Congenital Heart Disease in Adults, Third Edition

498 Pages·2011·49.66 MB·English

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CONGENITAL HEART DISEASE IN ADULTS, THIRD EDITION ISBN: 978-1-4160-5894-6
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The Publisher
Library of Congress Cataloging-in-Publication Data
Perloff, Joseph K., 1924-
Congenital heart disease in adults / Joseph K. Perloff, John S. Child, Jamil Aboulhosn.—3rd ed.
p. ; cm.
Includes bibliographical references and index.
ISBN 978-1-4160-5894-6
1. Congenital heart disease. I. Child, John S. II. Aboulhosn, Jamil.
III. Title
[DNLM: 1. Heart Defects, Congenital. 2. Adult. WG 220 P451ca 2008]
RC687.P39 2008
616.1’2043—dc22.
2008003109
Acquisitions Editor: Natasha Andjelkovic
Developmental Editor: Isabel Trudeau
Project Manager: Bryan Hayward
Design Direction: Gene Harris
Printed in United States of America
Last digit is the print number: 9 8 7 6 5 4 3 2 1
FM_i-xii-X5894.indd iv 6/25/08 10:39:39 AM

CONTRIBUTORS
JAMIL ABOULHOSN, MD BARRY H. GUZE, MD
Assistant Clinical Professor of Medicine Department of Psychiatry and Biobehavioural Sciences
Division of Cardiology David Geffen School of Medicine at UCLA
Ahmanson/UCLA Adult Congenital Heart Disease Resnick Neuropsychiatric Hospital at UCLA
Center Los Angeles, California
David Geffen School of Medicine at UCLA
Los Angeles, California LINDA HOUSER
Assistant Dean for Evaluation and Program
FABIAN CHEN, MD Improvement
Physician, Cardiology Indiana University
Assistant Professor, Medicine Bloomington, Indiana
Assistant Professor-In-Residence, Pediatric
Cardiology JON A. KOBASHIGAWA, MD
Member, ACCESS Department—MCIP Clinical Professor of Medicine
David Geffen School of Medicine at UCLA Cardiology
Los Angeles, California David Geffen School of Medicine at UCLA
UCLA Medical Center
JOHN S. CHILD, MD Medical Director
Professor of Medicine UCLA Heart Transplant Program
David Geffen School of Medicine at UCLA Los Angeles, California
Director
Ahmanson/UCLA Adult Congenital Heart Disease BRIAN KOOS, MD, DPHIL
Center Department of Obstetrics and Gynecology
UCLA Center for the Health Sciences UCLA Medical Center
Los Angeles, California Los Angeles, California
EMMANUÈLE DÉLOT, PhD HILLEL LAKS, MD
Department of Pediatrics Director, Heart and Heart-Lung Transplant Program
Pediatric Cardiology UCLA Medical Center
David Geffen School of Medicine at UCLA Division of Cardiac Surgery
Los Angeles, California David Geffen School of Medicine at UCLA
Los Angeles, California
HOWARD DINH, MD
Chief Cardiology Fellow DANIEL LEVI, MD
Cardiology/Medicine Assistant Professor
David Geffen School of Medicine at UCLA Division of Pediatric Cardiology
Los Angeles, California Mattel Children’s Hospital at UCLA
Los Angeles, California
JOHN PAUL FINN, MD
Department of Radiological Sciences DANIEL MARELLI, MD
David Geffen School of Medicine at UCLA David Geffen School of Medicine at UCLA
Los Angeles, California Los Angeles, California
v
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vi CONTRIBUTORS
PAMELA D. MINER, NP MARK D. PLUNKETT, MD
Nurse Practitioner Associate Professor
Ahmanson/UCLA Adult Congenital Heart Disease Division of Cardiothoracic Surgery
Center David Geffen School of Medicine at UCLA
UCLA Medical Center Los Angeles, California
Los Angeles, California
JEFFREY L. SAVER, MD
JOHN W. MOORE, MD Director, Stroke and Vascular Neurology
Director, Diagnostic and Interventional Catheterization Stroke Center and Department of Neurology
Program UCLA Medical Center
UCLA Medical Center Los Angeles, California
Los Angeles, California
KALYANAM SHIVKUMAR, MD, PhD
ELISA A. MORENO Director, Cardiac Arrhythmia Center
Department of Psychiatry and Biobehavioural Sciences Director, Interventional Cardiac Electrophysiology
David Geffen School of Medicine at UCLA Associate Professor of Medicine and Radiological
Resnick Neuropsychiatric Hospital at UCLA Sciences
Los Angeles, California Los Angeles, California
DAVID A. PEGUES, MD MICHAEL SOPHER, MD
Professor of Clinical Medicine Clinical Professor
Division of Infectious Disease, Department of Medicine Department of Anesthesiology
Hospital Epidemiologist David Geffen School of Medicine at UCLA
David Geffen School of Medicine at UCLA Los Angeles, California
Los Angeles, California
AMY VERSTAPPEN, MED
JOSEPH K. PERLOFF, MD President and Chief Executive Offi cer
Streisand/American Heart Association Professor of Adult Congenital Heart Association
Medicine and Pediatrics, Emeritus Philadelphia, Pennsylvania
Ahmanson/UCLA Adult Congenital Heart Disease
Center
David Geffen School of Medicine at UCLA
Los Angeles, California
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PREFACE
S
ince publication of the second edition of Congenital Heart Disease in Adults a de-
cade ago, progress has been rapid and impressive. The third edition is designed to
keep the reader abreast of the current status of this evolving fi eld. Worldwide recog-
nition is refl ected in the International Society for Adult Congenital Cardiac Disease
(ISACCD) that is represented in more than 30 countries (Chapter 2), and American
recognition is refl ected in the Adult Congenital Heart Association (ACHA), the fi rst
national patient advocacy organization (Chapter 2).
Survival has changed dramatically, literally reversing traditional norms. In devel-
oped countries, approximately 85% of infants born with congenital heart disease
are expected to reach adulthood. In the United States today, there are more adults
than infants and children with what was once considered a disorder of the young.
Congenital heart disease is no longer defi ned by the age of onset but more appro-
priately by the age range that survival permits. Interest has broadened to include the
quality of extended life spans.
The third edition consists of 23 chapters grouped in fi ve sections—Background and
Facilities, Survival Patterns, Medical Considerations, Surgical Considerations, and
Residua and Sequelae After Surgery or Interventional Catheterization. Three chapters
are entirely new—Chapter 2: “National and International Scope,” Chapter 7: “Cardiac
Magnetic Resonance Imaging and Computed Tomography in the Assessment of
Adult Congenital Heart Disease,” and Chapter 17: “Cardiac Transplantation in
Patients with Congenital Heart Disease.” Thirteen of the remaining chapters were
virtually rewritten—”Echocardiography in Anatomic Imaging and Hemodynamic
Evaluation of Adults with Congenital Heart Disease,” “Infective Endocarditis and
Congenital Heart Disease,” “Management of Pregnancy and Contraception in
Congenital Heart Disease,” “Genetics, Epidemiology, and Counseling,” “Exercise and
Athletics in Adults with Congenital Heart Disease,” “Cyanotic Congenital Heart Dis-
ease: A Multisystem Disorder,” “Psychiatric and Psychosocial Disorders in Congenital
Heart Disease,” “Neurologic Disorders,” “Cardiac Surgery in Adults with Congenital
Heart Disease: Operation and Reoperation,” “Prosthetic Materials: Selection, Use, and
Long-Term Effects,” “Transcatheter Interventions in Adult Congenital Heart Disease,”
“Electrophysiologic Abnormalities: Unoperated Occurrence and Postoperative Re-
sidua and Sequelae,” and “Myocardial Growth and the Development and Regression
of Increased Ventricular Mass.”
As was the case in the fi rst two editions, the fi nal version of each chapter was
drafted by one author (Joseph K. Perloff) to ensure that the book reads stylistically
as a single-authored text while retaining the advantages of expert collaborators, all
of whom were affi liated with the Ahmanson/University of California, Los Angeles
(UCLA) Adult Congenital Heart Disease Center.
Although the third edition, like its predecessors, is designed to chart advances
in the fi eld, it also recognizes that progress has not been without a price—indeed,
it has created problems of its own. There are too few pediatric cardiologists to care
for the large and growing adult population and too few adult congenital heart
disease facilities to handle the infl ux, concerns that are the subject of Chapter 3,
vii
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viii PREFACE
“Specialized Facilities for Adults with Congenital Heart Disease.” A generation ago,
survival was the chief objective—to make it possible for infants and children with
congenital heart disease to reach adulthood. However, prolongation of life is often
eclipsed by problems inherent in increased longevity, the very goal that we have la-
bored to achieve. Psychosocial and neurologic problems (Chapters 13 and 14), only
some of which were anticipated, accompany survival into adulthood. Cures in the
literal sense are few and far between. Postoperative residua and sequelae vary from
minor to serious and, with few exceptions, require long-term medical attention
(Chapter 3). Congenital heart disease is now recognized by the National Heart, Lung,
and Blood Institute as a chronic illness. Although life span has increased consider-
ably, longevity is not normal. Hence patients are confronted with the prospect of
premature mortality and with the emotional, social, educational, and occupational
problems associated with chronic illness (Chapter 13). Neurologic complications
have revealed themselves and are the subject of Chapter 14. The cardiovascular and
the central nervous systems form almost simultaneously in early gestation, so struc-
tural abnormalities of the heart may coincide with structural abnormalities of the
brain. Disorders of intellectual development, cognition, and higher cortical function
are major postoperative concerns.
Pediatric cardiology evolved as a specialized fi eld when advances in diagnostic
techniques and surgical management created an air of optimism for infants and
children. Congenital heart disease in adults evolved as a specialized fi eld because
the success of pediatric cardiologists and cardiac surgeons profoundly affected sur-
vival patterns. The third edition of this book brings up to date the gratifying progress
that has characterized this fi eld during the past decade.
In 1936, Maude Abbott published her landmark Atlas of Congenital Heart Disease,
and in 1944, Alfred Blalock sutured the end of a subclavian artery to the side of a
pulmonary artery in an infant with tetralogy of Fallot, establishing the now legend-
ary Blalock-Taussig anastomosis. A century has now passed, and a new patient
population has emerged—adults with congenital heart disease. The hopeless futili-
ties to whom Helen Taussig devoted herself to in the Harriet Lane Children’s Clinic
have come of age. Worldwide adult congenital heart disease facilities are gratifying
achievements and testimonies to progress.
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ACKNOWLEDGMENTS
O
ur largest debt is to the Ahmanson Foundation, whose generous endowment to
the UCLA Adult Congenital Heart Disease Center made possible the experience that
set the stage for the third edition.
Our thanks to Yelba Castellon, to whom we turned to resolve many problems and
who was responsible for the endnote arrangements of virtually all 23 chapters.
Pamela Miner and Linda Houser, nurse practitioners, were dedicated partners.
ix
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