Pediatric Cardiology for Practitioners

775 Pages·2007·20.37 MB·English

by  Myung K. Park MD FAAP FACC

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Contents
FOREWORD TO FIRST EDITION 9
PREFACE TO FIRST EDITION 10
PREFACE TO FIFTH EDITION 12
FREQUENTLY USED ABBREVIATIONS 14
Part I – Basic Tools in Routine Evaluation of Cardiac Patients 16
Chapter 1 – History Taking 16
Gestational and Natal History 17
Postnatal History 19
Family History 22
Chapter 2 – Physical Examination 23
Growth Pattern 23
Inspection 23
Palpation 32
Blood Pressure Measurement 35
Auscultation 43
Some Special Features of the Cardiac Examination of Neonates 65
Chapter 3 – Electrocardiography 68
What Is the Vectorial Approach? 68
Comparison of Pediatric and Adult Electrocardiograms 72
Basic Measurements and Their Normal and Abnormal Values Necessary for
Routine Interpretation of an Electrocardiogram 73
Atrial Hypertrophy 85
Ventricular Hypertrophy 86
Ventricular Conduction Disturbances 90
ST-Segment and T-Wave Changes 95
Chapter 4 – Chest Roentgenography 102
Heart Size and Silhouette 102
Evaluation of Cardiac Chambers and Great Arteries 105
Pulmonary Vascular Markings 108
Systematic Approach 108
Chapter 5 – Flow Diagrams 113
Part II – Special Tools in Evaluation of Cardiac Patients 117
Chapter 6 – Noninvasive Techniques 118
Echocardiography 118
Stress Testing 138
Long-term ECG Recording 146
Ambulatory Blood Pressure Monitoring 148
Chapter 7 – Invasive Procedures 150
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Catheter Intervention Procedures 155
Part III – Pathophysiology 159
Chapter 8 – Fetal and Perinatal Circulation 159
Fetal Circulation 160
Changes in Circulation after Birth 161
Premature Newborns 164
Chapter 9 – Pathophysiology of Left-to-Right Shunt Lesions 166
Atrial Septal Defect 167
Ventricular Septal Defect 169
Patent Ductus Arteriosus 171
Endocardial Cushion Defect 173
Chapter 10 - Pathophysiology of Obstructive and Valvular Regurgitation Lesions
176
Obstruction to Ventricular Output 176
Stenosis of Atrioventricular Valves 179
Valvular Regurgitant Lesions 181
Chapter 11 – Pathophysiology of Cyanotic Congenital Heart Defects 184
Clinical Cyanosis 184
Common Cyanotic Heart Defects 191
Part IV – Specific Congenital Heart Defects 205
Chapter 12 – Left-to-Right Shunt Lesions 206
Atrial Septal Defect 206
Ventricular Septal Defect 212
Patent Ductus Arteriosus 221
Patent Ductus Arteriosus in Preterm Neonates 226
Complete Endocardial Cushion Defect 228
Partial Endocardial Cushion Defect 235
Partial Anomalous Pulmonary Venous Return 238
Chapter 13 – Obstructive Lesions 242
Pulmonary Stenosis 242
Aortic Stenosis 248
Coarctation of the Aorta 257
Interrupted Aortic Arch 267
Chapter 14 – Cyanotic Congenital Heart Defects 269
Approach to a Cyanotic Neonate 269
Complete transposition of the great arteries 275
Congenitally Corrected Transposition of the Great Arteries 289
Tetralogy of Fallot 295
Tetralogy of Fallot with Pulmonary Atresia (Pulmonary Atresia and
Ventricular Septal Defect) 305
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Tetralogy of Fallot with Absent Pulmonary Valve 311
Total Anomalous Pulmonary Venous Return 313
Tricuspid Atresia 319
Pulmonary Atresia with Intact Ventricular Septum 332
Hypoplastic Left Heart Syndrome 338
Ebstein's Anomaly 342
Persistent Truncus Arteriosus 349
Single Ventricle 354
Double-Outlet Right Ventricle 360
Heterotaxia (Atrial Isomerism, Splenic Syndromes) 366
Persistent Pulmonary Hypertension of the Newborn 373
Chapter 15 – Vascular Ring 380
Pathology 380
Clinical Manifestations 383
Diagnosis 384
Management 385
Chapter 16 – Chamber Localization and Cardiac Malposition 386
Chamber Localization 386
Localization of the great arteries 389
Dextrocardia and Mesocardia 390
Chapter 17 – Miscellaneous Congenital Cardiac Conditions 392
Aneurysm of the Sinus of Valsalva 392
Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery
(Bland-White-Garland Syndrome, Alcapa Syndrome) 392
Aortopulmonary Septal Defect 394
Arteriovenous Fistula, Coronary 395
Arteriovenous Fistula, Pulmonary 396
Arteriovenous Fistula, Systemic 397
Atrial Septal Aneurysm 397
Cervical Aortic Arch 398
Common Atrium 398
Cor Triatriatum 398
DiGeorge Syndrome 399
Double-Chambered Right Ventricle 401
Ectopia Cordis 401
Hemitruncus Arteriosus 401
Idiopathic Dilatation of the Pulmonary Artery 402
Kartagener's Syndrome 403
Parachute Mitral Valve 403
Patent Foramen Ovale 403
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Pericardial Defect, Congenital 404
Pseudocoarctation of the Aorta 405
Pulmonary Artery Stenosis 405
Scimitar Syndrome 406
Systemic Venous Anomalies 406
Part V – Acquired Heart Disease 409
Chapter 18 – Primary Myocardial Disease 409
Hypertrophic Cardiomyopathy 411
Infants of Diabetic Mothers 419
Transient Hypertrophic Cardiomyopathy in Neonates 421
Dilated or Congestive Cardiomyopathy 421
Endocardial Fibroelastosis 425
Doxorubicin Cardiomyopathy 428
Carnitine Deficiency 429
Restrictive Cardiomyopathy 430
Right Ventricular Dysplasia 432
Noncompaction Cardiomyopathy 432
Chapter 19 – Cardiovascular Infections 434
Infective Endocarditis 434
Myocarditis 447
Pericarditis 450
Constrictive Pericarditis 453
Kawasaki Disease 453
Lyme Carditis 464
Postpericardiotomy Syndrome 466
Postperfusion Syndrome 467
Human Immunodeficiency Virus Infection 467
Chapter 20 – Acute Rheumatic Fever 469
Prevalence 469
Causes 470
Pathology 470
Clinical Manifestations 470
Diagnosis 473
Differential Diagnosis 474
Clinical Course 474
Management 475
Prognosis 476
Prevention 477
Chapter 21 – Valvular Heart Disease 478
Mitral Stenosis 479
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Mitral Regurgitation 483
Aortic Regurgitation 486
Mitral Valve Prolapse 490
Chapter 22 – Cardiac Tumors 493
Prevalence 493
Pathology 493
Clinical Manifestations 495
Management 498
Chapter 23 – Cardiovascular Involvement in Systemic Diseases 499
Mucopolysaccharidoses 499
Systemic Lupus Erythematosus 499
Rheumatoid Arthritis 500
Friedreich's Ataxia 501
Muscular Dystrophy 501
Myotonic Dystrophy 502
Marfan Syndrome 503
Acute Glomerulonephritis 504
Hyperthyroidism: Congenital and Acquired 504
Hypothyroidism: Congenital and Acquired 505
Sickle Cell Anemia 506
Part VI – Arrhythmias and Atrioventricular Conduction Disturbances 507
Chapter 24 – Cardiac Arrhythmias 507
Rhythms Originating in the Sinus Node 507
Rhythms Originating in the Atrium 512
Rhythms Originating in the Atrioventricular Node 521
Rhythms Originating in the Ventricle 524
Long QT Syndrome 535
Short QT Syndrome 543
Brugada Syndrome 543
Chapter 25 – Disturbances of Atrioventricular Conduction 544
First-Degree Atrioventricular Block 545
Second-Degree Atrioventricular Block 545
Third-Degree Atrioventricular Block 546
Atrioventricular Dissociation 548
Chapter 26 – Cardiac Pacemakers and Implantable Cardioverter-Defibrillators in
Children 548
ECGs of Artificial Cardiac Pacemakers 549
Pacemaker Therapy in Children 550
Implantable Cardioverter-Defibrillator Therapy 554
Part VII – Special Problems 558
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Chapter 27 – Congestive Heart Failure 558
Chapter 28 – Systemic Hypertension 574
Chapter 29 – Pulmonary Hypertension 590
Chapter 30 – Child with Chest Pain 606
Chapter 31 – Syncope 619
Chapter 32 – Palpitation 631
Chapter 33 – Dyslipidemia and Other Cardiovascular Risk Factors 635
Childhood Onset of Coronary Artery Disease 636
Cardiovascular Risk Factors and the Metabolic Syndrome 636
Dyslipidemia 640
Other Risk Factors 659
OBESITY 659
CIGARETTE SMOKING 668
Practice of Preventive Cardiology 670
Chapter 34 – Athletes with Cardiac Problems 676
Chapter 35 – Cardiac Transplantation 700
Appendix 713
Appendix A : Miscellaneous 713
Appendix B : Blood Pressure Values 717
Appendix C : Cardiovascular Risk Factors 727
Appendix D : Normal Echocardiographic Values and Images 735
Appendix E : Drugs Used in Pediatric Cardiology 743
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th
Park: Pediatric Cardiology for Practitioners, 5
ed.2008
Pediatric Cardiology for Practitioners

Fifth Edition
Myung K. Park, MD, FAAP, FACC
Professor Emeritus (Pediatrics), University of Texas Health Science Center, San Antonio, Texas
Clinical Professor of Pediatrics, College of Medicine, Texas A&M University System, Health Science
Center, College Station, Texas
Attending Cardiologist, Driscoll Children's Hospital, Corpus Christi, Texas
Dedication
This book is affectionately dedicated to my wife, Issun, our sons (Douglas, Christopher, and Warren), our
grandchildren (Natalie and Audrey), and their mother (Jin-Hee).
MOSBY ELSEVIER
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PEDIATRIC CARDIOLOGY FOR PRACTITIONERS
ISBN: 978-0-323-04636-7
Copyright © 2008, 2002, 1996, 1988, 1984 by Mosby, Inc., an affiliate of Elsevier Inc.
All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any
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Notice
Knowledge and best practice in this field are constantly changing. As new research and experience
broaden our knowledge, changes in practice, treatment, and drug therapy may become necessary or
appropriate. Readers are advised to check the most current information provided (i) on procedures
featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or
formula, the method and duration of administration, and contraindications. It is the responsibility of the
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practitioner, relying on their own experience and knowledge of the patient, to make diagnoses, to
determine dosages and the best treatment for each individual patient, and to take all appropriate safety
precautions. To the fullest extent of the law, neither the Publisher nor the Author assumes any liability for
any injury and/or damage to persons or property arising out or related to any use of the material contained
in this book.
The Publisher
Library of Congress Cataloging-in-Publication Data
Park, Myung K. (Myung Kun), 1934—Pediatric cardiology for practitioners/Myung K. Park.—5th ed.
p.; cm.
Includes bibliographical references and index.
ISBN 978-0-323-04636-7
1. Pediatric cardiology. I. Title.
[DNLM: 1. Heart Diseases. 2. Child. 3. Heart Defects, Congenital. 4. Infant. WS 290 P235p 2008]
RJ421.P37 2008
618.92'12—dc22
2007002639
Acquisitions Editor: Judith Fletcher
Developmental Editor: Colleen McGonigal
Publishing Services Manager: Frank Polizzano
Project Manager: Rachel Miller
Book Designer: Ellen Zanolle
Printed in the United States of America. Last digit is the print number: 9 8 7 6 5 4 3 2 1
FOREWORD TO FIRST EDITION
I was very honored by Dr. Park's request that I review his manuscript and write a foreword. Having carefully
read it, I am even more pleased to be able to write a foreword with unqualified praise and to recommend this
book to my colleagues in pediatrics and family medicine. I think that it is so well organized and logical that
serious students and even pediatric cardiologists in training will find it useful.
One of the more obvious difficulties encountered by a busy house officer or a practitioner is that most
textbooks are organized to be useful to the individual who already knows the diagnosis. This “Catch-22” is
resolved by Dr. Park's presentation, which permits scanning and rapid identification of the problem area, and
then progresses stepwise to the clinical diagnosis, the medical management of the problem, and the general
potential of surgical assistance.
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This book is both concise and thorough, a relatively rare combination in the medical literature. It spares the
practitioner the great detail usually found in a cardiology textbook on esoteric details of the echocardiogram,
catheterization, angiocardiogram, and surgical procedures. The presentations are practical, giving drug
dosages, intervals, and precautions. Although he presents alternative views where appropriate, Dr. Park is
courageous in presenting his own recommendations, which are well thought out and, above all, logical.
It is easy for me to see the impact of Dr. Park's career in this excellent book. He had a thorough general
pediatric training, followed by several years in pediatric cardiology and cardiovascular physiology in this
country. After some years in academic pediatric cardiology in Canada, he returned to this country and served
as a family practitioner in a small community in the state of Washington. He learned well the problems of
practicing in an area where consultants were not convenient for the practitioner, nor was it easy to transport
a patient quickly to a major center. He returned to a research fellowship in pharmacology, and consequently
knows more about the dynamics of cardiovascular drugs than anyone I can think of in the field of pediatric
cardiology. For the past several years he has actively taught and practiced pediatric cardiology in a
university setting and has learned the process of transmitting the information and the skills that he has
acquired in his career. This book reflects all of these experiences, and the balance between science and
practical considerations is reflected in every page, with the particular clarity of a natural teacher.
I am proud of my earlier association with Dr. Park, and particularly proud of his present contribution to
pediatric cardiology.
Warren G. Guntheroth, MD
Professor of Pediatrics, Head, Division of Pediatric Cardiology, University of Washington School of
Medicine, Seattle, Washington
PREFACE TO FIRST EDITION
Since teaching pediatric cardiology, I have felt that there was a need for a book that was written primarily
for noncardiologists, such as medical students, house staff, and practitioners. Although many excellent
pediatric cardiology textbooks are available, they are not very helpful to the noncardiologist, because they
are filled with many details that are beyond the need or comprehension of practitioners. In addition, these
books are not very effective in teaching practitioners how to approach children with potential cardiac
problems; they are usually helpful only when the diagnosis is known. This book is intended to meet the
needs of noncardiologist practitioners for improving their skills in arriving at clinical diagnoses of cardiac
problems, using basic tools available in their offices and community hospitals. This book will also serve as a
quick reference in the area of pediatric cardiology. Although echocardiograms, cardiac catheterization, and
angiocardiograms provide more definite information about the problem, these tools are not discussed at
length in this book because they are not routinely available to practitioners, and their use requires special
skills.
In writing a small yet comprehensive book, occasional oversimplification was unavoidable. Major emphasis
was placed on the effective utilization of basic tools: history taking, physical examination, ECGs, and chest
roentgenograms. Significance of abnormal findings in each of these areas is discussed, with differential
diagnoses whenever applicable. A section is provided for pathophysiology, for in-depth understanding of
clinical manifestations of cardiac problems. Accurate but succinct discussion of congenital and acquired
cardiac conditions is presented for quick reference. Indications, timing, procedures, risks, and complications
for surgical treatment of cardiac conditions are also briefly discussed for each condition. Common cardiac
arrhythmias are presented, with brief discussions of descriptions, causes, significance, and management of
each arrhythmia. A special section addresses cardiac problems of the neonate; another is devoted to special
problems, such as congestive heart failure, systemic hypertension, pulmonary hypertension, chest pain, and
syncope.