Adult Congenital Heart Disease - A Practical Guide - M. Gatzoulis, et. al., (Blackwell, 2005) WW

279 Pages·2005·3.8 MB·English

Adult Congenital Heart Disease

A PRACTICAL GUIDE

Adult Congenital
Heart Disease

A PRACTICAL GUIDE

Michael A. Gatzoulis
Royal Brompton Hospital and
National Heart & Lung Institute at the Imperial College
London, UK
Lorna Swan
Department of Cardiology
Western Infi rmary
Glasgow, UK
Judith Therrien
Sir M. Davis Jewish General Hospital
McGill University
Montreal, Quebec, Canada
George A. Pantely
Division of Cardiology
Oregon Health and Science University
Portland, Oregon, USA

INCLUDING
‘Pregnancy and Contraception’ chapter
by Philip J. Steer

FOREWORD BY
Eugene Braunwald

© 2005 by Blackwell Publishing Ltd
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First published 2005

Library of Congress Cataloging-in-Publication Data

Adult congenital heart disease : a practical guide / Michael A. Gatzoulis … [et al.] ; foreword by Eugene
Braunwald
p. ; cm.
Includes index.
ISBN 0-7279-1668-8
1. Congenital heart disease.
[DNLM: 1. Heart Defects, Congenital—Adult. WG 220 A2445 2005] I. Gatzoulis, Michael A.

RC687.A454 2005
616.1’2043—dc22
2004019723

ISBN 0-7279-1668-8

A catalogue record for this title is available from the British Library

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acceptable environmental accreditation standards.

v

Contents

Foreword, vii
Preface, ix
Acknowledgments, x

Part 1 General Principles, 1

1 Epidemiology of Congenital Heart Disease, 3
2 Services for the Adult with Congenital Heart Disease, 8
3 Pregnancy and Contraception, 16
4 Infective Endocarditis Prophylaxis, 36
5 Anticoagulation, 42
6 Lifestyle Issues, 49
7 Long-Term Outcome, 60

Part 2 Common Lesions, 65

8 Atrial Septal Defects and Anomalous Pulmonary Venous Drainage, 67
9 Ventricular Septal Defect, 82
10 Atrioventricular Septal Defect, 87
11 Left Ventricular Outfl ow Tract Disorders, 92
12 Coarctation of the Aorta, 97
13 Complete Transposition of the Great Arteries, 103
14 The Single Ventricle and Fontan Circulations, 112
15 Tetralogy of Fallot and Right Ventricular Outfl ow Tract Disorders, 125
16 Pulmonary Atresia with Ventricular Septal Defect, 132
17 Ebstein’s Anomaly of the Tricuspid Valve, 139
18 Patent Arterial Duct, 145
19 Marfan Syndrome, 153
20 Eisenmenger Syndrome, 161
21 Other Lesions, 174
22 Pulmonary Hypertension, 182

Part 3 Emergencies and Special Situations, 189

23 Arrhythmia and Syncope, 191
24 Suspected Infective Endocarditis, 196

vi Contents

25 Perioperative Care, 201
26 Heart Failure: Acute Management, 206
27 Care of the Cyanosed Patient, 209

Bibliography, 213
Glossary, 217
Appendix: Shunt Calculations, 257
Index, 261

vii

Foreword

The diagnosis and successful management of congenital heart disease rep-
resents one of the greatest triumphs of cardiovascular medicine and surgery
in the 20th century. As a consequence, the number of adults with congenital
heart disease – both with repaired and unrepaired lesions – has grown rapidly,
and is now approaching one million in North America. Similar increases have
occurred in Western Europe. The care of adults with congenital heart disease
represents a major challenge. They include a large number of diverse anatomic
malformations of varying severities at various stages of their natural history
and with different degrees of anatomic repair. Approximately one-third of
these patients are considered to have ‘simple’ congenital heart disease, such
as mild pulmonic stenosis or repaired ventricular septal defect. The majority,
however, such as those with cyanotic congenital heart disease, have lesions of
greater complexity.
The growing population of adults with congenital heart disease presents
unique problems in management. Arrhythmias are frequent and are often of
serious import. Pregnancy presents special problems. The risk of infective en-
docarditis and premature ventricular dysfunction may occur in patients even
following successful correction. There is a delicate interplay between manag-
ing the usual risks for the development of coronary artery disease in adults
and the residua of repaired congenital heart disease, such as coarctation of the
aorta.
During the past two decades the approach to the diagnosis and treatment of
adults with congenital heart disease has been totally transformed. For many
years the catheterization laboratory was the site of diagnosis while the oper-
ating room the site of treatment. Now, the imaging laboratory is the primary
diagnostic site and increasingly invasive treatment is carried out in the cath-
eterization laboratory.
Ideally, adults with congenital heart disease should be cared for at regional
adult congenital heart disease centers, staffed by cardiologists trained in both
pediatric and adult cardiology, who are trained in the special problems present-
ed by these patients. Regional centers alone, however, cannot do the whole job.
Cardiologists not specifi cally trained to care for patients with congenital heart
disease, other physicians and allied health professionals outside these regional
centers are required to participate in the care of these patients. Adult Congenital
Heart Disease: A Practical Guide by Gatzoulis, Swan, Therrien and Pantely will
be a valued resource to these practitioners. This guide describes the various
forms of congenital heart disease in suffi cient detail to allow the practitioner to

diagnose and manage most of their problems, yet it is not the encyclopedic text
required by the subspecialist and often overwhelming to the non-specialist.
The sections on general principles, including pregnancy, contraception, infec-
tive endocarditis, anticoagulation, arrhythmias, syncope, heart failure and the
care of the cyanosed patient, are particularly well written.
There has been fear that the adult with congenital heart disease could fall
between the cracks and become a medical orphan. Adult Congenital Heart Dis-
ease: A Practical Guide will become an important educational resource to help
diminish this fear.
Eugene Braunwald, MD
Boston, MA, USA

ix

Preface

Congenital heart disease with an approximate incidence of 1% is the most com-
mon inborn defect, which used to carry a very poor prognosis. Advances in di-
agnosis and management of patients with congenital heart disease over the latter
part of the 20th century, however, have led to the majority of such patients sur-
viving to adulthood. There are currently hundreds of thousands of individuals
around the world who either have survived life-saving childhood surgery or are
diagnosed with congenital heart disease in later life—often during pregnancy.
Most of these patients have ongoing lifelong congenital heart and other medi-
cal needs. They are regularly seen by their family physicians and, increasingly,
by offi ce and hospital specialists, presenting both a signifi cant workload and a
management challenge. While patients with congenital heart disease represent,
therefore, a common challenge to health care, there has been and continues to be
a marked shortfall in training programs and in resource allocation to provide for
them.
It is the aim of this smaller textbook to introduce this important subject to a
larger audience, namely general physicians, non-congenital heart specialists,
health allied professionals and trainees, all of whom are now involved with
the care of the congenital heart patient. Our main focus has been to introduce
general principles in recognizing the problem, discuss management of com-
mon lesions and provide essential information on dealing with emergencies.
Our ultimate goal was to familiarize the various disciplines involved with this
important subject and facilitate appropriate and timely specialist referral of
the congenital heart patient. Knowledge is power, and it is hoped that better
informed health professionals (and patients) will improve further the longer-
term prospects of what is considered to be one of the most successful endeavors
of modern medicine.
Michael A. Gatzoulis
Lorna Swan
Judith Therrien
George A. Pantely

x

Acknowledgments

The authors are deeply indebted to their colleagues Professor Philip Steer for the
pregnancy and contraception chapter, Professor Leon Gerlis for his morphologic
drawings, Drs Philip Kilner, Wei Li and Yen Ho for their cardiac MRI, echocar-
diographic and morphologic specimens, Drs Jack Colman, Erwin Oechslin and
Dylan Taylor for allowing us to use the congenital heart glossary, Dr Craig Brob-
erg for the appendix on shunt calculations, and to Mary Banks, Nick Morgan,
Tom Fryer and the BMJ Books/Blackwell team for their enthusiastic support
throughout the project. Dr Gatzoulis and the Royal Brompton Adult Congenital
Heart Program are supported by the British Heart Foundation.