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Clinical Cardiology
Clinical Cardiology
Current Practice Guidelines
Updated Edition
Demosthenes G. Katritsis, MD, PhD, FRCP, FACC
Athens Euroclinic, Athens, Greece
Beth Israel Deaconess Medical Center, Harvard Medical School,
Boston, MA, USA
Bernard J. Gersh, MB, ChB, DPhil, FRCP, FACC
Mayo Medical School, Rochester, MN, USA
A. John Camm, MD, FRCP, FACC
St George’s University of London, UK

1
3
Great Clarendon Street, Oxford, OX2 6DP,
United Kingdom
Oxford University Press is a department of the University of Oxford.
It furthers the University’s objective of excellence in research, scholarship,
and education by publishing worldwide. Oxford is a registered trade mark of
Oxford University Press in the UK and in certain other countries
© Oxford University Press 2016
The moral rights of the authors have been asserted
First Edition published in 2013
Updated Edition published in 2016
Impression: 1
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, without the
prior permission in writing of Oxford University Press, or as expressly permitted
by law, by licence or under terms agreed with the appropriate reprographics
rights organization. Enquiries concerning reproduction outside the scope of the
above should be sent to the Rights Department, Oxford University Press, at the
address above
You must not circulate this work in any other form
and you must impose this same condition on any acquirer
Published in the United States of America by Oxford University Press
198 Madison Avenue, New York, NY 10016, United States of America
British Library Cataloguing in Publication Data
Data available
Library of Congress Control Number: 2015947232
ISBN 978–0–19–873332–4
Printed in Great Britain by
Ashford Colour Press Ltd, Gosport, Hampshire
Oxford University press makes no representation, express or implied, that the
drug dosages in this book are correct. Readers must therefore always check
the product information and clinical procedures with the most up-to-date
published product information and data sheets provided by the manufacturers
and the most recent codes of conduct and safety regulations. The authors and
the publishers do not accept responsibility or legal liability for any errors in the
text or for the misuse or misapplication of material in this work. Except where
otherwise stated, drug dosages and recommendations are for the non-pregnant
adult who is not breast-feeding
Links to third party websites are provided by Oxford in good faith and
for information only. Oxford disclaims any responsibility for the materials
contained in any third party website referenced in this work.
Personal dedication by Demosthenes G. Katritsis
To Michael M. Webb-Peploe
Mentor, Teacher, Friend
 Foreword

Over the years I have had the pleasure of writing forewords for a number of books that I considered to be timely and to
fulfill important objectives. Without hesitation, I would say that Clinical Cardiology: Current Practice Guidelines, by D.G.
Katritsis, B.J. Gersh, and A.J. Camm, is the most outstanding book for which I have had the pleasure to write a foreword.
Further, this is probably the book that better serves the cardiovascular specialist in day-to-day practice than any other
written in the last two decades. This is not just a textbook; it is an extraordinary “toolkit” in the context of an evidence-
based cardiovascular practice in the midst of rapidly evolving scientific knowledge and guidelines.
Because of the need to integrate current knowledge on evidence-based cardiology, about three years ago, under the
auspices of the American Heart Association, we published a book that included the most recent guidelines by both the
ACC/AHA and the ESC. I believe that such integration was a step forward for the practicing cardiologist; indeed, in a
“synopsis” fashion, this aspect is well served in Clinical Cardiology: Current Practice Guidelines. However, in the excel-
lent compendium of my colleagues, three new components are incorporated, which we can describe as the “jewel” of the
book: a very succinct definition, classification, pathophysiology, diagnosis, management, and need of specific clinical in-
vestigation (including genetics and molecular biology) of the various disease entities; a regularly updated online version
on the most recent developments; and, most importantly a “user friendly, at a glance” presentation. These additional three
components, that make Clinical Cardiology: Current Practice Guidelines so unique, deserve a brief description.
1) In regard to the various disease entities, general textbooks tend to employ, from definition to management, a
rather long and descriptive format. In contrast, Clinical Cardiology: Current Practice Guidelines consolidates many
of the topics, regardless of their complexity, from definition to management, in a clear, concise and instructive way,
intermixed with the most recent guidelines. Thus, over 600 easily accessible tables dissect and summarize the key
points of all the latest ACC/AHA and ESC guidelines.
2) Rapidly evolving scientific knowledge, including the value of new diagnostic and management approaches and
their incorporation in practicing guidelines, makes it difficult for the cardiovascular specialist to be aware of the
latest clinical evidence-base. Written by three leading authorities in the field, its annually updated online version
provides the solution.
3) A novelty of this book is the “user-friendly, at a glance” way of presentation that makes it very useful to the practic-
ing cardiovascular specialist. Useful because of its combination of succinctness and clarity, the book is up to date
in every aspect of the cardiovascular science, and particularly on the most recent recommendations from both
sides of the Atlantic. Thus, these recommendations are summarized in tables derived from the guideline docu-
ments and incorporated in the appropriate diagnostic or management sections of the 87 comprehensive chapters.
For example, when confronted with complicated clinical issues that appear in everyday clinical practice (such as
modern antiplatelet therapy of ACS, differential diagnosis of wide complex tachycardia, or management of stable
CAD in view of COURAGE, FREEDOM or STICH) physicians consult general textbooks, or often several journal
articles, in order to obtain this information in a rather loose form. In contrast, Clinical Cardiology: Current Practice
Guidelines consolidates such topics in a summarized, succinct, and clear way.
This book is a tribute to the skill of the three editors who also served as the only authors. This limited, but unified and
hardworking, internationally known authorship is, without doubt, a great part of the success. It is with great pleasure that
I pen these words to relate my enthusiasm for their work as a remarkable addition to the cardiovascular field.

Valentin Fuster
Physician-in-Chief, Mount Sinai Medical Center
Director, Mount Sinai Heart
ΠΑΝΤΕΣ ΑΝΘΡΩΠΟΙ ΤΟΥ ΕΙΔΕΝΑΙ ΟΡΕΓΟΝΤΑΙ ΦΥΣΕΙ
All humans by nature desire to know

Aristotle
The Metaphysics
 Prologue

The entire field of cardiovascular medicine has witnessed an era of rapid scientific progress, accompanied by continu-
ous technological and applied innovation. This occurs against a backdrop of increasing emphasis on the importance of
evidence-based practice, and rapid development of guidelines by major professional societies. The resultant expansion of
our body of knowledge by evidence-based recommendations interjects a new set of challenges for the practicing clinician
with ever-extensive clinical responsibilities.
In order to practice evidence-based medicine, information must be easily accessible and, more importantly, easily
retrievable when the need arises; this may not always be easy with the current pace of dissemination of knowledge. The
rationale for writing this book reflects exactly this need, both ours and that of our potential readers: to organize our
continually evolving knowledge on often diverse cardiology issues, in our environment of networked and facilitated com-
munication. In other words, to provide a clinical tool that can be used in everyday clinical practice as a concise guide to
what we know and, more importantly, what we do not know, and what we think we know. To quote Mark Twain, “what
gets us into trouble is not what we don’t know, it is what we know for sure that just ain’t so.”
The prerequisites of informed clinical practice are: a satisfactory background of basic knowledge of disease entities,
remaining up-to-date on important clinical trials and emerging scientific evidence that shape current diagnosis and
therapy, and acquaintance with current practice guidelines from established professional societies such as the American
College of Cardiology Foundation/American Heart Association (ACC/AHA) and the European Society of Cardiology
(ESC), among many others.
Each chapter of this book has therefore been structured around the following parts:
1. A clear definition and modern classification of disease entities, followed by updated, focused information on
recent developments on the epidemiology and pathophysiology of each condition. Recent original articles and
reviews from leading journals were consulted and a summary of the most relevant information is included. Special
care was taken not to omit the most recent information on medical genetics, an expanding and promising aspect.
2. A description of the clinical presentation of the disease, with instructions on necessary clinical investigations.
Clinical investigations are presented in the context of recent evidence that dictates their current value or obsoles-
cence. An effort has been made to include the very latest published knowledge on the clinical value of existing and
evolving tests, based on recent randomized clinical trials and guidelines by both ACC/AHA and ESC.
3. Recommendations on management as derived from the most recent evidence available to the authors. Because
of the comprehensive nature of guidelines offered by learned societies, it was also decided to provide the most
recent recommendations in a summarized, tabulated format. These are not readily accessible since overlapping
guidelines may appear on the same condition from different working groups, and updated documents are continu-
ally appearing. Thus, all guideline documents and their updates published in the US and Europe were scrutinized
and classified according to year of publication. The most recent recommendations were defined, extracted and
tabulated. The resulting tables provided in the book offer the most recent recommendations on each disease entity
by both ACC/AHA and ESC. Where appropriate, new evidence that questions the validity of specific recommenda-
tions, as well as the opinions of established experts, and other data, such as FDA alerts are included.
4. Practical advice on “what and why to do”. Therapies, drug doses and selection of procedures are presented in a
clear and user-friendly way.
5. Carefully chosen references. Major randomized clinical trials and seminal scientific studies that define evidence-
based practice are included for further reference. In addition, recent, scholarly reviews are provided, which together
with the contents of the book should allow in-depth study of specific entities that may interest the individual reader.
6. Presentation of all recent guidelines. Guidelines are referenced and presented separately in order to guide the
reader to the most recent publications by ACC/AHA and ESC. Thus, the most recent recommendations on each
particular issue, as they appear in new and updated guidelines, are presented.
An inherent disadvantage of a medical textbook is inability to keep up-to-date with recent developments. To overcome
the problem, the online version of this book will be updated, initially on an annual basis. The updated edition of the book
emphasizes our commitment to this task.
 PROLOGUE ix

This book would have never been possible without the wholehearted support and commitment of Helen Liepman, our
Senior Editor at Oxford University Press. We are grateful for her acceptance of our view of a “next generation textbook”.
We are grateful to Dr P. Kostaki of Athens Euroclinic for her scholarship and dedication in proof-reading and correcting
our text. Finally, we also thank involved staff at Oxford University Press. Their professionalism and assistance throughout
the revision and production process are much appreciated.

Demosthenes G. Katritsis
Bernard J. Gersh
A. John Camm
 Contents

List of abbreviations xxviii

Part 1 Adult congenital heart disease

1 Adult congenital heart disease: general principles 3
Definition 3
Epidemiology 3
Aetiology 3
Recurrence rate 4
Clinical problems in ACHD 4
Imaging techniques and investigations 5
Principles of therapy 6
2 Ventricular septal defects 11
Definition and classification 11
Epidemiology 12
Aetiology 12
Pathophysiology 12
Presentation 12
Physical examination 13
Investigations 13
Therapy 13
Pregnancy 14
3 Atrioventricular septal defects 15
Definitions and classification of atrioventricular septal defects 15
Ostium primum atrial septal defects 16
4 Atrial septal defects18
Ostium secundum atrial septum defect 18
Sinus venosus defect 20
Patent foramen ovale 20
5 Patent ductus arteriosus 23
Definition 23
Epidemiology 23
Aetiology 23
Pathophysiology and natural history 23
Presentation 24
Physical examination 24
Investigations 24
Therapy 25
xii CONTENTS

6 Right ventricular outflow tract obstruction 26

Definitions and classification of RVOT obstruction 26
Valvular pulmonary stenosis 26
Subvalvular pulmonary stenosis 26
Supravalvular pulmonary stenosis 27
Branch pulmonary artery stenosis 28
7 Left ventricular outflow tract obstruction 29
Definitions and classification of LVOT obstruction 29
Valvular aortic stenosis (bicuspid aortic valve) 29
Subvalvular aortic stenosis 32
Supravalvular aortic stenosis 33
8 Coarctation of the aorta 34
Definitions 34
Epidemiology 34
Pathophysiology and natural history 34
Presentation 35
Physical examination 35
Investigations 35
Therapy 35
Pregnancy 36
9 Tetralogy of Fallot 37
Definition 37
Epidemiology 37
Aetiology 37
Pathophysiology 37
Presentation 37
Physical examination 37
Investigations 37
Therapy 38
Indications for intervention after repair 39
Participation in exercise 39
Pregnancy 39
10 Transposition of great arteries 40
Definitions and classification of transposition 40
Complete transposition (d-TGA) 40
Congenitally corrected transposition (l-TGA) 44
11 Ebstein’s anomaly 47
Definition 47
Epidemiology 47
Presentation 47
Physical examination 47
 CONTENTS xiii

Investigations 47
Therapy 47
Pregnancy 49
12 Anomalous PV connections, AV malformations, coronary
and LV abnormalities 50
Total anomalous pulmonary venous connection 50
Partial anomalous pulmonary venous connection 50
Pulmonary arteriovenous malformations 50
Aneurysms of the pulmonary artery 50
Congenital coronary anomalies 50
Coronary fistulas 51
Left ventricular protrusions 52
13 Univentricular heart (tricuspid atresia/single ventricle) 52
Definition 52
Pathophysiology and presentation 52
Therapy 53
14 The Fontan patient 53
Definition 53
Pathophysiology 53
Clinical problems 55
Therapy 55
15 Eisenmenger syndrome 56
Definition 56
Pathophysiology 56
Clinical problems 56
Physical examination 56
Investigations 56
Therapy 57

Part 2 Valve disease

16 General principles 61
Epidemiology 61
Cardiac auscultation 61
Investigations 62
Management 63
Rheumatic fever and endocarditis prophylaxis 63
Pregnancy 65
Non-cardiac surgery 65
17 Mitral stenosis
66
Epidemiology 66
Aetiology 66
xiv CONTENTS

Pathophysiology and natural history 66

Presentation 67
Physical examination 67
Investigations 67
Therapy 70
Non-cardiac surgery 74
Pregnancy 74
18 Mitral regurgitation 75
Classification 75
Epidemiology 75
Aetiology 75
Pathophysiology and natural history 76
Presentation 76
Physical examination 76
Investigations 77
Therapy 79
Non-cardiac surgery 84
Pregnancy 84
19 Aortic stenosis 86
Epidemiology 86
Aetiology 86
Pathophysiology and natural history 87
Presentation 90
Physical examination 90
Investigations 90
Therapy 92
Non-cardiac surgery 98
Pregnancy 101
20 Aortic regurgitation 104
Epidemiology 104
Aetiology 104
Pathophysiology and natural history 104
Presentation 106
Physical examination 106
Investigations 106
Therapy 107
Non-cardiac surgery 109
Pregnancy 110
21 Tricuspid valve disease 111
Tricuspid regurgitation 111
Tricuspid stenosis 116
 CONTENTS xv

22 Pulmonary valve disease 117

Pulmonary valve regurgitation 117
Pregnancy 119
Pulmonary valve stenosis 119
23 Prosthetic heart valves 119
Risk stratification for surgery 119
Mechanical valves 120
Tissue valves (bioprostheses) 120
Anticoagulation 122
Haemorrhage 125
Thrombosis of prosthetic valves 125
Haemolysis 128
Prosthetic valve stenosis and regurgitation 128
MRI 129
Pregnancy 129

Part 3 Systemic hypertension

24 Classification and pathophysiology of hypertension 135
Definition 135
Epidemiology 136
Aetiology 136
Pathophysiology 136
Subtypes of hypertension 137
Blood pressure measurement 138
25 Primary (essential) hypertension 141
Risk stratification 141
Physical examination 141
Investigations 141
Therapy 145
Resistant hypertension 162
Hypertensive emergencies 163
Hypertension in pregnancy 164
26 Secondary hypertension 170
Introduction 170
Obstructive sleep apnoea 170
Renal parenchymal disease 171
Renovascular hypertension 172
Primary aldosteronism 173
Phaeochromocytoma 174
Adrenal ‘incidentaloma’ 174
Other causes of hypertension 174
xvi CONTENTS

Part 4 Coronary artery disease

27 Epidemiology and pathophysiology of coronary
artery disease 179
Definitions and classification 179
Epidemiology 180
Aetiology 182
Pathophysiology 183
Assessment of cardiovascular risk 185
28 Non-ST elevation acute coronary syndromes 189
Definition 189
Presentation 189
Diagnosis 189
Therapy 193
Invasive vs conservative management 204
Specific clinical settings 210
Complications 220
Risk stratification before discharge 223
Post-hospital discharge care 223
29 Acute myocardial infarction 231
Definition 231
Presentation 232
Physical examination 232
Diagnosis 232
Initial therapy and medication 237
Reperfusion therapy 243
Complications of myocardial infarction 256
Discharge 264
Chronic therapy 265
Stem cell transplantation 267
30 Stable coronary artery disease 274
Definition 274
Presentation 274
Physical examination 274
Investigations 274
Differential diagnosis 284
Risk stratification 286
Therapy 294
Evaluation and risk assessment before non-cardiac surgery 341
 CONTENTS xvii

Part 5 Heart failure

31 Classification, epidemiology, and pathophysiology
of heart failure 365
Definitions and classification 365
Epidemiology 366
Aetiology 366
Pathophysiology 367
32 Chronic heart failure 369
Presentation 369
Physical examination 370
Investigations 371
Prognosis 378
Therapy 378
Pregnancy 407
33 Heart failure with preserved left ventricular ejection fraction 412
Definition 412
Aetiology and pathophysiology 412
Epidemiology 412
Clinical presentation 413
Diagnosis 413
Therapy 413
34 Acute heart failure and cardiogenic shock 414
Acute heart failure 414
Cardiogenic shock 421

Part 6 Cardiomyopathies
35 Classification of cardiomyopathies 427
Introduction 427
American Heart Association classification 427
European Society of Cardiology classification 427
MOGE(S) classification 428
36 Dilated cardiomyopathy 432
Definition 432
Epidemiology 432
Aetiology and pathophysiology 432
Presentation 435
Physical examination 435
Investigations 435
xviii CONTENTS

Risk stratification 436

Therapy 437
Prognosis 438
Pregnancy 438
37 Hypertrophic cardiomyopathy 440
Definition 440
Epidemiology 441
Aetiology 441
Pathophysiology 442
Presentation and natural history 442
Physical examination 442
Investigations 443
Differential diagnosis 450
Risk stratification 451
Therapy 453
Physical activity and sports 463
Follow-up 463
Family counselling and genetic testing 464
Pregnancy 467
38 Restrictive cardiomyopathy 469
Definition 469
Pathophysiology 469
Aetiology 469
Presentation 471
Physical examination 471
Investigations 471
Differential diagnosis 473
Therapy 474
39 Arrhythmogenic right ventricular
cardiomyopathy/dysplasia 476
Definition 476
Epidemiology 476
Aetiology 476
Pathophysiology 477
Presentation 477
Investigations 478
Differential diagnosis 482
Risk stratification 483
Therapy 484
Genetic testing 484
Pregnancy 484
 CONTENTS xix

40 Peripartum cardiomyopathy 486

Definition 486
Epidemiology 486
Aetiology 486
Presentation 486
Investigations 486
Therapy 487
Delivery 487
Prognosis 487
41 Tachycardiomyopathy 488
Definition 488
Epidemiology 488
Aetiology 488
Pathophysiology 489
Presentation 489
Diagnosis 489
Therapy 489
42 Stress cardiomyopathy 490
Definition 490
Pathophysiology 490
Diagnosis 490
Therapy 491
43 Iron overload cardiomyopathies 491
Definition 491
Aetiology 491
Pathophysiology 492
Presentation 493
Diagnosis 493
Therapy 493
44 Left ventricular non-compaction 494
Definition 494
Epidemiology 494
Aetiology 494
Presentation 494
Diagnosis 494
Therapy 494

Part 7 Myocarditis
45 Acute myocarditis 499
Definition and classification 499
xx CONTENTS

Epidemiology 499
Aetiology 499
Pathophysiology 500
Presentation 500
Physical examination 500
Investigations 500
Therapy 501
Prognosis 502

Part 8 Pericardial disease

46 Pericardial anatomy and congenital pericardial defects 507
Pericardial anatomy 507
Congenital pericardial defects 507
47 Acute and relapsing pericarditis 507
Acute pericarditis 507
Recurrent pericarditis 513
Specific conditions 515
48 Pericardial effusion and cardiac tamponade 516
Pericardial effusion 516
Cardiac tamponade 518
49 Constrictive pericarditis 522
Definition 522
Aetiology 522
Pathophysiology 522
Presentation 523
Physical examination 523
Investigations 523
Differential diagnosis 524
Therapy 524

Part 9 Tachyarrhythmias
50 Classification of tachyarrhythmias, mechanisms of arrhythmogenesis,
and acute management 529
Definitions and classification 529
Electrophysiological mechanisms of arrhythmogenesis 530
Differential diagnosis of tachyarrhythmias 535
51 Epidemiology, presentation, and therapy of supraventricular tachycardias 538
Definitions and classification 538
Epidemiology of SVT 538
Presentation of SVT 538
 CONTENTS xxi

Therapy of SVT 539

SVT in congenital heart disease 544
SVT in pregnancy 546
52 Atrial tachycardias 548
Atrial and junctional premature beats 548
Physiological sinus tachycardia 548
Inappropriate sinus tachycardia 549
Sinus reentrant tachycardia 549
Focal atrial tachycardia 550
Multifocal atrial tachycardia 554
Macro-reentrant atrial tachycardias (atrial flutters) 554
Cavotricuspid isthmus-dependent atrial flutter 555
Non-cavotricuspid isthmus-dependent atrial flutter 555
53 Atrial fibrillation 561
Definitions and classification 561
Epidemiology 561
Aetiology 562
Pathophysiology 563
Mortality 565
Diagnosis 566
Investigations 566
Risk stratification 566
Therapy 567
AF in specific conditions 599
AF in pregnancy 602
54 Atrioventricular junctional tachycardias 613
Atrioventricular nodal reentrant tachycardia 613
Non-reentrant junctional tachycardias 620
55 Atrioventricular reentrant tachycardias 622
Definitions 622
Epidemiology 622
Pathophysiology 622
Aetiology 624
Presentation 624
Diagnosis 624
Risk stratification in WPW syndrome 624
Therapy 625
56 Ventricular arrhythmias 630
Definitions and classification 630
Pathophysiology 631
Presentation 632
xxii CONTENTS

Differential diagnosis of wide QRS tachycardia 633

Acute therapy of ventricular arrhythmias 636
Risk stratification 640
Long-term therapy 645
Clinical forms of ventricular arrhythmias 651

Part 10 Genetic channelopathies

57 Definitions of inherited arrhythmias 679
Definitions 679
Glossary of terms 681
58 Long QT syndrome 684
Definition 684
Epidemiology 684
Genetics and pathophysiology 684
Presentation 685
Diagnosis 685
Genetic testing 689
Therapy 689
59 Short QT syndrome 693
Definition 693
Genetics and pathophysiology 693
Presentation 694
Diagnosis 694
Therapy 696
60 Brugada syndrome 697
Definition 697
Epidemiology 697
Genetics and pathophysiology 697
Presentation 698
Diagnosis 698
Risk stratification 702
Therapy 704
61 Catecholaminergic polymorphic ventricular tachycardia 706
Definition 706
Epidemiology 706
Genetics and pathophysiology 707
Presentation 707
Diagnosis 707
Therapy 708
 CONTENTS xxiii

62 Early repolarization syndromes 709

Definition 709
Genetics and pathophysiology 710
Clinical significance 711
Diagnosis 713
Therapy 713

Part 11 Bradyarrhythmias
63 The cardiac conduction system 717
Overview 717
64 Sinus nodal disease 719
Sinus bradycardia 719
Sick sinus syndrome 719
65 Atrioventricular and intraventricular block 724
Atrioventricular block 724
Atrioventricular dissociation 730
Intraventricular block 730
66 Conduction disease in specific conditions 735
Recent myocardial infarction 735
Congenital AV block 735
Pacing in adult congenital heart disease 736
Sleep apnoea 736
Neuromuscular disorders 736
Pacing after cardiac surgery, TAVI, and cardiac transplantation 737
Pacing in pregnancy 738
Other pacing indications 738

Part 12 Syncope and sudden cardiac death

67 Syncope 743
Definition 743
Epidemiology 743
Classification 743
Pathophysiology 744
Presentation 744
Aetiologic diagnosis 744
Investigations 746
Risk stratification 751
Therapy 751
Driving 756
xxiv CONTENTS

68 Sudden cardiac death 758

Definition 758
Epidemiology 758
Aetiology 760
Pathophysiology 761
Investigations in survivors 761
Management of cardiac arrest 764

Part 13 Implantable devices

69 Technical issues 769
Permanent pacemakers 769
Biventricular pacing 772
Implantable cardioverter-defibrillators 772
Magnetic resonance imaging 774
Electromagnetic interference 776
Radiotherapy 776
Driving, sports, and sexual activity 776
70 Procedural issues and complications of implantable devices 778
Device implantation in the anticoagulated patient 778
Reimplantation 779
Perioperative management of patients with devices 779
Implantation-related complications 782
Device-related infections 782

Part 14 Diseases of the aorta

71 Acute aortic syndromes 791
Definitions and classification 791
Epidemiology 793
Aetiology and pathophysiology 793
Presentation 794
Physical findings 795
Diagnosis 795
Therapy 800
Follow-up 804
72 Thoracic aortic aneurysms and other conditions 805
Thoracic aortic aneurysms 805
Aortic arch and thoracic aortic atheroma and atheroembolic disease 810
Cardiovascular conditions associated with thoracic aortic disease 811
 CONTENTS xxv

73 Genetic syndromes associated with thoracic aneurysm and dissection 812

Marfan’s syndrome 812
Other heritable syndromes and genetic defects associated with thoracic aortic disease 816
74 Inflammatory diseases associated with thoracic aortic disease 817
Introduction 817
Takayasu’s arteritis 817
Giant cell (temporal) arteritis 819

Part 15 Venous thromboembolism

75 Venous thromboembolism: epidemiology and aetiology 823
Definitions 823
Epidemiology 823
Aetiology 823
Diagnostic thrombophilia testing 824
76 Pulmonary embolism 825
Pathophysiology of PE 825
Presentation 826
Diagnosis 826
Risk stratification 830
Acute therapy 832
Extended therapy 833
Prevention of VTE 834
Special conditions 836
77 Deep vein thrombosis 841
Diagnosis and therapy 841

Part 16 Pulmonary hypertension

78 Definitions and classification of pulmonary hypertension 845
Definition and classification 845
Presentation and differential diagnosis 846
79 Pulmonary arterial hypertension and hypertension associated with pulmonary
venous abnormalities 851
Pulmonary arterial hypertension 851
Pulmonary hypertension associated with pulmonary venous capillary
abnormalities 865
80 Pulmonary hypertension associated with left heart disease and lung disease, high-
altitude disease, and chronic thromboembolic pulmonary hypertension 867
Pulmonary hypertension associated with left heart disease 867
xxvi CONTENTS

Pulmonary hypertension associated with lung disease 869

Chronic thromboembolic pulmonary hypertension 870

Part 17 Infective endocarditis

81 Infective endocarditis 879
Definition 879
Epidemiology 879
Aetiology 879
Pathophysiology 879
Presentation 881
Clinical features 881
Diagnosis 881
Therapy 885
Complications 891
Prognosis 894
Pregnancy 895
Prophylaxis 895

Part 18 Rheumatic fever

82 Rheumatic fever 901
Definition 901
Epidemiology 901
Aetiology 901
Pathophysiology 901
Presentation 901
Clinical forms 901
Diagnosis 902
Therapy 904
Prophylaxis 904

Part 19 Athlete’s heart

83 Athlete’s heart 909
Exercise-induced cardiac remodelling 909
Interpretation of the ECG in athletes 909
Arrhythmias in athletes 914
Classification of sports 914
Pre-participation screening, and recommendations for eligibility and
disqualification 915
 CONTENTS xxvii

Part 20 Cardiac tumours and pseudoaneurysms

84 Cardiac tumours 921
Primary cardiac tumours 921
Cardiac metastases 921
85 Pseudoaneurysms of the heart 923
Introduction 923
Aetiology 923
Diagnosis 923
Treatment 923

Part 21 Cardiovascular disease in pregnancy

86 Cardiovascular disease in pregnancy 927
Overview 927

Part 22 Cardiovascular drugs

87 Cardiovascular drugs 933
Drug interactions 933
Antiarrhythmic drugs 933
Antiplatelet agents 933
Anticoagulants 933
Beta blockers, diuretics, ACEIs, ARBs, CCBs 933
Statins, fibrates 933
Drugs for erectile dysfunction 933
Appendix 1: Recommendation classes and levels of evidence
used in guidelines 935
Appendix 2: Specific therapy of endocarditis 937
Appendix 3: ESC 2011 Guidelines on pregnancy 949

Index 953
 List of abbreviations

< less than AV atrioventricular; aortic valve

> more than AVNRT atrioventricular nodal reentrant tachycardia
≥ equal to or greater than AVR aortic valve replacement
≤ equal to or less than AVRT atrioventricular reentrant tachycardia
~ approximately BAV bicuspid aortic valve
≈ approximately equal to bd twice daily
= equal to BLS basic life support
α alpha bpm beat per minute
β beta BMS bare metal stent
δ delta BMV balloon mitral valvotomy
γ gamma BNP brain natriuretic peptide
$ Dollar BP blood pressure
€ Euro bpm beats per minute
ACC American College of Cardiology BrS Brugada syndrome
ACCP American College of Chest Physicians BSA body surface area
ACE angiotensin-converting enzyme BUN blood urea nitrogen
ACEI angiotensin-converting enzyme inhibitor Ca++ calcium
ACHD adult congenital heart disease CABG coronary artery bypass grafting
ACS acute coronary syndrome CAD coronary artery disease
ACT activated clotting time cAMP cyclic adenosine monophosphate
ADP adenosine diphosphate CAVF coronary arteriovenous fistula
AF atrial fibrillation CCB calcium channel blocker
AH atrial-His CCD cardiac conduction disease
AHA American Heart Association CCF congestive heart failure
AHF acute heart failure CCS Canadian Cardiovascular Society
AIDS acquired immunodeficiency syndrome CCT coronary artery computed tomography
AMI acute myocardial infarction CCTGA congenitally corrected transposition of the
AMP adenosine monophosphate great arteries
ANP atrial natriuretic peptide CCU Coronary Care Unit
Ao aorta CDT catheter-directed thrombolysis
AoD aortic dissection cGMP cyclic guanine monophosphate
AP action potential CHB congenital heart block
APB atrial premature beat CHD congenital heart disease
aPTT activated partial thromboplastin time CHF chronic heart failure
AR aortic regurgitation CIED cardiovascular implantable electronic device
ARB angiotensin receptor blocker CKD chronic kidney disease
ARF acute rheumatic fever CL cycle length
ARVC/D arrhythmogenic right ventricular cm centimetre
cardiomyopathy or dysplasia CMR cardiac magnetic resonance
AS aortic stenosis CMV cytomegalovirus
ASD atrial septal defects CO2 carbon dioxide
ASO arterial switch operation CoA coarctation of the aorta
AT atrial tachycardia COPD chronic obstructive pulmonary disease
AT1 angiotensin II type 1 CPAP continuous positive airway pressure
 LIST OF ABBREVIATIONS xxix

CPET cardiopulmonary exercise testing FA Friedreich’s ataxia

CPVT catecholaminergic polymorphic ventricular FDA Food and Drug Administration
tachycardia FDC familial dilated cardiomyopathy
CrCl creatinine clearance FFR fractional flow reserve
CRP C-reactive protein FMC first medical contact
CRT cardiac resynchronization therapy FIRM focal impulse and rotor modulation
CSNRT corrected sinus nodal recovery time g gram
CSM carotid sinus massage GAS group A Streptococcus
CSS carotid sinus syndrome GDF growth differentiation factor
CT computed tomography GFR glomerular filtration rate
CTEPH chronic thromboembolic pulmonary hyper- GI gastrointestinal
tension
GP glycoprotein
CTI cavotricuspid isthmus
GRACE Global Registry of Acute Coronary Event
CUS compression ultrasonography
h hour
CVA cerebrovascular accident
HA His-atrial
Cx circumflex
HBV hepatitis B virus
d day
HCM hypertrophic cardiomyopathy
2D two-dimensional
Hct haematocrit
3D three-dimensional
HCV hepatitis C virus
4D four-dimensional
HDL high density lipoprotein
Da Dalton
HELLP haemolysis, elevated liver enzymes, low plate-
DAD delayed after-depolarization let (count)
DAPT dual oral antiplatelet therapy HF heart failure
DC direct current HIV human immunodeficiency virus
DCC direct current cardioversion HIT heparin-induced thrombocytopenia
DCM dilated cardiomyopathy HLA human leucocyte antigen
DES drug-eluting stent H2O water
DFT defibrillator threshold HOCM hypertrophic obstructive cardiomyopathy
dL decilitre HRS Heart Rhythm Society
DNA deoxyribonucleic acid HRV heart rate variability
DSE dobutamine stress echocardiography Hz hertz
DTI direct thrombin inhibitor IABP intra-aortic balloon pump
DVT deep vein thrombosis IART intra-atrial reentrant tachycardia
dyn dyne ICD implantable cardioverter-defibrillator
EAD early after-depolarization IDC idiopathic dilated cardiomyopathy
EBV Epstein–Barr virus IE infective endocarditis
ECG electrocardiogram IFDVT iliofemoral deep vein thrombosis
ECS elastic compression stocking IHD ischaemic heart disease
EHRA European Heart Rhythm Association ILR implantable loop recorder
ELISA enzyme-linked immunosorbent assay IM intramuscular
ELT endless loop tachycardia IMH intramural haematoma
EMA European Medicines Agency IMT intima-media thickness
EP electrophysiology INR international normalized ratio
EPS electrophysiological study IOCM iso-osmolar contrast media
ERA endothelin receptor antagonist IPAH idiopathic pulmonary arterial hypertension
ERO effective regurgitant orifice (area) ISDN isosorbide dinitrate
ERS early repolarization syndrome IU international unit
ESC European Society of Cardiology IV intravenous
ESR erythrocyte sedimentation rate IVC inferior vena cava
xxx LIST OF ABBREVIATIONS

J Joule μm micron
JVP jugular venous pressure mm millimetre
K potassium mmHg millimetre mercury
KCl potassium chloride mmol millimole
kDa kilodalton μmol micromole
kg kilogram mo month
km kilometre mPAP mean pulmonary artery pressure
kPa kilopascal MPI myocardial perfusion imaging
L litre MPS myocardial perfusion stress
LA left atrium MRA magnetic resonance angiography; mineralo-
LAA left atrial appendage corticoid receptor antagonist
LAH left anterior hemiblock MRI magnetic resonance imaging
lb pound MRSA methicillin-resistant Staphylococcus aureus
LBBB left bundle branch block ms millisecond
LDL low density lipoprotein MS mitral stenosis
LDL-C low density cholesterol mSv milliSievert
LGE late gadolinium enhancement mV millivolt
LIMA left internal mammary artery MVA mitral valve area
LMWH low molecular weight heparin MVP mitral valve prolapse
LOCM low osmolar contrast media MVR mitral valve replacement
Lp(a) lipoprotein (a) Na sodium
LPH left posterior hemiblock NaCl sodium chloride
LQTS long QT syndrome ng nanogram
LVAD left ventricular assist device NIPPV non-invasive positive pressure ventilation
LVEDD left ventricular end-diastolic diameter NIV non-invasive ventilation
LVEDP left ventricular end-diastolic pressure NO nitric oxide
LVEF left ventricular ejection fraction NSAID non-steroidal anti-inflammatory drug
LVESD left ventricular end-systolic diameter NSTEMI non-ST elevation myocardial infarction
LVH left ventricular hypertrophy NSVT non-sustained ventricular tachycardia
LVNC left ventricular non-compaction NSTEACS non-ST elevation acute coronary syndrome
LVOT left ventricular outflow tract NTG nitroglycerin
LVOTO left ventricular outflow tract obstruction NYHA New York Heart Association
m metre O2 oxygen
MAT multifocal atrial tachycardia OAC oral anticoagulant
MBC mitral balloon commissurotomy od once daily
MBG myocardial blush grade OH orthostatic hypotension
MCT multidetector computed tomography OPAT outpatient parenteral antibiotic therapy
MDCT multidetector computed tomography OPCAB off-pump beating heart bypass surgery
MEN multiple endocrine neoplasia oz ounce
mEq milliequivalent p probability
METS metabolic equivalents PA pulmonary artery
mg milligram PAH pulmonary artery hypertension
mGy milligray PaO2 partial pressure of oxygen
MI myocardial infarction PAPVC partial anomalous pulmonary venous
connection
MIC minimum inhibitory concentration
PAU penetrating atherosclerotic ulcer
min minute
PAWP pulmonary artery wedge pressure
μL microlitre
PBV percutaneous balloon valvuloplasty
mL millilitre
PCC prothrombin complex concentrate
 LIST OF ABBREVIATIONS xxxi

PCDT pharmacomechanical catheter-directed RVEF right ventricular ejection fraction

thrombolysis RVOT right ventricular outflow tract
PCWP pulmonary capillary wedge pressure RVOTO right ventricular outflow tract obstruction
PCI percutaneous coronary intervention RWPT R wave peak time
PCR polymerase chain reaction s second
PDA patent ductus arteriosus SAECG signal-averaged electrocardiogram
PDE phosphodiesterase SAM systolic anterior motion
PDE-5I phosphodiesterase-5 inhibitor SaO2 oxygen saturation
PE pulmonary embolism SBP systolic blood pressure
PEEP positive end-expiratory pressure SC subcutaneous route
PES programmed electrical stimulation SCD sudden cardiac death
PFO patent foramen ovale SIHD stable ischaemic heart disease
pg pictogram SLE systemic lupus erythematosus
PH pulmonary hypertension SND sinus node dysfunction
PHV prosthetic heart valve SNP single-nucleotide polymorphism
PISA proximal isovelocity surface area SNRT sinus nodal recovery time
PJRT permanent junctional reciprocating SOBOE shortness of breath on exertion
tachycardia
SPECT single photon emission computed tomography
PMBV percutaneous mitral balloon valvotomy
SPERRI shortest pre-excited RR interval
PMC percutaneous mitral commissurotomy
sPESI simplified pulmonary embolism severity
po oral route index
PO2 partial pressure of oxygen SpO2 saturation of peripheral oxygen
POTS postural orthostatic tachycardia syndrome spp. species
PPCM post-partum cardiomyopathy SQTS short QT syndrome
PPM permanent pacemaker SR sinus rhythm
PMT pacemaker-mediated tachycardia SSS sick sinus syndrome
PR pulmonary regurgitation SSRI selective serotonin reuptake inhibitor
PV pulmonary vein STEMI ST elevation myocardial elevation
PVARP post-ventricular pacing atrial refractory SVC superior vena cava
period
SVR systemic vascular resistance
PVC premature ventricular contraction
SVT supraventricular tachycardia
PVOD pulmonary veno-occlusive disease
TAPSE tricuspid annular plane systolic excursion
PVR pulmonary vascular resistance; pulmonary
valve replacement TAPVC total anomalous pulmonary venous connec-
tion
Qp pulmonary flow
TAVI transcatheter aortic valve implantation
Qs systemic flow
TdP torsade de pointe
RA right atrium; rheumatoid arthritis
tds three times daily
RADT rapid antigen detection test
TEVAR thoracic endovascular aortic repair
RAO right anterior oblique
TGA transposition of great arteries
RAAS renin-angiotensin-aldosterone system
TIA transient ischaemic attack
RBBB right bundle branch block
TIC tachycardia-induced cardiomyopathy
RBC red blood cell
TIMI thrombolysis in myocardial infarction
RCA right coronary artery
TLR target lesion revascularization
RCM restrictive cardiomyopathy
TnI troponin I
RCT randomized controlled trial
TNK-tPA tenecteplase
RF radiofrequency; rheumatic fever
TnT troponin T
RNA ribonucleic acid
TOE transoesophageal echocardiogram
rPA rateplase
TOF tetralogy of Fallot
RVSP right ventricular systolic pressure
tPA tissue plasminogen activator
RV right ventricle
xxxii LIST OF ABBREVIATIONS

TR tricuspid regurgitation VHL von Hippel–Lindau

TS tricuspid stenosis VKA vitamin K antagonist
TTE transthoracic echocardiography VPB ventricular premature beat
TV tricuspid valve V/Q ventilation perfusion
TWA T wave alternans VSD ventral septal defect
U unit VT ventricular tachycardia
UA unstable angina VTE venous thromboembolism
UFH unfractionated heparin WBC white blood cell
ULN upper limit of normal WPW Wolff–Parkinson–White
URL upper reference limit WU Woods unit
V volt y year
VA ventricular arrhythmia
VD valve disease
VEGF vascular endothelial growth factor
VF ventricular fibrillation
 Part I

Adult congenital heart disease

Relevant guidelines with thoracic aortic disease. J Am Coll Cardiol. 2010;55:

e27–e129.
ACC/AHA 2008 Guidelines on ACHD
ACC/AHA 2008 guidelines for the management of adults with ESC 2014 Guidelines on aortic diseases
congenital heart disease. J Am Coll Cardiol. 2008;52:e1–e121. 2014 ESC Guidelines on the diagnosis and treatment of aortic dis-
eases. Eur Heart J. 2014;35:2873–926.
AHA 2015 Scientific Statement
American Heart Association Council on Clinical Cardiology. AHA/ACC 2014 Guidelines on valve disease
Congenital heart disease in the older adult: a scientific state- 2014 AHA/ACC Guideline for the management of patients with
ment from the American Heart Association. Circulation. valvular heart disease: J Am Coll Cardiol. 2014;63:e57–185.
2015;131:1884–931. AHA/ASA 2014 Guidelines on stroke and TIA
ESC 2010 Guidelines on ACHD Guidelines for the prevention of stroke in patients with stroke
ESC Guidelines for the management of grown-up congenital heart or transient ischemic attack: a guideline for healthcare profes-
disease. Eur Heart J. 2010;31:2915–57. sionals from the American Heart Association/American Stroke
Association. Stroke. 2014;45:2160–236.
PACES/HRS 2014 Consensus Statement on Arrhythmias
in ACHD ESC 2012 Guidelines on valve disease
Guidelines on the management of valvular heart disease.
PACES/HRS Expert Consensus Statement on the Recognition
Eur Heart J. 2012;33:2451–2496.
and Management of Arrhythmias in Adult Congenital Heart
Disease. Heart Rhythm. 2014;11:e102–e165. ESC 2011 Guidelines on pregnancy
ESC Guidelines on the management of cardiovascular diseases
ACC/AHA 2010 Guidelines on aortic disease
during pregnancy. Eur Heart J. 2011;32:3147–97.
2010 ACC/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM
Guidelines for the diagnosis and management of patients
 AETIOLOGY 3

Chapter 1

Adult congenital heart disease: general principles

Definition Aetiology
Congenital heart disease refers to a defect in the structure The causes of congenital heart disease in humans remain
of the heart and great vessels, which is present at birth. undefined in the majority of cases and probably depend on
the interplay of multiple genetic and environmental fac-
Epidemiology tors. There has been evidence that an intrinsically angio-
genic impairment exists in congenital heart disease, that
Approximately 0.8% of the population is born with con- appears to be present in both the maternal and fetal circu-
genital heart disease. Up to 40% of them are cured spon- lation and fetal heart.12
taneously (mainly small VSDs) and, with current surgical Environmental factors are rare: congenital rubella,
and interventional techniques, 56–98% survive into adult- maternal diabetes or SLE, paternal exposure to phthalates,
hood (adult congenital heart disease-ACHD, or grown-up maternal smoking, alcohol and drug abuse, air pollutants,
congenital heart disease-GUCH) (Table 1.1).1–3 According and pesticides.13,14 Weight control, smoking cessation, and
to data from Europe, Canada, and the USA, the live folic acid supplementation appear to decrease the risk of
birth prevalence of congenital heart disease is 7–12/1000 congenital heart disease in the offspring.8
births.2,3 Genetic factors Disruption at any point during cardiac
Adult congenital heart disease comprises a population primary morphogenesis (i.e. ornation of the heart tube,
that is currently estimated at one million in the USA and looping, septation, and resultant systemic and pulmo-
1.2 million in Europe, and admission rates in hospital nary circulations) results in the large spectrum of con-
are twice higher than in the general population.4–6 The genital heart defects. Genetic disorders responsible for
majority of patients with congenital heart disease are these alterations can be classified into three types: chro-
now adults.7 Congenital heart disease is the most com- mosomal disorders, single-gene disorders, and polygenic
mon form of heart disease complicating pregnancy in disorders.
the Western world (accounting for 74% of cases in the Chromosomal disorders (5–8% of congenital heart
Canadian Cardiac Disease in Pregnancy [CARPREG] disease patients), caused by absent or duplicated chro-
registry and 66% of cases in the European Registry on mosomes, include trisomy 21 (Down’s syndrome), 22q11
Pregnancy and Cardiac Disease [ROPAC] registry), deletion (DiGeorge syndrome), and 45X deletion (Turner’s
whereas, in less developed countries, rheumatic heart dis- syndrome). Recurrence risk in an offspring is that of the
ease plays a larger role.8 There is also a growing number chromosomal disorder.
of elderly ACHD patients (>60 years) with high mortality Single-gene disorders (3% of congenital heart disease
rates and a higher utilization of healthcare resources, com- patients) are caused by gene deletions, duplications, or
pared with younger patients.9 In adults, VSD and ASD are mutations. These disorders follow autosomal dominant,
the most common defects (each of them approximately autosomal recessive, or X-linked inheritance patterns.
20% of all defects), followed by PDA and pulmonary valve Some examples are Holt–Oram syndrome, atrial septal
stenosis.4 defect with conduction abnormalities, and supravalvular
Congenital heart defects are more common in twins aortic stenosis. Recurrence risk is high in first-degree rela-
than in singletons, and the increased occurrence is not tives of patients with these disorders.
restricted to monochorionic twins. Thus, intrauterine sur- Polygenic disorders result from environmental and
veillance and a post-natal comprehensive cardiac assess- genetic factors.
ment for both twins may be considered, regardless of The majority of cases (80%) occur as sporadic events
chorionicity and zygosity.10 Survival after operation is bet- but, in some, multiple family members are affected. In up
ter in patients without heterotaxy, i.e. randomized variation to 31% of families in which multiple relatives are affected
in the left-right asymmetry of visceral organs that differs by CHD, a genetic basis can be identified, and rapid
from complete situs solitus and situs inversus, probably due screening for disease-related genes can be facilitated using
to ciliary dysfunction that is associated with heterotaxy.11 advanced sequencing technologies.15
4 ADULT CONGENITAL HEART DISEASE: GENERAL PRINCIPLES

Recurrence rate abnormalities are associated with a high recurrence rate.

For isolated cases of congenital heart disease without a
The recurrence rate of congenital heart disease in offspring family history of CHD (e.g. sporadic defects), the recur-
ranges from 3% to 50% and is higher when the mother, rence risk of congenital heart disease in the offspring is
rather than the father, has congenital heart disease. 3-8%.8 The risk is higher if the mother is the affected par-
Diseases with a single-gene disorder and/or chromosomal ent or if more than one sibling is affected. In autosomal
dominant syndromes, such as Marfan’s, Noonan’s, and
Holt–Oram, there is a 50% risk of recurrence. Genetic
Table 1.1 Adult patients with congenital heart disease counselling should be offered to all patients with congeni-
Complex conditions tal heart disease, with referral for genetic testing in spe-
Eisenmenger syndrome
cific situations.
Double-outlet ventricle
Fontan procedure Clinical problems in ACHD
Mitral atresia
Patients with complex lesions and/or complications should
Pulmonary atresia
be managed in experienced ACHD centres.16–19
Pulmonary vascular obstructive diseases
Peripheral cyanosis may be due to peripheral vasocon-
Single ventricle (double inlet or outlet, common or primitive) striction, polycythaemia, or poor cardiac output.
Transposition of the great arteries Central cyanosis (arterial saturation <85% or >5g reduced
Tricuspid atresia haemoglobin) may be due to right-to-left shunting or
reduced pulmonary flow. Differential cyanosis may be
Truncus arteriosus/hemitruncus
seen with PDA and pulmonary hypertension or inter-
Other rare complex conditions include abnormalities of atrioventricular
rupted aortic arch. In cyanosis from pulmonary causes,
or ventriculoarterial connection, such as criss-cross heart, isomerism,
heterotaxy syndromes, and ventricular inversion. there is an increase of PO2 to, at least, >21 kPa (160 mmHg)
after breathing 100% O2 for 5 min.
Moderate conditions
In patients with ACHD, cyanosis and chronic hypox-
Anomalous pulmonary venous drainage (partial or total) aemia lead to marked erythrocytosis and, frequently, to
Aortic valve disease (valvar, supravalvar, subvalvar)
low platelet counts (<100 000), which may predispose
Atrioventricular septal defects
Coarctation of the aorta to bleeding. The absence of erythrocytosis (e.g. hae-
Coronary fistulae moglobin >17.0 g/dL) in such patients suggests a ‘rela-
Ebstein’s anomaly tive anaemia’. Phlebotomy should be undertaken with
Mitral valve disease haemoglobin >20 g/dL and Hct >65%, associated with
Patent ductus arteriosus headache, increasing fatigue, or other symptoms of
Pulmonary valve disease (valvar, supravalvar, subvalvar)
hyperviscosity in the absence of dehydration or anae-
Pulmonary arteriovenous malformations
Sinus of Valsalva fistula/aneurysm
mia (ACC/AHA 2008 GL on ACHD, Class I-C), under
Tetralogy of Fallot careful volume replacement with normal saline. Multiple
Ventricular septal defects phlebotomies result in iron deficiency that is associated
Simple conditions with impaired small-vessel blood flow and an increase in
the risk of reversible ischaemic neurological deficits and
Isolated aortic valve disease
stroke. The use of anticoagulation and antiplatelet agents
Isolated mitral valve disease (not parachute valve or cleft leaflet)
Small patent ductus arteriosus is controversial and should be reserved for well-defined
Mild pulmonary stenosis indications.
Small ASD Digital clubbing Apart from ACHD, it may be seen in
Small VSD pulmonary malignancy, chronic infection, and primary
1. Conditions may start acyanotic and become cyanotic with time: Fallot’s hypertrophic osteoarthropathy.
tetralogy, Ebstein’s anomaly, and left-to-right shunts, resulting in Renal function Sclerotic renal glomeruli leading to
Eisenmenger syndrome. increased creatinine levels, proteinuria, and hyperuricaemia.
2. Cardiac dextroversion with situs solitus (i.e. normal position of
viscera—gastric bubble on the left) is associated with congenital defects (TGA
Gallstones Increased breakdown of red cells results in
mainly, VSD, PS, tricuspid atresia) in 90% of cases. Dextrocardia with situs increased risk of calcium bilirubinate gallstones, especially
inversus (gastric bubble on the right) carries a low incidence of congenital in the cyanotic and Fontan populations.1
heart disease, whereas situs inversus with levocardia is invariably associated
Hypertrophic osteoarthropathy with thickened peri-
with complex congenital abnormalities.
The changing profile of congenital heart disease in adult life. J Am Coll osteum and scoliosis that may compromise pulmonary
Cardiol. 2001;37:1170–5 with permission from Elsevier. function.
 IMAGING TECHNIQUES AND INVESTIGATIONS 5

Cerebrovascular events (embolic or haemorrhagic), Pulmonary vascular (arterial) resistance (PVR) = (PA
brain abscess, cognitive and psychological problems are pressure–wedge pressure)/cardiac output (normal
also common. range: 0.25–1.5 Wood units (mmHg/L/min) or 20–120
Arrhythmias arise from the abnormal myocardial sub- dynes/cm5)
strate due to variable pressure/volume loads and/or scars Systemic vascular (arterial) resistance (SVR) = (Ao
following cardiac surgery (see also Chapter 51 and Chapter pressure–RA pressure)/cardiac output (normal
56).20,21 Malignant arrhythmias typically become manifest range: 9–20 Wood units (mmHg/L/min) or 700–1600
the third decade of life.21 Atrial fibrillation is usually a late dynes/cm5)
finding, and restoration of sinus rhythm may be difficult. If PVR is greater than two-thirds of SVR, vasodilat-
Atrial tachycardia (usually macroreentrant) is often seen ing challenge, either acute in the catheter laboratory or
in tetralogy of Fallot and following Fontan, Mustard, and chronic, with oxygen, nitric oxide, adenosine, epopros-
Senning procedures. These arrhythmias can be treated tenol, calcium channel blockers, endothelin antagonists,
with catheter ablation, usually assisted by electroanatomic and phosphodiesterase inhibitors, is indicated to inves-
mapping. Atrioventricular reentrant tachycardia (acces- tigate the responsiveness of the pulmonary vascular bed.
sory pathways) in Ebstein’s anomaly and corrected trans- With fixed values, irreversible damage and Eisenmenger
position. Ventricular tachycardia in conditions with the syndrome have developed.
greatest known risk of late sudden cardiac death, such as Pulmonary flow/systemic flow (Qp/Qs)—usually
tetralogy of Fallot, d- or l-transposition, aortic stenosis, derived by echocardiography.
and univentricular hearts.16,22 Sick sinus syndrome in ASD, According to the Fick method, Qp/Qs is calculated by
post-operative Fontan, Mustard, Senning. AV block in oximetry as:
ASD, corrected transposition, VSD closure, AVR.
Qp/Qs = (Ao saturation–mixed venous saturation) /
(PV–PA saturation), where
Imaging techniques and Mixed venous saturation = (3 × SVC saturation + IVC
investigations saturation)/4

Two- or three-dimensional echocardiography with If PV saturation is not available, the value of 98 is used
Doppler imaging and cardiac magnetic resonance have instead.
now replaced cardiac catheterization as a diagnostic tool Routine saturation run during catheterization for exclu-
in most patients with ACHD.23 sion of shunt involves blood sampling from: high SVC,
MRI is considered superior to echocardiography for: RA/SVC junction, high RA, mid-RA, low RA, IVC, RV
inflow, RV body, RV outflow, main PA, PV and LA if pos-
◆ Quantification of RV volume and function, and PR sible, LV, and Ao.
◆ Evaluation of the RVOT, RV-PA conduits, and great A step-up of saturation >10% indicates shunt.
vessels
◆ Tissue characterization (fibrosis, fat, iron, etc.).
Coronary angiography
CT is superior to MRI for: Coronary angiography, or computed coronary angiogra-
◆ Collaterals, arteriovenous malformations, and coro- phy in low or intermediate pretest probability,1 is indicated
nary anomalies preoperatively in patients >40 years, post-menopausal
◆ Evaluation of intra- and extra-cardiac masses. women, adults with multiple risk factors for coronary
artery disease, and children with suspicion of congenital
Haemodynamic assessment coronary anomalies.
Haemodynamic measurements of cardiac output and
systemic and pulmonary flow are derived by Doppler Exercise testing
echocardiography that has replaced calculations by The presence of chronotropic incompetence on con-
the Fick method. However, verification of pressures by ventional exercise testing is a predictor of pregnancy
direct measurement at cardiac catheterization is neces- outcome.8 Cardiopulmonary exercise testing provides
sary for therapeutic decision making in the presence of strong prognostic information in adult patients with
pulmonary hypertension (>½ of systemic pressure) and congenital heart disease. Peak oxygen consumption
for angiographic delineation of defects and selection of (max VO2) is one of the best predictors of morbidity
appropriate closure devices. and mortality.24,25
6 ADULT CONGENITAL HEART DISEASE: GENERAL PRINCIPLES

Spirometry Table 1.2 AHA 2015 statement on congenital heart

There is a high prevalence of markedly abnormal forced disease in the older adult
vital capacity (FVC) in patients with ACHD, and reduced Recommendations for Genetic Screening and
FVC is associated with increased mortality.26 Counseling of the Older ACHD Patient
A detailed family history for CHD and other birth defects that I-C
Assessment of arrhythmia spans at least 3 generations to identify familial inheritance.
Parental consanguinity should be documented, along with a
Surveillance for adults with moderate or severe CHD history of miscarriages and stillbirths
should include a 12-lead ECG at least once per year, and Detailed history and physical examination for dysmorphic I-C
periodic Holter monitoring in adults with transposition features, extracardiac malformations, and other organ system
of the great arteries and atrial switch surgery, Fontan pal- involvement,including neuromuscular abnormalities, mental
retardation, psychiatric abnormalities, short stature, visual or
liation, and in patients with tetralogy of Fallot >35 years hearing loss, immune deficiency, endocrine disorders, and other
of age.18 systemic disorders
Electrophysiologic testing is indicated in patients Family member screening through history, physical examination, IIa-C
with unexplained syncope and ‘high-risk’ substrates and/or echocardiographic screening, particularly in patients
associated with primary ventricular arrhythmias or reporting a positive family history This may aid in the detection
of clinically silent defects such as ASDs, small VSDs, BAV, and
poorly tolerated atrial tachyarrhythmias, such as tetral-
right aortic arch in asymptomatic family members.
ogy of Fallot, transposition of the great arteries with atrial
switch surgery, or significant systemic or single ventricu- Recommendations for Genetic Testing
lar dysfunction. Programmed ventricular stimulation Patients with a history of parental consanguinity or a family I-C
may also be useful in risk-stratifying adults with tetral- history of CHD that includes frequent miscarriages or stillbirths
ogy of Fallot who have additional risk factors for sudden Patients with associated clinical features suggestive of an IIa-C
cardiac death (see Chapter 9).20 Prior to cardiac surgery, underlying genetic syndrome, such as facial dysmorphism,
extracardiac malformations, cognitive impairment,
EPS is indicated in the presence of history of syncope, VT, neuropsychiatric disorders, or multisystem involvement (eg,
SVT (not AF), and ventricular pre-excitation. It may also hepatic, renal, hematologic, immunologic, endocrinologic, and
be considered in patients with a history of palpitations sensorineural abnormalities)
or nonsustained atrial or ventricular arrhythmias, and in Patients with certain types of isolated cardiac defects commonly IIa-C
cases known to be at high risk for atrial arrhythmia devel- associated with genetic syndromes even in the absence of
syndromic features. Common examples are screening for
opment.20 A detailed discussion is presented in Chapter 22q11.2 deletion syndrome in patients with interrupted aortic
50 and Chapter 55. arch, truncus arteriosus, TOF, VSD with aortic arch anomaly,
right aortic arch, or discontinuous branch PAs and screening
for 7q11.23 deletion or Williams-Beuren syndrome in patients
Genetic screening with supravalvar AS, coronary stenosis, and supravalvar and
The AHA recommendations are provided in Table 1.2. peripheral pulmonary stenosis.

American Heart Association Council on Clinical Cardiology. Congenital heart

disease in the older adult: a scientific statement from the American Heart
Principles of therapy Association. Circulation. 2015;131:1884–931 with permission from Wolters
Kluwer.
General measures are presented in Table 1.3. Specific man-
agement is discussed in relevant chapters. In neonates who
undergo cardiac surgery, optimal early outcomes are asso- major treatment advances during the past 50 years, surgi-
ciated with delivery at 39–40 weeks’ gestation. Birth during cal correction of nearly all congenital heart defects is now
the early term period of 37–38 weeks’ gestation is associ- possible, and substantial improvements in short-term sur-
ated with worse outcomes after neonatal cardiac surgery.27 vival have been documented for most conditions.29,30
The IMPACT (Improving Pediatric and Adult Congenital
Treatment) database that was launched by the National Arrhythmias
Cardiovascular Data Registry (NCDR) provides measures The most common indications for permanent pacemaker
and predictors of procedural success that may be con- implantation in children, adolescents, and patients with
sidered in evaluating patients with congenital heart dis- congenital heart disease are symptomatic sinus brady-
ease for common interventional procedures. Procedures cardia, the bradycardia–tachycardia syndromes, and
directed at PDA and ASD closure are among the safest and advanced second- or third-degree AV block, either con-
most successful procedures for congenital heart disease in genital or post-surgical.22,31 Indications for pacing are pre-
the US compared with aortic valvuloplasty and coarcta- sented in Chapter 56 and Chapter 66. Indications for CRT
tion angioplasty and/or stenting, which are generally less in patients with congenital heart disease are presented
successful and are associated with greater risk.28 Following in Chapter 32. The management of tachyarrhythmias
 PRINCIPLES OF THERAPY 7

Table 1.3 ESC 2010 GL on ACHD. Risk reduction strategies Table 1.4 ACC/AHA 2008 GL on ACHD
in patients with cyanotic congenital heart disease Recommendations for infective endocarditis (IE)
Prophylactic measures are the mainstay of care to prophylaxis in patients with adult congenital heart
avoid complications. The following exposures/activities disease
should be avoided: Patients must be informed of their potential risk for IE and I-B
• Pregnancy should be provided with the AHA information card with
instructions for prophylaxis.
• Iron deficiency and anaemia (no routine, inappropriate phlebotomies
to maintain predetermined haemoglobin) When patients present with an unexplained febrile illness and I-B
potential IE, blood cultures should be drawn before antibiotic
• Dehydration treatment is initiated to avoid delay in diagnosis due to
‘culture-negative’ IE.
• Infectious disease: annual influenza vaccination, Pneumovax (every
5 years) Transthoracic echocardiography (TTE) when the diagnosis of I-B
native-valve IE is suspected.
• Cigarette smoking, recreational drug abuse, including alcohol
Transoesophageal echocardiography if TTE windows are I-B
• Transvenous PM/ICD leads
inadequate or equivocal, in the presence of a prosthetic valve
• Strenuous exercise or material or surgically constructed shunt, in the presence
of complex congenital cardiovascular anatomy, or to define
• Acute exposure to heat (sauna, hot tub/shower)
possible complications of endocarditis.
Other risk reduction strategies include: Patients with evidence of IE should have early consultation I-C
• Use of an air filter in an intravenous line to prevent air embolism with a surgeon with experience in adult congenital
heart disease (ACHD) because of the potential for rapid
• Consultation of a ACHD cardiologist before administration of any deterioration and concern about possible infection of
agent and performance of any surgical/interventional procedure prosthetic material.
• Prompt therapy of upper respiratory tract infections Antibiotic prophylaxis before dental procedures that involve IIa-B
• Cautious use or avoidance of agents that impair renal function manipulation of gingival tissue or the periapical region of teeth
or perforation of the oral mucosa, in patients with CHD with
• Contraceptive advice the highest risk for adverse outcome from IE:
a. Prosthetic cardiac valve or prosthetic material used for cardiac
ESC Guidelines for the management of grown-up congenital heart disease.
valve repair.
Eur Heart J. 2010;31:2915–57 with permission from Oxford University Press.
b. Previous IE.
c. Unrepaired and palliated cyanotic CHD, including surgically
constructed palliative shunts and conduits.
d. Completely repaired CHD with prosthetic materials, whether
in ACHD is discussed in Chapter 51, Chapter 53, and placed by surgery or by catheter intervention, during the first
Chapter 56. Recommendations for ICD are not, in gen- 6 months after the procedure.
eral, different than that to other patients with cardiac e. Repaired CHD with residual defects at the site or adjacent to
disease. Indications for ICD are discussed in detail in the site of a prosthetic patch or prosthetic device that inhibits
endothelialization.
Chapter 56. Patients with AF should receive anticoagula-
tion regardless of other risk factors (AHA 2015 statement, Antibiotic prophylaxis against IE before vaginal delivery at the IIa-C
time of membrane rupture in select patients with the highest
I-C).1 Recommendations for concomitant arrhythmia risk of adverse outcomes:
surgery are provided in Chapter 51 and Chapter 56. a. Prosthetic cardiac valve or prosthetic material used for cardiac
valve repair.
b. Unrepaired and palliated cyanotic CHD, including surgically
Endocarditis prophylaxis constructed palliative shunts and conduits.
The risk of endocarditis in patients with ACHD is esti- Prophylaxis against IE is not recommended for non-dental III-C
mated to 4.1 first cases/10 000 person-years. The greatest procedures (such as oesophagogastroduodenoscopy or
risk is seen in children with cyanotic congenital heart dis- colonoscopy) in the absence of active infection.
ease, endocardial cushion defects, or left-sided lesions, and ACC/AHA 2008 guidelines for the management of adults with congenital
recent cardiac surgery and young age.32 heart disease. J Am Coll Cardiol. 2008;52:e1–e121 with permission from
Elsevier.
Prophylaxis is now indicated only in high-risk patients
and only before dental procedures that involve manipula-
tion of gingival tissue or the periapical region of teeth or
perforation of the oral mucosa, or before vaginal deliv- Hypertension
ery.16,17 Congenital conditions for which endocarditis In the treatment of the hypertensive patient with cyanotic
prophylaxis is recommended before the aforementioned CHD, ACE inhibitors, angiotensin receptor blockers,
procedures are presented in Table 1.4. A detailed discus- and diuretic agents should be used cautiously, and care
sion and specific recommendations are provided in the should be coordinated at an ACHD center (AHA 2015
chapter on infective endocarditis. statement, I-C).1
8 ADULT CONGENITAL HEART DISEASE: GENERAL PRINCIPLES

Table 1.5 AHA 2015 statement on congenital heart Table 1.6 ACC/AHA 2008 GL on ACHD
disease in the older adult Recommendations for non-cardiac surgery in patients
Recommendations for Heart Failure (HF) in the Adult with adult congenital heart disease (ACHD)
With CHD Preoperative assessment with systemic arterial oximetry, ECG, I-C
Patients with moderate to complex ACHD are at risk for I-C chest X-ray, TTE, and blood tests for full blood count and
development of HF, and early referral to an ACHD center with a coagulation screen.
HF service and electrophysiological service is indicated When possible, the preoperative evaluation and surgery for I-C
The ACHD specialist should lead the direction of care, because I-C ACHD patients should be performed in a regional centre
these patients are not directly comparable to heart failure specializing in congenital cardiology, with experienced surgeons
patients with acquired disease (ischemic and nonischemic) and cardiac anaesthesiologists.

Transplant evaluation, when considered, should include in the I-C High-risk patient should be managed at centres for the care of
risk-benefit assessment not only the mortality or morbidity of ACHD under all circumstances, unless the operative intervention
transplantation but also the presence of antibodies secondary is an absolute emergency. High-risk categories:
to multiple prior surgeries in some patients and the coexistence a. Prior Fontan procedure. I-C
of multisystem dysfunction (ie, renal, hepatic, pulmonary
hypertension) b. Severe pulmonary arterial hypertension. I-C

American Heart Association Council on Clinical Cardiology. Congenital heart

c. Cyanotic CHD. I-C
disease in the older adult: a scientific statement from the American Heart d. Complex CHD with residua, such as heart failure, valve I-C
Association. Circulation. 2015;131:1884–931 with permission from Wolters disease, or the need for anticoagulation.
Kluwer.
e. Patients with CHD and malignant arrhythmias. I-C
Consultation with ACHD experts regarding the assessment I-C
Heart failure of risk for patients with CHD who will undergo non-cardiac
surgery.
Recommendations by AHA are provided in Table 1.5.
Consultation with a cardiac anaesthesiologist for moderate- and I-C
high-risk patients.
Psychosocial issues and sexual
ACC/AHA 2008 guidelines for the management of adults with congenital
dysfunction heart disease. J Am Coll Cardiol. 2008;52:e1–e121 with permission from
Elsevier.
Individual and family psychosocial screening should be
part of the care of ACHD patients.1 Phosphodiesterase
5 inhibitors are safe provided the patient is not taking recent statement, AHA recognized the importance of phys-
nitrates (AHA 2015 statement, I-C). It is reasonable to treat ically active lifestyles to the health and well-being of chil-
dyspareunia in women with nonsystemic estrogen therapy, dren and adults with congenital heart defects.34 There is no
which has not been shown to increase cardiovascular risk evidence regarding whether or not there is a need to restrict
(AHA 2015 statement, IIa-C).1 recreational physical activity among patients with congeni-
tal heart defects, apart from those with rhythm disorders.
Non-cardiac surgery Counselling to encourage daily participation in appropri-
ate physical activity should be a core component of every
Preoperative evaluation and surgery for patients with con-
patient encounter. As a general recommendation, dynamic
genital heart disease should be performed in specializing
exercise is more suitable than static exercise. Conditions
centres with experienced surgeons and cardiac anaesthe-
that are not compatible with competitive sports are:
siologists. The ACC/AHA recommendations are provided
in Table 1.6. ◆ Eisenmenger syndrome
Risk factors of non-cardiac perioperative risk are: ◆ Pulmonary hypertension
◆ Univentricular heart physiology
◆ Cyanosis and/or pulmonary hypertension
◆ Ebstein’s anomaly
◆ LVEF <35% and/or NYHA III or IV
◆ Transposition of great arteries
◆ Prior Fontan procedure
◆ Coronary artery anomalies.
◆ Complex congenital heart disease with heart fail-
ure, severe left-sided obstructive lesions, malignant Maximal exercise testing is contraindicated in all patients
arrhythmias, or the need for anticoagulation. with pulmonary hypertension.

Exercise Long-distance flights

Adults with congenital heart disease have subnormal exer- Cyanotic patients should use only pressurized com-
cise tolerance. However, participation in regular exercise is mercial airplanes and should drink non-alcoholic and
beneficial for fitness and psychological well-being.33 In a non-caffeinated fluids frequently on long-distance flights
 PRINCIPLES OF THERAPY 9

100%

90%

80%
Abortions
70% Miscarriages
Completed pregnancies
60%

50%

40%

30%

20%

10%

0%
D

SD

PS

CC n

an

ic

ll
ge
TO

ra
AO
ei

io
AS

VS

G
TG

VS

ot
nt
AV

st

ve
at

-T

en
an
PA

Fo
Eb

ct

O
nm
Cy
ar
Co

se
Ei
Figure 1.1 Distribution of miscarriages, completed pregnancies (>20 weeks pregnancy duration), and elective abortions
for each congenital heart disease separately and the overall rates (from ESC 2011 guidelines on pregnancy).
ASD, atrial septal defect; AVSD, atrioventricular septal defect; AOS, aortic stenosis; CC-TGA, congenital corrected transposition of the great arteries;
coarctation, aortic coarctation; Ebstein, Ebstein’s anomaly; Eisenmenger, Eisenmenger syndrome; Fontan, patients after Fontan repair; PAVSD, pulmonary
atresia with ventricular septal defects; PS, pulmonary valve stenosis; TGA, complete transposition of the great arteries; TOF, tetralogy of Fallot; VSD, ventricular
septal defect.
ESC Guidelines on the management of cardiovascular diseases during pregnancy. Eur Heart J. 2011;32: 3147–97 with permission from Oxford University Press.

to avoid dehydration. Oxygen therapy, although often Table 1.7 ACC/AHA 2008 GL on ACHD
unnecessary, may be suggested for prolonged travel in Recommendations for pregnancy and contraception
cyanotic patients. Similarly, residence at high altitude is
Consultation with an expert in adult congenital heart disease I-C
detrimental for patients with cyanosis. (ACHD) before patients plan to become pregnant.
Patients with intracardiac right-to-left shunting should have I-C
Pregnancy fastidious care taken of intravenous lines to avoid paradoxical
Uteroplacental Doppler flow parameters are abnormal in air embolus.
pregnant women with CHD and are related to offspring Pre-pregnancy counselling is recommended for women I-B
outcome.35 Generally, pregnancy is not recommended receiving chronic anticoagulation with warfarin.
in Eisenmenger syndrome. In women with congenital Meticulous prophylaxis for deep venous thrombosis, including IIa-C
defects not complicated by Eisenmenger syndrome, signifi- early ambulation and compression stockings, for all patients
with an intracardiac right-to-left shunt. Subcutaneous
cant pulmonary hypertension or Marfan’s syndrome (and heparin or LMWH for prolonged bed rest. Full anticoagulation
Ehlers–Danlos or Loeys–Dietz syndromes) with aortic for high-risk patients.
root >40 mm, pregnancy can be tolerated (Figure 1.1). The The oestrogen-containing oral contraceptive pill is not III-C
most prevalent cardiac complications during pregnancy are recommended in ACHD patients at risk of thromboembolism,
arrhythmias, heart failure, and hypertensive complications. such as those with cyanosis related to an intracardiac shunt,
severe pulmonary arterial hypertension (PAH), or Fontan
Oestrogen-only contraceptives potentially increase the repair.
thrombotic risk and should be avoided. Risk factors are dis-
ACC/AHA 2008 guidelines for the management of adults with congenital
cussed in the chapter on pregnancy (miscellaneous topics).
heart disease. J Am Coll Cardiol. 2008;52:e1–e121 with permission from
The ACC/AHA recommendations, as well as the ESC Elsevier.
guidelines on pregnancy,36 are presented in Tables 1.7
and 1.8.
10 ADULT CONGENITAL HEART DISEASE: GENERAL PRINCIPLES

Table 1.8 ESC 2011 GL on pregnancy

Recommendations for the management of congenital heart disease
Pre-pregnancy relief of stenosis (usually by balloon valvulotomy) in severe PV stenosis (peak Doppler gradient >64 mmHg). I-B
Follow-up should range from twice during pregnancy to monthly. I-C
Symptomatic patients with Ebstein’s anomaly with cyanosis and/or heart failure should be treated before pregnancy or advised against I-C
pregnancy.
Pre-pregnancy pulmonary valve replacement (bioprosthesis) in symptomatic women with marked dilatation of the RV due to severe I-C
pulmonary regurgitation (PR).
Pre-pregnancy pulmonary valve replacement (bioprosthesis) in asymptomatic women with marked dilatation of the RV due to severe PR. IIa-C
All women with a bicuspid aortic valve should undergo imaging of the ascending aorta before pregnancy, and surgery should be IIa-C
considered when the aortic diameter is >50 mm.
Anticoagulation during pregnancy in Fontan patients. IIa-C
Anticoagulation in pulmonary arterial hypertension (PAH) with suspicion of pulmonary embolism as the cause (or partly the cause) of the IIa-C
pulmonary hypertension.
In patients who are already taking drug therapy for pulmonary arterial hypertension before becoming pregnant, continuation should be IIa-C
considered after information about the teratogenic effects.
Women with pulmonary hypertension should be advised against pregnancy. III-C
Women with an oxygen saturation <85% at rest should be advised against pregnancy. III-C
Patients with TGA and a systemic RV with more than moderate impairment of RV function and/or severe TR should be advised against III-C
pregnancy.
Fontan patients with depressed ventricular function and/or moderate to severe atrioventricular valvular regurgitation or with cyanosis or III-C
with protein-losing enteropathy should be advised against pregnancy.

ESC Guidelines on the management of cardiovascular diseases during pregnancy. Eur Heart J. 2011;32: 3147–97 with permission from Oxford University Press.

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10. Herskind AM, et al. Increased prevalence of congeni-
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2. Dolk H, et al. Congenital heart defects in Europe: preva-
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3. Mozaffarian D, et al. American Heart Association
12. Llurba E, et al. Maternal and foetal angiogenic imbalance in
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Heart disease and stroke statistics–2015 update: a report
13. Vinete E, et al. Increased congenital heart defects in chil-
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2015;131:e29–322
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4. Hoffman JI, et al. Prevalence of congenital heart disease.
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Am Heart J. 2004;147:425–39
14. Liu S, et al. Association between maternal chronic condi-
5. Moons P, et al. Delivery of care for adult patients with con-
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15. Gillian M, et al. Targeted next-generation sequencing iden-
6. Verheugt CL, et al. The emerging burden of hospital
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2010;96:872–8
16. Baumgartner H, et al. ESC guidelines for the manage-
7. Marelli AJ, et al. Lifetime prevalence of congenital heart
ment of grown-up congenital heart disease. Eur Heart J.
disease in the general population from 2000 to 2010.
2010;31:2915–57
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17. Warnes CA, et al. ACC/AHA 2008 guidelines for the man-
8. Brickner ME. Cardiovascular management in pregnancy:
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Cardiol. 2008;52:e143–263
9. Tutarel O, et al. Congenital heart disease beyond the age
18. Baumgartner H, et al. Recommendations for organiza-
of 60: emergence of a new population with high resource
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 DEFINITION AND CLASSIFICATION 11

training in the subspecialty of ‘Grown-up Congenital Heart 28. Moore JW et al. Procedural results and safety of common
Disease’ in Europe: a position paper of the Working Group interventional procedures in congenital heart disease:
on Grown-up Congenital Heart Disease of the European Initial report from the national cardiovascular data registry.
Society of Cardiology. Eur Heart J. 2014;35:686–90 J Am Coll Cardiol. 2014;64:2439–51.
19. Mylotte D, et al. Specialized adult congenital heart dis- 29. Raissadati A, et al. Progress in late results among pedi-
ease care: the impact of policy on mortality. Circulation. atric cardiac surgery patients: A population-based
2014;129:1804–12 6-decade study with 98% follow-up. Circulation. 2015;
20. PACES/HRS expert consensus statement on the recognition 131:347–53.
and management of arrhythmias in adult congenital heart 30. Erikssen G, et al. Achievements in congenital heart defect
disease. Heart Rhythm. 2014;11:e102–e165. surgery: A prospective, 40-year study of 7038 patients.
21. Walsh EP. Sudden death in adult congenital heart dis- Circulation. 2015;131:337–46.
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1735–1742. the ACC/AHA/HRS 2008 guidelines for device-based
22. Sherwin ED, et al. Update on interventional electrophysiol- therapy of cardiac rhythm abnormalities. J Am Coll Cardiol.
ogy in congenital heart disease: evolving solutions for com- 2013;61:e6–75
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23. Hundley WG, et al. ACC/ACR/AHA/NASCI/SCMR 2010 congenital heart disease: cumulative incidence and predic-
expert consensus document on cardiovascular magnetic tors. Circulation. 2013;128:1412–19
resonance: a report of the American College of Cardiology 33. Budts W, et al. Physical activity in adolescents and adults
Foundation Task Force on expert consensus documents. with congenital heart defects: individualized exercise pre-
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24. Inuzuka R, et al. Comprehensive use of cardiopulmonary 34. Longmuir PE, et al., on behalf of the American Heart
exercise testing identifies adults with congenital heart Association Atherosclerosis, Hypertension and Obesity in
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Circulation. 2012;125:250–9 in the Young. Promotion of physical activity for children
25. Rhodes J, et al. Exercise testing and training in children with and adults with congenital heart disease: a scientific state-
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26. Alonso-Gonzalez R, et al. Abnormal lung function in adults 2013;127:2147–59
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diac anatomy, and association with survival. Circulation. tion, and pregnancy outcome in women with congenital
2013;127:882–90 heart disease. Circulation. 2013;128:2478–87
27. Costello JM, et al. Gestational age at birth and outcomes 36. Regitz-Zagrosek V, et al. ESC guidelines on the management
after neonatal cardiac surgery: an analysis of the Society of cardiovascular diseases during pregnancy. Eur Heart J.
of Thoracic Surgeons Congenital Heart Surgery Database. 2011;32:3147–97
Circulation. 2014;129:2511–17

Chapter 2

Ventricular septal defects

Definition and classification VSD (70–80%) and are called perimembranous, para-
membranous, or infracristal. Perimembranous defects
The ventricular septum can be divided into two mor- may extend into the adjacent muscular septum and,
phological components, the membranous septum and in this case, are called perimembranous inlet, per-
the muscular septum. The membranous septum is small imembranous muscular, and perimembranous outlet
and located at the base of the heart between the inlet and (Figure 2.1).1,2
outlet components of the muscular septum, behind the The muscular septum can be divided into inlet, tra-
septal leaflet of the tricuspid valve and below the right becular, and infundibular components. Defects in the inlet
and non-coronary cusps of the aortic valve. Defects that muscular septum, i.e. inferoposterior to the membranous
involve the membranous septum are the most common septum, are called inlet VSD (usually part of a complete
12 VENTRICULAR SEPTAL DEFECTS

Pulmonary
Membranous trunk
Infundibular Membranous
Aorta

Muscular
Left
Right atrium
atrium
Left
ventricle
Right
ventricle

Right heart Inlet Left heart

Figure 2.1 Location of VSDs.

Minette MS, Sahn DJ. Ventricular septal defects. Circulation. 2006;114:2190–7 with permission from Wolters Kluwer.

AV canal defect) (5%). A defect in the trabecular septum is subaortic, subpulmonary (Taussig-Bing anomaly), or
is called muscular VSD if the defect is completely rimmed noncommited (remote from the outlets).
by muscle (15–20%). Muscular VSDs may be multiple and
can be acquired after a septal myocardial infarction. The Pathophysiology
infundibular septum separates the right and left ventricular
outflow tracts. Defects in the infundibulum (5%) are called The shunt volume in a VSD depends on the size of the defect
infundibular, subarterial, or supracristal (also referred and the pulmonary vascular resistance. Without pulmonary
to as doubly committed and juxta-arterial), subpulmo- hypertension or obstruction to the right ventricle, the direc-
nary or subaortic, outlet, conal, and conoventricular. tion of shunt is left to right, with decreased LV output and
Perimembranous or infundibular VSDs are often associ- compensatory intravascular volume overload. Thus, pulmo-
ated with progressive AR due to prolapse of an aortic cusp. nary artery, left atrial, and left ventricular volume overload
develop. Moderate or large VSDs result in the transmission
Epidemiology of LV pressure to pulmonary vascular bed with increased
shear forces. This combination of high volume and pressure
VSD is the most common congenital heart defect after the contributes to the development of irreversible pulmonary
bicuspid aortic valve, occurring in 40% of all children with vascular disease.5 VSD is the most common cause of pulmo-
congenital heart disease and with an estimated prevalence nary hypertension. Eventually, the elevated pulmonary vas-
of 5% in newborn babies.2 With paternal VSD, the recur- cular resistance becomes irreversible and leads to reversal of
rence risk in an offspring is 2%. Maternal VSD has a recur- shunt and cyanosis, and Eisenmenger syndrome develops.
rence risk of 6–10%. In the setting of elevated pulmonary vascular resistance or
right ventricular obstruction resulting from muscle bundles
Aetiology or pulmonary stenosis, the shunt volume is limited and may
be right to left, depending on the difference in pressure.
The origins of VSD are not known, and as in most cases of Spontaneous closure Muscular or membranous VSDs
ACHD, they are most probably multifactorial (see Chapter can undergo spontaneous closure, usually in the first years
1). Initial reports about the teratogenic effects of selective of life. Up to 90% of such defects close spontaneously by
serotonin uptake inhibitors in this respect have not been one year of age.6
verified.3 Recently a locus on chromosome 10p15 was asso-
ciated with familial ventricular aneurysms and VSDs,4 and Presentation
mutations in the transcription factors TBX5 and GATA4
have been identified in familial cases of VSD.2 No direct Adults with small defects and normal pulmonary artery pres-
genetic testing at this time for VSD exists. Associated dis- sure are generally asymptomatic. Patients with large defects
orders are tetralogy of Fallot, AV canal, aortic coarctation, who survive to adulthood usually have left ventricular failure
and, rarely, double-outlet right ventricle in which the VSD or pulmonary hypertension with associated RV failure.7
 THERAPY 13

Physical examination Table 2.1 ACC/AHA 2008 GL on ACHD

Recommendations for cardiac catheterization
Physical signs depend on the size of VSD.
Cardiac catheterization to assess the operability of adults with I-C
Holosystolic (pansystolic) murmur, with or without a VSD and PAH should be performed in an ACHD regional centre
thrill, with moderate or large defects. The grade of mur- in collaboration with experts.
mur depends on the velocity of flow. Very small or large In adults with VSD in whom non-invasive data are inconclusive
defects with no shunt and defects with Eisenmenger physi- and further information is needed for management. Data to be
ology and right-to-left shunt may not have a VSD mur- obtained include the following:
mur. Muscular defects can be heard along the lower left a. Quantification of shunting. IIa-B
sternal border and may vary in intensity, as the defect size b. Assessment of pulmonary pressure and resistance in patients IIa-B
changes with muscular contraction throughout systole. with suspected PAH. Reversibility of PAH should be tested
Infundibular defects close to the pulmonary valve can be with various vasodilators.
heard best at the left upper sternal border. c. Evaluation of other lesions, such as AR and IIa-C
Short, mid-diastolic apical rumble (increased mitral double-chambered right ventricle.
flow) may be heard. d. Determination of whether multiple VSDs are present before IIa-C
Decrescendo murmur in the presence of AR. surgery.
Cyanosis with clubbing and peripheral oedema due to e. Performance of coronary arteriography is indicated in patients IIa-C
right-sided heart failure gradually appear. at risk for coronary artery disease.
f. VSD anatomy, especially if device closure is contemplated. IIa-C

ACC/AHA 2008 guidelines for the management of adults with congenital

Investigations heart disease. J Am Coll Cardiol. 2008;52:e1–e121 with permission from
Elsevier.
ECG is normal in small VSD. With large defects, there is
LA and LV hypertrophy. When pulmonary hypertension VSDs) and endocarditis. Endocarditis is a lifelong risk in
develops, there is right axis deviation and RV hypertrophy. unoperated patients, being six times higher than in the nor-
Chest radiography is normal with small VSDs. With mal population, but is primarily associated with the associated
large defects, there is ‘shunt vascularity’, i.e. well-visualized valve disease rather than the VSD itself.9,10 Routine endocar-
small pulmonary arteries in the periphery of both lungs. ditis prophylaxis, however, is not recommended any more
When pulmonary hypertension develops, there is marked for unrepaired VSDs. For closed VSDs, prophylaxis is recom-
enlargement of the proximal pulmonary arteries, rapid mended for 6 months after the procedure.9,10 Patients with
tapering of the peripheral arteries (pruning), and oligae- unrepaired VSDs are advised on dental hygiene and the phy-
mic lung fields. sician should be alert of suspicious symptoms (see Chapter 1
Transthoracic or transoesophageal echocardiography and Chapter 79 on endocarditis). Survival up to 40 years after
with colour flow mapping are used for quantification of the successful surgical VSD closure is slightly lower than in the
shunt, assessment of pulmonary artery pressure, distortion general population, and follow-up for the identification of LV
of the aortic valve, and obstruction of the right ventricular or RV dysfunction may be advisable. Aortic cross-clamp time
outflow tract (double-chamber RV). Three-dimensional and, most probably, post-operative arrhythmia are unfavour-
echocardiography is useful for defects that are difficult to able prognostic signs.11 In adults with inoperable VSDs with
evaluate by two-dimensional imaging. progressive/severe pulmonary vascular disease, pulmonary
Cardiac magnetic resonance is very useful with com- vasodilator therapy may be considered (ACC/AHA 2008 GL
plex associated lesions. on ACHD, Class IIb-B).
Cardiac catheterization is no longer necessary.
However, it can be used to determine Qp/Qs by oximetry, Indications for closure
and pulmonary artery pressure and resistance in case of Indications are presented in Tables 2.2 and 2.3.9,10 Main
anticipated closure (Table 2.1). It can also assess response indications are:
to pulmonary vasodilators that can guide therapy and eval-
uate coexistent AR, dual-chamber RV, or multiple VSDs. ◆ Qp/Qs >1.5
◆ History of endocarditis
Therapy ◆ Progressive AR
◆ LV volume overload.
Medical
Adult patients with small VSD without evidence of left ven- Contraindications for closure
tricular volume overload or AR do not require intervention.8 Irreversible pulmonary arterial hypertension, i.e. PA pres-
These patients, as well as patients who had VSD repair, need sure >2/3 systemic pressure or PVR >2/3 SVR at baseline
surveillance for AR (perimembranous and infundibular or after oxygen or vasodilation.
14 VENTRICULAR SEPTAL DEFECTS

Catheter closure Pregnancy

Currently, defect-specific devices are in the investigational
Contraindicated in Eisenmerger syndrome.8,9 Women
stage and have been used both for congenital and post-MI
with large shunts and pulmonary arterial hypertension
VSD. They may interfere with AV or TV and carry a higher
may have arrhythmias, LV dysfunction, and progression of
risk for AV block than surgical closure. Recent experience
pulmonary hypertension. Combinations of epoprostenol
with closure of perimembranous VSDs indicates a <1%
and sildenafil may improve outcome in pregnant women
risk of complete AV block, which is comparable to that
with severe pulmonary hypertension who choose to con-
after surgical closure.12,13
tinue pregnancy (see also General principles).2 The esti-
mated recurrence rate in the offspring is 6–10%.9
Table 2.2 ACC/AHA 2008 GL on ACHD
References
Recommendations for Ventricular Septal Defect Closure
1. Minette MS, et al. Ventricular septal defects. Circulation.
Surgeons with training and expertise in CHD should perform VSD I-C
2006;114:2190–7
closure operations.
2. Penny DJ, et al. Ventricular septal defect. Lancet.
Closure of VSD when there is a Qp/Qs ≥2.0 and clinical evidence I-B 2011;377:1103–12
of LV volume overload.
3. Huybrechts KF, et al. Antidepressant use in preg-
Closure of VSD with a history of endocarditis. I-C nancy and the risk of cardiac defects. N Engl J Med.
Closure of VSD with net left-to-right shunting and PA pressure IIa-B 2014;370:2397–407
<2/3 systemic pressure and PVR <2/3 SVR. 4. Tremblay N, et al. Familial ventricular aneurysms and
septal defects map to chromosome 10p15. Eur Heart J.
Closure of VSD with net left-to-right shunting and Qp/Qs >1.5 IIa-B
and LV systolic or diastolic failure. 2011;32:568–73
5. Sommer RJ, et al. Pathophysiology of congenital heart
Device closure of a muscular VSD may be considered, especially IIb-C
disease in the adult: part I: shunt lesions. Circulation.
if the VSD is remote from the tricuspid valve and the aorta,
if the VSD is associated with severe left-sided heart chamber 2008;117:1090–9
enlargement, or if there is PAH. 6. Hoffman JI, et al. The incidence of congenital heart disease.
J Am Coll Cardiol. 2002;39:1890–1900
VSD closure in severe irreversible PAH. III-B
7. Brickner ME, et al. Congenital heart disease in adults.
ACC/AHA 2008 guidelines for the management of adults with congenital Second of two parts. N Engl J Med. 2000;342:334–42
heart disease. J Am Coll Cardiol. 2008;52:e1–e121 with permission from 8. Gabriel HM, et al. Long-term outcome of patients with
Elsevier. ventricular septal defect considered not to require sur-
gical closure during childhood. J Am Coll Cardiol.
2002;39:1066–71
Table 2.3 ESC 2010 GL on ACHD 9. Baumgartner H, et al. ESC guidelines for the manage-
Indications for intervention in VSD ment of grown-up congenital heart disease. Eur Heart J.
2010;31:2915–57
Surgical VSD closure in patients with symptoms that can be I-C
10. Warnes CA, et al. ACC/AHA 2008 guidelines for the man-
attributed to L–R shunting through the (residual) VSD and who
have no severe pulmonary vascular disease. agement of adults with congenital heart disease. J Am Coll
Cardiol. 2008;52:e143–263
Surgical VSD closure in asymptomatic patients with evidence of I-C 11. Menting ME, et al. The unnatural history of the ventricular
LV volume overload attributable to the VSD.
septal defect: outcome up to 40 years after surgical closure.
Surgical VSD closure in patients with a history of IE. IIa-C J Am Coll Cardiol. 2015;65:1941
Surgery for patients with VSD-associated prolapse of an aortic IIa-C 12. Yang J, et al. Transcatheter device closure of perimembra-
valve cusp, causing progressive AR. nous ventricular septal defects: mid-term outcomes. Eur
Surgery for patients with VSD and PAH when there is still net IIa-C Heart J. 2010;31:2238–45
L–R shunt (Qp/Qs >1.5) present and PAP or PVR are <2/3 of 13. Zhang GC, et al. Transthoracic echocardiographic guidance
systemic values (baseline or when challenged with vasodilators, of minimally invasive perventricular device closure of per-
preferably nitric oxide, or after targeted PAH therapy). imembranous ventricular septal defect without cardiopul-
Surgery in Eisenmenger VSD and when exercise-induced. III-C monary bypass: initial experience. Eur Heart J Cardiovasc
Imaging. 2012;13:739–44
Surgery if the VSD is small, not subarterial, does not lead to LV III-C
volume overload or pulmonary hypertension, and if there is no
history of IE.

ESC Guidelines for the management of grown-up congenital heart disease.

Eur Heart J. 2010;31:2915–57 with permission from Oxford University Press.
 DEFINITIONS AND CLASSIFICATION OF ATRIOVENTRICULAR SEPTAL DEFECTS 15

Chapter 3

Atrioventricular septal defects

Definitions and classification Ostium secundum defect involves the region of the
fossa ovalis (80%).
of atrioventricular septal defects Sinus venosus defect at the junction of the right atrium
The primordial single atrium divides into right and left and superior vena cava (5%).
sides by formation and fusion of the septum primum and Coronary sinus septal defect (‘unroofed’ coronary
septum secundum. The septum primum grows from the sinus) and inferior sinus venosus defect (at the junc-
primordial atrial roof toward the endocardial cushions, tion of the right atrium and inferior vena cava): very rare
and the septum secundum grows from the ventrocranial (<1%).
atrial wall on the right side of the septum primum. Patent foramen ovale is the incomplete septal partition
Atrioventricular septal defects (AV canal or endocar- (usually an oval-shaped window) at the point where the
dial cushion defects) are complete (large VSD, common septum secundum overlaps perforations of the septum pri-
AV junction, and common AV valve with five leaflets) or mum (i.e. the foramen secundum).
partial (ostium primum ASD with a common AV junc- Complete AV canals rarely reach adulthood with-
tion, but two separate AV valves) (Figure 3.1).1,2,3 out Eisenmenger syndrome. Partial AV canal defects
Ostium primum defect at the lower part of the atrial (including ostium primum ASD) are not uncommon in
septum is a partial atrioventricular septal defect and may, adults.
or may not, have a VSD component (15% of ASDs).

A B

Normal

SVC SVC

Complete
E AVSD
B
D
F
C

IVC IVC
Partial
AVSD

Figure 3.1 Anatomy of ASDs. A: superior sinus venosus ASD; B: secundum ASD; C: inferior sinus venosus ASD; D: ostium
primum ASD or partial AV septal defect; E, secundum ASD without posterior septal rim; F: coronary sinus ASD.
Webb G, Gatzoulis MA. Atrial septal defects in the adult: recent progress and overview. Circulation. 2006;114:1645–53 with permission from Wolters Kluwer.
Other documents randomly have
different content
Leipzig. — S. Anhang, A, Nr. 33. 44) Originalstimmen auf der
Bibliothek der Thomasschule. Eine Abschrift Penzels von 1757 auf
der künigl. Bibliothek zu Berlin. — S. Anhang B, Nr. 38. 45)
Originalstimmen auf der Bibliothek der Thomasschule. — S. Anhang
A, Nr. 36. — Diese Cantate hängt ebenso wie »Lobe den Herren
meine Seele"
The text on this page is estimated to be only 29.62%
accurate

— . 287 — Flemmings bekanntes Reiselied » In allen

meinen Thaten « aus dem Jahre 1633 bearbeitete Bach 1734, man
weiß nicht für welche Gelegenheit. 4C) Die Compositionen von
Martin Rinckarts »Nun danket alle Gott« (1644) und Jacob Schützens
»Sei Lob und Ehr dem höchsten Gut« (1673) entbehren ebenfalls
der Angabe eines besondern Zweckes. Es läßt sich auch über das
Jahr ihrer Entstehung nur sagen, daß dasselbe ungefähr in den
Zeitabschnitt fallen muß, mit welchem wir uns hier beschäftigen,
während Johann Heermanns »Was willst du dich betrüben« (um 1
630) und Rodigasts »Was Gott thut, das ist wohlgethan« (1675)
einer etwas späteren Zeit anzugehören scheinen47) Allen diesen
Cantaten ist es gemeinsam , daß sie mit einem großen Ghoralchor
beginnen , der in der Form der sogenannten Choralfantasie gehalten
ist. Bei »Nun danket alle Gott« hat diese Form nur insofern eine
Abwandlung erfahren, als frei erfundene Chorsätze die Durchführung
des Chorals einleiten, unterbrechen und beschließen. In der Cantate
»In allen meinen Thaten« stellt sich die Choralfantasie als
französische Ouvertüre dar, in deren fugirtes Allegro der Chor mit
bewunderungswürdiger Kunst hineingebaut ist. Vernehmlich mahnt
dieses Stück an den Anfangschor der Cantate »0 Ewigkeit, du
Donnerwort«, welchen wir indessen in eine frühere Periode verlegen
zu müssen glaubten. Mannigfaltigere Gestaltung weisen die
Schlußchoräle auf. Bald erscheinen sie als einfach vierstimmige
Tonsätze, bald, wie in »Lobe den Herren« und »In allen meinen
Thaten« durch drei selbständige instrumentale Oberstimmen
prachtvoll erweitert ; dann wieder in Choralfantasieform , oder auch
, wie in »Gelobet sei der Herr« und »Was Gott thut, das ist
wohlgethan« in einer an Böhm erinnernden Behandlungsart , indem
eine gewisse dem Orchester zuertheilte Phrase zwischen den Zeilen
und wo(s. S. 236 f. dieses Bandes) augenscheinlich mit dein
Rathswechsel zusammen. Der 12. Trinitatis-Sonntag war 1732 der
erste Sonntag nach Barttioloinäi, mit welchem Tage der
Rathswechsel einzutreten pflegte; der Kathswahlgottesdienst selbst
fand am 25. August statt. 46) B.-G. XXII, Nr. 97. - S. Anhang A, Nr.
39. 47) »Sei Lob und Ehr« B.-G. XXIV. Nr. 117. »Was willst du dich
betrüben« XXIII, Nr. 107. »Was Gott thut, das ist wohlgethan« XXII,
Nr. 100. — »Nun danket alle Gott« ist in, leider unvollständigen,
Originalstimmen auf der königl. Bibliothek zu Berlin. — S. Anhang A,
Nr. 33.
The text on this page is estimated to be only 27.31%
accurate

— 288 — lieh auch neben denselben unablässig

wiederkehrt. Außer im Anfangs- und Schlußsatze wird aber die
Melodie des Kirchenliedes vollständig nur noch zweimal, nämlich zur
zweiten und vierten Strophe von »Lobe den Herren« durchgeführt;
in letzterer ist sie einer concertirenden Trompete zuertheilt. In der
Mehrzahl der Fälle wird weiter garkeine Rücksicht auf sie genommen
; so durchweg in den Cantaten »Der Herr ist mein getreuer Hirt«,
»Ich ruf zu dir«, »In allen meinen Thaten « ; hier dienen die übrigen
Strophen nur als Texte zu frei erfundenen Arien , Duetten und
Recitativen. Manchmal aber treibt auch der Componist mit der
Melodie ein launiges Spiel, indem er sie bald stärker bald schwächer
anklingen läßt. Hiermit entwickelt Bach eine neue Seite seiner
Kirchenmusik ; bisher war uns derartiges nur in der Cantate »Wer
nur den lieben Gott« fluchtig begegnet und ist dort in seiner
ästhetischen Bedeutung gewürdigt worden. 4S) Vom musikalischen
Standpunkte betrachtet bieten die Suiten etwas ähnliches , in denen
der Anfang der übrigen Tänze jedesmal wieder an den Anfang der
Allemande anknüpft. 49) Die Anspielungen beziehen sich gewöhnlich
auf die erste, oder die ersten beiden Choralzeilen, Z.B.
Choralmelodie : **££ B E£ =&=t ■ß- ■#■ß P Ge - lo - bet sei der
Herr, mein Gott, mein Licht, mein Le - ben. c * Dritte Arie der
Cantate : ffef^g^^^^^^^^fg^i Ge - lo - bet sei der Herr, mein
Gott, der e - wig le - bet. Choralmelodie : ffif — g Was Gott thut, das
ist wohl-ge-than. 48) Der Anklang an den Choral »Nun danket alle
Gott« in der Rathswahl? musik »Preise Jerusalem den Herrn« (s S.
194 dieses Bandes) kann hier nicht in Betracht kommen, da jener
Choral in der Cantate sonst nirgends vorkommt. Die Nachbildung hat
dort nur eine poetisch-symbolische Bedeutung. 49) S. Band I, S.
C93.
The text on this page is estimated to be only 20.90%
accurate

— 289 Vierter Vers der Cantaie : m =f i ß—0-M—ggb Was

Gott thut, das ist wohl-ge - than. In der Cantate »Lobe den Herren«
wird einmal die Melodie *=fc lÜ^P^g £ igül Lo-be den Her-ren, den
mäch -ti- gen Kö-nig der nach Moll versetzt und als Duett bearbeitet,
nämlich : Eh - ren ^ :t: £: i^tzl :r£: ^=m^m^ Lo-be den Her-ren,
der künst-lich und fein dicli be - rei - tet. Im fünften Vers von » Sei
Lob und Ehr « . der als Recitativ beginnt, stößt man plötzlich auf
eine Phrase, welche an die vier ersten Töne der Choralmelodie : ;p:
feÖEf Sei Lob und Ehr dem hb'ch-sten Gut anknüpft; der Bass imitirt
und es entwickelt sich ein Spiel, das lebhaft an eine Choralarbeit aus
Bachs Jugendzeit erinnert. 50) Auer auch andre als die
Anfangszeilen werden zuweilen flüchtig und wie im Vorübergehen
gestreift ; in dieser Beziehung ist namentlich Vers 4 von »Was Gott
thut , das ist wohlgethan« merkwürdig. Einmal , in Vers 5 von » Was
willst du dich betrüben« . der mit einer geistreichen Fantasie über
die ersten Zeilen beginnt und sich dann ungebunden als Arie weiter
entwickelt , ereignet es sich , daß am Schluß die letzte Choral-Zeile
ganz schmucklos und einfach eintritt, gleichsam als sei die Phantasie
des Componisten zu ihrer Quelle zurück gekehrt. Auch der zweite
Vers von »Nun danket alle Gott« wird mit freier Benutzung der
Choralmelodie gebildet , so daß diese nun in allen Theilen der
mächtigen und äußerst brillanten Compositum theils voll und klar,
theils verhüllt dem Hörer entgegentritt. Daß eine solche Behandlung
des Kirchenliedes und derChoralmelodie ihr bedenkliches hat .
konnte indessen dem Meister auf die Dauer nicht verborgen bleiben.
Wenn er auch durch mehre Jahre 50) S. Band I, S. 20S. Si'iTTA, J. S.
Bach. II. 19
The text on this page is estimated to be only 20.30%
accurate

— 290 — von Zeit zu Zeit wieder auf sie zurück kam. so

ergiebt ein Blick auf seine spatere Thätigkeit als Cantatenconiponist
doch, dafi wir in den eben beschriebenen Cantaten Übergangs- oder
abschweifende Bildungen zu erkennen haben, jenseits deren eine
vollkommenere Form lag. Schon während der ersten Leipziger
Periode erscheinen einige Werke, in denen diejenige Form, welche
flir Bach in seiner letzten Lebensperiode Ideal und Typus der Kirch
encantate wurde, mit voller Klarheit zu Tage kommt. Es tritt in ihr
ebenfalls ein bestimmtes Kirchenlied mit der zugehörigen Melodie in
den Mittelpunkt, aber weder wird der kirchliche Text für Arien und
Recitative verbraucht, noch die kirchliche Melodie phantastischen
Spielen preisgegeben. Vielmehr dienen der persönlicheren, durch
das kirchliche angeregten Empfindung nun wieder besondere, aber
aus dem Kirchenlied entwickelte Dichtungen und freie
Comyjositiouen : dem Choral wird sein unnahbares,
unveränderliches Wesen gewahrt, und doch durch dringt er, auch wo
weder Originaltext noch Originalniclodie gehört werden, als
einheitgebende Macht das Ganze. Sehr nahe dieser Idealform steht
die bewunderungswürdige Composition . mit der Bach den 27.
Trinitatis Sonntag des Jahres 1731 (25. Nov.j festlich beging. Der
Sonntag kommt bekanntlich im Kirchenjahre sehr selten vor: deshalb
und wegen seines hochpoetisehen. geheimnisvoll feierlichen
Evangeliums sah sich Bach wohl veranlaßt, ihn durch eine
Tonschöpfung ersten Kanges zu schmücken. Sinnvoll ist Nicolais
dreistrophiges Kirchenlied »Wachet auf. ruft uns die Stimme«,
welches an das Evangelium von den zehn Jungfrauen (Matth. 25, 1
— 13) direct anknüpft, um dann in die Anschauungen des
Hohenliedes und der Offenbarung Johannis Cap. 21 hinüber zu
leiten, zur Grundlage des Werkes gewählt worden51 Zwischen die
Strophen schieben sich Recitative und Zwiegesänge Christi und
seiner Braut, kunstvolle Duette, die eine keusche Innigkeit athmen
ohne sich zu wreit ins Gebiet persönlicher Leidenschaftlichkeit zu
verlieren. Die drei Choralstrophen bilden genau Anfang. Mitte und
Ende, und stellen den mystischen Grundton des Werkes her, welchen
die Vorstellungen von der feierlichen Stille der Nacht. 51) Die
Cantate, deren Originalstinimen die Bibliothek der Thomasschule
aufbewahrt, ist jetzt nur bei Winterfeld, Evang. Kirchenges. III,
Beilage S. 172 ff. veröffentlicht. — Über das Entstehuogsjahr s.
Anhang A. Nr. 33.
The text on this page is estimated to be only 26.58%
accurate

— 291 — . in welcher der himmlische Bräutigam erwartet

wird, und der unsäglichen Freuden in der Herrlichkeit des neuen
Jerusalem bedingten. Die erste Strophe ist Choralfantasie : in die
majestätischen Rhythmen des Orchesters mischt das mit dem
fünften Takte auftretende Motiv 7hRt — ^SFJ1-1^-*1--*—^^
^zqzip ^ em heimliches Glück, das manchmal in seligem Ausdrucke
überströmt: der Sopran führt die Melodie, deren poetischen Gehalt
die andern Singstimmen durch Tonreihen von außerordentlicher
Plastik ausdeuten. In der zweiten Strophe, einem Trio für Tenor,
Geigen und Bass, klingt der mystische Ton wohl am vollsten aus. Es
ist wie ein Reigen seliger Geister , was sich hier in den tiefen Lagen
sämmtlicher Geigen mit seltsamem, unerhörtem Ausdruck hin und
her wiegt : Zion und die Gläubigen sind mit Christus eingegangen
zum Fest in den Freudensaal. Die letzte Strophe, ein »Gloria, mit
Menschen und Engel-Zungen« angestimmt, tritt in schmuckloser
Einfachheit auf; die herrliche Melodie erhält hier noch einmal
Gelegenheit , vorzugsweise durch sich selbst zu wirken. Die Cantate
»Was Gott thut, das ist wohlgethan«, von der oben die Rede war, ist
in ihrem Anfangs- und Schlußsatze nur eine erweiterte und
bereicherte Bearbeitung älterer Stücke. Der Schlußsatz findet sich
schon in der Cantate von 1723 »Die Elenden sollen essen « 52j . Der
Anfangssatz in einfacherer Fassung leitet ein Werk ein, welches um
zwei oder drei Jahre früher, also gegen 1733 geschrieben sein mag.
Es repräsentirt den Typus der Cantate »Wachet auf« in etwas
vereinfachter Gestalt , in dem nur zwei Strophen des Chorals benutzt
sind : sie haben am Anfang und Ende ihren Platz und dieselben
Formen, die wir dort an diesen Stellen finden; zwischen ihnen steht
eine Anzahl madrigalischer Gesangstücke. 53j Eine dritte mit
demselben Choral anhebende Composition sollte dem 21 . Trinitatis-
Sonntage dienen, und wird 1731 (21. October) oder 1732 (2.
November) die erste Aufführung erlebt haben. 54) Sie enthält gar
nur eine Strophe des Chorals , welche am Anfange steht und auch
die Form der Choralfantasie aufweist ; doch will das instrumentale
52) S. S. 185 dieses Bandes. 53) B.-G. XXII,. Nr. 99. 54 B.-G. XXII,
Nr. 98. — S. Anhang A, Nr. 38. 19*
 — 292 — Tonbild sich anfänglich nicht recht zur Einheit
zusammenfügen und nähert sich während des Aufgesanges mehr
dem Charakter eines Ritornells. Im Verlauf des Werkes wird die
Choralmelodie nirgends weiter verwendet, nicht einmal am Schlüsse,
die Cantate läuft in eine Bassarie aus. Sie ist denmach formell
unvollkommen, mehr nur ein Ansatz, dem die entsprechende
WeiterfUhrung fehlt; auch der musikalische Gehalt der einzelnen
Stücke steht hinter dem der andern beiden Cantaten zurück.
Hingegen gewährt eine Cantate auf den sechsten Trinitatis-Sonntag
» Es ist das Heil uns kommen her « durch ihre meisterwürdige
Formrundung wieder vollste Befriedigung. Wenn Ähnlichkeiten in der
Gestaltung des Einzelnen gleiche Entstehungszeit voraussetzen
lassen — und dieses ist ja bei Bach der Fall — so muß sie mit der
Cantate » Wachet auf« in demselben Jahre (1731) geschrieben sein.
Von dem Liede des Paul Speratus wird nur die erste und zwölfte
Strophe verwendet , um Ausgangs- und Endpunkt des Werkes
festzustellen, zwischen welchen neben andern madrigalischen
Stücken ein bewundernswerthes canonisches Duett seinen Platz
findet; die Behandlung der ersten Strophe aber gleicht derjenigen
der ersten Strophe von »Wachet auf« in auffälligem Grade,
namentlich in den zweistimmigen Imitationen und theilweise dem
begleitenden Rhythmus des instrumentalen Tonsatzes. 55) Einer
neuen, tiefsinnigen Composition zum 16. Trinitatis-Sonntage
(wahrscheinlich 28. September 1732) »Christus der ist mein Leben«
muß an dieser Stelle Erwähnung geschehen, obwohl sie ihrer Form
nach nur halb hierher paßt. Sie beginnt mit einem Choralchor über
das genannte Lied , verwerthet außerdem aber noch drei andre
Choräle, welche sämmtlich zu den schönsten und bekanntesten
Sterbeliedern der protestantischen Kirche gehören. Hierdurch
gelangt die poetisch -musikalische Grundempfindung der Cantate
freilich zu einer mächtigen Intensität. Doch läßt sich ein Mangel an
Einheitlichkeit nicht verkennen. Die Bedeutung des Chorals in den
Bachschen Cantaten ist eine andre und größere, als nur die welche
einem gehaltvollen Gedichte mit einer schönen Melodie zukommt ;
er soll unter den subjectiveren Arien und Recitativen den
gemeinverständlichen Mittelpunkt bilden. Berechtigung hat es noch
wenn am Schlüsseein andrer als der einleitende Choral erscheint, in
einem solchen 55} B.-G. I, Nr. 9. — S. Anhang A, Nr. 33.
 — 293 — Falle entwickelt sich die Empfindung von einem
strengkirchlicheu Ausgangspunkte derart, daß sie an einer andern
Stelle in das engere kirchliche Gebiet wiedei einströmt. Statt dieses
einen Ausgangspunktes aber deren zwei oder drei zu nehmen, muß
beunruhigend wirken. Wir wissen, wie sehr Bach es liebte in der
Stimmung von Sterben und Tod zu schwelgen. Er hat dieser Vorliebe
hier einmal die Zügel schießen lassen; ist er doch soweit gegangen
im ersten Choralchor dem Worte »Sterben« zu Gefallen durch eine
lange Dehnung die Proportionen der vier Choralzeilen gänzlich zu
zerstören. Sehr fein und sinnig ist es gemacht , wie dieser Chor sich
ohne musikalische Unterbrechung in den Sologesang hinein fortsetzt,
erst ein Arioso, dann ein Recitativ , an welches sich nun unmittelbar
wieder ein neuer 5 in einfacher Pachelbelscher Form gehaltener
Choralchor »Mit Fried und Freud ich fahr dahin« anschließt. Der
dritte Choral »Valet will ich dir geben« ist als Trio behandelt und
kann seine Verwandtschaft mit dem Choraltrio aus » Wachet auf«
nicht verbergen. Am Schlüsse steht die vierte Strophe des Liedes
»Wrenn mein Stündlein vornanaen ist. « 56) Wenn die Form der
chorischen Choralfantasie für den ersten Satz von nun an mehr und
mehr das Feststehende wird , so ist doch einstweilen der Drang nach
Mannigfaltigkeit bei Bach immer noch so stark, daß er auch andere
Gebilde an die erste Stelle rückt. Solches geschieht in einer Musik
zum Sonntag nach Neujahr 1 733 (4. Januar) . Hier ist der zum
Tonbilde des Orchesters sich gesellende Gesang nur zweistimmig:
der Sopran singt die erste Strophe des Chorals »Ach Gott, wie
manches Herzeleid«, der Bass stellt sich ihm mit einem besondern
Charakter entgegen und ermahnt zur Geduld und Standhaftigkeit.
Derselbe Gegensatz ist auch im Schlußsatze musikalisch verwerthet,
übrigens keiner Choralfantasie sondern einer Bassarie, in die der
vom Sopran vorgetragene Choral hineingeflochten ist. Den Text zu
diesem Choral bildet die zweite Strophe des Martin Böhmschen
Liedes »0 Jesu Christ, meins Lebens Licht«, während die Melodie
dieselbe ist wie am Anfang. Von dem klagenden und dem tröstenden
Charakter, die in dieser Cantate, wenn.auch nur in den Hauptsätzen
derselben durchgeführt sind, hat das Werk 56) B.-G. XXII, Nr, 95. —
Aniiang A, Nr. 40.
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— 294 — den Namen Dialogus erhalten57) Es ist ebenfalls

nicht ohne Seitenstlick geblieben. Auch zum 24. Trinitatifl-Sonntage
wahrscheinlich 1732, also 23, November schrieb Bach einen
Dialogus: »Furcht« und »Hoffnung« heißen liier die Charaktere,
ihren Gegensatz ausgleichend tritt schließlich ein Bass hinzu . unter
welchem wir midie »Stimme vom Himmel« vorstellen dürfen, der in
der Offenbarung Johannis (14, 13) die hier gesungenen Worte
zuertheilt werden. Der erste Satz ist dem der vorigen Cantate ganz
analog gestaltet. Der Alt singt die Melodie »0 Ewigkeit, du
Donnerwort«. Zum Beschluß kehrt indessen diese nicht wieder ,
sondern es ertönt Joh. Rudolph Ahles empfindungsvolle Arie »Es ist
genug« in vierstimmigem Tonsatz. 5S) — Wir verlassen hiermit die
Choralcantaten und verweilen abschließend noch mit kurzem Blicke
auf einer Gruppe solcher Kirchenmusiken, welche überwiegend oder
gänzlich auf freier Erfindung beruhen. In der Mehrzahl derselben
bildet ein Bibelspruch den poetischen Grundgedanken, der nach dem
allgemein üblichen und auch von Bach durch die That als begründet
anerkannten Verfahren gewöhnlich in eine Chorform gekleidet ist.
Zwei Werke ragen unter ihnen hervor. Eine Cantate zum zehnten
Trinitatis-Sonntag (wahrscheinlich 29. Juli 1731) »Herr deine Augen
sehen nach dem Glauben« gehört zur Gattung derjenigen, welche
ich mit dem Namen orthodoxe Compositionen (s. S. 255) belegen, zu
dürfen glaubte. 5
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— 295 — That plante, könnte er seinen Gehülfen einmal

energisch aufgerüttelt haben. Während er in der Cantate »Schauet
doch und sehet« in unersättlicher Klage schwelgt , gleich als wolle er
dem Prophetenworte Genüge thun »Meine Augen rinnen mit
Wasserbächen über dem Jammer der Tochter meines Volks. Meine
Augen fließen und können nicht ablassen« (Klagelieder Jeremiae 3,
48 und 49), erscheint er hier als glühender, fast fanatischer
Bußprediger. Der erste Chor stellt wie immer die Grundempfindung
fest. Seine Disposition ist meisterhaft und durchaus neu. Ein Vorspiel
legt die Hauptgedanken im Zusammenhange dar, welche sodann
getrennt und in mannigfacher Versetzung die Grundlage des Chors
bilden. Dies Verfahren findet man bei Bach öfter. Überraschend aber
ist, daß zwei weitausgeführte Fugensätze in die Entwicklung
eingewoben werden. Von unerhörter Kühnheit und
niederschmetternder Gewalt sind ihre Themen : m
^^^E^^^|E^E#^^ Du schlä gest sie und 9-^fc=f * ■fs h *-0 «= £
-^ 1 Sie ha-ben ein här-ter An- ge - sieht, denn ein Fels und S ¥ ? j?
^ ^_" M! h— ^ — . 60) Den oratorienhaften wol-len sich nicht be-
keh - - - (ren) Zug in diesem gewaltigen Chore wird man nicht
verkennen. Er erhebt sich in den Arien und dem Bass-Arioso
manchmal zu fast dra60) Die Bedenken, welche M. Hauptmann
gegen die Stelle Takt 37 — 44 erhob, sind von Rust durch den
Hinweis auf die Disposition des Ganzen hinfallig gemacht, wenn auch
nicht zu leugnen ist, daß die Vorwegnahme der Worte »Du schlagest
sie, aber sie fühlen es nicht, du plagest sie, aber sie bessern sich
nicht«, welche sich überdies nicht einmal mit dem gehörigen
Nachdruck auseinander legen, etwas befremdendes hat.
 — 296 matischer Schärfe. Eine formelle Merkwürdigkeit
haftet der TenorArie an Ihr Text lautet : 1 >u allzu sichre Seele
Erschrecke doch ! Denk, was dich würdig zähle Der Sünden Joch !
Die Gottes-Langmuth geht auf einem Fuß von Blei, Damit ihr Zorn
hernach dir desto schwerer sei. Für die aufgeregte Empfindung ,
welche Bach in dieser Cantate überall zum Ausdruck bringen wollte ,
war der Inhalt der ersten Textzeile nicht sprechend genug. Er
begann deshalb mit der zweiten und in Tonreihen, welche das
»Erschrecken« mit greifbarer Deutlichkeit malen. Die Arie bietet aber
einen der bei Bach nicht häufigen Fälle, wo der musikalische
Hauptgedanke , der im Ritomell vorgespielt wird, durchgängig nur in
dem Jnstrumentalpart verbleibt, in der Singstimme dagegen garnicht
auftritt. Die Hauptmelodie desselben scheint nun unter der
Vorstellung der Textworte in ihrer richtigen Ordnung erfunden zu
sein : -&* m feü^ (Du allzu sichre See - le er - schrecke doch ! Trifft
diese Vermuthung das wahre , so ist die Arie ein lehrreiches Beispiel,
wie bei Bach vocale. und instrumentale Anschauung zu unlöslicher
Einheit in einander griffen. Die andre Cantate, welche wir dieser als
ebenbürtig beiordnen, hat einen Psalmspruch (Ps. 38, 4) zum
poetischen Hauptgedanken. Sie paßt auch ihrem Inhalte nach mit
der vorigen zusammen. Man kann sich vorstellen, daß die in jener
mit feurigen Zungen gepredigte Buße nunmehr die Herzen der
Sünder erfüllt hat. »Es ist nichts gesundes an meinem Leibe vor
deinem Dräuen und ist kein Friede in meinen Gebeinen vor meiner
Sünde«, beten sie. 61) Der Chor ist eine in sich abgeschlossene
Doppelfuge trüben, zerknirschten Ausdrucks. Er hat aber außerdem
complicirte poetische Beziehungen. Von Takt 1 5 ab klingt in
gemessenen Intervallen, von Flöten. Zink und dreiPosau01) B.-G. V1,
Nr. 25. — S. Anhang A, Nr. 33. Das diplomatische Merkzeichen ist
nur im Umschlage der Originalstimmen einigermaßen deutlich.
 — 297 nen geblasen, der vierstimmige Choral » Ach Herr
mich armen Sünder«02) in die durch Streichinstrumente theilweise
mit besondern Tonreihen begleitete Fuge hinein. Der vierstimmige
Satz ist gleichfalls in sich abgeschlossen und könnte, wie die Fuge,
auch allein mit befriedigender Wirkung zu Gehör gebracht werden.
Trotzdem vereinigen sich beide Tonkörper, als seien sie aus einer
Wurzel gewachsen. Die Tiefe der Empfindung, welche sich öffnet ,
wenn das kirchliche Bußlied wie von unsichtbaren Stimmen
erklingend über die reuig im Staube betende Menge dahinzieht, ist
unsagbar und unergründlich. Vor den beiden Stollen des
Aufgesanges erscheint es jedesmal im Instrumentalbass in der
Verlängerung. Wir haben noch nicht alles gesagt. Die beiden
Fugenthemen sind aus zwei Zeilen des Chorals gebildet, das erste
aus der letzten , das zweite aus der ersten. Die Weise , wie dies
geschehen ist , mahnt an die Melodieumbildungen der Cantaten,
denen der vollständige Text eines Kirchenliedes zu Grunde liegt ; es
ist mir aus diesem Grunde wahrscheinlich., daß die Cantate »Es ist
nichts gesundes« mit jenen in eine und dieselbe engere Zeit gehört.
Das aus all diesen musikalischen und poetischen Motiven entwickelte
Musikstück ist höchst merkwürdig und in seiner Art einzig. Den Satz
»Es ist der alte Bund« aus der Cantate »Gottes Zeit« (s. Band I, S.
453 ff.) kann man deshalb nicht vergleichen, weil dort die Chorsätze
mehr nur als Zwischenstücke des Chorals auftreten. Hier aber
erblicken wir die auf Chor und Orchester übertragene Form der
Choralfantasie in ihrer Umkehrung : die Aufgabe der Instrumente
löst der Chor , die des Chors ein Instrumentencomplex. Es ist weder
ein freigestalteter biblischer noch ein Chöralchör; es ist ein aus
beiden gewordenes, höheres, das man staunend zu fassen sich
bemüht. Eine schöne Bassarie mit selbständig entwickeltem ,
charaktervollem Instrumentalbass bringt dem Hörer die
übermächtige Erscheinung des ersten Satzes persönlich näher: sie
steht noch in jenem Empfindungskreise, leitet aber in eine tröstliche
Stimmung hinüber, in welcher das Werk zu Ende geht. Ein helleres
Bild entrollt die Himmelfahrts-Cantate »Wer da 62) Nur dieses
Gedicht kann gemeint sein. Die Melodie gehört bekanntlich auch zu
dem Liede » 0 Haupt voll Blut und Wunden «.
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accurate

— 298 — glaubet und getauft wird«63) In dein Hauptchor

werden
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— 299 — Fugirung allmählig an den großen Chor

übergeht.05) Der homophoner gehaltene Schlußchor ist breit und
kräftig; beide umrahmen eine Bassarie im sogenannten
lombardischen Stil, G,)j die mit einem an italiänische Schönheit
mahnenden Melodienflusse ebenfalls einen hervorragend festlichen
Charakter verbindet. Ein warmer Hauch lagert über dem ganzen
Werke, das aber in dieser Gestalt schwerlich Originalcomposition ist,
sondern aus Überarbeitung einer wohl der früheren Leipziger Zeit
ungehörigen Composition hervorgegangen sein wird07) Es scheint
dieselbe Entstehungsgeschichte zu haben wie die Trauungs-Cantate
»Herr Gott, Beherrscher aller Dinge«, die wenigstens zum Theil auf
einer älteren Rathswahl-Cantate »Gott, man lobet dich in der Stille«,
zuni Theil auch auf einem Adagio der G dur- Violinsonate 6S) beruht.
Sehr verschlungen sind oft die Beziehungen, welche durch
Überarbeitungen und Übertragungen zwischen den verschiedenen
Werken Bachs sich geknüpft haben. Die genannte Rathswahl-
Cantate hat allem Anscheine nach auch als zweite Jubelcantate zur
Saecularfeier der Augsburgischen Confession (26. Juni 1730) dienen
müssen und dürfte endlich so wie sie vorliegt in abermaliger
Bearbeitung für ihren anfänglichen Zweck erscheinen. Der
Bibelspruch (Ps. 65, 1) ist hier nicht als Chor eomponirt, was sein
Inhalt verwehrt zu haben scheint, sondern als Alt-Solo, dessen
blühendes Wesen doch die Feststimmung aufs deutlichste ausdrückt.
Der Hauptchor folgt nach und hat madrigalischen Text. Jene Trias
von Jubelcantaten, welche Bach 1730 an drei Tagen hintereinander
aufführte, bS)) stellt sich, wie hier beiläufig bemerkt werden soll,
auoh in ihren beiden andern Theilen als Überarbeitung früherer
Werke heraus. Die erste »Singet dem Herrn ein neues Lied« ist die
Neujahrscantate von 1724 70), die dritte »Wünschet Jerusalem
Glück« eine Rathswahl-Cantate von 1727 (25. August), welche als
solche am 18. August 1741 wiederholt wurde71) ; sie ist aber sammt
ihrem 05) S. S. 187 dieses Bandes. 66) S. Band I, S. 413. 67) B.-G.
XIIIi, S. 3 ff. 68) B.-G. XXIV, Nr. 120. — Vrgl. B.-G. IX, S. 252 ff., und
Band I, S. 724 f. — S. Anhang A, Nr. 42. 69) Vrgl. S. 70 dieses
Bandes. 70) S. S. 215 f. dieses Bandes. 7 1 Jedoch mit einigen
Auslassungen und Zusätzen; s. Nützliche Nachrichten von Denen
Bemühungen derer Gelehrten u. s. w. Leipzig, 1741. S. 82 ff.
 — :*00 — Urbilde verloren gegangen. Und noch eine vierte
Umarbeitung dürfen wir wohl auf 1730 verlegen. Die Behauptung
wird begründet sein, daß in diesem Jahre aueh die Feier des
Reformationsfestes eine besondere Bedeutung erhielt und
demgemäß begangen wurde. Augenscheinlich sollte die Cantate »Ein
feste Burg ist unser Gott einer solchen außergewöhnlichen Feier
dienen. 72) Bach suchte die weimarische Musik »Alles was von Gott
geboren«, welche er mit ihrer Bestimmung für den Sonntag Oculi in
Leipzig doch nicht gebrauchen konnte , wieder hervor und fügte an
erster und fünfter Stelle neue Stücke ein. Es sind Choral chöre über
die erste und dritte Strophe des Lutherschen Liedes. Der kühne,
urkräftige Geist, der die deutsche Reformation ins Leben rief und in
Bachs Kunst noch mit voller Stärke weiter wirkte , hat nie einen
künstlerischen Ausdruck gefunden, weicherauch nur von ferne an
diese beiden kolossalen Gestalten heranreichte. Das erste, 228 Takte
zählende Stück hat die Pachelbelsche Form , nur daß der Cantus
ßrmus von der Trompete und den Instrumentalbässen canonisch
geführt wird ; wie eine unbezwingliche Riesenburg ragt es auf. Das
zweite ist eine Choralfantasie mit motivischer Benutzung aer ersten
Melodiezeile; den Cantus firmus singt der gesammte Chor im
Einklänge , während das Orchester ein Getümmel grotesker , wild
anspringender Gestalten ausbreitet , durcü welches der Chor
unentwegt hindurchschreitet — eine Illustration der dritten Strophe
(» Und wenn die Welt voll Teufel war«) wie sie großartiger und
charakteristischer nicht zu denken ist. 73) Im Vorübergehen sei hier
noch zweier andrer Uber72) B.-G. XVIII, Nr. SO. — S. Band I, S. 810
f. Möglich ist allerdings auch, daß diese Cantate für 1 739
geschrieben wurde , unter Hinblick auf das 200 jährige Jubiläum der
Annahme der evangelischen Lehre in Sachsen. Die Jubelfeier selbst
fand in den Hauptkirchen am Pfingstfeste (17. Mai) statt, jedoch auf
ausdrückliche Verordnung ohne besonderes Ceremoniel. Die
Universität beging das Andenken an jenes Ereigniß am 25. August;
Görner hatte zu diesem Zwecke eine lateinische Ode eomponirt
(Gretschel, Kirchliche Zustände Leipzigs vor und während der
Reformation im Jahre 1539. Leipzig, 1S39. S. 292 f.). — Daß die
Reformations- Cantate »Gott der Herr ist Sonn uud Schild« weder für
1730 noch 1739 eomponirt ist, wird später gezeigt werden. 73) Die
Cantate erschien um 1822 bei Breitkopf und Härtel in Leipzig als die
erste, welche nach Bachs Tode durch den Stich veröffentlicht wurde.
Rochlitz widmete ihr eine Besprechung (Für Freunde der Tonkunst.
Band 3, S 229 ff.)
 — 301 — arbeitungen gedacht, von denen wir nur sagen
können , daß sie ungefähr in dieser Zeit, also um 1730, ausgeführt
zu sein scheinen. Die Cöthener Serenade »Durchlauchtger Leopold«
benutzte Bach zu einer Cantate auf den zweiten Pfingsttag
(»Erhöhtes Fleisch und Blut«) und machte zu diesem Zwecke aus
dem Schluß -Duett einen Chor. 74) Der Geburtstags-Cantate auf die
Fürstin von Anhalt-Cöthen aber gab er die Bestimmung einer Musik
zum ersten Adventsonntage. Als solche wurde sie durch zwei
vortreffliche Bearbeitungen des Chorals »Nun komm der Heiden
Heiland« und zwei einfache Choräle bereichert. Die eine der
Bearbeitungen ist Choralfantasie mit einem vom Tenor
vorgetragenen Cantus firmus . In der andern dagegen wird — ein
bei Bach seltener Fall — die Choralmelodie motettenartig von einem
Sopran und Alt Zeile für Zeile durchgearbeitet. Man wird dabei an
den schönen zweistimmigen Choralsatz in der Cantate »Wer da
glaubet und getauft wird« erinnert, doch ist in der Advents-Cantate
die Durcharbeitung eine ausführlichere75). Wenn Bibelworte für
Sologesang benutzt wurden, so war die feststehende Form das Bass-
Arioso. Im allgemeinen ist auch Bach von diesem Brauche nicht
abgewichen. Doch hat er ihn manchmal nur äußerlich beobachtet,
indem er zwar die Bezeichnung »Arie« vermied, aber doch
Musikstücke schrieb, die man als frei gestaltete Arien auffassen muß.
Dies gilt für das Solo der Cantate »Herr, deine Augen sehen nach
dem Glauben«, welches den Namen »Arioso«, und das
ausnahmsweise einem Alt zugetheilte Solo der Rathswahl-Cantate
»Gott man lobet dich in der Stille«, das garkeine Bezeichnung trägt.
Es gilt ferner für den Gesang, mit welchem eine Cantate auf den 22.
Trinitatis-Sonntag »Was soll ich aus dir machen, Ephraim« (Hosea
11,8) anhebt. Dagegen ist dem Anfangs-Solo einer Cantate auf den
5. Trinitatis-Sonntag »Siehe, ich will viel Fischer aussenden« (Jerem.
16,61) schlankweg der Name »Arie« beigefügt. Beide Werke
gehören in diese Zeit, doch läßt sich der genauere Termin ihrer
Entstehung nur bei der letzteren mit Wahrscheinlichkeit angeben
(13. Juli 1732). 76) 74) S. Band I, S. 618 f. — S. Anhang A, Nr. 33.
75) B.-G. VII, Nr. 36. Vrgl. Band I. S.765 f. - S. Anhang A, Nr. 33. 76)
B.-G. XX1, Nr. 88 und 89. Zu ersterer Nr. s. Anhang A, Nr. 38. Zu
letzterer Anhang A, Nr. 33; das Wasserzeichen ist nur in der
Hornstimnie zu erkennen. Nicht entstanden kann diese Cantate sein
im Jahre 1733, weil da das Reformationsfest auf den 22. Trinitatis-
Sonntag fiel..
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