1 2 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 3 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 4 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 5 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 6 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 7 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 8 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 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 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 means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Permissions may be sought directly from Elsevier's Health Sciences Rights Department in Philadelphia, PA, USA: phone: (+1) 215 239 3804, fax: (+1) 215 239 3805, e-mail: [email protected] . You may also complete your request on-line via the Elsevier homepage (http://www.elsevier.com ), by selecting “Customer Support” and then “Obtaining Permissions”. 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 9 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. 10 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.