ventricular infarction Inferior MI


High JVP with hypotension
Clear CXR
Acute mitral regurgitation Pansystolic murmur, although this may be absent if there is rapid equalisation of pressure between left
atrium and left ventricle
Severe pulmonary oedema
Echocardiography will be diagnostic (Fig. 28)
Pulmonary artery (PA) catheter may confirm this diagnosis by revealing a giant v wave
Acquired VSD Very high JVP
Pansystolic murmur
Pulmonary oedema, but less severe than acute mitral regurgitation
Echocardiography will detect location
Intermittent ischaemia from Severe coronary disease (especially critical left main-stem disease)
severe ischaemic heart disease Recurrent chest pain associated with widespread ischaemic changes on ECG
High risk of death; refer patient for urgent revascularisation
Pump failure Raised JVP
Pulmonary oedema
Echocardiogram shows poor left ventricular function, but no evidence of VSD or mitral regurgitation
PA catheter may be helpful if there is doubt about left ventricular filling. In general, fill with cautious boluses
of fluid until PA wedge pressure is 15 mmHg; if the patient is hypotensive and anuric despite PA wedge
>15 mmHg, start inotropes
Retroperitoneal bleeding This may be spontaneous in response to thrombolytic and antiplatelet agents, but may also occur from a
leaking femoral artery puncture site after angiography and PCI
Tachycardia
Low BP and JVP
Good BP response to fluids
Evidence of dropping haemoglobin on serial tests
Consultant advice should be sought regarding urgent CT scan of the abdomen, and stopping glycoprotein
IIb/IIIa antagonists
Gastrointestinal bleeding Recognised complication of thrombolytic and antiplatelet agents
Tachycardia
Low BP and JVP
Good BP response to fluids
Evidence of dropping haemoglobin on serial tests
Watch for melaena

MI, myocardial infarction; PCI, percutaneous coronary intervention; VSD, ventricular septal defect.

1.4.5 Breathlessness and particularly if there is no prior


collapse are bleeped urgently by the history of cardiorespiratory disease.
Emergency Department to assess The episode of syncope is of
Scenario and instigate initial management. particular concern and confirms
the life-threatening nature of this
A 55-year-old woman is brought presentation.
to hospital by ambulance. Her Introduction
husband called 999 this morning What is the differential diagnosis
when he found her acutely in this patient? Common causes
In cases of breathlessness and
breathless at home. She had a (Table 16) should be considered collapse, always consider
brief syncopal episode on being as you assess and resuscitate the tension pneumothorax.
moved into the ambulance, but patient. Preliminary assessment will
is now conscious, although direct specific investigations and
extremely dyspnoeic. Her BP treatment. Pulmonary embolism History of the presenting problem
is only 80 mmHg systolic. You (PE) should be high on the list of If you can obtain a history from the
diagnoses for a patient such as this, patient and/or her husband, ask

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such as orthopnoea, paroxysmal


nocturnal dyspnoea or pink
frothy sputum?

• Does the patient have a history of


asthma and has it been getting
worse?

• Has the patient suffered from


abdominal pain, vomiting,
haematemesis or melaena:
could there have been an
intra-abdominal catastrophe?

• Symptoms suggestive of sepsis:


the most common infective cause
of hypotension and breathlessness
would be septicaemia and acute
›Fig. 28 Transoesophageal echocardiogram showing prolapse of the posterior mitral valve leaflet
respiratory distress syndrome, but
(indicated) into the left atrium (LA) after papillary muscle rupture. LV, left ventricle. with ’flu-like illness also consider
pericardial effusion with sudden
decompensation.
TABLE 16 DIFFERENTIAL DIAGNOSIS OF ACUTE DYSPNOEA
AND HAEMODYNAMIC COLLAPSE • Exposure to a known allergen.

• Drugs: illicit or prescribed.


General cause Comment Specific cause

Cardiac Common MI/complications (acute mitral regurgitation, VSD, acute


left ventricular failure)
Arrhythmia (VT) When dealing with patients
Less common Aortic dissection who are very ill, history-taking,
Cardiac tamponade examination, investigations and
Acute aortic regurgitation treatment should all begin together.
Cardiorespiratory Common Massive PE (occluding >50% of pulmonary vasculature) Finishing a very complete history at
the same time as the patient expires is
Respiratory Common Acute life-threatening asthma
to be avoided!
Less common Tension pneumothorax1
Other Common Sepsis
Less common Intra-abdominal catastrophe
Severe haemorrhage (‘air hunger’) Other relevant history
Anaphylaxis This is not the time for a lengthy
medical history, but ask about the
1. Remember that although tension pneumothorax is uncommon, this diagnosis should be
considered before all others because immediate treatment is life-saving. following.
MI, myocardial infarction; VSD, ventricular septal defect; VT, ventricular tachycardia.
• PE or deep venous thrombosis
and the relevant risk factors
particularly about the following, • Is there central chest or (see Section 2.18).
doing so at the same time as you interscapular pain? This
• Cardiac: MI, angina, previously
examine and organise initial suggests MI or aortic dissection,
undiagnosed anginal pain, valve
investigations such as ECG, respectively.
disease, rheumatic fever, ‘heart
arterial blood gases and CXR.
• Presence of pleuritic chest pain murmur’ (and if so any recent
• Did the breathlessness have a or haemoptysis: either suggests dental work or surgery – could
sudden or gradual onset? Sudden smaller PEs preceding a larger she have acute valve dysfunction
onset suggests pneumothorax, one. caused by infective endocarditis?)
massive PE, acute valve and hypertension (risk factor for
dysfunction, dissection • Are there any symptoms ischaemic heart disease and aortic
or arrhythmia. suggestive of pulmonary oedema, dissection).

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• Respiratory: asthma, chronic Take samples for FBC, electrolytes,


airflow obstruction and sounds in the opposite axilla). These renal and liver function tests,
signs are not subtle, but they require a
pneumothorax. cardiac enzymes and blood
clear head to recognise in the context
cultures.
• Abdominal: peptic ulcers, of a patient who is desperately ill.
pancreatitis and gallstones.
ECG
• Anaphylaxis: allergy to anything? Look for the following (and repeat
• Does the pulse become impalpable
after 1 hour, or sooner if clinically
• Drugs: a useful rapid check of past on inspiration? This indicates
indicated).
medical history in this context. severe paradox, so consider
life-threatening asthma or • Arrhythmia (see Section 2.2).
If no history is available, check bags
tamponade.
and pockets for inhalers, glyceryl • Localised ST-segment
trinitrate, etc. • Where is the JVP? A high JVP elevation of acute MI (see
suggests a cardiac or respiratory Section 2.1.3).
Examination cause for collapse, whereas a low
• ECG changes compatible with
one suggests bleeding, intra-
acute PE, the most common
General features abdominal catastrophe or sepsis.
being sinus tachycardia and
• Specifically look for evidence T-wave inversion in leads
of massive PE: high JVP, right V1–V4. The ‘typical’ right-axis
If the patient looks in extremis, ventricular heave and tricuspid deviation and ‘S1Q3T3’ are
call for help from the intensive
regurgitation. actually quite unusual (see
care unit or the cardiac resuscitation
team immediately. Do not wait until Section 2.18).
• Heart sounds: is there a
she has a cardiac arrest if she looks as
pansystolic murmur? If • Generalised low voltages
though she is deteriorating.
so, consider acute mitral of pericardial effusion
regurgitation or VSD (see Section 2.6).
Immediately assess the following. (see Section 1.4.4)?
Chest radiograph
• Vital signs: pulse (rate and
Respiratory system Look for pneumothorax,
rhythm), BP, respiratory rate
Are there reasonable breath sounds pulmonary oligaemia (resulting
and temperature.
in both lungs? If there are many from PE), pulmonary oedema,
• Is there cyanosis? Check pulse crackles and/or bronchial breathing, consolidation, effusions, heart
oximetry (but do not remove consider pulmonary oedema, size, mediastinal shift and the
the high-flow oxygen to ‘check pneumonia or adult respiratory widened mediastinum of aortic
value on air’ in someone who is distress syndrome. dissection.
desperately ill).
Abdominal
• Is there swelling of the lips and
Peritonism indicates an intra-
tongue (anaphylaxis)?
abdominal catastrophe. Feel A normal mediastinal width
• Conscious level: establish baseline deliberately for an abdominal on the CXR does not exclude
Glasgow Coma Score. aortic aneurysm. aortic dissection.

Cardiovascular system Investigation


The following investigations are Echocardiography
required immediately in all patients This is the examination of choice
Is there a tension who present with severe hypotension for effusion, valve regurgitation and
pneumothorax? Look for and breathlessness. VSD, and for assessing ventricular
asymmetry of the chest, shift of function. Remember that right
trachea from midline, shift of
Blood tests ventricular dysfunction in the
mediastinum from midline (percuss
area of cardiac dullness) and silent
Check fingerprick blood glucose context of acute severe dyspnoea
chest on one side (with a few breath immediately in anyone who is and hypoxia is highly suspicious
severely ill. Check arterial gases. of massive PE.

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Other investigations • Arrhythmia (see Section 2.2). Further comments


As determined by clinical
• Complications of MI
assessment and the findings of Communication
(see Section 2.1).
preliminary tests, eg pulmonary After making your clinical
CT angiography for suspected • Tamponade: urgent assessment, performing immediate
massive PE, or CT of the pericardiocentesis (see investigations and initiating
abdomen if an abdominal Section 2.6.2). management, you will need to
cause is likely. speak to the patient’s husband
• Asthma (see Acute Medicine,
to explain the situation.
Section 1.2.9 and Respiratory
Management
Medicine, Section 2.2.2).
Key priorities when you 1.4.6 Pleuritic chest pain
reach the patient include the • Intra-abdominal catastrophe:
following. call for surgical help immediately. Scenario
Resuscitate the patient while
• Resuscitate (Airway, Breathing
considering surgery – do not A 50-year-old woman gives
and Circulation) and secure
say you will resuscitate the a 1-day history of left-sided
venous access while taking a
patient and then call the pleuritic chest pain and
history.
surgeons. progressive breathlessness
• Give high-flow oxygen. on minimal exertion. Routine
• Anaphylaxis (see Acute Medicine,
observations reveal her
• Exclude tension pneumothorax: Section 1.2.33 and Rheumatology
temperature is 37.9°C, pulse
insert a large-bore cannula and Clinical Immunology,
80 bpm, BP 130/80 mmHg
into the silent side of the chest Section 1.4.2).
and respiratory rate 18/minute.
(second intercostal space, mid- You are asked to see her in
Also, if you do not know the
clavicular line or mid-axillary the Medical Assessment Unit.
diagnosis, give broad-spectrum
line, above the level of the
antibiotics to cover sepsis
nipple) if this is the clinical
(see Infectious Diseases,
diagnosis (see Acute Medicine,
Section 1.3.2).
Sections 1.2.14 and 3.5; and Introduction
Respiratory Medicine, Sections 3.2 Arrange for the patient to be The differential diagnosis of the
and 3.4) transfered to an appropriate patient presenting with pleuritic
high-dependency area for pain is shown in Table 17. The two
• Exclude other diagnoses that
continuing management most important acute diagnoses to
can be established quickly, eg
(high-dependency unit, consider in this case are obviously
arrhythmia or acute MI with
critical care unit or intensive pulmonary embolism (PE) and
definitive ECG changes.
treatment unit). pneumonia, although pneumothorax
General supportive measures will be
required by all patients, in particular
rapid restoration of intravascular
volume in those who are volume TABLE 17 DIFFERENTIAL DIAGNOSIS OF PLEURITIC CHEST PAIN
depleted (low JVP and postural
Frequency Cause
hypotension when lying and sitting,
as standing will clearly not be Common PE
possible). Pneumothorax
Pneumonia
Specific management depends on Musculoskeletal: rib fracture, costochondritis, Bornholm myalgia
the diagnosis. (Coxsackie B virus, self-limiting), non-specific
Less common Autoimmune/rheumatic disease
• PE: the two major treatment Pericarditis
options for massive PE with Neoplasia: primary or secondary
Herpes zoster (‘shingles’, difficult to diagnose before the rash)
haemodynamic collapse are
thrombolysis and surgical PE, pulmonary embolism.
embolectomy (see Section 2.18).

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also needs to be considered. A CXR • Position: it is important to identify Also consider risk factors for
will easily exclude a pneumothorax the location of pain and any pneumothorax (chronic respiratory
and infection if the infective process radiation. disease, recent flights or diving)
is advanced to have formed and for pneumonia (exposure to
pulmonary changes. However, Associated clinical features others with the condition and travel,
the diagnosis of PE, be it an early which is especially relevant for
• Dyspnoea: when did this occur
infection or an atypical infection, atypical pneumonias such as
in relation to the pain? A sudden
that does not result in typical Legionella).
onset of pain and breathlessness
radiographic changes may be
together supports PE (although Examination
difficult.
different timing does not exclude
it). How severe is the breathlessness General features
(occurs at rest/patient cannot The first priority is always to
walk/patient cannot hurry)? establish how unwell the patient is.
Beware of the following
in a patient with pleuritic • Cough: a non-specific symptom, • Is she breathless at rest?
chest pain.
but production of purulent
• A low-grade fever (<38°C) does not • Does she look cyanosed? Check
sputum indicates likely infection
necessarily indicate infection; it can pulse oximetry.
and haemoptysis is a feature of
be associated with inflammation of
PE, pneumonia and (exceedingly • Can she speak easily?
any cause, including pulmonary
infarction. unlikely in this case) malignancy.
• Check peripheral perfusion and
• Pneumonia may present in a variety
• Fever: sweats, rigors and confirm pulse, BP, respiratory rate
of ways and sputum production is
not generally an early feature. temperature >38.5°C would and temperature.
• Rib fracture tends to be associated suggest pneumonia. It could also
In this case the routine observations
with a clearly memorable episode of be a feature of autoimmune disease
trauma and is therefore not usually
do not indicate that the patient is
(although unlikely in this case).
a diagnostic difficulty, but remember severely compromised, although she
the possibility of pathological • Unilateral leg pain, swelling clearly has significant symptoms:
fracture. examination can therefore be
or tenderness: all of these
are suggestive of venous completed before a decision
thrombosis and strongly regarding investigation. In patients
support the diagnosis of who appear very unwell with
History of presenting problem PE in this clinical context. cardiorespiratory compromise,
Bear in mind the diagnoses listed in history-taking, examination,
Table 17 when taking the history. investigations and treatment
Other relevant history
Which is most likely? would all begin concurrently
Have there been similar previous
episodes? This is always a good (see Section 1.4.5).
Description of the pain
question to ask – the patient may Note any of the following.
• Character: it is important to tell you the diagnosis! Enquire
• Habitus: tall thin ‘marfanoid’
ensure that the pain is definitely specifically about previous history
young men have an increased risk
pleuritic, ie sharp, localised and of pneumothorax, PE or deep
of spontaneous pneumothorax.
exacerbated by deep inspiration venous thrombosis and
and coughing. autoimmune/rheumatic disease. • Labial herpes: this is often seen in
pneumonia.
• Onset: was it sudden and Pursue other risk factors for
associated with coughing or PE, such as recent immobility, • Signs of autoimmune or
straining (this would indicate surgery, travel, dehydration, rheumatic disease, eg joint
pneumothorax or PE), or gradual? smoking and a family history of PE, deformity in rheumatoid arthritis
Did it follow a ’flu-like illness and deep venous thrombosis or or butterfly rash in systemic lupus
(suggesting pneumonia or hypercoagulable states. In a woman, erythematosus.
Bornholm myalgia) or an incident ask about pregnancy or use of the
of chest trauma (suggesting a oral contraceptive/hormone- Cardiovascular system
pneumothorax or rib fracture)? replacement therapy. Note particularly the following.

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• Pulse: rate and rhythm. Tachycardia secondary to an infection, patient presenting to hospital (sinus
would be expected in a patient percussion will be dull and localised tachycardia will be the most likely
such as this and is non-specific. course crackles and/or bronchial finding in this case).
Atrial fibrillation can occur breathing may be heard.
secondary to PE or pneumonia, Blood tests
If you suspect a PE, examine
but is once again non-specific. FBC, electrolytes, renal and liver
the abdomen for masses and
function tests and a clotting screen
• Signs of right ventricular organomegaly, along with the breasts
would be routine in all cases with
dysfunction, such as elevated JVP, in a woman or the testes in a man.
this presentation. Blood cultures,
left parasternal heave, loud P2 and
atypical respiratory serology screen,
pansystolic murmur of tricuspid
inflammatory markers and tests
regurgitation. Any or all of these
To hear a pleural rub, ask the for autoimmune rheumatic disease
would support the diagnosis of a
patient to point to the place may all be indicated in some patients.
large PE, but a patient presenting that hurts most and listen here and Note that measurement of D-dimers
with a small peripheral PE just around it – you may miss it if you
is not an appropriate investigation in
causing lung infarction and do not take care to do this.
this situation: the clinical probability
pleurisy (as perhaps in this case)
of PE is high and clinical decision-
would not be expected to have
Investigation making will (or should) not be
any of these findings.
The most important investigations affected by the result since imaging
• Heart sounds: is there a in making the diagnosis will be to exclude PE will be required.
pericardial rub? imaging of the chest. Look at the
• Calf swelling and tenderness: CXR carefully for the features listed
measure both sides. A difference in Table 18.
• Do not measure D-dimers if
of >2 cm may indicate venous the clinical probability of PE is
thrombosis and would strongly ECG high: definitive imaging is required
support the diagnosis of PE in Look for arrhythmia, right whatever the result might be.
this clinical context. However, an ventricular strain and pericarditis • Do measure D-dimers if the clinical
(see Section 3.1). Remember that the probability of PE is low. Patients can
absence of swelling does not rule
be reassured that they are extremely
out a PE. ECG is most likely to be normal in
unlikely to have had a blood clot in
someone with PE, and also that pain
their lungs if the result is normal,
Respiratory and other systems and fear are the most common and further imaging is not required.
Thoracic wall tenderness or rib causes of sinus tachycardia in a
crepitus Exquisite local tenderness
clearly suggests a musculoskeletal TABLE 18 SIGNS TO LOOK FOR ON THE CXR IN THE PATIENT
cause, but there can be local
WITH PLEURITIC CHEST PAIN
tenderness with pleurisy. Rib
crepitus proves that a rib has Radiological sign Comment
been broken.
Pneumothorax Look very carefully at the lung apex and bases. Is
Expansion, percussion and there an area within the chest that does not have
auscultation of the chest Is there any lung markings? Can you see a line indicating the
edge of the lung? Are you absolutely sure?
a pleural rub? This can be very
localised. Ask the patient to point Lobar oligaemia A rare sign, but suggesting large PE
to the place that hurts most and Pleural effusion This may be small and visible only as blunting of the
costophrenic angle. This would be consistent with
listen here and just around it. In
the diagnosis of PE or pneumonia
pneumothorax expansion may be
Wedge-shaped peripheral infarcts Typical of PE (but rare)
reduced, the percussion note may
be hyperresonant and breath sounds Consolidation Typical of pneumonia
may be diminished on the affected Ribs and bony structures Look carefully at anterior and posterior aspects of
ribs for fracture lines and more obvious displacement
side. However, with a small
pneumothorax examination may PE, pulmonary embolism.
be normal. If there is consolidation

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Arterial blood gases • Musculoskeletal pain: analgesia and may be septic from a primary
If the patient is unwell or pulse and reassurance. Particularly in infection of his knee joint. A careful
oximetry indicates oxygen saturation the case of rib fracture, adequate history and examination are
<95%, check arterial blood gases. analgesia is essential to allow the essential to help rule out other non-
Some would recommend that this be patient to inspire fully and avoid infective and non-cardiac casuses for
performed for all patients presenting hypostatic pneumonia. Local his symptoms, although these would
with pleuritic chest pain. Typical intercostal nerve blocks can be clearly be very unlikely in this case.
findings in cases of PE where the very effective.
patient presents with pleurisy are History of the presenting problem
• Pericarditis (see Section 2.6).
normal PO2 (but there may be The history (and examination)
hypoxia) and reduced PCO2, both will be dominated by consideration
as a result of hyperventilation. of the most likely diagnosis (IE),
If you diagnose a PE for which
but clues may emerge that take
Sputum you in another direction. Bear the
there is no obvious cause (eg
If present, sputum should be sent for postoperative), perform rectal (and, in diagnoses listed in Table 19 in mind
microscopy, culture and sensitivity, a woman, pelvic) examination and as you take the history and examine
and also for cytology and acid-fast consider pelvic ultrasonography to the patient.
bacilli (indicating tuberculosis) if exclude masses causing deep venous
the clinical picture is appropriate. thrombosis by compression. Gauge the severity of the patient’s
debilitation. In acute IE, the fever is
Other imaging high with rigors and prostration. Ask
If a confident diagnosis cannot be ‘Have you had attacks of really bad
made to explain the pleuritic pain 1.4.7 Fever, weight loss and a shivering and shaking? Have you
and breathlessness, then ventilation– murmur sweated so much that you had to
perfusion lung scanning (see Section change your clothes or the sheets
3.9) or pulmonary CT angiography Scenario on the bed?’ The current history
(see Section 3.8) is required. is more suggestive of a subacute
A 26-year-old man with a presentation, which is associated
Management previous history of intravenous with a low-grade fever, malaise
drug abuse presents with a 6- and weight loss.
week history of recurrent sweats
and weight loss. He comes to the
It is clearly critical to explore
If the probable diagnosis is PE,
Emergency Department because
whether the patient has been
then give anticoagulation
(low-molecular-weight heparin) he is feeling increasingly unwell.
injecting drugs at any point over
immediately while you are waiting On examination he is tachycardic
the past 2–3 months, emphasising
for confirmatory investigations.
and has a swollen, hot and
the point that even a single episode
tender left knee joint and a faint
may be enough to result in a very
pansystolic murmur at the left
dangerous infection. Also enquire
sternal edge. You are called to
about dental procedures or medical
This depends on the specific
assess him.
investigations (particularly if
diagnosis.
invasive), which are other well-
• PE (see Section 2.18; see also recognised risk factors for
Acute Medicine, Section 1.2.10 and endocarditis.
Haematology, Section 3.6). Introduction
Possible causes of this presentation Ask the patient about symptoms of
• Pneumothorax (see Acute
are shown in Table 19. However, in heart failure: has he been breathless
Medicine, Sections 1.2.14 and 3.5;
the presence of a murmur, whether when walking, at rest or lying flat
and Respiratory Medicine,
new or not, the diagnosis of infective in bed at night? Have his ankles
Sections 3.2 and 3.4).
endocarditis (IE) must be the become swollen? These signs may
• Pneumonia (see Acute Medicine, favoured differential diagnosis. This be insidious. If present they raise
Section 1.2.11; Infectious Diseases, remains the case even though the the possibility of haemodynamic
Section 1.3.4 and Respiratory patient is, according to the scenario, compromise from aortic or mitral
Medicine, Section 1.4.1). no longer using intravenous drugs regurgitation. Sudden episodes

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Also rare, but much more suggestive,


TABLE 19 DIFFERENTIAL DIAGNOSIS OF FEVER AND WEIGHT are transient changes in the hands
LOSS OF 6 WEEKS’ DURATION and feet: painful lesions in the finger
or toe pulps (Osler’s nodes) or
Category Common example In presence of a murmur painless ones in the palms or soles
(Janeway’s spots or lesions).
Infective Infective endocarditis Infective endocarditis is the most
Tuberculosis likely The glomerulonephritis that may
Liver abscess
Primary joint accompany IE or autoimmune
infection/osteomyelitis conditions often results in
Soft-tissue infection microscopic haematuria, which
Rheumatic fever (very
unlikely in UK) goes unnoticed by the patient,
although macroscopic haematuria (a
Autoimmune disorders SLE SLE (also some other rheumatic
and/or vasculitis Rheumatoid arthritis disorders) can affect the heart valves symptom that can also be caused by
Polymyalgia rheumatica and cause substantial diagnostic renal infarction or hypernephroma)
Potentially any other difficulty is also possible.
vasculitic condition
Malignancy Lymphoma Atrial myxoma possible, but extremely Back pain may simply result from
Hypernephroma rare. Marantic endocarditis is possible myalgia, but severe loin pain
suggests renal infarction, abscess
SLE, systemic lupus erythematosus.
or tumour, although the latter is not
normally painful. Similarly, pain in
the left hypochondrium radiating to
the left shoulder may result from
of pulmonary oedema may be infective seeding from bacteraemia
splenic infarction or abscess.
suggestive of significant valve or the result of a mycotic embolus
degeneration. from the heart (in the latter, the Regarding other infective causes, ask
infection would have to affect a the patient the following questions.
Ask about chest pain and
left-sided cardiac valve). Ask about
haemoptysis: myocardial infarction • Have you had a cough? Does this
systemic manifestations of the
is rare in endocarditis, but can arise produce any phlegm or blood?
other conditions listed in Table 19,
from coronary artery embolism.
especially autoimmune disorders, • Have you travelled abroad
Pleuritic chest pain and/or
TB and (in this case because of the recently?
haemoptysis would suggest
knee problem) joint/bone infections.
pulmonary abscess or infarction, • Have you ever had TB? Have you
commonly from tricuspid valve The following can be seen with been in contact with anyone who
endocarditis. In this patient, where both IE as well as autoimmune has TB?
tricuspid endocarditis is a real disorders:
• Have you had any injuries to your
possibility, there might be mycotic
• skin rashes; knee?
pulmonary emboli from the right
side of the heart. Remember that • changes in the nails; • Did your cough or knee injury
pulmonary tuberculosis (TB) and start before or after your sweats
• blood in the urine;
(much less likely in this case) and temperature?
other lung pathologies such as • back or abdominal pain;
• Have you had any swollen glands?
malignancies can also present
• changes in vision; Have you had any problems with
with haemoptysis.
the blood or the lymph glands in
• sudden periods of arm or leg
the past?
Other relevant history weakness;
Endocarditis has a very wide range • Have you had arthritis?
• episodes of difficulty speaking.
of extracardiac manifestations (see
• Have you had any odd illnesses in
Section 2.8), and hence many other Although uncommon, vasculitic
the past?
aspects of the history could be rashes can occur with IE, but they
relevant. For instance, the swollen are not specific and may occur with Concerning past history, also ask the
knee joint could be secondary to several of the differential diagnoses. following.

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CARDIOLOGY: PACES STATIONS AND ACUTE SCENARIOS

• Has anyone ever told you that you Cardiovascular tender (consider liver abscess),
have a ‘mumur’ or ‘hole’ in your Take particular note of the and can you feel a renal mass
heart? following. (hypernephroma)?

• Have you had rheumatic fever? • Peripheral perfusion. • Neurological: are there any focal
signs? These are likely to have
• Have you had any antibiotics • Pulse: check rate (often
been caused by emboli from an
recently? Are you absolutely sure tachycardic as in this case),
infected valve in this clinical
about that? (A common reason rhythm and character (‘collapsing’
situation.
for negative blood cultures in pulse in significant aortic
endocarditis is partial treatment regurgitation). • Fundi: is there any evidence
with antibiotics, which render the of endocarditic lesions (see
blood cultures sterile but do not • JVP: this may be elevated if there Section 2.8)?
cure the condition.) is tricuspid valve regurgitation
(often seen in right heart infective Investigations
Examination endocarditis) and/or heart failure.

• Apex: will be hyperdynamic


General features
in sepsis and if displaced is
Just as the history may be relatively
suggestive of long-standing Critical investigations in the
non-specific, the examination patient with chronic fever,
heart disease.
findings may also be so. As always, malaise and weight loss include the
get an overall impression. Patients following.
• Heart sounds: are there any
with IE are likely to look unwell, murmurs or added sounds? The • Blood cultures: at least three
although elderly patients presenting pansystolic murmur could be taken 1 hour apart from separate
atypically may simply be confused. well-cleaned sites.
tricuspid or mitral regurgitation,
• FBC, erythrocyte sedimentation rate
General points must include the but take care to listen carefully (ESR) and C-reactive protein (CRP): is
following. for aortic and/or pulmonary there evidence of systemic
incompetence. Be aware that a inflammation?
• Temperature: a fever of <39°C is difficult murmur/funny sound • Urine: look for haematuria and
typical of endocarditis, although could (extremely rarely) be a proteinuria, which suggest
higher is occasionally seen. glomerulonephritis (autoimmune,
‘tumour plop’.
vasculitic or endocarditic).
• Pallor and anaemia: these can • Chest radiograph: look for TB, lung
suggest chronic disease. Other systems abscess or lymphadenopathy.
Check specifically for the following. • Echocardiography: check for
• Look at the hands, feet, skin, evidence of endocarditis.
conjunctiva and mucous • Lymphadenopathy: this is not a
membranes for splinters/vasculitic feature of endocarditis and would
manifestations of endocarditis. point towards another infective The following are the key
cause or a lymphoproliferative investigations in the patient
condition. with chronic fever, malaise and
Examination of the patient weight loss.
with chronic fever, malaise and • Chest: are there any signs at all?
weight loss Consider TB but remember, as
stated previously, that right heart
Blood cultures
• How does the patient look? These are the single most important
• A thorough examination of all IE may give rise to pulmonary
investigation and should be carried
systems is essential. mycotic emboli.
• Look carefully for skin rashes and out as soon as possible. Three
nail changes. • Abdomen: can you feel the spleen? or more blood samples should
• Look carefully for signs of embolic The splenic tip or a mildly be taken from separate sites at
phenomena. enlarged spleen can be felt in different times, ideally over 24
• Is there lymphadenopathy?
endocarditis, but a moderately hours. Seriously ill patients thought
• Is there evidence of significant
or grossly enlarged spleen to have endocarditis should have
valvular regurgitation?
• Can you feel the patient’s spleen? would favour lymphoma as the samples taken over 1–2 hours and
diagnosis. Is the liver palpable or then be given antibiotics.

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(a)

(b)

(c)

›Fig. 29 Chest radiographs demonstrating (a) TB, (b) lung tumour and (c) abscess.

Other blood tests microscopy to look for casts abscess, pneumonia, mediastinal
FBC, inflammatory markers (indicating renal inflammation), lymphadenopathy or (unlikely here)
(ESR and CRP), electrolytes, and and culture and sensitivity if lung tumour (Fig. 29).
renal, liver and bone function in all dipsticks show abnormality.
cases. A range of further studies, in Echocardiography
particular serological tests for other ECG This is crucial for the detection
infective conditions or autoimmune Look particularly for evidence of of vegetations (or cardiac tumours)
disease, may be indicated if there conduction disturbance (consider and the assessment of valvular
are appropriate clues from the aortic root abscess in this context) regurgitation and paravalvular
history or examination. and atrial fibrillation. abscesses. Transoesophageal
echocardiography may be needed
Urine Chest radiograph (Fig. 30) if good views cannot
Use dipsticks to detect haematuria Look for pulmonary oedema, be obtained on transthoracic
and proteinuria in all cases, heart contour, pulmonary echocardiography. This is

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(b)

(a)

›Fig. 30 (a) Aortic vegetation and (b) para-aortic abscess (arrow). Ao, aorta; LA, left atrium; RV, right ventricle. (Courtesy of Dr J. Chambers.)

particularly relevant if the patient 1.4.8 Chest pain following a Introduction


has prosthetic heart valves or the ’flu-like illness The history immediately suggests
clinical suspicion for endocarditis an acute viral pericarditis. This is
is high even if transthoracic Scenario usually a mild and self-limiting
echocardiography is completely condition, so the greatest danger
normal. A 25-year-old woman presents arises from failure to recognise more
to the Emergency Department serious pathology that presents in a
Management complaining of a 4-day history of similar manner (Table 20). You must
This depends on the specific chest pain. She has been unwell initially decide whether the pain is
diagnosis. with ’flu for the last week. You caused by pericarditis, excluding
are asked to review the patient. other causes of chest pain along
• IE: see Section 2.8.1.
the way. If you decide it is, then you
• Left atrial myxoma: see
Section 2.9.

• See Infectious Diseases, TABLE 20 DIFFERENTIAL DIAGNOSIS OF PERICARDITIC CHEST PAIN

Haematology, Oncology, and


Comment Diagnosis
Rheumatology and Clinical
Immunology modules for further Common Acute pericarditis
information on the many diseases Musculoskeletal
Oesophagitis
that can present with fever and
which are differential diagnoses Must consider Pneumonia
Pulmonary embolism
of IE. Autoimmune rheumatic disease
Do not completely forget Myocardial ischaemia
Aortic dissection

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need to be aware of the more serious Pericarditic pain is usually


underlying causes of pericarditis and continuous, but does not typically • Musculoskeletal pain: history of
unaccustomed activity with chest
must not immediately assume viral have a sudden onset, unlike PE.
wall tenderness.
aetiology. It is exacerbated by inspiration,
• Oesophagitis: suggested by dyspeptic
movement and lying supine, and is symptoms, particularly belching and
As there is overlap between the
typically eased by sitting forward. reflux, and is worse at night.
symptoms of pericardial and • Pneumonia: fever (sometimes
Aside from movement itself, there is
pleural inflammation, you will need rigors), breathlessness, malaise and
no relationship with exertion, unlike
to consider pulmonary pathologies, (sometimes) pleuritic pain; also a
angina.
particularly pneumonia and painful cough that is dry initially,
pulmonary embolism (PE). but later productive of sputum.
Other symptoms • PE: sudden onset of pleuritic pain
Remember that serositis is also
This woman has had ’flu-like with breathlessness and
a feature of some autoimmune haemoptysis.
symptoms recently, but ask any
rheumatic diseases, such as • Myocardial infarction or aortic
patient presenting with chest pain
systemic lupus erythematosus and dissection: however unlikely you
the following questions.
rheumatoid arthritis. consider these to be in a young
patient, think of them if the
• Have you been feeling ‘under the
Both myocardial infarction symptoms are ‘ischaemic’ or
weather’ or feverish recently?
and aortic dissection would be ‘tearing’ in nature.

extremely unlikely in this patient, • Do you feel breathless?


but because of their potentially fatal
• Have you coughed up any Other relevant history
consequences they should always be
phlegm? If so, what colour was You will obviously ask whether there
considered, even if only briefly and
it and did it contain any blood? have been any similar episodes in
to dismiss them, in any patient
presenting with chest pain. • Have you had any joint pains? the past and, if so, what diagnosis
(if any) was made. This woman
• Do you ever get an acid taste at is very likely to have a viral
History of the presenting problem
the back of your mouth? pericarditis, but ask about other
Get as much information about
the pain as possible. Ask the patient Although painful breathing may conditions that could be associated
‘Show me where you feel it? What cause dyspnoea in pericarditis, with acute pericarditis:
is it like? Does it go anywhere else?’ prominent respiratory symptoms • rheumatoid arthritis and other
If a clear description of the pain is are clearly more in keeping with autoimmune rheumatic disease;
not forthcoming, offer suggestions pulmonary pathology. Haemoptysis
• renal failure;
such as ‘like a knife’ or ‘raw’, both would suggest PE. The presence of
of which suggest pericarditic arthralgia may simply reflect the • hypothyroidism;
pain, or ‘like a heavy weight’ or associated viraemia, but could also
• rheumatic fever;
‘squeezing’, which do not. The pain be due to autoimmune rheumatic
of pericarditis is usually located disease. Acid reflux suggests • tuberculosis (TB) and contact
retrosternally, and like angina it may oesophagitis, as would a history with TB;
radiate to the neck or shoulders. of indigestion.
• malignancy, eg breast;
Ask specifically about the following.
• chest radiotherapy.
• Did the pain start suddenly? When taking a history from a
young patient at low risk of Examination
• Do you have pain all the time? ischaemic heart disease who presents
with chest pain, look for the following General features
• If not, when do you get it? patterns. As always, form an overall
• Does it hurt when you breathe in? • Pericarditis: indicated by a impression first. A patient with
continuous sharp or raw retrosternal uncomplicated pericarditis is
• When you sit forward, does the discomfort, which is worse on lying
unlikely to look very unwell: he or
pain change? down and relieved by sitting
she may be in pain, which may be
forwards. This is often accompanied
• Is the pain affected by lying by a history of a ’flu-like illness. severe, and is likely to be sitting
down? forward rather than lying supine.

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If the patient with suspected • Pulse: check rate, rhythm and


pericarditis is unwell, then you character.
should assess immediately for When examining a young
• BP: a significant (>10 mmHg) fall patient at low risk of ischaemic
evidence of tamponade, PE or
on inspiration (pulsus paradoxus) heart disease who presents with
pneumonia, which may be life- chest pain, look specifically for the
indicates cardiac tamponade.
threatening. following.
• JVP: should be normal in • Pericarditis: is there a pericardial
In all cases check temperature uncomplicated pericarditis. rub? Look for an underlying
(pyrexia indicates an inflammatory Gross elevation could be due to aetiology.
cause of pain), examine the sputum cardiac tamponade or PE. • Is there evidence of cardiac
pot (haemoptysis suggests PE) and tamponade? Look for poor
ask ‘Does it hurt when I press on • Heart sounds: a pericardial rub peripheral perfusion, elevated
would clinch the diagnosis. venous pressure and tachycardia
the chest where the pain is?’ This
with a small-volume pulse
would clearly suggest a local exhibiting pulsus paradoxus
musculoskeletal cause, but Respiratory and other systems (BP falls significantly on inspiration).
remember that there can be This is an emergency and requires
• Look for chest signs suggestive of
tenderness in pleurisy. pericardiocentesis.
pneumonia or PE. • Musculoskeletal pain: pain that is
well localised and reproduced by
Cardiovascular system • Is the patient euthyroid?
local pressure on the chest wall.
Perform a full cardiovascular • Pneumonia: fever with signs of focal
• Are there any signs of malignancy,
assessment, taking particular lung consolidation. Listen for a
eg breast?
pleural rub.
note of the following.
• PE: is there haemoptysis in the
• Is there evidence of autoimmune
sputum pot? Listen for a pleural
• Peripheral perfusion: is this rheumatic disorder, eg joint
rub. Are there signs of pulmonary
impaired? It should not be in inflammation or deformity, hypertension? (See Sections 2.12.1.)
uncomplicated pericarditis. or a rash?

›Fig. 31 ECGs of (a) anterior myocardial infarction.

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›Fig. 31 ECGs of (b) acute pericarditis, where widespread ST-segment elevation, concave upwards, is seen.

evidence of autoimmune rheumatic Chest radiograph


• Aortic dissection: a rank outsider. disorders (see Rheumatology and Look for cardiomegaly, suggesting
However, does the woman look
Clinical Immunology, Section 3.2). an effusion, and for any areas of
as though she may have Marfan’s
Checking ‘viral titres’ is not generally consolidation or pleural effusion
syndrome? Can you feel the
left radial pulse? Is the BP useful. (Fig. 32).
the same in both arms?
(See Section 2.11.1.)

Investigations

ECG
Along with chest pain and
pericardial rub, the changes
in the ECG encountered in
acute pericarditis form a triad
of characteristic findings that
can establish the diagnosis
(Fig. 31).

Blood tests
FBC, electrolytes, renal and
liver function, thyroid function,
inflammatory markers (erythrocyte
sedimentation rate and C-reactive
protein) and cardiac enzymes.
If clinically indicated, check
blood cultures and serology for ›Fig. 32 Cardiomegaly. Note the increased cardiothoracic ratio in this posteroanterior film.

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Other tests
Ventilation–perfusion scanning
should be performed if clinically
indicated.

Management
Management depends on the
specific diagnosis. Uncomplicated
viral pericarditis is a self-limiting
illness. If patients are reasonably
well, they should be reassured that
they have not had a heart attack and
can be discharged home with simple
analgesia, NSAIDs often being
particularly effective. Follow-up in a
few weeks’ time should be organised
›Fig. 33 Large pericardial effusion: the heart is surrounded by an echo-free space (e) formed by the to ensure that the symptoms have
effusion. a, left atrium; b, left ventricle; c, right atrium; d, right ventricle. (Courtesy of Dr J. Chambers.) settled. Admission is warranted
if the pain is severe, if there is
Echocardiography not exclude this diagnosis. This evidence of a large pericardial
An echocardiogram that reveals a investigation is mandatory, and effusion or tamponade (a medical
small amount of pericardial fluid urgent if the cardiac shadow is emergency) or if treatment of an
can be very helpful in making the enlarged and you suspect cardiac underlying aetiology is indicated.
diagnosis of pericarditis, but the tamponade (Fig. 33).
absence of pericardial fluid does

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CARDIOLOGY: SECTION 2
DISEASES AND TREATMENTS

Physical signs • Stress imaging includes


2.1 Coronary artery Examinations are often normal, myocardial nuclear perfusion,
disease but watch for signs of aortic stress echocardiography and
stenosis and anaemia, and check magnetic resonance stress
the peripheral pulses and BP. imaging. This may be used in
Coronary artery disease (CAD) is the patients unable to exercise or
cause of two of every 10 deaths in who have bundle branch block
Investigations
the UK, accounting for 114,000 (see Section 3.11).
Figure 34 shows an investigation
deaths in the UK in 2005. In the UK
algorithm. • Cardiac catheterisation
260,000 people have a myocardial
infarction every year. • Resting ECG: this is often normal (see Section 3.12).
and does not exclude coronary • Blood tests: FBC and fasting
2.1.1 Stable angina disease. lipids.

Aetiology/pathophysiology/ • Exercise ECG: this may show Patients who have chest pain
pathology exercise-induced ischaemic without diagnostic ECG changes
Atheromatous plaques in the changes, confirming the diagnosis during exercise testing may have
coronary arteries reduce blood flow. and giving objective evidence of important coronary disease.
The myocardial oxygen supply exercise capacity and prognosis It is important to stratify patients
cannot meet the demand, resulting (see Section 3.1.1). by their presentation and risk
in myocardial ischaemia and chest
pain (angina pectoris).

Epidemiology
The prevalence of angina in the UK
is estimated as 3% of the population,
ie 2,000,000 individuals.

Clinical presentation

Common
• Predictable exertional central
chest tightness.

• Worse in cold weather and after


meals.

• May radiate to jaw or the left arm.

• Pain resolves with rest.

Uncommon
• Exertional dyspnoea (angina
equivalent). This is more common
in females. ›Fig. 34 Algorithm for patients presenting with suspected stable angina.

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• left main-stem stenosis (>50%);

• proximal left anterior descending


stenosis (>70%);

• three-vessel disease, especially


with impaired left ventricular
function.

PCI is carried out more


commonly than CABG, and is
currently indicated for symptomatic
stenoses of >70%. At present an
improvement in life expectancy has
not been demonstrated, but trials
are ongoing. Patients who are unfit
for CABG may be considered for
PCI. See Fig. 36 for an investigation
algorithm.

Prognosis
Generally, stable CAD confers a good
prognosis. Cardiac catheterisation of
›Fig. 35 Left coronary arteriogram demonstrating major stenosis in two major vessels with retrograde patients identified to be at high risk
filling of a blocked right coronary artery (arrow) via collaterals. This patient has three-vessel coronary
disease. by non-invasive stress testing will
identify those with prognostically
important disease.
factors into low- and high-risk enzyme inhibitors should be
groups. Patients at high risk of prescribed to patients with normal
CAD with exertional chest pain left ventricular function and
but without diagnostic ischaemic ischaemic heart disease.
Important information for
changes should proceed to
patients
diagnostic cardiac catheterisation Revascularisation
(Fig. 35). Those at low risk of There are two methods of • The condition is stable.
• There are important narrowings in
ischaemic heart disease should revascularisation: coronary
the arteries.
undergo stress imaging to search artery bypass grafting (CABG) • Continue exercise but avoid
for ischaemia. If these investigations and percutaneous coronary strenuous exertion, especially heavy
demonstrate ischaemia, then intervention (PCI) involving lifting.
cardiac catheterisation should angioplasty and stenting. Currently, • GTN before exertion may be helpful.
• Chest pain at rest continuing for
be considered. the evidence is that CABG confers
greater than 15 minutes despite GTN
prognostic advantage in certain
warrants immediate hospital
Treatment groups, with diabetics showing a admission.
particular improvement in:
Lifestyle advice
Encourage regular exercise.
Prophylactic sublingual glyceryl
trinitrate (GTN) administered before
exertion may be helpful. Stress the
importance of stopping smoking.

Medical therapy
First-line therapy is with aspirin,
GTN spray, beta-blockers and a
›Fig. 36 Algorithm for interventional treatment of coronary artery disease. PTCA, percutaneous coronary
statin. Angiotensin-converting angioplasty.

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• Cardiac biomarkers
(see Section 3.7).

• Cardiac catheterisation (Fig. 38)


(see Section 3.12).

Differential diagnosis
After excluding ST-segment
elevation myocardial infarction,
other diagnoses should be
considered. These are the same
›Fig. 37 Schematic diagram illustrating the sequence of events within a coronary artery during an acute
as the differential diagnoses for
coronary syndrome. Fissuring or erosion of an atherosclerotic plaque (yellow) leads to thrombosis (red), a ST-segment elevation myocardial
rapid reduction in coronary blood flow and myocardial infarction. Sustained occlusion of the coronary artery
leads to Q-wave (transmural) infarction. Highly sensitive troponin assays can detect microembolic heart infarction.
muscle damage, enabling identification of those at higher risk of further infarction. CK, creatine kinase.

Treatment
2.1.2 Unstable angina and Physical signs The aim of treatment is to limit
non-ST-elevation myocardial Often there are no abnormalities myocardial damage by ‘pacifying’
infarction on examination, but other causes the vulnerable plaque with
of chest pain may be excluded. medication. Patients who fall into
Aetiology/pathophysiology/ high-risk groups undergo cardiac
pathology Investigation catheterisation.
An atheromatous plaque within a
• ECG: often normal, but look
coronary artery reduces the blood Emergency
for ST-segment changes and
flow to such an extent that there is
T-wave inversion. T-wave • Aspirin, sublingual GTN followed
ischaemia at rest (unstable angina).
inversion alone has no by an infusion, beta-blocker,
The plaque may fissure or erode
prognostic significance. low-molecular-weight heparin
exposing the dense lipid core.
The lipid is very thrombogenic,
causing platelets to clump on it. If
the thrombus is not sufficient to
occlude the artery, it reduces blood
flow downstream and pieces of the
thrombus may break off and pass
downstream to lodge in small end
vessels, causing non-ST-elevation
myocardial infarction (Fig. 37).

Clinical presentation
• Rapid onset of central chest pain
at rest, unrelieved by glyceryl
trinitrate (GTN).

• Pain may escalate, and occur with


increasing frequency and severity
(crescendo angina).

• Pain may radiate to jaw and arm.

• Severe breathlessness may reflect


pulmonary oedema due to
transient impairment of left
›Fig. 38 Right coronary angiogram in a patient with non-ST-elevation myocardial infarction. There is
ventricular function. extensive thrombus present, seen as filling defects within the lumen (arrows).

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and a statin. Chronic obstructive Uncommon • Vomiting and sweating without


pulmonary disease and pain.
• Pulmonary oedema.
peripheral vascular disease
• Painless infarct (diabetics and
are not contraindications • Ventricular arrhythmias.
the elderly).
to beta-blockers, and these
groups benefit greatly from Prognosis
The risk of death or non-fatal Physical signs
cardioselective beta-blockers.
myocardial infarction is 7% at
• Clopidogrel (with an initial 6 months with appropriate Common
loading dose). revascularisation of high-risk cases. Examination may be normal.
Patients are pale, sweaty and
• Glycoprotein IIb/IIIa antagonists
are recommended for patients 2.1.3 ST-elevation myocardial often appear to be in pain. The

with unstable symptoms and a infarction patient may be hypotensive or


hypertensive. Listen for pulmonary
positive troponin assay.
Aetiology/pathophysiology/ oedema.
pathology
In-hospital management
Continue antianginals and
Rupture of an atheromatous plaque Uncommon
exposes the rich lipid core to the Epigastric tenderness.
antithrombotics. Patients who
circulating platelets and fibrin,
do not have elevated troponin
which forms thrombus, occluding Investigations
are diagnosed with unstable
the artery. Occlusion will lead to full-
angina and do not require urgent • ECG: ST-segment elevation and
thickness muscle necrosis. Transient
cardiac catheterisation unless new onset left bundle-branch
occlusion leads to limited heart
they fall into a high-risk group block are indications for
muscle death.
(see below). Cardiac catheterisation reperfusion treatment (Fig. 39).
with a view to revascularisation
Epidemiology • Biochemical markers
is indicated in high-risk
groups: • Approximately 300,000 myocardial (see Section 3.7).
infarctions occur every year in the • Cholesterol (level may drop
• positive troponin assay;
UK. after 24 hours for up to
• ongoing ischaemic pain; 3 months).
• One-quarter of patients do not
• ST-segment changes with pain; reach hospital. • Echocardiogram: indicated in
• Death rates are declining. cardiogenic shock.
• recurrent unstable angina;

• ventricular arrhythmias. Clinical presentation Differential diagnosis


• Unstable angina/non-ST-elevation
Long-term treatment Common
myocardial infarction.
Patients admitted with unstable
• Rapid onset, severe and crushing
angina are treated medically, and • Thoracic aortic dissection.
central chest pain radiating to
if the pain settles they are assessed
the jaw/left arm that is unrelieved • Musculoskeletal pain.
in the same way as patients with
by glyceryl trinitrate (GTN) or
stable angina. Medications should • Pain of gastric/oesophageal
oxygen.
be continued, and if the troponin origin.
is positive clopidogrel is continued • Acute breathlessness (pulmonary
for 1 year. oedema). Treatment
• Cardiac arrest (ventricular
Complications fibrillation). Emergency
It is vital to start reperfusion
Common Uncommon treatment as soon as possible,
either with thrombolysis or primary
• Recurrent angina. • Epigastric, arm or back pain.
percutaneous coronary intervention
• Myocardial infarction. • Collapse. (PPCI).

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›Fig. 39 Twelve-lead ECG showing acute anterior myocardial infarction.

• Aspirin 300 mg if no
contraindications. thrombolysis should be obtained
Indications for thrombolysis from the patient, and the risk of
• Clopidogrel 600 mg. haemorrhagic stroke should be
• ST-segment elevation >1 mm
explained. The following are
in the standard leads, >2 mm in the • Once ST-segment elevation considered contraindications to
chest leads or new bundle branch myocardial infarction (STEMI) thrombolysis:
block.
is confirmed on ECG, quickly • active internal bleeding;
• Typical chest pain within 12 hours of
onset. examine the patient and ensure • active peptic ulcer;
• Bundle branch block: if there are no there are no contraindications to • uncontrolled bleeding tendency;
old ECGs and there is a good history thrombolysis/angiography and • stroke in the last 6–12 months or
for a myocardial infarction, then the antiplatelet therapy. any previous haemorrhagic stroke
patient should be thrombolysed. (obtain neurosurgical advice in the
• Transfer to coronary care unit case of clipped subarachnoid
Indications for PPCI after reperfusion initiated. haemorrhage aneurysm);
• aortic dissection;
• ST-segment elevation >1 mm in the
• Intravenous insulin for diabetics, • uncontrolled BP >180/110 mmHg
standard leads, >2 mm in the chest
or where blood glucose (treat with intravenous GTN, then
leads or new bundle branch block.
thrombolyse);
• Typical chest pain within 24 hours of >11 mmol/L.
• major trauma or surgery within the
onset.
See Fig. 40. past year.
• In the presence of cardiogenic shock,
PPCI is more effective than
thrombolysis.

Contraindications to
thrombolysis Ninety minutes after thrombolysis
• Take history whilst obtaining
intravenous access. Consultant advice should be sought the ECG should be recorded. If the
before deciding not to reperfuse a ST segments do not show greater
• Simultaneous recording of ECG patient with STEMI, and PPCI should
than 50% resolution, then rescue
and observations by nursing staff. be considered. Prolonged resuscitation
percutaneous coronary intervention
is not a contraindication unless there
• Oxygen, opiate analgesia and is obvious trauma. Oral consent to may be appropriate. Obtain
antiemetic. consultant advice.

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Contraindications to PPCI

Consultant cardiologist advice


should be sought prior to transfer to a
catheter laboratory.

• Unable to take aspirin and


clopidogrel.
• Severe peripheral vascular disease
making femoral or radial
catheterisation impossible.
• Active bleeding, preventing
administration of antiplatelet drugs.
• Thrombocytopenia.
• Allergy to iodine (contrast contains
iodine).
• Inability to lie flat: intubation may
be necessary if patient has resistant
pulmonary oedema.

Patients who present within (a)


2 hours of pain have similar
outcomes with both treatments.
Thereafter, morbidity and mortality
are lower with PPCI compared
with thrombolysis. Patients in
cardiogenic shock are best treated
by PPCI and may have an intra-
aortic balloon pump inserted.

Short term
Standard treatment should include
the following.

• Beta-blocker: taken orally within


24 hours and continued indefinitely.

• An angiotensin-converting
enzyme (ACE) inhibitor is of
particular benefit in patients
with left ventricular (LV)
(b)
impairment, but improves
the prognosis of all patients.
›Fig. 40 Left coronary arteriogram showing a severe stenosis in the left anterior descending artery.
• Statin: for all patients unless (a) This patient had a prolonged ventricular fibrillation arrest due to an anterior myocardial infarct that
contraindicated. was initially thrombolysed. (b) The stenosis has been stented.

• Sliding insulin scale for patients • Clopidogrel: given for up to small biomarker rises suggesting
with blood sugar >11 mmol/L or 30 days after thrombolysis, or little damage from the infarct.
known diabetics. for up to 12 months after PPCI.
Long term
• Warfarin: indicated for patients • Cardiac catheterisation and Continued secondary prevention
with atrial fibrillation, severe revascularisation is recommended is important. Glucose tolerance
LV dysfunction due to risk of for all patients within 24 hours of testing should be considered as a
LV thrombus, or who exhibit STEMI. Definite indications include high proportion of patients have
presence of LV thrombus (Fig. 41). recurrent pain, reinfarction or diabetes mellitus (30% of these

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CARDIOLOGY: DISEASES AND TREATMENTS

• 25% treated conservatively


develop unstable angina;

• 10% treated conservatively have a


further infarct;

• 25% with a first myocardial


infarction do not reach hospital
alive;

• 10% die before discharge;

• 10% die in the year after discharge.

Prevention

Primary
Prevention involves regular exercise,
a healthy diet and stopping smoking.

Secondary
• Aspirin, beta-blocker, ACE
inhibitor, statin and clopidogrel.
›Fig. 41 Left ventriculogram showing apical mural thrombus (arrow) after anterior myocardial infarction.
• Patients with poor LV function
(ejection fraction <35%) undergo
patients are missed when fasting • Stroke: urgent CT of head required.
repeat echocardiography at 6
glucose alone is tested). • Deep vein thrombosis/pulmonary weeks to see if remodelling has
embolus. occurred. If LV function remains
Complications
poor, an implantable cardioverter
Uncommon defribrillator may be indicated
Common
• LV rupture (Fig. 42). (see Section 3.4.2).
• Haemorrhage induced by
• Ventricular septal defect. • Patients with poor LV function may
thrombolysis: transfuse as required.
• Severe mitral regurgitation from be treated with spironolactone
• Haemorrhage from arterial or eplerenone if creatinine
papillary muscle rupture or
puncture site: apply direct <180 µmmol/L.
ischaemia.
pressure, transfuse as required.
• Dressler’s syndrome: fever,
• Ventricular fibrillation/tachycardia:
pleuropericarditis, anaemia, raised
cardiovert prompty. Advice to patients
erythrocyte sedimentation rate,
• Atrial fibrillation. usually 1– 4 weeks after infarct. On admission:

• Complete heart block. Prognosis • Inform the patient that he or she is


having a heart attack and that
• Pulmonary oedema. This is determined by LV function, effective treatment is available.
comorbidity and whether the patient • Advise that the risk of treatment is
• Cardiogenic shock: seek expert
has been revascularised. Stress testing less than the risk of the heart attack.
advice. • Caution that the main risk is of
identifies patients with ischaemic
myocardium who require cardiac bleeding, and that thrombolysis
• Post-infarct unstable angina.
carries the risk of stroke.
catheterisation and revascularisation.
• Reinfarction.
Patients with poor LV function On discharge:
• Post-infarct pericarditis: usually undergo repeat echocardiography at
• A good recovery is expected.
benign and responds to NSAIDs; 6 weeks to see if remodelling has
• Cardiac rehabilitation: patients are
echocardiogram is needed to occurred. Of patients suffering a seen prior to discharge by the
exclude contained rupture. STEMI:

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CARDIOLOGY: DISEASES AND TREATMENTS

(a)

(b)

(c)

›Fig. 42 Rupture of the inferior–posterior wall of the left ventricle (LV) after an acute myocardial infarction. (a) Transoesophageal echocardiogram showing flow
through the defect in the LV wall. (b) Left ventriculogram: a pigtail catheter has been passed through the defect, outlining the LV, the defect and the pericardium.
(c) LV at operation showing the hole on the inferior–posterior surface of the ventricle.

specialist nurses, an exercise FURTHER READING 2.2 Cardiac arrhythmia


programme is arranged and support Hobbs FDR. Cardiovascular disease:
to help them stop smoking is different strategies for primary and
offered. Car drivers must cease secondary prevention. Heart 2004; 90: 2.2.1 Bradycardia
driving for 4 weeks, and heavy 1217–23.
goods drivers must inform the Aetiology
DVLA (Driver and Vehicle Licensing
Fox KAA. Management of acute Virtually any condition that has
Agency) in the UK and must satisfy
coronary syndromes: an update. Heart a pathophysiological effect on
requirements prior to relicensing
2004; 90: 698–706. the heart might affect normal
(see Section 2.19).
• The usual advice regarding fitness electrophysiological properties and
for sexual activity is that the patient De Jaegere PP, Serruys PW and Simoons
thus cause bradycardias. The more
should be able to climb one flight of ML. Should all patients with an acute
myocardial infarction be referred for
common conditions are listed in
stairs prior to intercourse.
direct PTCA? Heart 2004; 90: 1352–7. Table 21.
• Smoking cessation is vital: it reduces
risk of reinfarction by 50%.
• Always carry GTN, even if free of Kristensen SD, Andersen HR, Thuesen L,
angina. et al. Heart 2004; 90: 1358–63.

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• beat-to-beat variation in intensity


TABLE 21 POSSIBLE CAUSES OF BRADYCARDIA of first heart sound in complete
heart block;
Sinoatrial disease Atrioventricular block
• hypotension;
Ischaemic heart disease Ischaemic heart disease
Idiopathic fibrosis Aortic stenosis • pulmonary oedema.
Infective Cardiomyopathy
Pericardial disease Infection Consider using carotid sinus
Post radiotherapy Sarcoidosis massage to provoke the bradycardia.
Post cardiac surgery Congenital
Do not perform this in patients who
Trauma Connective tissue disease
Antiarrhythmic drugs Antiarrhythmic drugs have had a stroke or are known
Amyloidosis Post radiotherapy to have atherosclerotic carotid
Post cardiac surgery disease.
Trauma
Hypothermia
Investigations
In most cases the diagnosis
will be made with one of the
Pathology/pathophysiology Clinical presentation following:

Sinoatrial dysfunction Common • 12-lead ECG (see Section 3.1);

• Abnormality of neurohormonal • Dizziness (presyncope). • Holter monitor (see Section 3.3);


input to sinoatrial (SA) node,
• Syncope. • patient-activated device;
eg sympathetic/parasympathetic.
• tilt-table testing.
• Abnormality of SA node leading Uncommon
to slow or failed conduction to
• Dyspnoea. Treatment
atrial tissue.
• Exertional fatigue. Emergency/short term
Atrioventricular block In a patient with haemodynamic
• Heart failure.
• Abnormality in conduction compromise consider the
through atrioventricular (AV) following:
Rare
node.
Palpitations are unusual. • intravenous atropine;
• Failure to conduct rapidly
• temporary pacing, either
throughout the ventricles. Physical signs
transvenous or transcutaneous
These include the following:
(short-term measure).
Classification of bradycardias
• slow regular/irregular pulse;
Bradycardias can be divided Address any potential reversible
clinically into SA dysfunction • cannon waves in complete heart causes of bradycardia:
and AV block (Table 22). block;
• hypothyroidism;

• drugs (Fig. 43);

TABLE 22 CLINICAL CLASSIFICATION OF BRADYCARDIAS • hypothermia;

Sinoatrial dysfunction Atrioventricular block • electrolyte imbalance.

Sinus bradycardia First degree Long term


Vasovagal syndrome Second degree: Mobitz I (Wenckebach’s)
Consider whether permanent
Carotid sinus hypersensitivity Second degree: Mobitz II
Junctional rhythm Third degree: complete pacemaker implantation is
appropriate (see Section 3.5).

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CARDIOLOGY: DISEASES AND TREATMENTS

›Fig. 43 Twelve-lead ECG of patient with ischaemic heart disease who presented with presyncope and was taking beta-blockers. Heart rate is <40/minute.

tachycardia. On an ECG it may look antegradely (atrium to ventricle) via


FURTHER READING similar to sinus tachycardia but with the atrioventricular (AV) node and
Zipes DP. Specific arrhythmias: abnormal P-wave morphology. retrogradely through the accessory
diagnosis and treatment. In: pathway (concealed accessory
Braumwald E, ed. Heart Disease: A
Atrial fibrillation/flutter pathway). If conduction is in an
Textbook of Cardiovascular Medicine,
6th edn. Philadelphia: WB Saunders; Atrial fibrillation affects 0.4% of the antegrade direction through the
2001: 815–90. whole population, rising to 2– 4% in pathway (eg Wolff–Parkinson–White
people over 60 years old and >11% syndrome), pre-excitation is seen on
in those over 75 years old. Its causes the surface ECG.
2.2.2 Tachycardia are numerous:
For practical purposes it is easiest to Ventricular tachycardias
• hypertension;
divide tachyarrhythmias into: Almost any pathological process
• ischemic heart disease; affecting the ventricles may
• atrial tachycardia;
predispose to VT:
• atrial fibrillation/atrial flutter; • congestive heart failure;
• myocardial infarction
• atrioventricular nodal re-entry • valvular heart disease; (acute/chronic);
tachycardia (AVNRT) and • thyroid dysfunction; • dilated cardiomyopathy;
atrioventricular re-entry
tachycardia (AVRT); • pulmonary abnormalities, eg • hypertrophic cardiomyopathy;
pulmonary embolism;
• ventricular tachycardia (VT). • valvular heart disease (especially
• pericardial disease. aortic stenosis and mitral
Aetiology/epidemiology prolapse);
AVNRT/AVRT
• hypertension;
Atrial tachycardia These are the result of the presence
Atrial tachycardia is caused by an of an additional conducting • congenital heart disease;
ectopic source of atrial tissue firing pathway, allowing a re-entry
• long QT syndrome;
in a rhythmical manner faster than mechanism. In most cases, the
the sinus node. It is a rare cause of electrical impulse is conducted • cardiac tumours.

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CARDIOLOGY: DISEASES AND TREATMENTS

cases, establish the diagnosis


TABLE 23 CLINICAL FEATURES DISTINGUISHING ATRIAL (see Section 3.1). In some
FIBRILLATION FROM ATRIAL FLUTTER cases where there is evidence
of pre-excitation, the diagnosis
Atrial fibrillation Atrial flutter can be relatively confidently
made in sinus rhythm.
Pulse Irregularly irregular May be regular
JVP Absence of a waves Rapid flutter waves Distinguishing some arrhythmias
First heart sound Variation in intensity Constant intensity can be difficult.

• Atrial flutter versus atrial


fibrillation: look for
Pathophysiology/pathology AVNRT/AVRT characteristic flutter waves
Tachyarrhythmias occur as a Physical signs are not especially (Figs 44 and 45).
result of: helpful in making the diagnosis:
• AVNRT versus AVRT:
• abnormal automaticity, eg VT • regular pulse; distinguishing these is rarely
after myocardial infarction; • JVP may be raised, but waveform of clinical importance because,
is normal; in most cases, management is
• triggered activity, eg VT with long
similar (Fig. 46).
QT syndrome; • constant intensity of first heart
sound. • VT versus AVNRT/AVRT
• re-entry, eg atrial fibrillation,
AVRT/AVNRT, VT, ventricular with aberrant conduction
Ventricular tachycardias (see Section 3.1).
fibrillation.
Patients may or may not be
significantly compromised. Ambulatory monitoring
Clinical presentation
Physical signs include: Documentation of an infrequent
Common • hypotension; rhythm may be possible using
24-hour Holter monitoring or
• Palpitations. • cannon waves.
patient-activated devices
• Presyncope/syncope. Investigations (see Section 3.3).
Investigations aim to exclude
• Breathlessness.
a structural thoracic/cardiac or Electrophysiological studies
• Chest pain. metabolic cause. Aside from an See Section 3.2.
ECG, CXR, echocardiogram, thyroid
Uncommon function tests, renal function and Treatment
Patients may complain only of electrolytes are appropriate in most There are many different
lethargy. cases. classifications of antiarrhythmic
agents. The Vaughan Williams
Rare Twelve-lead ECG classification is the most commonly
Thromboembolism is unusual, Documenting the arrhythmia used and is based on the cellular
except in atrial fibrillation. with a 12-lead ECG will, in most action of the drug (Table 24).

Physical signs
Examination of sinus rhythm TABLE 24 VAUGHAN WILLIAMS CLASSIFICATION OF
may be unremarkable. However, ANTIARRHYTHMIC DRUGS
during tachycardia some physical
signs may help to establish a Class Action Example
diagnosis. IA Prolong action potential Quinidine, procainamide, disopyramide
IB Shorten action potential Lidocaine, mexiletine
Atrial fibrillation/flutter IC Slow conduction Propafenone
II Block β-adrenergic receptors Propranolol, atenolol, metoprolol
For the physical signs of atrial
III K+ channel blockers, prolong repolarisation Sotalol, amiodarone
fibrillation/atrial flutter, IV Block slow calcium channels Verapamil, diltiazem, nifedipine
see Table 23.

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CARDIOLOGY: DISEASES AND TREATMENTS

›Fig. 44 ECG of atrial flutter: note the characteristic saw-tooth appearance of the baseline.

›Fig. 45 ECG of atrial fibrillation. Notice the ‘chaotic’ baseline in comparison with Fig. 44 and the complete irregularity of QRS complexes.

Emergency Atrial fibrillation/flutter If the Verapamil may be used if you


Atrial tachycardia Most patients patient is compromised, consider are confident that the rhythm
will not be compromised. DC DC cardioversion or intravenous is not VT.
cardioversion or intravenous amiodarone.
Ventricular tachycardias For
amiodarone will restore sinus
AVNRT/AVRT Most will respond resuscitation, see Acute Medicine,
rhythm in the majority.
to intravenous adenosine. Section 1.2.1.

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›Fig. 46 ECG of AVNRT. Note the very rapid rate, regular rhythm and absence of discernible P waves.

Short term AVNRT/AVRT Consider the following: Atrial tachycardia The focal origin
Atrial tachycardia Consider the of these arrhythmias makes them
• class IA and IC agents;
following: particularly suited to catheter
• class II (beta-blockers); ablation.
• class I agent (if structurally normal
heart and no coronary disease); • class III (sotalol); Atrial fibrillation/flutter Consider
• beta-blocker; the following:
• class IV (verapamil).
• class III agent; Ventricular tachycardias Consider • anticoagulation;

• calcium antagonist. the following:


• ablation for atrial flutter
• class III agents; (see Section 3.4.1);
Atrial fibrillation/flutter The main
aim of treatment is the restoration • class I agents; • ablation for paroxysmal
of sinus rhythm. This may be achieved atrial fibrillation (see
pharmacologically or electrically • class II agents;
Section 3.4.1);
with DC cardioversion. In all cases,
• temporary pacing may prevent
the risk of thromboembolism and • ablation of AV node and
VT in patients with bradycardia-
the requirement to anticoagulate permanent pacemaker
induced VT or long QT syndrome.
should be considered. Consider the for atrial fibrillation not
following: controlled with drugs (see
Long term
Section 3.4.1).
• anticoagulation; Most short-term drugs may be
used long term, but more definitive AVNRT/AVRT Consider
• DC cardioversion;
therapies should be considered. ablation (see Section 3.4.1).
• class III agent; Particular caution should be
applied to commiting any patient Ventricular tachycardias Consider
• digoxin (rate control only);
to long-term use of amiodarone. referral for ablation/implantable
• class I agent (if no coronary artery Its side-effect profile is worse cardioverter defribrillator
disease). with chronic use. (see Section 3.4.2).

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TABLE 25 CAUSES OF HEART FAILURE


Cardioversion of atrial (APPROXIMATE RELATIVE FREQUENCY)
fibrillation/atrial flutter

The following can be used as Cause Relative frequency (%)


guidelines for anticoagulation.
Ischaemic heart disease 50
• Duration <48 hours: proceed Valve disease 10
without anticoagulation. Hypertension 5
• Duration >48 hours: anticoagulation Dilated cardiomyopathy/unknown (see text) 35
for 4–6 weeks before cardioversion,
and for 4 weeks afterwards.
• Duration >48 hours and no evidence
of intracardiac thrombus on After a single episode of cardiac
transoesophageal echocardiography: 2.3 Cardiac failure damage, eg a myocardial infarction
proceed without anticoagulation.
(MI), LV dysfunction is often
• Sinus rhythm is achieved in
approximately 85% of cases of atrial Aetiology/pathophysiology/ progressive even in the absence of
fibrillation/atrial flutter, but pathology further cardiac insults. This appears
recurrence rates may be high (up to The common causes of to result from the neurohumoral
75% at 12 months). response to reduced cardiac output,
heart failure are listed in
Table 25. which is initially compensatory but
becomes detrimental in the long
Left ventricular (LV) systolic term (Fig. 47).
Complications dysfunction is commonly
associated with ventricular Epidemiology
Atrial fibrillation/flutter dilatation. Other causes include The prevalence of heart failure is
Thromboembolism is the most viral myocarditis, toxins (eg alcohol, approximately 4 per 1,000 (28 per
significant and devastating cocaine and chemotherapeutic 1,000 in those aged over 65 years).
condition. agents), metabolic abnormailites Heart failure is the primary diagnosis
(eg thyroid disease and acromegaly) in about 4% of general medical
AVNRT/AVRT and inflammatory conditions admissions to hospital. Increasing
Complications are uncommon, (eg sarcoidosis and connective prevalence is the result of:
but with Wolff–Parkinson–White tissue disorders). In patients with • an ageing population;
syndrome, rapid conduction of idiopathic dilated cardiomyopathy,
atrial fibrillation down an accessory around 25% are thought to have a • better survival after MI;
pathway may precipitate ventricular familial origin. • better survival with heart failure.
fibrillation.

Ventricular tachycardias
Haemodynamic collapse and death is
a potential risk in many cases of VT.

Prevention
Primary and secondary prevention
of stroke/transient ischaemic attack
(see Neurology, Sections 2.8.1 and
2.8.2).

FURTHER READING
Crystal E and Connolly SJ. Role of oral
anticoagulation in management of
atrial fibrillation. Heart 2004; 90:
813–17.
›Fig. 47 The vicious cycle of progressive left ventricular damage.

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CARDIOLOGY: DISEASES AND TREATMENTS

Clinical presentation reproducibility). Pansystolic Echocardiography


Patients suffering from heart failure murmur of functional mitral Gold standard for diagnosis of
commonly present with the regurgitation (other murmurs heart failure; use for assessment
following: may be present and relate to of LV systolic function, filling
aetiology). pressures and valvular function
• exertional breathlessness;
(see Section 3.10).
• Basal lung crackles.
• fatigue;
• Bilateral ankle oedema with or Differential diagnosis
• paroxysmal nocturnal dyspnoea;
without ascites. Consider the following:
• cough productive of clear frothy
• cor pulmonale;
sputum; Investigations
• nephrotic syndrome;
• ankle swelling;
ECG
• renal failure;
• orthopnoea. A completely normal ECG is rare in
heart failure. Look for: • liver failure.

• rhythm (eg AF);


Treatment
Severity of breathlessness in
heart failure is graded • LV hypertrophy;
according to the New York Heart Emergency
• previous MI;
Association (NYHA) classification. In someone suffering from acute
• NYHA class I: impaired LV function • left bundle-branch block; pulmonary oedema, carry out the
but asymptomatic on ordinary following.
activity.
• left axis deviation.
• NYHA class II: symptoms resulting in • Sit the patient up.
slight limitation of ordinary activity. Chest radiograph
• NYHA class III: symptoms on
• Give oxygen (monitor blood gases).
In addition to excluding lung
minimal exertion, eg walking around pathology, look for: • Give intravenous diamorphine
the house.
• NYHA class IV: symptoms present
(venodilator).
• heart size;
at rest.
• Offload with intravenous infusion
• pulmonary oedema;
of nitrate titrated to maximum
• pleural effusions. tolerated dose (but keep BP
Physical signs >90 mmHg systolic).
The physical signs may include the Blood tests
following. • Give intravenous furosemide in
• Urea and electrolytes: associated small aliquots (eg 40 – 80 mg).
• Pulse: tachycardia or atrial hyponatraemia, hypokalaemia
fibrillation (AF). • Check FBC, urea and electrolytes,
(diuretic treatment) and renal
and cardiac enzymes.
• JVP: elevated. If it is up to angle dysfunction.
of the jaw, then suspect tricuspid • Monitor clinical response
• Liver function tests: often
regurgitation (TR). Check for any including urine output
mildly deranged in chronic
systolic v waves that coincide with (catheterise).
heart failure.
contralateral carotid; also check
• Consider ventilatory support
for pulsatile liver. • Thyroid function tests (aetiology).
(continuous positive airway
• Left parasternal heave (usually • Haemoglobin: mild anaemia pressure or intubation) and/or
right ventricular hypertrophy, common and associated with inotropic support where
occasionally the result of greatly adverse outcomes. If present appropriate.
enlarged left atrium). check haematinics.
• Invasive monitoring may be
• Heart sounds: third heart sound • Brain natriuretic peptide required if the patient gives a poor
(probably the most sensitive (see Section 3.7): a normal response (arterial line and central
and specific physical sign for value virtually excludes heart venous line, or pulmonary artery
LV dysfunction, but has poor failure. catheter).

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Short term Following discharge an early symptoms. Only start when


Hospital treatment of review is important to prevent clinically stable and euvolaemic:
decompensated chronic heart re-decompensation. Check renal start low, go slow. Titrate to
failure includes the following. function and up-titrate medication. maximum tolerated dose.
Hypotension may be avoided by
• Monitor fluid balance, daily
Long term reducing other drugs including
weight (aim to lose 0.5–1 kg daily),
diuretics where possible. If fluid
and daily urea and electrolytes.
retention occurs increase the loop
• No-added-salt diet. diuretic. Try to continue the beta-
‘You are a physician, doctor.
You would promise life to a
blocker if at all possible, because
• Intravenous loop diuretic, eg
corpse if he could swallow pills.’ side effects are usually transient.
furosemide once or twice daily
(Napoleon Bonaparte)
(dose will depend on prior • Candesartan (angiotensin receptor
exposure). Counselling a patient with heart blocker) can be added in patients
failure can be very difficult as the
who remain symptomatic despite
• Angiotensin-converting enzyme prognosis is often poor. Yet education
is key to enhancing patient
ACE inhibitors and beta-blockers.
(ACE) inhibitor; angiotensin II
compliance. It is important to judge
receptor blocker can be used if • Spironolactone in patients
each case individually and not give the
ACE inhibitor not tolerated. with NYHA III–IV, creatinine
patient unrealistic expectations.
<200 µmol/L and K+ <5.5 mmol/L.
• Consider anticoagulation
Check electrolytes weekly for
(AF and LV thrombus).
Figure 48 shows long-term 2 weeks and stop spironolactone
• Avoid calcium antagonists and treatment options for those with if K+ <6.0 mmol/L.
NSAIDs. heart failure. The following improve
• Digoxin does not prolong life, but
symptoms and life expectancy.
If there is a good response, change improves symptoms and reduces
to oral diuretics when approaching • ACE inhibitor for all patients, hospital admissions in more
euvolaemia. Aim to continue unless contraindicated (titrate severe cases of heart failure.
hospital treatment until oedema is to maximum tolerated dose).
• Cardiac resynchronisation therapy
clearly improved and the patient is
• Beta-blocker, eg bisoprolol, (dual-chamber pacemaker with
stable on oral therapy for 48 hours.
carvedilol or nebivolol, in patients additional LV lead) may be
In patients with impaired renal
with stable NYHA II–IV considered in symptomatic
function it may be necessary to
accept some residual oedema rather
than precipitate acute-on-chronic
renal failure. Do not use JVP as the
sole guide for treatment because
this is often persistently elevated
as a result of TR.

If weight loss is not satisfactory


on twice-daily furosemide,
add a thiazide diuretic
(bendroflumethiazide 2.5 mg or
metolazone 2.5–5 mg daily) but
watch renal function closely. If
diuresis remains unsatisfactory,
establish continuous intravenous
furosemide infusion (eg 5 –10
mg/hour). Fluid restriction should
be held in reserve for resistant
cases. Rarely, inotropes (dopamine
or dobutamine) are required for a
few days to assist diuresis. ›Fig. 48 Escalation of treatment for left ventricular dysfunction.

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CARDIOLOGY: DISEASES AND TREATMENTS

Complications
• AF.

• Ventricular tachycardia.

• Sudden death.

• Progressive heart failure.

• Renal impariment.

Prognosis
• Mortality related to ejection
fraction and NYHA class.

• Chronic stable heart failure: overall


annual mortality rate is 10%.

• Following hospitalisation, annual


mortality rate is 30 –50%.

• Mortality rate of NYHA IV is up to


60% in 1 year.

Prevention

Primary
• Prevention of MI (see Section 2.1).

• Prompt reperfusion therapy for


acute MI.

• Avoid excess alcohol.

Secondary
›Fig. 49 Left ventricular assist device.
ACE inhibitors, beta-blockers and
patients with poor LV function be used as a ‘bridge to spironolactone all reduce progression
and left bundle-branch block transplantation’ if a suitable of heart failure and mortality.
(see Section 3.4.3). donor is not immediately available
(Fig. 49). The function of the heart
• Implantable cardioverter may improve (and transplantation
defibrillator (see Section 3.4.2) avoided) when it is ‘rested’ by one Important information for
in selected patients. of these devices. However, use patients
of a ventricular assist device is • Advise a no-added-salt diet.
Surgical intervention may be
frequently complicated by • Moderate alcohol intake.
beneficial in carefully evaluated
thromboembolism and infection. • Avoid heavy lifting (potentially
patients with valvular disease and
arrhythmogenic).
those with ischaemic aetiology • Chronic progressive heart failure • May feel worse for a few days after
and ongoing angina. Cardiac in young patients with very poor starting beta-blocker, or if the dose
transplantation (of which there are prognosis and no comorbidity. is increased.
around 200 annually in the UK) is • Must weigh themselves daily and
report to their GP or increase dose of
indicated for the following.
diuretic if they gain weight (>1–2 kg
‘. . . it is infinitely better to in 3 days or >2.5 kg in 2 weeks).
• Acute heart failure not responding
transplant a heart than to bury • Education and monitoring ideally
to ventilation and inotropic
it so it can be devoured by worms.’ performed in conjunction with a
support. A ventricular assist (Christiaan N. Barnard) specialist heart failure nurse.
device (artificial heart) may

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FURTHER READING
Cowie MR and Zaphiriou A.
Management of chronic heart failure.
BMJ 2002; 325: 422–5.

Millane T, Jackson G, Gibbs CR, et al.


ABC of heart failure: acute and chronic
management strategies. BMJ 2000;
320: 559–62.

2.4 Diseases of heart


muscle ›Fig. 50 Effect of asymmetrical septal hypertrophy in HCM. In late systole the septum contracts down on
the outflow tract, obstructing flow and generating a gradient. This generates a negative pressure (Venturi
effect) just proximal to the obstruction, sucking the mitral valve anteriorly (systolic anterior motion) and
producing mitral regurgitation. AO, aorta; LA, left atrium; LV, left ventricle; MV, mitral valve.
2.4.1 Hypertrophic
cardiomyopathy
Epidemiology Uncommon
Aetiology/pathophysiology/ The prevalence of hypertrophic
• Fourth heart sound: often easier
pathology cardiomyopathy (HCM) is 1 in 500
to feel (as a double apical
and it is the most common single-
• Autosomal dominant. impulse) than hear.
gene cardiac disorder.
• Mutations found in at least
Investigations
10 genes (all encode contractile Clinical presentation
The ECG and echocardiogram must
proteins, eg myosin β heavy
be interpreted together because they
chain and troponin T). Common
provide complementary information.
• Unexplained hypertrophy of the • Exertional chest pain.
left (and occasionally the right) ECG
• Palpitations.
ventricle, which is usually focal, The ECG is sensitive but not very
eg asymmetrical septal • Asymptomatic murmur. specific. It varies from T-wave
hypertrophy and apical inversion to overt left ventricular
• Abnormal ECG on screening.
hypertrophy. hypertrophy (LVH).

• Mechanism of hypertrophy Uncommon


Echocardiography
unknown: possibly secondary to
• Syncope. Echocardiography is specific
impaired function of contractile
but less sensitive than the ECG.
proteins (ie a compensatory
Rare Classically, there is asymmetrical
phenomenon).
septal hypertrophy with systolic
• Sudden death.
• Degree of hypertrophy variable anterior motion of the mitral valve
even between individuals with the leaflet, left ventricular outflow tract
Physical signs
same mutation. obstruction and secondary mitral
There may be no abnormal findings.
regurgitation. Alternative patterns
• Left ventricular outflow tract
include apical, free wall or
obstruction may occur secondary Common
concentric LVH.
to septal hypertrophy (Fig. 50).
• Jerky pulse.
• Mitral regurgitation may also be a Ambulatory monitoring
• Prominent apical impulse.
feature, usually as a result of the This is used to identify the cause of
Venturi effect in the presence of • Systolic murmur at left lower palpitations or detect asymptomatic
septal hypertrophy (Fig. 50). sternal edge/apex. arrhythmia.

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CARDIOLOGY: DISEASES AND TREATMENTS

septum relieves gradient, but


mortality rate is 1–2% at best.

• Percutaneous transluminal
septal myocardial ablation is
a promising new technique. A
selected area of the obstructing
septum is destroyed by alcohol
injected into a carefully chosen
septal artery (Fig. 52).

Complications

Common

• Atrial fibrillation: always


anticoagulate because there is
a high risk of thromboembolism.
Atrial fibrillation is often
poorly tolerated, so consider
cardioversion along with
antiarrhythmic drugs to maintain
›Fig. 51 MRI of the heart in short-axis view, showing asymmetrical hypertrophy of the interventricular sinus rhythm. Note that digoxin
septum in HCM (arrow). LV, left ventricular cavity; RV, right ventricular cavity. is contraindicated if there is a
significant left ventricular outflow
tract gradient (>5 mmHg), so use
Exercise ECG weight-lifters, rowers and a beta-blocker or calcium
This is used to provoke arrhythmia cyclists) have an identical pattern antagonist for rate control.
and assess the BP response of physiological hypertrophy.
(important for prognosis or for However, this will regress if Uncommon
vocational driving licence). training is discontinued. A septal
thickness >1.6 cm is likely to be • Ventricular tachycardia (VT):
Magnetic resonance imaging pathological. sustained VT is associated with
MRI may confirm the diagnosis if high risk of sudden death and
echocardiographic images are not Treatment requires an implantable
clear (Fig. 51). No treatment is indicated in cardioverter defribrillator
asymptomatic patients who do (see Section 3.4.2).
not have significant arrhythmia. • Progression to dilated
It is possible to have HCM Antibiotic prophylaxis is generally cardiomyopathy: documented
without any hypertrophy. The recommended for dental and in up to 15% of early series, but
diagnosis may be made on the family certainly less common than
surgical procedures likely to
history plus an abnormal ECG.
produce a bacteraemia. this in modern practice.
Breathlessness and chest pain • Sudden death.
can be treated with beta-blockers or
Differential diagnosis
calcium antagonists, but often these
Rare
• Hypertensive cardiac hypertrophy: only partially relieve symptoms.
a concentric pattern of Severe breathlessness associated • Endocarditis.
hypertrophy with documented with a left ventricular outflow tract
hypertension. gradient may be treated in a number Prognosis
of non-medical ways. Risk of premature death is
• Athlete’s heart: differentiation
associated with the following:
may be difficult because some • Surgical myectomy: partial
highly trained athletes (especially excision of the hypertrophied • cardiac arrest or sustained VT;

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CARDIOLOGY: DISEASES AND TREATMENTS

›Fig. 52 Septal ablation in hypertrophic obstructive cardiomyopathy. (a) A wire is passed through a coronary guide catheter into the target septal artery (arrow).
A balloon catheter is passed, the wire is removed and the balloon inflated to occlude the artery. (b) Dye is injected into the lumen of the balloon catheter and into
the distal septal artery to confirm correct positioning. (c) Absolute alcohol is then injected to destroy selectively the septal artery, leaving a stump. Simultaneous
pressure recordings reveal a left ventricular outflow tract gradient (peak ventricular minus peak aortic pressure) of approximately 100 mmHg before the procedure
(d), falling to 15 mmHg afterwards (e).

• syncope (especially when Occupational aspects


Important information for Patients should not be
recurrent or associated with
patients professionals in sports requiring
exertion);
• It is an inherited condition. vigorous physical exertion. They
• strong family history of sudden • There is a 50% chance of may still hold vocational driving
early death; transmission to their children.
licences if they meet the DVLA
• It is benign in most cases, so
• diagnosis of HCM in childhood; (Driver and Vehicle Licensing
reassure that it is low risk if
appropriate. Agency) criteria (see Section 2.19).
• VT on 24-hour ECG monitoring; • Continue as far as possible with a
normal life, but avoid competitive
• BP drop on exercise;
physical sports.
• presence of certain high-risk • Seek medical advice in the event of
palpitations, dizziness or blackouts.
mutations; FURTHER READING
• Carefully discuss before you begin
• extreme LVH (>3 cm). screening: no treatment is indicated Frenneaux MP. Assessing the risk of
in the absence of symptoms and sudden cardiac death in a patient with
knowledge of the diagnosis will hypertrophic cardiomyopathy. Heart
Disease associations adversely affect life insurance, 2004; 90: 570–5.
Friedreich’s ataxia and Wolff– mortgages, etc.
Parkinson–White syndrome.

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CARDIOLOGY: DISEASES AND TREATMENTS

Treatment/prognosis Investigations
Maron BJ, Nishimura RA, Tajik AJ, et al. See Section 2.4.2.
Efficacy of implantable cardioverter-
Chest radiograph
defibrillators for the prevention of
sudden death in patients with The heart size may be normal
FURTHER READING or increased. Pericardial
hypertrophic cardiomyopathy. N. Engl.
J. Med. 2000; 342: 365–73. Graham RM and Owens WA. calcification suggests constrictive
Pathogenesis of inherited forms of
pericarditis rather than restrictive
dilated cardiomyopathy. N. Engl. J. Med.
Spirito P, Seidman CE, McKenna WJ, et cardiomyopathy (see Sections 2.4.3
1999; 341: 1759.
al. The management of hypertrophic
and 2.6.3).
cardiomyopathy. N. Engl. J. Med. 1997;
336: 775–85.
Echocardiography
2.4.3 Restrictive
Ventricular cavities are usually not
cardiomyopathy
dilated, but atrial cavities are often
2.4.2 Dilated cardiomyopathy
greatly enlarged. Rapid ventricular
Aetiology/pathophysiology/
filling may be seen at the onset of
Aetiology/pathophysiology/ pathology
diastole, which stops abruptly in
pathology This is a chronic progressive
early diastole.
This is a chronic progressive condition characterised by
disorder of unknown aetiology, excessively rigid ventricular walls
Cardiac catheterisation
characterised by dilatation and that impair ventricular filling
May be diagnostic in restrictive
systolic dysfunction of the left (diastolic dysfunction). Contractile
cardiomyopathy. Rapid ventricular
(and sometimes the right) ventricle. (systolic) function is preserved.
filling in early diastole produces a
Some cases are probably the result Causes are divided into:
‘square root sign’ appearance of the
of unrecognised alcohol abuse,
• myocardial, eg amyloid, sarcoid and left ventricular diastolic pressure
‘burnt-out’ hypertension or acute
storage diseases (often idiopathic); trace, which is also seen in
myocarditis. Familial dilated
pericardial constriction. However,
cardiomyopathy caused by • endomyocardial, eg
other catheter data help differentiate
mutations in cytoskeletal proteins endomyocardial fibrosis and
the two conditions (Table 26).
has been described and is present hypereosinophilic syndrome.
in 35% of individuals with
Myocardial biopsy
idiopathic cardiomyopathy. Epidemiology
Biopsy is sometimes useful to
Dilated cardiomyopathy also This condition is rare in Western
identify the cause of a restrictive
complicates muscular dystrophy. countries. Endomyocardial fibrosis
cardiomyopathy.
is common in the tropics,
Clinical presentation particularly in Africa.
Differential diagnosis
This condition presents with
Restrictive cardiomyopathy must
congestive cardiac failure or Clinical presentation
be distinguished from pericardial
arrhythmia (atrial or ventricular).
constriction, which is readily
Symptoms
treated by surgery. Table 26 gives
Investigations
• Breathlessness. distinguishing features, but in up to
• ECG: often shows poor R-wave 25% of patients it is not possible to
• Fatigue.
progression or left bundle-branch differentiate the two conditions and
block. • Ankle swelling. in these circumstances exploratory
surgery may be justified.
• Echocardiography: dilated left
Signs
ventricle with globally impaired
Treatment
contraction. Focal areas of • Elevated JVP, which rises on
The response of patients to medical
hypokinesia suggest ischaemic inspiration (Kussmaul’s sign).
treatment of heart failure is often
damage or prior myocarditis.
• Third and/or fourth heart sound. poor. Successful combined heart
• Cardiac catheterisation: ensures and liver transplantation has
• Peripheral oedema.
that there is no occult coronary been described in amyloid
disease and confirms diagnosis. • Ascites. cardiomyopathy.

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CARDIOLOGY: DISEASES AND TREATMENTS

2.4.5 Left ventricular


TABLE 26 FEATURES OF RESTRICTIVE CARDIOMYOPATHY non-compaction
AND PERICARDIAL CONSTRICTION
Aetiology/pathophysiology/
Restrictive cardiomyopathy Pericardial constriction pathology
Third heart sound Present Absent This a recently described form
Pericardial calcification Absent In 50% of cardiomyopathy that is a result
CT of the chest Normal pericardium Thickened pericardium of persistence of the embryonic
PA systolic pressure Usually >50 mmHg <50 mmHg
Diastolic pressure LV > RV LV = RV pattern of myocardial architecture.
It is rare and has not been fully
LV, left ventricular; PA, pulmonary artery; RV, right ventricular. characterised. However, there are
some associations with other
inherited cardiomyopathies.

Prognosis ventricular arrhythmias arise from


Clinical presentation
The disease is generally relentlessly the right ventricle (commonly left
The most commmon presentation
progressive with a high mortality. bundle-branch block). In latter
is in adulthood with signs and
stages there may be progressive
symptoms of congestive cardiac
right ventricular dilatation leading
FURTHER READING failure.
to right heart failure and in some
Doughan AR and Williams BR. Cardiac case biventricular failure.
sarcoidosis. Heart 2006; 92: 282–8. Investigations
Echocardiography can demonstrate
Investigations
Wynne J and Braunwald E. The a very trabeculated left ventricle.
cardiomyopathies and myocarditides. • ECG: may show ST abnormalities
In Braunwald E, ed. Heart Disease.
in the right precordial leads, Treatment/prognosis
Philadelphia: WB Saunders, 2001:
although in some cases they See Section 2.3.
1751–806.
may be normal.

• Echocardiography: often FURTHER READING


normal but may show dilatation
2.4.4 Arrhythmogenic right Hughes S and McKenna J. New insights
of the right ventricle in some into the pathology of inherited
ventricular cardiomyopathy
cases. cardiomyopathy. Heart 2005; 91:
257–64.
Aetiology/pathophysiology/ • MRI: best method of
pathology demonstrating fatty infiltration
Arrythmogenic right ventricular of the right ventricle.
cardiomyopathy is a disease of
• Electrophysiological studies: may
primarily the right ventricular
myocardium. It is characterised
induce ventricular arrhythmias 2.5 Valvular heart
(see Section 3.2).
by myocyte death and replacement disease
with fibro-fatty tissue. In some cases Diagnosis is often difficult if
the left ventricle is also involved. the ECG and imaging are not
2.5.1 Aortic stenosis
Its aetiology is not known but conclusive. The main differential
30 –50% are thought to be familial diagnosis is benign right outflow
Aetiology/pathophysiology/
(autosomal dominant) and recent tract tachycardia, which can respond
pathology
studies have highlighted to beta-blockers.
abnormalities in genes that • Senile (calcific/degenerative):
code for cell adhesion molecules. Treatment this is the most common form
In the case of aborted cardiac death, of aortic stenosis, especially in
Clinical presentation the treatment is an implantable those aged over 65 years. Diabetes,
The condition usually presents with cardioverter defibrillator. Heart hypercholesterolaemia and
ventricular arrhythmias or sudden failure is treated in the usual way chronic renal failure are
death and affects young adults. The (see Section 2.3). predisposing factors. Coexistent

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CARDIOLOGY: DISEASES AND TREATMENTS

coronary artery disease is Chest radiograph Coronary angiography


common. May be normal. Poststenotic aortic Coronary angiography will be
dilatation may be seen. Suspect required in most cases to assess
• Congenital bicuspid valve:
aortic (or mitral) regurgitation or LV the coronary arteries before surgery.
symptoms usually appear at
dilatation if cardiomegaly is present. The peak–peak withdrawal gradient
the age of 40–50 years. There
across the aortic valve can also be
is a male predominance.
Echocardiography determined.
• Rheumatic heart disease: Echocardiography determines
this is an unusual cause of whether the valve is tricuspid,
aortic stenosis. bicuspid or rheumatic in
Consider the following in your
appearance. The degree of valve differential diagnosis:
Stenosis results from a combination
thickening, leaflet mobility and
of fibrosis and calcification, with • innocent systolic murmur
calcification (Fig. 53) can be (pregnancy, fever, anaemia and
additional commissural fusion
determined. Continuous-wave thyrotoxicosis);
and reduced cusp separation.
Doppler enables estimation of the • aortic sclerosis;
The increased left ventricular
pressure drop across the aortic valve • mitral regurgitation;
(LV) pressure load results in • hypertrophic obstructive
and the aortic valve area (Table 27).
compensatory left ventricular cardiomyopathy;
LVH and LV dilatation with reduced
hypertrophy (LVH) and diastolic • atrial or ventricular septal defect;
systolic function will be seen with • pulmonary stenosis.
dysfunction. Subendocardial
severe disease.
ischaemia and fibrosis is common.
Untreated, LV dilatation and failure
will occur. There is an increased risk
of ventricular arrhythmia. Atrial
arrhythmias are usually poorly
tolerated.

Clinical presentation

Common
Common symptoms are exertional
angina, dyspnoea and syncope, and
occasionally palpitations. There are
symptoms of LV failure if
presentation is late.

Uncommon
• Embolic phenomena from calcific
emboli.

• Gastrointestinal bleeding
(idiopathic/angiodysplasia).

• Infective endocarditis.

Physical signs
See Section 1.2.7.

Investigations

ECG
Look for LVH (85% of cases) and, ›Fig. 53 (a) Calcific aortic stenosis. In this parasternal long-axis view, the aortic valve cusps (arrow) appear
markedly thickened and calcified. Note the hypertrophy of the septum and posterior wall. (b) The peak
rarely, conduction disturbance. velocity across the valve is 4 m/s. The calculated peak valve gradient is 64 mmHg.

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2.5.2 Aortic regurgitation


TABLE 27 GRADING OF AORTIC STENOSIS BY AORTIC VALVE
GRADIENT AND AREA Aetiology/pathophysiology/
pathology
Aortic stenosis Peak aortic valve gradient (mmHg) Aortic valve area (cm2) Aortic regurgitation (AR) may result
Mild <50 >1.5 from primary disease of the valve
Moderate 50–70 1.1–1.5 leaflets, dilatation of the aortic root,
Severe >70 <0.8–1.0 loss of commissural support or
failure of valve prosthesis, either
alone or in combination. The result
is the addition of a regurgitant
volume to the normal inflow from
Treatment Complications
the left atrium.
The following are associated with
aortic stenosis:

Severe aortic stenosis is • cardiac failure and pulmonary


Aetiology of AR
associated with a peak gradient hypertension;
of >70 mmHg. However, with LV
• sudden death; Dilatation of the aortic root:
impairment the aortic valve gradient
may underestimate the degree of • Degenerative (senile).
stenosis. In this situation, valve area is • infective endocarditis;
• Cystic medial necrosis:
a more reliable measurement and a isolated/associated with Marfan’s
• embolic disease;
dynamic assessment of the valve syndrome.
gradient with dobutamine stress may • complete heart block. • Aortic dissection.
be required. • Systemic hypertension.
• Aortitis (connective tissue disorders
Prognosis
and syphilis).
Symptoms occur only after the
stenosis has become severe. Mild Primary disease of valve leaflets:
Emergency aortic stenosis progresses to severe
• Rheumatic heart disease.
Admit the patient if there is heart stenosis in about 20% of cases, two- • Infective endocarditis.
failure and treat with diuretics thirds remaining unchanged. On • Bicuspid valve.
with a view to early inpatient valve average, the valve gradient will • Myxomatous degeneration with
replacement. Try to avoid inotropes. increase by 4–8 mmHg per year. prolapse.
• Trauma.
Exercise great caution with Extensive valve calcification, the
angiotensin-converting enzyme presence of a bicuspid valve and Loss of support of the aortic valve cusps:
inhibitors and other vasodilators, coexistent coronary artery disease
• High ventricular septal defect.
and never use them if the patient predispose those affected to more
• Fallot’s tetralogy.
is hypotensive. rapid stenosis progression.
Asymptomatic patients have an Failure of a prosthetic valve
Long term excellent prognosis. In symptomatic
Follow moderate disease with disease, the average survival with
repeat echocardiography at yearly angina or syncope is 2–3 years,
intervals. Severe stenosis requires and with heart failure 1 year. Clinical presentation
closer supervision to detect onset The patient may present with the
of symptoms. Antibiotic prophylaxis following:
is required for dental procedures, FURTHER READING
• exertional dyspnoea, orthopnoea
etc. Valve replacement should be Baumgartner H. Aortic stenosis:
and paroxysmal nocturnal
considered in all patients with medical and surgical management.
Heart 2005; 91: 1483–8. dyspnoea;
severe aortic stenosis who become
symptomatic. It is also indicated in • lethargy;
Decena BF III and Tischler MD. Stress
asymptomatic patients who develop
echocardiography in valvular heart • palpitations;
LV dysfunction or prior to
disease. Cardiol. Clin. 1999; 17: 555–72.
non-cardiac surgery. • angina.

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CARDIOLOGY: DISEASES AND TREATMENTS

assessment. Antibiotic prophylaxis


is required. All patients with
severe symptomatic AR should
be considered for surgery. Early
surgery is indicated if there is
evidence of LV dilatation or LV
systolic dysfunction. Patients
with coexistent aortic root
dilatation >5 cm and AR of
any severity should have aortic
root reconstruction and valve
resuspension or replacement.

Complications
Complications commonly
encountered include the following:

• progressive heart failure;


›Fig. 54 Parasternal long-axis view showing severe AR with colour flow mapping. In early diastole there is
a broad-based regurgitant jet (yellow–blue) filling the whole of the left ventricular outflow tract.
• mitral regurgitation;

• atrial fibrillation;

Investigations may be required to exclude • sudden death.


dissection and endocarditis.
ECG Prognosis
Look for the following: Coronary angiography The risk of developing symptoms
Coronary angiography will be and /or LV dysfunction in severe
• normal/ left ventricular (LV)
required before surgery to assess AR with normal LV function is 4%
hypertrophy;
the coronary arteries in most cases. annually. If there is LV dysfunction,
• left atrial enlargement; The AR can be assessed with an the risk is >25% annually. Prognosis
aortogram. is excellent in mild or moderate
• prolongation of PR interval;
disease. In symptomatically severe
• non-specific ST-segment and Differential diagnosis AR, the yearly mortality rate is >10%.
T-wave changes. Consider pulmonary regurgitation
and mitral stenosis with Graham
Chest radiograph Steell murmur. FURTHER READING
This is normal or shows Bonow RO. Chronic aortic
cardiomegaly, which may be gross. Treatment regurgitation: role of medical therapy
There is pulmonary oedema in acute and optimal timing for surgery. Cardiol.
cases. Look for evidence of aortic Emergency Clin. 1998; 16: 449–61.
dilatation or dissection. In patients with acute severe AR or
severe decompensated chronic AR, Carabello BA and Crawford FA Jr.
Valvular heart disease. N. Engl. J. Med.
Echocardiography treat heart failure aggressively with
1997; 337: 32–41.
Echocardiography may enable diuretics, vasodilators and inotropes.
diagnosis of the aetiology from Look for the underlying cause and
the anatomy of the aortic valve plan early or emergency valve
and root. Severity assessment is replacement.
semi-quantitative and derived 2.5.3 Mitral stenosis
from colour and continous-wave Long term
Doppler (Fig. 54). LV dilatation Patients with asymptomatic Aetiology
and reduced ejection fraction mild/moderate AR with normal Most mitral stenosis (MS) is
occur with untreated severe disease. ventricular function require annual acquired through rheumatic heart
Transoesophageal echocardiography clinical and echocardiographic disease. It is more common in

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CARDIOLOGY: DISEASES AND TREATMENTS

women, presenting in developed


countries in the fourth or fifth
decades of life.

Clinical presentation
MS commonly presents with the
following:

• exertional dyspnoea;

• orthopnoea;

• paroxysmal nocturnal dyspnoea;

• haemoptysis;

• palpitations;

• fatigue;

• weight loss;

• embolic phenomena in up
to 15%.
›Fig. 55 CXR showing left atrial enlargement in a patient with mitral valve disease: note the double atrial
shadow (left atrial border indicated by broken line) and dilatation of the left atrial appendage (arrow).
Physical signs (Reproduced with permission from Axford JS, ed. Medicine. Oxford: Blackwell Science, 1996.)

See Section 1.2.6.


• Atrial double shadow along right for valvuloplasty if this is being
Investigations cardiac border (Fig. 55). considered (Fig. 56).

ECG Echocardiography Cardiac catheterisation


This enables visualisation of This is advisable when patient
• Normal.
leaflet mobility and calcification. symptoms and echocardiographic
• Left atrial enlargement or atrial Quantification of the valve area findings are discordant or coexistent
fibrillation (AF). and mean gradient (Table 28) are coronary artery disease is suspected.
derived from continous-wave The mean mitral valve (MV) gradient
• Right venricular hypertrophy.
Doppler. Left atrial size and right can be calculated from the
ventricular function should be difference between left ventricular
Chest radiograph
assessed. Left ventricular size is end-diastolic pressure and
• Normal. usually small and left ventricular pulmonary artery wedge pressure
systolic function normal. Doppler recorded simultaneously.
• Straightening of left cardiac
echocardiography allows estimation
border as a result of dilated left
of the pulmonary artery pressure Dynamic assessment
atrial appendage.
(PAP). A transoesophageal study is Some symptomatic patients may
• Pulmonary oedema. usually required to assess suitability have evidence of only mild or
moderate MS at rest. However,
during exercise the rate of mitral
inflow increases, which may cause
TABLE 28 SEVERITY OF MITRAL STENOSIS ASSESSED BY MEAN the transvalvular gradient to
GRADIENT AND MITRAL VALVE AREA increase significantly. Diastolic filling
time may also be reduced, causing
Severity Mean gradient (mmHg) Mitral valve area (cm2) raised left atrial pressure. Exercise
echocardiography should therefore
Mild 0–6 <1.5
be considered in patients who are
Moderate 6–11 1.0–1.5
Severe >12 <1.0 symptomatic with apparent mild or
moderate disease only, and no other

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CARDIOLOGY: DISEASES AND TREATMENTS

FURTHER READING
Bruce CJ and Nishimura RA. Newer
advances in the diagnosis and
treatment of mitral stenosis. Curr.
Probl. Cardiol. 1998; 23: 125–92.

Lawrie GM. Mitral valve repair


vs. replacement. Current
recommendations and long-term
results. Cardiol Clin 1998; 16: 437–48.

2.5.4 Mitral regurgitation

Aetiology/pathophysiology/
pathology
›Fig. 56 Rheumatic MS: note thickening of the leaflet tips and subvalvar apparatus causing marked Abnormalities of the mitral valve
restriction of leaflet excursion in diastole. There is marked left atrial enlargement with a relatively small left annulus, valve leaflets, chordae
ventricular cavity. Spontaneous echo contrast (smoky appearance) can be seen in left atrium, suggestive of
a prothrombotic state. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. tendineae, papillary muscles or
adjacent left ventricular (LV) wall
explanation for their symptoms (eg of severity. Intervention is required may cause mitral regurgitation
anaemia, other valvular disease or if there is severe stenosis and (MR).
coronary artery disease). An increase symptoms. If the MV has minimal
in mean MV gradient to >15 mmHg calcification, there is merely mild
or PAP to >60 mmHg with exercise is mitral regurgitation and there is
Common causes of MR in the
considered significant. no evidence of left atrial thrombus,
adult
then mitral valvuloplasty should
Differential diagnosis be considered (see Section 3.8). • Idiopathic mitral valve prolapse
(MVP): most common cause.
Consider the following: Otherwise, open valvuloplasty
• After myocardial infarction:
or MV replacement is required. (a) Papillary muscle dysfunction
• Austin Flint murmur (of aortic
(ischaemia or rupture).
regurgitation);
Complications (b) Ruptured chordae tendineae.
• left atrial myxoma; The following are possible: (c) Annular dilatation (ischaemic
heart disease or dilated
• tricuspid stenosis. • AF; cardiomyopathy).
• Rheumatic heart disease.
Treatment • pulmonary hypertension or • Infective endocarditis.
infarction; • Atrial septal defect.
• Failure of valve prosthesis/
Emergency • chest infections; paraprosthetic leak.
Treat acute pulmonary oedema with
diuretics. • tricuspid regurgitation;

• right ventricular failure;


Short term Epidemiology
Beta-blockers or rate-limiting • thromboembolic disease. The prevalence of MVP varies from
calcium antagonists may help 1 to 6%, and is twice as common in
symptoms. AF may require Prognosis women. After myocardial infarction,
treatment (see Section 2.2.2). In severe MS, 5-year survival rates the prevalence is 20%.
range from 62% with New York
Long term Heart Association class III Clinical presentation
Formal anticoagulation with symptoms to 15% with class IV. Common symptoms include
warfarin is required. Antibiotic After surgery, 5-year survival rates exertional dyspnoea, orthopnoea,
prophylaxis is needed irrespective are between 90 and 96%. fatigue and lethargy. Occasionally,

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CARDIOLOGY: DISEASES AND TREATMENTS

In the case of prolapse, a precise


assessment of the scallops involved
is required. Severity assessment is
semi-quantitative from colour and
continous-wave Doppler.
Measurements of left and right
ventricular size, systolic function
and pulmonary artery pressure are
required (Fig. 58).

Coronary angiography
Coronary angiography may be
required if there is a suspicion of
coronary artery disease.

Dynamic assessment
In patients with ischaemic MR,
baseline MR may only be mild to
moderate. However, during exercise
ischaemia of the papillary muscles
and adjacent myocardium may
cause the MR to become severe.
Exercise (stress) echocardiography
›Fig. 57 Mitral annular calcification: a ring of calcification can be seen within the heart shadow. should therefore be considered
in patients with ischaemic heart
there are palpitations and, in severe A murmur may not be heard due to disease and severe cardiac
acute MR, the patient may be very very rapid equalisation of pressures symptoms that cannot be
unwell with severe dyspnoea. between the left atrium and left explained by baseline MR or
ventricle during early diastole. any other cause.
Physical signs
In mild disease, there are few signs Investigations Treatment
apart from an apical pansystolic
murmur radiating to the axilla. In Emergency
ECG
more haemodynamically significant Treat acute pulmonary oedema and
This may be normal, but look for
regurgitation, there can be the shock. Vasodilator therapy reduces
AF, left atrial enlargement or LV
following: the afterload and is of benefit.
hypertrophy.
Intravenous nitroprusside may
• atrial fibrillation (AF);
be life-saving. Urgent surgery is
• laterally displaced, hyperdynamic Chest radiograph required.
apex beat with systolic thrill; This can be normal, or may
show cardiomegaly with left Short term
• left parasternal late systolic heave atrial enlargement. Mitral annular Symptomatic patients with severe
(atrial filling) in severe MR; calcification may be seen (Fig. 57). MR who are awaiting surgery should
• soft first heart sound, wide There is pulmonary oedema in receive diuretic and vasodilator
splitting of the second heart acute MR. therapy. Digoxin is of particular
sound, and a third heart sound; benefit in the treatment of AF.
Echocardiography Anticoagulation will be required.
• late systolic murmur in
The presence of excess leaflet
association with a systolic click
motion, restricted leaflet motion Long term
suggests MVP.
and annular size must be carefully All patients should receive antibiotic
In acute severe MR, there is poor assessed with both transthoracic and prophylaxis. Mild to moderate
perfusion with pulmonary oedema. transoesophageal echocardiography. disease requires annual monitoring

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of right ventricular (RV) dilatation


and high pressure resulting from
severe pulmonary hypertension.
Tricuspid stenosis (TS) is almost
invariably rheumatic in origin, and
accompanies mitral stenosis. In
both, right atrial enlargement and
hypertrophy occur with the risk of
atrial fibrillation.

Clinical presentation
This is usually asymptomatic, and in
the case of TR is usually discovered
secondary to other more significant
cardiac pathology. TR and TS may
cause a sensation of neck pulsation,
›Fig. 58 Apical four-chamber view of a patient with prolapse of the posterior mitral valve leaflet. An
anteriorly directed jet of mitral regurgitation can be seen with colour flow mapping (coloured green).
right upper quadrant discomfort and
peripheral oedema. Occasionally,
a low cardiac output syndrome
only. All patients with symptomatic • thromboembolism (more common comprising fatigue, weight loss
severe MR require surgery. in MVP); and syncope may be present.
Asymptomatic patients with severe
• sudden death (more common in
MR should be referred once LV Physical signs
flail leaflet).
function starts to decline or LV TR causes prominent v waves in the
dilatation occurs. Generally, surgical JVP, whereas TS causes prominent
Prognosis
outcome is better with mitral valve a waves in sinus rhythm. In more
Progression of MR depends on the
repair than replacement. severe cases, both cause pulsatile
aetiology, but it develops in 15% of
hepatomegaly, ascites and peripheral
patients with MVP over 10–15 years.
oedema. In TR, a pansystolic
Without surgery, patients with severe
Indications for surgery in
murmur that increases on
MR have a 5-year survival rate as
severe MR inspiration and is heard best at
low as 45%. After surgery, the 5-year
the lower left sternal edge is usual.
• If surgical repair is possible, it should survival rates vary from 40% in MR
be considered in all patients aged The corresponding murmur in
caused by ischaemic heart disease to
<75 years who have a flail leaflet or TS is a presystolic murmur in sinus
over 75% in rheumatic mitral valve
persistent AF. rhythm with a mid-diastolic
disease.
• Deteriorating ventricular function murmur.
(ejection fraction <60% or end-
systolic diameter >45 mm). FURTHER READING Investigations
• The presence of symptoms, although
Cooper HA and Gersh BJ. Treatment of
careful consideration of the aetiology
and severity of LV dysfunction is
chronic mitral regurgitation. Am. Heart ECG
J. 1998; 135: 925–36. This is usually normal, but right
needed in older patients.
atrial enlargement is a feature of
Quinones MA. Management of mitral tricuspid valve disease. There is
regurgitation: optimal timing for
Complications evidence of RV hypertrophy in TR.
surgery. Cardiol. Clin. 1998; 16: 421–35.
The following are possible:
Chest radiograph
• LV failure;
• Often normal, but there may be
2.5.5 Tricuspid valve disease
• AF; an enlarged right atrium and
superior vena cava in both TR
• infective endocarditis; Aetiology/pathophysiology/
and TS.
pathology
• pulmonary hypertension;
Tricuspid regurgitation (TR) is • RV enlargement may be evident
• right ventricular failure; usually secondary to a combination in TR.

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CARDIOLOGY: DISEASES AND TREATMENTS

Investigations

ECG
This is normal, or there is RAH and
RVH.

Chest radiograph
This is normal, or shows right atrial
and ventricular enlargement.

Treatment
Pulmonary valvotomy may be
necessary in severe PS. Severe PR
may require surgery if the patient is
symptomatic.

›Fig. 59 TR: a broad band seen mainly as blue extends back into the right atrium.
FURTHER READING
(Courtesy of Dr J. Chambers.) Waller BF, Howard J and Fess S.
Pathology of pulmonic valve stenosis
and pure regurgitation. Clin. Cardiol.
Echocardiography invariably results from dilatation
1995; 18: 45–50.
This gauges the severity (from of the pulmonary annulus,
colour and continuous-wave which may occur with pulmonary
Doppler) and enables calculation hypertension, leading to right
of pulmonary artery pressure from ventricular hypertrophy (RVH) and
a TR jet (Fig. 59). right atrial hypertrophy (RAH).
2.6 Pericardial disease

Treatment Clinical presentation 2.6.1 Acute pericarditis


Both TR and TS are usually well Both PR and mild PS are
tolerated irrespective of their asymptomatic. More severe disease Aetiology/pathophysiology/
severity. When TR is the result presents with a low cardiac output pathology
of a correctable left-sided cause, syndrome and right heart failure. There are many causes of acute
eg mitral valve disease, annuloplasty pericarditis (Table 29). In the
at the time of surgery is corrective. Physical signs developed world the cause of
TS rarely requires valvotomy. There is a characteristic harsh many cases is never established
ejection systolic murmur at the (idiopathic) but a viral cause is
left sternal edge in the second often suspected, coxsackievirus
FURTHER READING
intercostal space, which is louder B being most often incriminated.
Kratz J. Evaluation and management of on inspiration. Other signs include Tuberculosis is a major cause
tricuspid valve disease. Cardiol. Clin.
the following: in the developing world.
1991; 9: 397–407.
• prominent a wave in JVP; There is inflammation of the
pericardium with infiltration of
• thrill over pulmonary area, right
2.5.6 Pulmonary valve disease polymorphonuclear leucocytes,
ventricular heave;
increased pericardial vascularity and
Aetiology/pathophysiology/ • soft pulmonary second heart sound; deposition of fibrin. Inflammation
pathology can involve the superficial
• right ventricular fourth heart
Pulmonary stenosis (PS) is usually myocardium and fibrinous adhesions
sound.
congenital in origin, and may form may form between the pericardium
part of Fallot’s tetralogy. Rarely, PR is characterised by a decrescendo and epicardium, and between the
it may be the result of rheumatic early-diastolic murmur heard in the pericardium, adjacent sternum and
fever or the carcinoid syndrome. pulmonary area (Graham Steell pleura. The visceral pleura may exude
Pulmonary regurgitation (PR) murmur). fluid, leading to pericardial effusion.

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seen with ST-elevation myocardial


TABLE 29 CAUSES OF ACUTE PERICARDITIS infarction.

Pathogenesis Cause Blood tests


Look for the following:
Acute idiopathic pericarditis Unknown
Infectious Viral • elevated white cell count,
TB erythrocyte sedimentation rate
Other bacteria
and C-reactive protein;
Fungi
Inflammatory Post myocardial infarction/cardiotomy • blood culture, atypical bacterial
Autoimmune rheumatic disorder antibody and viral titres;
Other Neoplastic
• renal function to exclude uraemia;
Uraemia
Trauma • serial cardiac enzymes, which may
Aortic dissection
Hypothyroidism show a modest rise;
Irradiation
• as directed by suspicion of
Drugs, eg hydralazine
underlying cause (Table 29).

Chest radiograph
Clinical presentation • evidence of an underlying disease; This is often normal, but look for
evidence of pericardial effusion,
• signs associated with tamponade.
Common malignancy, tuberculosis or aortic
dissection.
• Chest pain: usually retrosternal Investigations
or left precordial in location, Echocardiography
radiating to the neck. The pain is Echocardiography is useful to
aggravated by supine posture, exclude pericardial effusion or
coughing, deep inspiration and The diagnosis of acute
suspicion of aortic dissection.
pericarditis is based on the
swallowing; it is eased by sitting
following triad:
up and leaning forward. It may be Differential diagnosis
preceded by a few days of malaise. • typical chest pain;
Consider the following:
• pericardial friction rub;
• Fever. • classical ECG changes. • acute coronary syndrome;

• aortic dissection;
Uncommon
ECG • pulmonary embolism;
• Dyspnoea.
The ECG may be normal. There
• musculoskeletal pain.
• Symptoms of any underlying is an initial, widespread (not V1
cause. or aVR), upwardly concave ST Treatment
elevation, followed by return of
• Acute epigastric pain mimicking
the ST segments to baseline and Emergency
an acute abdomen.
flattened T waves. T waves then Pericardiocentesis if there is cardiac
• Anginal type pain. become inverted before returning to tamponade.
normal over 1 week. It is necessary
• Cardiac tamponade.
to distinguish these changes from Short term
those of acute myocardial infarction, Admit if there is severe pain or
Physical signs
in which ST elevation is convex and large effusion, or for treatment of
The patient may present with the
regional, R waves are lost, Q waves underlying condition. Treatment
following:
form and conduction abnormalities should include bed-rest with oral
• pericardial friction rub; may develop (Fig. 60). Acute NSAIDs given for symptomatic
pericarditis generally affects the relief. Consider corticosteroids if
• fever;
ECG more extensively, with little in there is severe pain not responding
• atrial fibrillation; the way of the ‘reciprocal changes’ to NSAIDs after 48 hours.

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CARDIOLOGY: DISEASES AND TREATMENTS

›Fig. 60 ECG showing changes of acute pericarditis.

tamponade may cause death if starts to rise. Once intrapericardial


untreated, as may some of the pressure exceeds intracardiac
In cases of acute pericarditis
precipitating conditions. pressure, left ventricular filling
stop any oral anticoagulants
and cardiac output decline. With
because of the risk of intrapericardial
haemorrhage and tamponade. a gradual accumulation of fluid,
FURTHER READING volumes of up to 2 L may be
Maisch B. Pericardial diseases, with a present before left ventricular
focus on aetiology, pathogenesis, filling becomes compromised.
Long term pathophysiology, new diagnostic
If idiopathic relapsing pericarditis imaging methods, and treatment. Acute cardiac tamponade typically
develops, corticosteroids or Curr. Opin. Cardiol. 1994; 9: 379–88. follows cardiac trauma (which may
colchicine may be effective. be iatrogenic), aortic dissection,
Soler-Soler J, Sagrista-Sauleda J spontaneous bleeding or cardiac
Complications and Peranyer-Miralda G. Relapsing rupture after a myocardial
pericarditis. Heart 2004; 90: 1364–8.
Beware of the following: infarction. Chronic tamponade
usually results from malignancy,
• pericardial effusion;
idiopathic pericarditis or uraemia,
• idiopathic relapsing pericarditis; 2.6.2 Pericardial effusion although almost any cause of acute
pericarditis may be responsible.
• cardiac tamponade;
Aetiology/pathophysiology/
• constrictive pericarditis. pathology Clinical presentation
Pericardial effusion may develop in
Prognosis cases of acute pericarditis from any Common
There is complete resolution within cause. The normal pericardial space In chronic cases the patient may
3 months in 80% of patients, although contains 15–50 mL fluid and can be asymptomatic despite a large
20% develop idiopathic relapsing only accommodate a rapid increase effusion. Typical symptoms include
pericarditis with chronic symptoms in pericardial volume to 150–200 mL shortness of breath and and mild
of pericardial inflammation. Cardiac before the intrapericardial pressure chest discomfort.

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CARDIOLOGY: DISEASES AND TREATMENTS

Uncommon
In a large pericardial effusion without
tamponade, compression of adjacent
structures may lead to the following:

• dysphagia (oesophagus);

• cough (bronchus/trachea);

• hiccups (phrenic nerve);

• hoarseness (laryngeal nerve);

• abdominal bloating and nausea


(abdominal viscera).

Physical signs
In most patients, the examination
will be normal. In large effusions
without tamponade, there may be
›Fig. 61 (a) CXR demonstrating enlarged globular-shaped heart. (b) Transthoracic echocardiography
muffled heart sounds, crackles confirmed this was due to a large pericardial effusion, seen as an echo-free space around the heart (arrow)
in apical four-chamber view. After drainage of the effusion, cardiomegaly resolved on CXR (c) and an
(compression of lung parenchyma) effusion can no longer be seen around the heart at echocardiography (d).
or Ewart’s sign (patch of dullness
below the angle of the left scapula
caused by compression of the base Investigations Differential diagnosis
of the left lung). There may also In chronic cases consider the
be pericardial friction rub or signs ECG following:
of cardiac tamponade. Cardiac This is usually normal, although
tamponade should be suspected in a • constrictive pericarditis;
changes of acute pericarditis may be
shocked patient with apparent clear present. There may be a non-specific • restrictive cardiomyopathy.
lung fields and elevated JVP. reduction in QRS voltage and T- In those with tamponade
wave flattening. Electrical alternans consider causes of circulatory
is suggestive of a large effusion. collapse (see Acute Medicine,
Cardiac tamponade may Section 1.2.2), in particular massive
present in three ways: Chest radiograph pulmonary embolus and severe
1. cardiac arrest; This is often normal, although a asthma.
2. a severely ill patient who is large effusion may cause an enlarged
stuporous or agitated and restless globular cardiac silhouette with Treatment
(survivor of acute tamponade); clear lung fields. Look for separation
3. with dyspnoea, chest pain, weight
Consider the following.
of the pericardial fat lines and a
loss, anorexia and weakness (more • Urgent pericardiocentesis is
slowly developing tamponade). left-sided pleural effusion (Fig. 61).
essential in cardiac tamponade.
Rapid recognition of the clinical signs It is also needed for diagnosis
Echocardiography
of tamponade is essential. if there is suspicion of purulent
Echocardiography is the most or tuberculous pericarditis, or
• Tachypnoea and tachycardia.
• Pulsus paradoxus (pulse becomes sensitive test for detection of prolonged and otherwise
impalpable on inspiration in severe pericardial fluid (as little as 20 mL). unexplained illness.
cases). This appears as an echo-free space
• Elevated JVP with prominent systolic • Symptom relief as for
around the heart (Fig. 61b).
x descent and absence of diastolic y acute pericarditis (see
Diastolic left and right heart
descent. Section 2.6.1).
• Rarely, normal JVP in severe
collapse, marked decrease in mitral
dehydration. inflow with inspiration and a dilated • Recurrent or persistent
inferior vena cava that fails to symptomatic effusions may
If you suspect the diagnosis, organise
an urgent echocardiogram. collapse with inspiration suggest require balloon pericardiostomy
tamponade. or surgical pericardiectomy.

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CARDIOLOGY: DISEASES AND TREATMENTS

Complications Before the 1960s, tuberculous Investigations


constrictive pericarditis was the
Common most common cause of pericardial ECG
Complications arise more commonly constriction worldwide. In the This may be normal or show
from the underlying condition than developed world its importance non-specific generalised T-wave
from the effusion, although chronic has declined, and the aetiology changes, low-voltage complexes
pericardial effusion lasting more is usually idiopathic, post or atrial fibrillation.
than 6 months may be seen. This radiotherapy or post surgery.
is more likely after idiopathic, Chest radiograph
uraemic, myxoedematous or Clinical presentation This is usually normal, but the
malignant pericarditis. cardiac silhouette may be either
Common reduced or enlarged. Left atrial
Uncommon Oedema, abdominal swelling enlargement, pleural effusions
Uncommon complications are and discomfort caused by ascites and pericardial calcification are
cardiac tamponade and constrictive or hepatic congestion are most non-specific findings.
pericarditis. frequent. Vague abdominal
symptoms such as postprandial Echocardiography
fullness, dyspepsia, flatulence Echocardiography shows pericardial
FURTHER READING and anorexia may also be present. thickening. Septal motion is abnormal
Chong HH and Plotnick GD. Pericardial Cachexia and fatigue suggest a and the left ventricular posterior
effusion and tamponade: evaluation, reduced cardiac output. wall flattens abruptly in early
imaging modalities, and management.
diastole due to rapid equalisation of
Compr. Ther. 1995; 21: 378–85.
Uncommon left and right ventricular pressures.
Exertional dyspnoea and During inspiration there is a marked
Devlin GP, Smyth D, Charleson HA,
et al. Balloon pericardiostomy: a new orthopnoea may occur when reduction in diastolic mitral inflow
therapeutic option for malignant ventricular pressures become (Fig. 62).
pericardial effusion. Aust. NZ J. Med. severely elevated, as may
1996; 26: 556–8. Cardiac catheterisation
platypnoea (dyspnoea in
upright position). This is usually needed to confirm
Tsang TS, Oh JK and Seward JB.
the diagnosis, with characteristic
Diagnosis and management of
Physical signs equalisation of end-diastolic
cardiac tamponade in the era of
echocardiography. Clin. Cardiol. 1999; pressures in the two ventricles,
22: 446–52. Common persisting with respiration and
Elevation of the JVP with fluid challenge (Fig. 63).
prominent x and y descents is the
2.6.3 Constrictive pericarditis most important clinical sign, plus CT/MRI
the following: These imaging techniques may be
Aetiology/pathophysiology/ used to demonstrate the extent and
pathology • atrial fibrillation; distribution of pericardial thickening
Constrictive pericarditis is • Kussmaul’s sign (inspiratory rise (Fig. 64).
characterised by an abnormally in JVP);
thickened and non-compliant Differential diagnosis
pericardium, which abruptly limits • pericardial knock (third heart Consider the following:
ventricular filling in mid to late sound);
• chronic pericardial effusion;
diastole. This results in elevated
• hepatosplenomegaly, ascites and
end-diastolic cardiac filling pressures • restrictive cardiomyopathy
peripheral oedema;
and the equalisation of pressure (see Section 2.4.3);
in all four chambers at end diastole. • cachexia.
• superior vena cava obstruction
As cardiac filling is compromised,
(excluded if there is a pulsatile
cardiac output is reduced. The Uncommon
waveform in JVP);
clinical features are secondary Pulsus paradoxus and signs of severe
to systemic venous congestion. liver failure. • congestive cardiac failure;

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CARDIOLOGY: DISEASES AND TREATMENTS

pericardiectomy: an early operation


is recommended.

Complications
Severe venous congestion with
chronic hepatic impairment is
common. Death results from the
consequences of an inadequate
cardiac output.

Prognosis

Morbidity
Without treatment, most patients
deteriorate progressively with
severely limiting symptoms. With
pericardiectomy, 90% improve and
›Fig. 62 M-mode parasternal long-axis view of a patient with pericardial constriction demonstrating 50% may gain complete relief of
thickened pericardium with small effusion. Note the notching of the septum and abrupt flattening of the
posterior wall in diastole. Both these features are due to abrupt equalisation of left and right ventricular symptoms.
pressures in early diastole, with cessation of further cardiac filling.

Mortality
• nephrotic syndrome (see Treatment The outlook in untreated cases is
Nephrology, Section 2.1.4); A minority of patients poor. Hospital mortality rate after
may be managed medically pericardiectomy is 5 –16%, and
• malignant hepatic or with diet and diuretic 5-year survival rate after surgery
intra-abdominal disease. therapy. Most will require is 74 – 87%.

›Fig. 63 Pericardial constriction: the top section of the image shows pressure tracings from catheters placed simultaneously in the left and right ventricles. The
pressures from the two chambers are seen to equalise at the end of diastole. (Courtesy of E. Tomsett.)

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CARDIOLOGY: DISEASES AND TREATMENTS

›Fig. 64 Pericardial thickening: these sections (a–d) through the heart show a markedly thickened pericardium.

cardiac abnormalities can be


FURTHER READING 2.7 Congenital heart associated with genetic syndromes
Mehta A, Mehta M and Jain AC.
Constrictive pericarditis. Clin. Cardiol.
disease or other diseases (Table 30).
Abnormalities are divided into
1999; 22: 334–44.
cyanotic and acyanotic conditions.
Advances in paediatric cardiology The most common conditions are
Tuna IC and Danielson GK. Surgical
management of pericardial diseases. over the past three decades have reviewed in this section (Table 31).
Cardiol. Clin. 1990; 8: 683–96. resulted in the survival of a large Diagnosis of these conditions
number of individuals with depends on accurate imaging of
Vaitkus PT and Kussmaul WG. congenital heart disorders into underlying cardiac abnormalities.
Constrictive pericarditis vs. restrictive adulthood. Management of these This is increasingly performed using
cardiomyopathy: a reappraisal and
patients is a challenging area of non-invasive techniques such as
update of diagnostic criteria. Am. Heart
J. 1991; 122: 1431–41.
cardiology, with over 100 separate echocardiography and cardiac MRI.
diagnostic conditions. Congenital Both of these diagnostic methods

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CARDIOLOGY: DISEASES AND TREATMENTS

venosus in type (Fig. 65). The


TABLE 30 EXAMPLES OF GENETIC DISEASES AFFECTING THE haemodynamic consequences
CARDIOVASCULAR SYSTEM depend on the size of the defect.
In larger defects blood can shunt
Syndrome Inheritance Cardiac manifestations Management from the left atrium to the right
because the right ventricle (RV)
Marfan’s AD Aortic root Echo surveillance, beta-blockade
dilatation/rupture and elective aortic root repair is more compliant than the left.
when >5.5 cm in diameter As a result, over time the RV
Turner’s Coarctation of aorta and Echo surveillance of valve and may dilate and fail leading to
bicuspid aortic valve surgery for coarctation the development of pulmonary
Noonan’s AD Pulmonary stenosis most hypertension and possibly
common Eisenmenger’s syndrome
Down’s Atrioventricular canal Surgical (see Section 2.7.3).
defects
DiGeorge’s AD or sporadic Conotruncal Surgical
Williams’ Supravalvar aortic Echo surveillance
stenosis A patent foramen ovale is
Holt–Oram AD Septal defects See Sections 2.7.1.1 and 2.7.1.2 common in young people. It
(hand–heart) does not permit left-to-right shunting
but allows right-to-left shunting when
Muscular Various Conduction defects. Pacing for heart block, standard
dystrophies Cardiomyopathy may treatment for left ventricular right atrial pressure exceeds left (eg
also be seen in female dysfunction, echocardiography Valsalva manoeuvre). This can result in
carriers of X-linked and ECG screening of carriers an increased risk of stroke as a result
dystrophies of paradoxical embolism.

See also cardiomyopathy (Section 2.4) and muscular dystrophies (Neurology, Section 2.2.3).
AD, autosomal dominant.

Epidemiology
ASDs are the most common
TABLE 31 COMMON CONGENITAL HEART DISORDERS congenital heart abnormality and
account for around 30% of all
Cyanotic Acyanotic
congenital heart defects in adults
Tetralogy of Fallot Atrial septal defect (female/male ratio 2:1 and 75%
Complete transposition of the great arteries Ventricular septal defect are ostium secundum).
Ebstein’s anomaly Patent ductus arteriosus
Coarctation of the aorta
Clinical presentation
In most adults ASDs are
can also be used to calculate asymptomatic and discovered
intracardiac shunts, which play • tetralogy of Fallot; incidentally as a result of other
• Ebstein’s anomaly;
an important part in determining investigations. Symptoms are more
• ventricular septal defect;
treatment. As a consequence cardiac • patent ductus arteriosus; likely if there is a left-to-right shunt
catheterisation is performed less • coarctation of the aorta. and include:
frequently.
• atrial arrhythmias (including
atrial fibrillation, atrial flutter
2.7.1 Acyanotic congenital
and sick sinus syndrome);
In congenital heart disease heart disease
particular care should be taken • exertional fatigue or dyspnoea;
to prevent infective endocarditis 2.7.1.1 Atrial septal defect
associated with dental or surgical
• right heart failure;
procedures by using appropriate
Anatomy/pathophysiology/ • stroke/other arterial territory
prophylactic antibiotics. The highest
risk is associated with high-pressure
pathology infarcts (eg lower limbs)
jets of blood: An atrial septal defect (ASD) may be secondary to paradoxical
ostium primum, secundum or sinus embolism.

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CARDIOLOGY: DISEASES AND TREATMENTS

Transoesophageal echocardiography
with microbubble contrast study
is ideal for visualising shunts
(Fig. 66).

Treatment
Surgical and percutaneous
closure are both possible.
Haemodynamically non-significant
ASDs do not require closure unless
to prevent paradoxical emboli.
In asymptomatic patients with a
pulmonary/systemic flow ratio
(shunt) in excess of 1.5:1 or mild to
moderate pulmonary hypertension,
closure should be considered.

›Fig. 65 Sites of defects in the atrial septum seen from the right atrium. AV, atrioventricular; IVC, inferior
vena cava; SVC, superior vena cava. Prognosis
• Shunt <1.5:1 has an excellent
long-term outcome.
Physical signs pulmonary arteries and pulmonary
plethora indicating increasing • Shunt >1.5:1, if untreated,
• Along with increasing flow
pulmonary blood flow. restricts life expectancy to
through the right heart, an
fifth decade (RV failure, rarely
ejection systolic pulmonary flow
Echocardiography Eisenmenger’s syndrome). Good
murmur and wide fixed splitting
Transthoracic and transoesophageal long-term outcome if ASD is
of S2 (equalisation of right and
echocardiography can be used for closed before development of
left ventricular stroke volumes
confirmation of diagnosis. pulmonary hypertension.
throughout the respiratory cycle)
can be heard.

• RV heave with large shunts.

There is no murmur across an


ASD itself.

Investigations

ECG
Look for the following:

• right-axis deviation and right


bundle-branch block;

• left-axis deviation in ostium


primum defects;

• atrial arrhythmias.

Chest radiograph
Look for cardiomegaly (from right
›Fig. 66 Transoesophageal echocardiographic image of left-to-right flow across a large atrial septal
atrial and RV enlargement), dilated defect. A, atrial septum; B, left atrium; C, flow into right atrium. (Courtesy of Dr L.M. Shapiro.)

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2.7.1.2 Isolated ventricular Chest radiograph with maternal rubella and in


septal defect Normal in small defects. preterm infants.
With progressive shunts there
Anatomy/pathophysiology/ are radiographic signs of Clinical presentation
pathology pulmonary hypertension, including
• Small PDA: patients are
Defects can occur at any level in cardiomegaly, enlarged pulmonary
asymptomatic and PDA is detected
the ventricular septum. A small trunk and reduced lung markings in
incidentally from murmur or
defect causes a high-pressure left- the periphery.
cardiac imaging for other causes.
to-right jet but no haemodynamic
abnormality. Large defects with Echocardiography • PDA with moderate or large shunt:
a shunt can eventually result Used to confirm diagnosis and patients present with progressive
in Eisenmenger’s syndrome calculate shunt. fatigue, dyspnoea, palpitations or
(see Section 2.7.3). Small defects in eventually Eisenmenger’s
the membranous part of the septum Treatment syndrome.
noted during childhood often close Surgical closure of the defect should • Infective endocarditis is a risk and
by the age of 2 years. be considered. Small VSDs without may be the first presentation.
a shunt do not need closure. VSDs
Epidemiology with a haemodynamically significant
Physical signs
Ventricular septal defect (VSD) is shunt should be closed if pulmonary
the second most common congenital hypertension is not severe and/or • Continuous machinery murmur in
malformation of the heart, reversible. the second left intercostal space.
accounting for 20% of all • Significant shunt: wide pulse
congenital malformations. Prognosis pressure, hyperdynamic apex and
Long-term outcome in patients who signs of left ventricular failure
Clinical presentation have successful closure of their VSD (in large shunts).
Small defects with no shunt are
prior to development of pulmonary • Pulmonary hypertension:
usually asymptomatic and detected
hypertension is excellent. machinery murmur shortens
incidentally, secondary to the
(as proportion of cardiac cycle
identification of a cardiac murmur.
2.7.1.3 Patent ductus arteriosus during which pulmonary pressure
Defects with a significant shunt
is lower than systemic pressure
present with progressive dyspnoea,
Anatomy/pathophysiology/ reduces) and eventually
reduced exercise tolerance and
pathology disappears.
eventually cyanosis in adult life.
The ductus arteriosus is part of
the lung bypass circuit in the fetal Investigations
Physical signs
circulation (connecting the left
• Pansystolic murmur and thrill at pulmonary artery to the descending ECG
the lower left sternal edge. aorta) that ensures oxygenated blood Normal in small PDA. With large
from the right heart is delivered shunts there is evidence of left atrial
• Signs of pulmonary hypertension.
directly to the systemic circulation. and left ventricular hypertrophy.
• Clubbing and cyanosis of The ductus normally closes soon
Eisenmenger’s syndrome. after birth. However, in a small Chest radiograph
number of people it can remain Normal in small PDA. With large
Investigations patent, resulting in a left-to-right shunts there is initially left-sided
shunt that leads to left ventricular and then right-sided cardiomegaly,
ECG enlargement and eventually prominent proximal pulmonary
In a small VSD it is often normal. pulmonary hypertension. arteries/ascending aorta and
However, with progressive pulmonary plethora.
worsening of the shunt there Epidemiology
may be atrial arrhythmias (eg atrial Patent ductus arteriosus (PDA) Echocardiography
fibrillation), right-axis deviation accounts for 10% of all congenital Can be used to identify defect,
and right ventricular hypertrophy. heart cases. The incidence is higher calcuate extent of the shunt and

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estimate pulmonary arterial


pressures.

Treatment
Both surgical (very low mortality
of <0.5%, as does not require
cardiopulmonary bypass) and
percutaneous closure (suitable
anatomy) of PDA are possible.
Closure of all clinically detectable
PDA (in the absence of irreversible
pulmonary hypertension) is
recommended.

Prognosis
Excellent outcome if PDA
closed before development of
any complications. Without closure,
one-third of patients will be dead
by the age of 40 years and two-thirds
by 60 years. Potential complications
include: ›Fig. 67 MRI scan of coarctation of the aorta (arrow).

• infective pulmonary artery


endarteritis and consequent
Epidemiology • Left sternal edge and/or
septic pulmonary emboli;
This condition is more common in interscapular systolic murmur
• ductal aneurysm and calcification males than in females (3:1). emanating from the coarctation.
can also occur with risk of
rupture. Clinical presentation Investigations

2.7.1.4 Coarctation of the aorta Common ECG


Often normal, but there may
• Asymptomatic: incidental
Anatomy/pathophysiology/ be evidence of left ventricular
finding of murmur or systolic
pathology hypertrophy.
hypertension in the upper
Aortic coarctation is the result
limbs.
of a flow-limiting narrowing Chest radiograph
in the aorta at the site of the Rib notching of posterior third
fetal ductus arteriosus (see Uncommon to eighth ribs (from enlarged
Section 2.7.1.3), most commonly • Symptoms of hypertension intercostal arteries because of
just distal to the left subclavian (epistaxis, headache and collateral flow; Fig. 13). Prestenotic
artery (may also be positioned haemorrhagic stroke). and poststenotic dilatation of the
more proximally in the aortic aorta can give rise to a ‘3’ sign
arch; Fig. 67). With time an • Palpitations. appearance.
arterial supply to the lower • Claudication in the legs.
body is achieved by extensive Echocardiography
collateralisation. Often hypertension • Heart failure. Can be used for diagnosis of
develops during childhood. coarctation and calculation of
Most patients have other Physical signs gradient acoss stenosis.
abnormalities, including
• Systolic BP is usually greater in
bicuspid aortic valve (50 – 80% MRI and CT
the arms than in the legs.
of cases) and intracranial Both can be used for accurate
aneurysms of the circle of Willis. • Radiofemoral delay. diagnosis.

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altered mental activity, fatigue,


myalgia and visual problems.
Dehydration can worsen the
situation. Recurrent venesection
is one possible treatment.

Iron-deficiency anaemia
This is often a result of excessive
venesection, but can also occur
secondary to haemoptysis and
epistaxis.

Stroke
Arterial thrombosis may be
associated with polycythaemia.
Alternatively, thrombotic strokes
may occur secondary to paradoxical
emboli. Haemorrhagic strokes
are also possible as coagulation
›Fig. 68 Angiogram showing coarctation of aorta, which can be seen to taper just after the arch. On a
plain radiograph the feature to look for is rib notching produced by collateral vessels (Fig. 13). pathways may be deranged, thereby
increasing the risk of bleeding.
Angiography • Corrected: survival depends on
May aid in diagnosis, although MRI the age at surgery, the younger Bleeding abnormalities
and CT may preclude the need the better. There is a range of presentations,
(Fig. 68). ranging from easy bruising to
Disease associations epistaxis and haemoptysis.
Treatment Bleeding can be catastrophic.
• Turner’s syndrome.
• Surgical repair is often the first-
line treatment if gradient across • Bicuspid aortic valve. Renal impairment
the coarctation is >30 mmHg. Overt renal failure is rare and
• Circle of Willis aneurysms.
often associated with increased uric
• Percutaneous dilatation with or acid absorption from excessive
without stent deployment is also 2.7.2 Cyanotic congenital erythrocyte degradation.
possible. However, routine current heart disease
use is confined to treatment of Patients with cyanotic congenital
2.7.2.1 Tetralogy of Fallot
paediatric or adolescent cases, heart disease are chronically
and for the dilatation of repeat hypoxaemic. This causes a number
Anatomy/pathophysiology/
coarctation. Percutaneous treatment of adaptive physiological changes
pathology
in adults remains experimental. leading to complications. These
Four anatomical defects contribute
adaptive changes are common
to this abnormality (Fig. 69):
Complications to all patients in this group with
These may include the following: significant cyanosis regardless of the 1. large ventricular septal defect
underlying anatomical abnormality. (VSD);
• left ventricular failure;
These changes must be treated
2. overriding of the aorta;
• aortic dissection; accordingly as detailed below.
3. right ventricular outflow tract
• premature coronary disease.
Polycythaemia and hyperviscosity obstruction (RVOTO);
syndrome
Prognosis 4. compensatory right ventricular
Polycythaemia increases
hypertrophy (RVH).
• Uncorrected: mean survival is oxygen delivery but the resultant
around 35 years and 75% will die hyperviscocity can result in multiple The large VSD results in equal left
by the age of 50 years. symptoms including headaches, and right ventricular pressures.

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• Patients with partial correction


develop increasing cyanosis in
adult life as RVOTO increases.

• Patients who have had total


correction can present later in life
with palpitations and syncope
(from atrial and ventricular
arrhythmias) as well as heart
failure as pulmonary and/or aortic
valve regurgitation develops.

• All patients have complications


of cyanosis.

Physical signs
• Cyanosis.

• Clubbing (Fig. 70).


›Fig. 69 Tetralogy of Fallot with left Blalock–Taussig shunt. Ao, aorta; LA, left atrium; LV, left ventricle;
PA, pulmonary artery; PV, pulmonary veins; RA, right atrium; RV, right ventricle. • There may be unequal upper
limb pulses in patients with
However, there is right-to-left Blalock–Taussig shunts.
shunting (cyanosis) as result of
RVOTO. Shunting (and hence level
of cyanosis) increases with any
decrease in systemic vascular
resistance. Most patients require
some form of partial or complete
surgical correction to ensure
their survival into adult life.
Partial surgical correction involves
a surgical shunt to increase
pulmonary flow and reduce cyanosis,
commonly achieved by a Blalock–
Taussig shunt (Fig. 69), which
connects the subclavian artery to
the pulmonary artery.

Epidemiology
This is the most common cyanotic
congenital heart defect to occur after
infancy and accounts for around
10% of all cogenital heart
abnormalities.

Clinical presentation
• Patients who have had no
correction present with cyanosis
from birth or early infancy and
become progressively more short
of breath on exertion as their age
increases. ›Fig. 70 Example of digital clubbing in a patient with cyanotic heart disease.

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• Right ventricular heave and right Complications circulations are connected in


ventricular outflow tract thrill. These are often seen in patients who parallel. The aorta arises anteriorly
have complete correction as a child: from the right ventricle (RV),
• Normal S1, inaudible P2.
the systemic ventricle, and the
• ventricular arrhythmias;
• Right ventricular outflow tract pulmonary artery (PA) from the
ejection systolic murmur. • atrial fibrillaton and/or flutter; left ventricle (LV). As a result the
two circulations are completely
• pulmonary regurgitation with right
Investigations separate. A communication such
ventricular dilatation and failure;
as a patent foramen ovale, patent
ECG • recurrent RVOTO. ductus arteriosus or atrial septal
Can illustrate right-axis deviation, defect/ventricular septal defect
RVH and right bundle-branch Prognosis between the two circulations is
block. necessary for survival (Fig. 71).
• Without surgical repair, the
Other anatomical variants, such
survival rate is 66% at age 1
Chest radiograph as congenitally corrected TGA,
and 10% at age 20 years.
Will detect boot-shaped heart. also exist.
• With complete surgical correction
Echocardiography outcomes are excellent, with over Clinical presentation
Demonstrates all anatomical 94% of patients surviving at least The majority of patients who survive
features which constitue the 25 years. to adulthood will have had a surgical
condition. corrective procedure soon after
2.7.2.2 Complete transposition birth.
Cardiac MRI of great arteries
• Cyanosis is present from birth.
As with echocardiography, cardiac
MRI can be used to identify Anatomy/pathophysiology/ • Heart failure and valvular
anatomical abnormalities, but has pathology regurgitation (secondary to
the added advantage of identifying In patients with complete degenerative changes) are
other thoracic (pulmonary vascular transposition of the great arteries commonly seen in adults who
and aortic) abnormalities. (TGA) the pulmonary and systemic have had corrective surgery.

Cardiac catheterisation
Useful preoperatively for identifying
coronary arterial abnormalities.

Treatment
Most patients now undergo
complete repair during infancy. In
adulthood most of these individuals
will require repeat surgical
procedures for repair of recurrent
VSDs and regurgitation in the right
ventricular outflow tract/pulmonary
valve and/or aortic valve. Adults
who have had partial repair (eg
Blalock–Taussig shunt) should be
considered for complete repair
in adult life as cyanosis worsens
or other complications develop.
Surgical repair is still recommended
›Fig. 71 TGA with the circulations mixing through a patent ductus arteriosus. The effect of an atrial
for adults who have had no form of switch operation is shown in dashed lines, re-routing the venous return to the correct great vessel. In the
correction. This is now extremely arterial switch, the great vessels are transected above the valves and switched, with reimplantation of the
coronaries. Ao, aorta; IVC, inferior vena cava; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PV,
rare in the developed world. pulmonary veins; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

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Physical signs restore physiological circulation. patients survive more than


Blood from the pulmonary veins is 20–30 years after corrective
• Cyanosis.
directed through the mitral valve surgery.
• Signs of heart failure. and blood from the systemic venous
return through the tricuspid valve. 2.7.2.3 Ebstein’s anomaly
• Single loud S2 (anterior aorta).
However, the RV remains the
systemic ventricle and commonly Anatomy/pathophysiology/
Investigations
fails in adult life (Fig. 71). pathology
An abnormal tricuspid valve
ECG Arterial switch procedures Blood is
(regurgitant or stenotic) is displaced
Findings are variable and depend on redirected at the arterial level by
down into the right ventricle, leaving
exact anatomy and type of surgical switching the position of the aorta
a small functional right ventricle.
correction. These include normal and the PA. Therefore, the LV is
A majority of patients (80%)
traces, atrial arrhythmias, RV connected to the aorta and the
have other associated cardiac
hypertrophy and right bundle- RV to the PA.
abnormalities, including atrial septal
branch block.
Heart transplantation Potential defect or patent foramen ovale (most
option for patients with significant common, seen in 50% of cases),
Chest radiograph
heart failure subsequent to aberrant conduction pathways,
Narrrow vascular pedicle and
corrective surgery. ventricular septal defect, coarctation
cardiomegaly.
of the aorta, patent ductus arteriosus
Prognosis and, on rare occasions, other more
Echocardiography
complex abnormalities. If the
Demonstrates anatomical changes, • Without intervention the mortality
abnormality is mild, most patients
calculates shunts and shows valvular rate is around 90% by 6 months.
remain asymptomatic well into adult
abnormalities post repair.
• Life expectancy is shortened in life. Right-to-left shunting secondary
most patients even if they do have to rising right atrial pressure
Cardiac MRI
surgical correction. Only 70% of (Fig. 72) leads to symptoms.
Very effective at demonstrating
anatomical abnormalities and
useful in monitoring patients
postoperatively.

Treatment

Emergency
Mixing of blood between the
pulmonary and systemic circulation
is vital for survival at birth. This can
be achieved with prostaglandin E to
maintain a patent ductus arteriosus
and/or atrial septostomy.

Surgery
A number of complex surgical
procedures have been developed.

Atrial switch procedures (Mustard


or Senning procedures) Blood is
redirected at the level of the atria
using a baffle made from either
Terylene (Dacron)/pericardium
(Mustard procedure) or atrial flaps ›Fig. 72 Ebstein’s anomaly with shunting across an associated atrial septal defect. Ao, aorta; IVC, inferior
vena cava; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PV, pulmonary veins; RA, right atrium; SVC,
(Senning procedure) in order to superior vena cava.

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Clinical presentation considered if there is progressive follow-up to ensure symptoms do


There is a wide spectrum of symptoms, cyanosis or heart not recur.
presentation. failure. Multiple surgical

• In mild disease patients remain


procedures are available 2.7.3 Eisenmenger’s syndrome
depending on the exact anatomy,
entirely asymptomatic well into
including tricuspid valve Aetiology/pathophysiology/
adult life and diagnosis is made
repair/replacement and more pathology
incidentally.
complex surgical corrective A large left-to-right shunt causes
• Progressive dyspnoea, reduced procedures. increased pulmonary blood flow.
exercise tolerence and palpitations Over time this can result in vascular
can occur in the event of a • Complications (such as heart
obstructive disease leading to
worsening shunt. failure and arrhythmias) should
pulmonary hypertension and right
be treated conventionally.
ventricular hypertrophy. As the
• In rare instances sudden death
pulmonary arterial pressures
can occur as a result of
Complications approach and exceed systemic
conduction abnormalities.
Common complications include pressures, the original left-to-right
arrhythmias arising from the atria, shunt becomes bidirectional and
Physical signs
often from aberrant conduction then reverses (right to left). This
• Low JVP secondary to a large pathways (eg Wolff–Parkinson– shunt reversal results in cyanosis.
compliant right atrium. White syndrome). These changes and their associated
complications are known as
• Pansystolic murmur of tricuspid
regurgitation.
Prognosis Eisenmenger’s syndrome and can
Patients with no symptoms have complicate both ‘simple’ (eg atrial
• Hepatomegaly. a good prognosis without surgical septal defect and ventricular septal
correction and can survive well defect) as well as more ‘complex’
• Cyanosis depending on the extent
into adult life. The medium-term congenital cardiac abnormalities.
of the right-to-left shunt.
prognosis of patients who have Shunt reversal often takes many
undergone successful surgery is years and typically occurs in the
Investigations
good, but they require long-term third decade (Fig. 73).

ECG
An ECG may be normal, but common
abnormalities include tall/broad P
waves, right bundle-branch block,
first-degree heart block, delta waves
if abnormal conduction pathways
present and atrial arrhythmias
(atrial fibrillation and flutter).

Chest radiograph
Convex right heart border secondary
to right atrial enlargement.
Cardiomegaly is also a possibility.

Echocardiography and cardiac MRI


Both can be used to make the
diagnosis and outline other
associated cardiac abnormalities.

Treatment
• Repair of the anatomical
›Fig. 73 Eisenmenger’s syndrome secondary to ventricular septal defect. Ao, aorta; LA, left atrium; LV, left
abnormality should be ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle.

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Clinical presentation Echocardiography


Can be used to identify the 2.8 Infective diseases of
• Cyanosis.
anatomic site and direction of the heart
• Limiting exertional dyspnoea. the shunt. The shunt can also
be quantified.
• Palpitations secondary to atrial
2.8.1 Infective endocarditis
arrhythmias (atrial fibrillation
Cardiac catheterisation
and flutter) and ventricular
Pulmonary pressure and saturation Aetiology/pathophysiology/
arrhythmias (ventricular
can be measured directly. It also pathology
tachycardia).
allows assessment of pulmonary Microbial infection of the
• Haemoptysis. vascular reactivity, which may endocardium develops as a result
determine the succes of future of a two-stage process.
• Syncope and sudden death.
surgical procedure.
1. Non-bacterial thrombotic
• Congestive heart failure.
endocarditis (NBTE): a sterile
Treatment
mass arises from the deposition
Physical signs The main management principle
of platelets and fibrin on areas
in this group of patients is to avoid
• Cyanosis. of endocardium injured from
any factors that may destabilise
exposure to high-velocity jets,
• Clubbing. the circulation and to treat any
flow from high- to low-pressure
complications. In those with
• Signs of pulmonary hypertension: chambers, and flow across a
reactive pulmonary vessels,
right ventricular heave, right narrow orifice.
surgical correction of the underlying
ventricular outflow tract thrill,
shunt may be of value. In the 2. Bacteraemia with organisms that
loud P2, pulmonary ejection flow
remaining patients, a combination have the capacity to adhere to
murmur and high-pitched early
of oxygen therapy, prostacyclin NBTE result in the formation of
diastolic murmur of pulmonary
and/or calcium channel blockers infected vegetation. Bacteraemia
regurgitation (Graham Steell
may provide variable levels of rates are higher in the presence
murmur).
disease stability and marginal of diseased mucosa, especially if
• Signs of tricuspid regurgitation symptom improvement. Lung infected.
(elevated JVP and pansystolic and/or heart transplantation
murmur). may be required (see The consequences of infective
Section 2.12.1). endocarditis (IE) vary from trivial
to catastrophic valvular and
Prognosis paravalvular tissue destruction.
After diagnosis, the 10-year survival Risk factors for IE are classified as
In Eisenmenger’s syndrome,
is 80% and the 25-year survival 40%. cardiac or non-cardiac and are
the murmur of the original
A poor prognosis is associated with summarised in Table 32.
shunt will have disappeared.
the following:
Epidemiology
• syncope.
The incidence of IE in North
Investigations • signs of right ventricular failure; America and Western Europe
remains unchanged at around
• low cardiac output;
ECG 1.7–6.2 per 100,000 person-years.
Peaked P waves (right atrial • severe hypoxaemia. The risk of IE increases with age
enlargement), right ventricular and it is more common in men.
hypertrophy and atrial Prevention However, the risk profile of IE
arrhythmias. Early closure of haemodynamically patients has changed over the past
significant left-to-right shunts and/or decade. Rheumatic heart disease is
Chest radiograph protective pulmonary artery banding no longer the leading cause of IE
Prominent central pulmonary will reduce pulmonary flow and and has been replaced by increasing
arteries and ‘pruning’ of peripheral avoid development of pulmonary cases of IE ocurrring secondary to
pulmonary vessels. vascular disease. intravenous drug abuse,

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• pulmonary, causing lung abscess


TABLE 32 RISK FACTORS FOR IE or pneumonia;

System Level of Risk Cause • other arterial territories including


joints and soft-tissue infections.
Cardiac High Prosthetic heart valves
Aortic and mitral (especially regurgitation) valve disease
Cyanotic congenital heart disease
Uncorrected left-to-right shunts (except atrial septal defect)
Moderate Mitral valve prolapse/isolated mitral stenosis IE must always be considered
Tricuspid and pulmonary valve disease in a patient presenting with
Hypertrophic cardiomyopathy chronic fever, weight loss and malaise,
irrespective of the presence of a
Low Isolated ASD
MVP with no regurgitation murmur. Elderly patients may present
Corrected left-to-right shunt with little apart from confusion. The
Atrial myxoma absence of the much over-emphasised
Cardiac pacemakers ‘classic’ signs of Janeway’s lesions,
Osler’s nodes, etc does not exclude
Non-cardiac Recurrent bacteraemia (intravenous drug abuse and
periodontal disease) the diagnosis.
Medical conditions increasing risk of infection (eg diabetes,
renal failure and immunosuppression)

ASD, atrial septal defect; IE, infective endocarditis; MVP, mitral value prolapse. Investigations
A definite pathological diagnosis
can be achieved only via isolation
implantation of intracardiac devices, • Renal: features of or culture of the bacteria from
haemodialysis and increasing age. glomerulonephritis and renal material obtained from a vegetation
impairment. or abscess. This is not possible
Clinical presentation and physical in most patients, and diagnosis
signs Embolic events depends on a combination of clinical
IE can present rapidly (acute IE) Septic emboli seen in 20 –40% of criteria. The modified Duke Criteria
or insidiously (subacute IE) over a cases including: (Table 33) based on a number
number of days to weeks. Clinical of major and minor clinical and
• cerebral, leading to abscess
features of IE are varied and at microbiological features are
formation and focal neurological
times it can be difficult to initially designed to assist diagnosis of IE.
abnormalities;
differentiate from other chronic Cases are defined as follows.
medical conditions (Fig. 74). • spleen, leading to pain secondary
• Definite IE: presence of two major
to infarction;
criteria, one major and three minor
Features of systemic sepsis
• renal, resulting in renal failure; criteria or five minor criteria.
Fever and rigors, sweats, general
malaise, anorexia and weight loss.

Local cardiac manifestations


TABLE 33 DUKE CRITERIA FOR THE DIAGNOSIS OF IE
New cardiac murmur, heart failure,
Criteria Grade Clinical Evidence
pericarditis and conduction
abnormalities. Major Microbiological: typical microorganism isolated from two separate blood
cultures or persistently positive blood cultures
Evidence of cardiac involvement: echocardiographic evidence of new
Features of immune complex
native or prosthetic valve regurgitation, vegetation and/or intracardiac
deposition abscess
• Dermatological: petechiae (most Minor Presence of risk factor predisposing to IE (see Table 32)
common), splinter haemorrhages, Fever >38°C
Vascular complications
Osler’s nodes and Janeway lesions. Immune complications
Microbiological findings that do not meet major criteria
• Ophthalmological: Roth spots,
conjunctival and retinal IE, infective endocarditis.
haemorrhage.

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›Fig. 74 Peripheral manifestations of IE including: (a) finger splinter haemorrhage; (b) Osler’s nodes; (c) Conjunctival petechiae; (d) Janeway’s lesions.

• Possible IE: presence of one major Blood tests Biochemical profile:


and one minor criteria or three FBC:
minor criteria. • Renal function (urea
• Mild-to-moderate normochromic/ and creatinine) may be
• Unlikely diagnosis: presence normocytic anaemia. deranged.
of firm alternative diagnosis,
• Neutrophil leucocytosis is usual in
or sustained resolution of • Liver function tests may be
acute IE.
clinical features with less abnormal, especially alkaline
than 4 days of antibiotic • Thrombocytopenia in chronic phosphatase and γ-glutamyl
therapy. infections. transpeptidase.

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›Fig. 74 Peripheral manifestations of IE including: (e) angiogram demonstrating mycotic aneurysm of iliac artery; (f) CT brain scan showing cerebral infarction in a
patient with staphylococcal endocarditis; (g) coronary angiogram showing embolic obstraction of the left anterior descending artery in a parent with streptococcus
bovis endocarditis; and (h) the same patient as in panel (g) after angioplasty. (Adapted and with permission from BMJ Publishing Group; from Habib G. Management
of infective endocarditis. Heart 2006; 92: 124–30.)

Acute-phase markers: • Reduced complement levels. sites, leaving around 60 minutes


between each puncture.
• C-reactive protein (CRP) and • Positive rheumatoid factor.
erythrocyte sedimentation rate • Discuss cultures with
(ESR) are typically elevated. Microbiology microbiologist to ascertain if there
Blood culture: is a need for prolonged cultures
Immunology:
and the use of specific culture
• Polyclonal increase in serum • Take at least three to four sets media for infections caused by
immunoglobulins. of blood cultures from different fastidious organisms and fungi.

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TABLE 34 INFECTIVE ORGANISMS ISOLATED IN MICROBIOLOGICAL


INVESTIGATIONS A normal echocardiogram does
not exclude a diagnosis of IE.

Infective organisms Frequency (%)

Streptococci (especially Streptococcus viridans group) 50–60 Treatment


Staphylococci (coagulase-positive Staphylococcus aureus, 25
coagulase-negative Staphylococcus epidermidis) Medical treatment
Enterococci 10 Antibiotics are the main therapy.
‘Blind’ treatment (Table 35 shows a
Culture-negative organisms 5–10
Fastidious organisms (Mycobacteria, Brucella, Neisseria, Legionella sample protocol) should be started
and the HACEK group of oropharyngeal organisms) as soon as a clinical diagnosis of IE
Previous antibiotic therapy is suspected (after multiple blood
Chlamydia
Rickettsiae (eg Coxiella) cultures have been taken). Early
disscussion with microbiology and
Gram-negative bacteria, mixed organisms and fungi <1
cardiothoracic teams is important.
HACEK, Haemophilus, Actinobacillus, Cardiobacterium, Eikinella, Kingella. Identification of the infective
organism is invaluable and antibiotic
treatment should be modified