Look for these other titles in Catherine M. Otto’s Practical Echocardiography Series Donald C. Oxorn Intraoperative Echocardiography Linda D. Gillam & Catherine M. Otto Advanced Approaches in Echocardiography Martin St. John Sutton & Susan E. Wiegers Echocardiography in Heart Failure Echocardiography in Congenital Heart Disease P R A C T I C A L E C H O C A R D I O G R A P H Y S E R I E S Mark B. Lewin, MD Professor and Chief Division of Pediatric Cardiology University of Washington School of Medicine Heart Center Co-Director and Director of Pediatric Echocardiography Seattle Children’s Hospital Seattle, Washington Karen Stout, MD Director, Adult Congenital Heart Disease Program Associate Professor, Departments of Medicine and Pediatrics University of Washington School of Medicine Attending Cardiologist University of Washington Medical Center and Seattle Children’s Hospital Seattle, Washington 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 ECHOCARDIOGRAPHY IN CONGENITAL HEART DISEASE ISBN: 978-1-4377-2696-1 Copyright © 2012 by Saunders, an imprint of Elsevier Inc. 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. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein). Notices Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. With respect to any drug or pharmaceutical products identified, 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 practitioners, relying on their own experience and knowledge of their patients, 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 authors, contributors, or editors, assume any liability for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Library of Congress Cataloging-in-Publication Data Echocardiography in congenital heart disease/[edited by] Mark B. Lewin, Karen Stout. p. ; cm.—(Practical echocardiography series) Includes bibliographical references and index. ISBN 978-1-4377-2696-1 (hardcover : alk. paper) I. Lewin, Mark B. II. Stout, Karen. III. Series: Practical echocardiography series. [DNLM: 1. Echocardiography—methods—Handbooks. 2. Heart Defects, Congenital— ultrasonography—Handbooks. WG 39] LC classification not assigned 618.92'1207543—dc23 2011036498 Senior Acquisitions Editor: Dolores Meloni Editorial Assistant: Brad McIlwain Publishing Services Manager: Pat Joiner-Myers Project Manager: Marlene Weeks Designer: Steven Stave Working together to grow libraries in developing countries Printed in the United States of America. www.elsevier.com | www.bookaid.org | www.sabre.org Last digit is the print number: 9 8 7 6 5 4 3 2 1 Contributors Peter J. Cawley, MD, FACC Denise Joffe, MD Acting Assistant Professor of Medicine, Univer­ Associate Professor of Anesthesiology, Depart­ sity of Washington School of Medicine; Attend­ ment of Anesthesiology, University of Washing­ ing Cardiologist, University of Washington ton School of Medicine; Seattle Children’s Medical Center, Seattle, Washington Hospital, Seattle, Washington Thromboembolic Phenomena and Vegetations Intraoperative Transesophageal Echocardiography Nadine F. Choueiter, MD Troy Johnston, MD Pediatric Cardiology Fellow, University of Wash­ Associate Professor of Pediatrics, University of ington School of Medicine; Seattle Children’s Washington School of Medicine; Attending Hospital, Seattle, Washington Cardiologist, Seattle Children’s Hospital, Echocardiographic Imaging of Single-Ventricle Seattle, Washington Lesions Echocardiography in the Cardiac Catheterization Laboratory Raylene M. Choy, RDCS Cardiac Sonographer, Heart Center, Seattle Chil­ Mariska Kemna, MD dren’s Hospital, Seattle, Washington Assistant Professor of Pediatrics, University of Echocardiographic Imaging of Single-Ventricle Washington School of Medicine; Attending Lesions Cardiologist, Seattle Children’s Hospital, Seattle, Washington Myocardial Pathology Jeffrey A. Conwell, MD Echocardiographic Assessment After Heart Associate Professor of Pediatrics, Division of Transplantation Pediatric Cardiology, University of Washington School of Medicine; Attending Cardiologist, Seattle Children’s Hospital, Seattle, Wash­ Joel Lester, RDCS ington Atrioventricular Septal Defect: Echocardiographic Echocardiography Laboratory Supervisor, Heart Assessment Center, Seattle Children’s Hospital, Seattle, Kawasaki Disease: Echocardiographic Assess- Washington ment The Pediatric Transthoracic Echocardiogram Brandy Hattendorf, MD Mark B. Lewin, MD Assistant Professor of Pediatrics, University of Professor and Chief, Division of Pediatric Car­ Washington School of Medicine; Attending diology, University of Washington School of Cardiologist, Seattle Children’s Hospital, Medicine; Heart Center Co­Director and Direc­ Seattle, Washington tor of Pediatric Echocardiography, Seattle Chil­ Shunting Lesions dren’s Hospital, Seattle, Washington Implications of Pediatric Renal, Endocrine, and The Pediatric Transthoracic Echocardiogram Oncologic Disease Right Heart Anomalies v vi    Contributors Maggie L. Likes, MD Brian D. Soriano, MD Assistant Professor of Pediatrics, University of Assistant Professor of Pediatrics, University of Washington School of Medicine; Attending Washington School of Medicine; Attending Cardiologist, Seattle Children’s Hospital, Cardiologist, Seattle Children’s Hospital, Seattle, Washington Seattle, Washington Right Heart Anomalies Venous Anomalies Left Heart Anomalies Thromboembolic Phenomena and Vegetations David S. Owens, MD Karen Stout, MD Acting Assistant Professor of Medicine, Univer­ sity of Washington School of Medicine; Attend­ Director, Adult Congenital Heart Disease ing Cardiologist, University of Washington Program, Associate Professor, Departments of Medical Center, Seattle, Washington Medicine and Pediatrics, University of Wash­ Left Heart Anomalies ington School of Medicine; Attending Cardiolo­ Myocardial Pathology gist, University of Washington Medical Center and Seattle Children’s Hospital, Seattle, Washington Amy H. Schultz, MD Assistant Professor of Pediatrics, University of Margaret M. Vernon, MD Washington School of Medicine; Attending Assistant Professor of Pediatrics, University of Cardiologist, Seattle Children’s Hospital, Washington School of Medicine; Attending Seattle, Washington Cardiologist, Seattle Children’s Hospital, Conotruncal Lesions Seattle, Washington Transposition of the Great Arteries The Fetal Echocardiogram Foreword Echocardiography is a core component of every principles presented in the Textbook of Clinical aspect of clinical cardiology and now plays an Echocardiography, fourth edition, and can be used essential role in daily decision making. Both echo- as a supplement to that text or can be used by cardiographers and clinicians face unique chal- physicians interested in a focused introduction lenges in interpretation of imaging and Doppler to echocardiography in their area of clinical data and in integration of these data with other practice. clinical information. However, with the absorp- Patients with congenital heart disease are tion of echocardiography into daily patient care, increasingly encountered in clinical practice due there are several unmet needs in our collective to the success of surgical and medical treatment knowledge base. First, clinicians caring for of these conditions, allowing survival into adult- patients need to understand the value, strengths, hood. At the same time, each of us may see only and limitations of echocardiography relevant to a few cases of each type of congenital heart disease their specific scope of practice. Second, echocar- because of the wide range of congenital lesions diographers need a more in-depth understanding and the variety of surgical repair techniques. of the clinical context of the imaging study. Thus, easily accessible and concise information is Finally, there often are unique aspects of data needed when these patients are seen to ensure acquisition and analysis in different clinical that the echocardiographic study is performed and situations, all of which are essential for accurate interpreted correctly. echocardiographic diagnosis. The books in the The editors of Echocardiography in Congenital Practical Echocardiography Series are aimed at Heart Disease, Mark B. Lewin and Karen Stout, filling these knowledge gaps, with each book are recognized experts with robust clinical experi- focusing on a specific clinical situation in ence that includes pediatric, adolescent, and which echocardiographic data are key for optimal adult patients with congenital heart disease. In patient care. this book the editors provide a comprehensive In addition to Echocardiography in Congenital discussion of echocardiography in the patient with Heart Disease, edited by Mark B. Lewin, MD, and congenital heart disease, spanning the entire age Karen Stout, MD, other books in the series are range from birth to old age. This book is aimed Intraoperative Echocardiography, edited by Donald at all clinicians who care for patients with congeni- C. Oxorn, MD; Echocardiography in Heart Failure, tal heart disease, whether in the pediatric or edited by Martin St. John Sutton, MD, and Susan adult setting, including cardiologists, cardiology E. Wiegers, MD; and Advanced Approaches in Echo- fellows, cardiac sonographers, anesthesiologists, cardiography, edited by Linda D. Gillam, MD, and and cardiac surgeons. myself. Information is presented as concise bul- The wealth of information provided in this leted text accompanied by numerous illustrations book is truly awesome. Every clinician who sees and tables, providing a practical approach to data patients with congenital heart disease and every acquisition and analysis, including technical echocardiography laboratory will want a copy details, pitfalls, and clinical interpretation, supple- close at hand. mented by web-based video case examples. Each volume in this series expands on the basic Catherine M. Otto, MD vii Preface This text is one of four in the Practical Echocar- chapters devoted to congenital transesophageal diography Series, which covers the range of echo­ echo as well as echo imaging in the cardiac cath­ cardiographic topics. The topics in the other three eterization laboratory. volumes in this set include Intraoperative Echocar- Each chapter includes a step-by-step approach to diography, Echocardiography in Heart Failure, and patient examination, bulleted points of major prin- Advanced Approaches in Echocardiography. This ciples, and lists of key points. Those areas where volume provides a resource for those interested echo can serve as a resource for accurately working in pediatric and adult congenital echocardio­ through a differential diagnosis are also pointed graphy. The chapters are designed to review out. Methods regarding quantitative data analysis basic principles, provide details of image acquisi­ and calculations are also included. Numerous tion and interpretation, and describe how echo­ echo images and illustrations with detailed figure cardiography is used to develop management legends demonstrate important principles. This strategies. book does not replace formal training in pediatric This book will be of interest to cardiology and congenital echocardiography but rather serves and sonographer trainees, as well as practicing as a supplement to this training. Accredited train­ cardiologists and sonographers, as an overview ing is the only method of obtaining all the tools of pediatric and congenital echocardiography. needed to obtain accurate echocardiographic data, The chapters cover general pediatric echo imaging and we fully endorse this process. protocols, individual congenital cardiac diagnoses, cardiomyopathies, and other pediatric organ system disorders in which cardiac structural or Mark B. Lewin, MD functional assessment is necessary. There are also Karen Stout, MD ix Acknowledgments We could never have completed this work if not Zwink, RDCS; Pamela Clark, RDCS; Sarah for the dedication and skills of our authors. The Curtis, RDCS; Jennifer Gregov, RDCS; Carol cardiac sonographers at Seattle Children’s Hospi- Kraft, RDCS; Chris McKenzie, RDCS; Joannalyn tal and the University of Washington deserve rec- Sangco, RDCS; and Rebecca G. Schwaegler, ognition for their commitment to superb imaging RDCS. Special thanks to Catherine Otto, MD, and the dedication they show to patients, families, for her careful attention to detail and dedication and their colleagues. From Seattle Children’s to this project. We also wish to acknowledge these include Heidi Borchers, RDCS; Colleen Natasha Andjelkovic, Bradley McIlwain, and Cailes, RDCS; Raylene Choy, RDCS; Mikki Marla Sussman at Elsevier, who kept us on track Clouse, RDCS; Judy Devine, RDCS; Alison Free- and on time. berg, RDCS; Laura Huntley, RDCS; Mary Jordan, Of course, finally (and most importantly) the RDCS; Joel Lester, RDCS; Danielle Saliba, unwavering support of our families cannot be RDCS; Pauline Suon, RDCS; Shelby Thomas- overlooked. Deb, Johanne, Julien, and Cal are Irish, RDCS; and Erin Trent, RDCS. From the always in our hearts! University of Washington these include Caryn D’Jang, RDCS; Michelle Fujioka, RDCS; Yelena Mark B. Lewin, MD Kovalenko, RDCS; Amy Loscher, RDCS; Todd Karen Stout, MD xi Glossary 2C two-chamber view DA ductus arteriosus 4C four-chamber view DAo descending aorta 5C five-chamber view DCM dilated cardiomyopathy 2D two-dimensional DCRV double-chamber right ventricle 3D three-dimensional DILV double-inlet left ventricle A4C apical four-chamber view DKS Damus-Kaye-Stansel (procedure) AA aortic arch DORV double-outlet right ventricle AAO aortic arch obstruction dP/dt rate of change in pressure over time ACC American College of Cardiology DSE dobutamine stress echocardiography AHA American Heart Association dT/dt rate of increase in temperature AI aortic insufficiency d-TGA dextro-transposition of the great arteries ALCAPA anomalous origin of the left coronary  E early diastolic peak velocity artery from the pulmonary artery E′ early diastolic tissue Doppler velocity Ao aorta ECG electrocardiogram APB absent pulmonary valve echo echocardiography AR aortic regurgitation EF ejection fraction aRV atrialized right ventricle EFE endocardial fibroelastastosis AS aortic stenosis; atrial septum ET ejection time ASD atrial septal defect FAC fractional area change ASO arterial switch operation FO foramen ovale AV atrioventricular; aortic valve FS fractional shortening AVC atrioventricular canal; aortic valve closure GOS Great Ormond Street AVR aortic valve replacement GV great vessel AVS atrioventricular septum HCM hypertrophic cardiomyopathy AVSD atrioventricular septal defect HIV human immunodeficiency virus AVV atrioventricular valve HLHS hypoplastic left heart syndrome AVVR atrioventricular valve regurgitation HR heart rate bpm beats per minute IAS interatrial septum BSA body surface area ICE intracardiac echocardiography BT Blalock-Taussig ILB inferior limbic bands BVF bulboventricular foramen IVA isovolumic acceleration cc-TGA congenitally corrected transposition of  IVC inferior vena cava; isovolumic contraction the great arteries IVCT isovolumic contraction time CFD color flow Doppler IVRT isovolumic relaxation time CHD congenital heart disease IVS interventricular septum; intact ventricular  CI confidence interval system CM cardiomyopathy IVSD inlet ventricular septal defect CMR cardiac magnetic resonance imaging LA left atrium CoA coarctation of the aorta LAA left atrial appendage CPB cardiopulmonary bypass LAD left descending artery CS coronary sinus LAE left atrial enlargement CT computed tomography LAX long axis view CW continuous wave LCA left coronary artery Cx circumflex coronary artery LCC left coronary cusp xv