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Echocardiography in Pediatric and Adult Congenital Heart Disease S E C O N D E D I T I O N EDITED BY Benjamin W. Eidem, MD, FACC, FASE Professor of Pediatrics and Medicine Divisions of Pediatric Cardiology and Cardiovascular Diseases College of Medicine, Mayo Clinic Rochester, Minnesota Patrick W. O’Leary, MD, FACC, FASE Professor of Pediatrics and Medicine Divisions of Pediatric Cardiology and Cardiovascular Diseases College of Medicine, Mayo Clinic Rochester, Minnesota Frank Cetta, MD, FACC, FASE Professor of Pediatrics and Medicine Divisions of Pediatric Cardiology and Cardiovascular Diseases College of Medicine, Mayo Clinic Rochester, Minnesota 91226_fm_pi-x.indd 1 08/08/14 3:36 PM Acquisitions Editor: Julie Goolsby Product Development Editor: Andrea Vosburgh Production Project Manager: Alicia Jackson Design Coordinator: Teresa Mallon Manufacturing Coordinator: Beth Welsh Marketing Manager: Stephanie Manzo Kindlick Prepress Vendor: Integra Software Services Pvt.Ltd. Second edition Copyright © 2015 Wolters Kluwer Health Copyright © 2010 Wolters Kluwer Health / Lippincott Williams & Wilkins. All rights reserved. This book is protected by copyright. No part of this book may be reproduced or transmitted in any form or by any means, including as photocopies or scanned-in or other electronic copies, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright. To request permission, please contact Wolters Kluwer Health at Two Commerce Square, 2001 Market Street, Philadelphia, PA 19103, via email at permissions@ lww.com, or via our website at lww.com (products and services). 9 8 7 6 5 4 3 2 1 Printed in China Library of Congress Cataloging-in-Publication Data Echocardiography in pediatric and adult congenital heart disease / [edited by] Benjamin W. Eidem, Patrick W. O’Leary, Frank Cetta. — Second edition. p. ; cm. Includes bibliographical references and index. ISBN 978-1-4511-9122-6 (hardback) I. Eidem, Benjamin W., editor. II. O’Leary, Patrick W., editor. III. Cetta, Frank, editor. [DNLM: 1. Heart Defects, Congenital—ultrasonography. 2. Echocardiography—methods. WG 220] RC687 616.1'207543—dc23 2014017820 This work is provided “as is,” and the publisher disclaims any and all warranties, express or implied, including any warranties as to accuracy, comprehensiveness, or currency of the content of this work. This work is no substitute for individual patient assessment based upon healthcare professionals’ examination of each patient and consideration of, among other things, age, weight, gender, current or prior medical conditions, medication history, laboratory data and other factors unique to the patient.  The publisher does not provide medical advice or guidance and this work is merely a reference tool.  Healthcare professionals, and not the publisher, are solely responsible for the use of this work includ- ing all medical judgments and for any resulting diagnosis and treatments.  Given continuous, rapid advances in medical science and health information, independent pro- fessional verification of medical diagnoses, indications, appropriate pharmaceutical selections and dosages, and treatment options should be made and healthcare professionals should consult a vari- ety of sources. When prescribing medication, healthcare professionals are advised to consult the product information sheet (the manufacturer’s package insert) accompanying each drug to verify, among other things, conditions of use, warnings and side effects and identify any changes in dosage schedule or contradictions, particularly if the medication to be administered is new, infrequently used or has a narrow therapeutic range. To the maximum extent permitted under applicable law, no responsibility is assumed by the publisher for any injury and/or damage to persons or property, as a matter of products liability, negligence law or otherwise, or from any reference to or use by any person of this work. LWW.com 91226_fm_pi-x.indd 2 08/08/14 3:36 PM C O N T R I B U T O R S Naser Ammash, MD, FACC, FASE Allison K. Cabalka, MD Benjamin W. Eidem, MD, FACC, Professor of Medicine Associate Professor of Pediatrics FASE Mayo Clinic Mayo Clinic College of Medicine Professor of Pediatrics and Medicine Rochester, Minnesota Rochester, Minnesota Divisions of Pediatric Cardiology and Cardiovascular Diseases Nandan Sadanand Anavekar, Frank Cetta, MD, FACC, FASE College of Medicine, Mayo Clinic MBBCh Professor of Pediatrics and Rochester, Minnesota Assistant Professor of Medicine Medicine Department of Medicine, Division of Divisions of Pediatric Cardiology and Gregory J. Ensing, MD Cardiovascular Diseases Cardiovascular Diseases Clinical Professor of Pediatrics Department of Radiology, Division of College of Medicine, Mayo Clinic University of Michigan Congenital Cardiac Radiology Rochester, Minnesota Heart Center Mayo Clinic C.S. Mott Children’s Hospital Rochester, Minnesota Meryl S. Cohen, MD Ann Arbor, Michigan Associate Professor of Pediatrics Nancy A. Ayres, MD, FACC, FASE Michele A. Frommelt, MD Perelman School of Medicine Medical Director of Non-Invasive Assistant Professor The University of Pennsylvania Imaging Department of Pediatric Cardiology Medical Director Echocardiography Texas Children’s Hospital Medical College of Wisconsin Laboratory Associate, Pediatric Cardiology Director, Fetal Echocardiography Associate Program Director Cardiology Baylor College of Medicine Children’s Hospital of Wisconsin Fellowship Houston, Texas Milwaukee, Wisconsin The Children’s Hospital of Philadelphia Peter Bartz, MD Peter C. Frommelt, MD, FACC, FASE Philadelphia, Pennsylvania Associate Professor of Pediatrics and Professor Medicine Department of Pediatrics Timothy Colen, MBBS, FRACP Divisions of Cardiology and Children’s Hospital of Wisconsin Assistant Clinical Professor Cardiovascular Medicine Medical College of Wisconsin Department of Pediatrics Medical College of Wisconsin Milwaukee, Wisconsin University of Alberta Children’s Hospital of Wisconsin Pediatric Cardiologist Milwaukee, Wisconsin Javier Ganame, MD, PhD Stollery Children’s Hospital Division of Cardiology, Department of Edmonton, Alberta, Canada Stuart Berger, MD Medicine Professor of Pediatrics McMaster University Heidi M. Connolly, MD Division of Pediatric Cardiology Hamilton, Ontario, Canada Professor of Medicine The Leigh Gabrielle Herma Chair for Cardiology Division of Cardiovascular Sarah Gelehrter, MD Medical College of Wisconsin Diseases Associate Professor Medical Director, The Herma Heart Mayo Clinic Division of Pediatric Cardiology Center Rochester, Minnesota University of Michigan Congenital Children’s Hospital of Wisconsin Heart Center Milwaukee, Wisconsin David J. Driscoll, MD C.S. Mott Children’s Hospital Professor of Pediatrics Ann Arbor, Michigan Crystal R. Bonnichsen, MD Departments of Cardiovascular Diseases Instructor in Medicine and Pediatrics Edmund Gillis, BS, RDCS Division of Cardiovascular Diseases College of Medicine, Mayo Clinic Mayo Clinic Mayo Clinic Rochester, Minnesota Rochester, MN Rochester, Minnesota Michael G. Earing, MD Martha Grogan, MD William L. Border, MBChB, MPH Director Consultant Director of Noninvasive Cardiac Imaging Wisconsin Adult Congenital Heart Cardiovascular Diseases Children’s Healthcare of Atlanta Disease Program Professor of Medicine Emory University School of Medicine Medical College of Wisconsin Mayo Clinic Atlanta, Georgia Milwaukee, Wisconsin Rochester, Minnesota iii 91226_fm_pi-x.indd 3 08/08/14 3:36 PM iv n CONTRIBUTORS Donald J. Hagler, MD Steven E. Lipshultz, MD, FAAP, FAHA Erik C. Michelfelder, MD Professor of Pediatrics and Medicine Schotanus Family Endowed Chair of Director, Fetal Heart Program Divisions of Pediatric Cardiology and Pediatrics Co-Director, Cardiac Imaging Cardiovascular Diseases Professor and Chair, Carman and Ann The Heart Institute College of Medicine, Mayo Clinic Adams Department of Pediatrics Cincinnati Children’s Hospital Medical Rochester, Minnesota Professor of Medicine (Cardiology), Center Oncology, Obstetrics and Gynecology, Professor of Pediatrics Lindsey E. Hunter, MBChB, MRCPCH Molecular Biology and Genetics, Family University of Cincinnati College of Fetal Cardiology Unit Medicine and Public Health Sciences, Medicine Department of Congenital Heart and Pharmacology Cincinnati, Ohio Disease Wayne State University School of Fletcher A. Miller, Jr., MD, FASE, Evelina Children’s Hospital Medicine FACC, FAHA Guy’s and St. Thomas’ NHS Trust President, University Pediatricians London, United Kingdom Pediatrician-in-Chief Professor of Medicine Children’s Hospital of Michigan Mayo Clinic College of Medicine Jennifer A. Johnson, MD Interim Director Consultant Assistant Professor of Pediatrics Children’s Research Center of Michigan Division of Cardiovascular Diseases University of Pittsburgh School of Detroit, Michigan Mayo Clinic Medicine Rochester, Minnesota Children’s Hospital of Pittsburgh of Leo Lopez, MD, FACC, FAAP, FASE L. LuAnn Minich, MD UPMC Director of Pediatric Cardiac Professor of Pediatrics Pittsburgh, Pennsylvania Noninvasive Imaging Division of Pediatric Cardiology Children’s Hospital at Montefiore Jonathan N. Johnson, MD, FAAP, Associate Professor of Clinical University of Utah FASE Pediatrics Salt Lake City, Utah Associate Professor Albert Einstein College of Medicine Jae K. Oh, MD Department of Pediatrics New York, New York Professor of Medicine Mayo Clinic College of Medicine Division of Cardiovascular Diseases Medical Director, Pediatric Heart Joseph Mahgerefteh, MD Mayo Clinic Transplantation Pediatric Cardiology Division Rochester, Minnesota Senior Associate Consultant The Children’s Hospital at Montefiore Department of Pediatrics, Division of Albert Einstein College of Medicine Patrick W. O’Leary, MD, FACC, FASE Pediatric Cardiology New York, New York Professor of Pediatrics and Medicine Mayo Clinic Divisions of Pediatric Cardiology Rochester, Minnesota Joseph J. Maleszewski, MD and Cardiovascular Diseases Associate Professor of Pathology and College of Medicine, Mayo Clinic Frederick D. Jones, RDCS Medicine Rochester, Minnesota Senior Pediatric Echocardiographer College of Medicine Kentucky Children’s Hospital Consultant, Divisions of Anatomic Angira Patel, MD, MPH Lexington, Kentucky Pathology and Cardiovascular Diseases Assistant Professor of Pediatrics Mayo Clinic Pediatric Cardiology Thomas R. Kimball, MD Rochester, Minnesota Ann and Robert H. Lurie Children’s Professor of Pediatrics Hospital of Chicago University of Cincinnati College of Colin J. McMahon,MB, BCh, DCH, Chicago, Illinois Medicine FRCPI, FAAP Medical Director, Heart Institute Consultant Pediatric Cardiologist Sabrina Phillips, MD, FACC, FASE Cincinnati Children’s Hospital Our Lady’s Hospital for Sick Children Consultant Cincinnati, Ohio Crumlin, Dublin, Ireland Division of Cardiovascular Diseases Mayo Clinic John P. Kovalchin, MD Shaji C. Menon, MD Rochester, Minnesota Director of Echocardiography Assistant Professor of Pediatrics Director of Advanced Noninvasive Adjunct Assistant Professor of Radiology Ricardo H. Pignatelli, MD Cardiac Imaging Fellowship Department of Pediatric Cardiology Assistant Professor of Pediatrics The Heart Center University of Utah Texas Children’s Hospital Nationwide Children’s Hospital Salt Lake City, Utah Baylor College of Medicine Professor of Pediatrics Houston, Texas The Ohio State University Luc Mertens, MD, PhD Columbus, Ohio Professor in Pediatrics Joseph T. Poterucha, DO The University of Toronto Assistant Professor of Pediatrics Robert Lichtenberg, MD Section Head, Echocardiography Division of Pediatric Cardiology Heart Care Centers of Illinois The Hospital for Sick Children Mayo Clinic College of Medicine Berwyn, Illinois Toronto, Ontario, Canada Rochester, Minnesota 91226_fm_pi-x.indd 4 08/08/14 3:36 PM n CONTRIBUTORS v Muhammad Yasir Qureshi, MBBS John M. Simpson, MD, FRCP, FESC Lloyd Y. Tani, MD Assistant Professor of Pediatrics Professor of Paediatric and Fetal Professor of Pediatrics Division of Pediatric Cardiology Cardiology Division Chief, Pediatric Cardiology Mayo Clinic College of Medicine Department of Congenital Heart University of Utah School of Medicine Rochester, Minnesota Disease Co-Director, Heart Center Evelina Children’s Hospital Primary Children’s Medical Center Bernadette Richards, RDCS London, United Kingdom Salt Lake City, Utah Technical Director Echocardiography Department Dongngan T. Truong, MD Jeffrey F. Smallhorn, MBBS, FRACP, Nationwide Children’s Hospital FRCP(C) Pediatric Cardiology Fellow Columbus, Ohio Department of Pediatrics Professor University of Utah Ritu Sachdeva, MD, MBBS, FACC, Department of Pediatrics Salt Lake City, Utah FAAP University of Alberta Associate Professor Stollery Children’s Hospital Himesh V. Vyas, MD Emory University School of Medicine Edmonton, Alberta, Canada University of Florida Director, Cardiovascular Imaging Gainesville, Florida Research Core Brian D. Soriano, MD, FASE Children’s Healthcare of Atlanta Associate Professor Luciana T. Young, MD Sibley Heart Center Department of Pediatrics Associate Professor Atlanta, Georgia Adjunct Associate Professor Department of Pediatrics Department of Radiology Northwestern University Feinberg School Amy H. Schultz, MD, MS University of Washington School of of Medicine Assistant Professor of Pediatrics Medicine Director, Echocardiography Laboratory University of Washington School of Seattle Children’s Heart Center Ann & Robert H. Lurie Children’s Medicine Seattle, Washington Hospital of Chicago Attending Physician Chicago, Illinois Division of Cardiology Ronald Springer, BS, RDCS Seattle Children’s Hospital Mayo Clinic Robert Young, BA, RDCS Seattle, Washington Rochester, Minnesota Cardiac Sonographer Mayo Clinic Girish S. Shirali, MBBS, FACC, FASE Rochester, Minnesota Melva and Randall L. O’Donnell, PhD Nathaniel W. Taggart, MD Chair in Pediatric Cardiology Assistant Professor of Pediatrics Division Director, Cardiology Mayo Clinic College of Medicine Co-Director Consultant, Division of Pediatric The Ward Family Heart Center Cardiology The Children’s Mercy Hospital and Clinics Department of Pediatric and Adolescent Professor, Department of Pediatrics Medicine University of Missouri, Kansas City Mayo Clinic Kansas City, Missouri Rochester, Minnesota 91226_fm_pi-x.indd 5 08/08/14 3:36 PM P R E F A C E know that his greatest contributions were in education and mentorship. Bill is one of the most delightful lectur- ers and has the gift to make any topic interesting. One of his most recent contributions “A Light-Hearted Look …” had his usual mix of rigorous anatomic detail and humor (see figures on the next page). He is able to distill very logical and straightforward messages from complex ana- tomic details and nomenclature. Bill Edwards instilled in many echocardiographers the mantra of describing what one sees in precise and accurate detail with simple jargon. He is not a disciple of “tomb- stone medicine” whereby one loosely utilizes jargon which many times is an uninterpretable code. Bill spent much of his career crafting echocardiographers into noninva- sive junior pathologists. Many of the pathology images included in the 1st and 2nd editions of this textbook are from the extensive image library that Bill created over decades of devotion to this field. Frequently, our task in the echocardiography laboratory is to create images which accurately reflect Bill’s pathologic specimens. This exer- cise enhanced our imaging skills and our understanding of the patient’s anatomy and physiology. In this way, Bill impacted the clinical practice on a daily basis. We are indebted to Bill for his friendship, professional- ism and talent. He understood 3½ decades ago the impor- The 2nd edition of Echocardiography in Pediatric and tance of transitioning from “bench to bedside.” Through Bill’s detailed work on anatomic specimens, he improved Adult Congenital Heart Disease is dedicated to William the clinical acumen of many cardiologists. Included in this D. Edwards, M.D., the congenital cardiac pathologist preface are some images from Bill’s famous talks. Bill is at Mayo Clinic for the last 34 years. Bill will be retir- renowned for his analogies: why a right ventricle is like a ing from Mayo at the end of 2014. Over his career Bill strawberry (see figures on the next page), why an atrio- made tremendous contributions to the field of congenital ventricular septal defect looks like Batman, why it is an cardiology. He was the recipient of numerous teaching “illustration” or a “diagram” and not a “cartoon.” awards and he participated in the publication of over 250 Thank you, Bill. Your decades of devotion to the field of peer-reviewed manuscripts. He authored numerous book congenital cardiac imaging are on display in this textbook. chapters and co-edited, in 1987, the original congenital It would have been impossible to produce this text without echocardiography textbook when he teamed with Drs. A. your tireless efforts. Jamil Tajik, James Seward, and Donald Hagler. Although Bill made numerous contributions to the literature, those who had the privilege to work with him at Mayo Clinic Ben Eidem, Pat O’Leary, Frank Cetta vi 91226_fm_pi-x.indd 6 08/08/14 3:36 PM n PREFACE vii 91226_fm_pi-x.indd 7 08/08/14 3:36 PM A C K N O W L E D G M E N T S To Jori, Katie, Gannon, and Matthew for all of your love and support. —BWE Cate, Mike, and Matt Cetta, thanks for the sacrifices you have made for my career. You guys are stars! —FC To my mentors, patients, and, most of all, my family. Thank you for the examples you have provided, the patience you have shown, and the love you have given. —PWO The editors would like to sincerely thank Mark Zangs and Jeff Stelley for their significant efforts in the preparation of many of the figures and videos throughout this textbook. viii 91226_fm_pi-x.indd 8 08/08/14 3:36 PM C O N T E N T S Contributors iii 13 Abnormalities of Right Preface vi Ventricular Outflow .................. 250 Acknowledgments viii Erik C. Michelfelder and William L. Border 1 Principles of Cardiovascular Ultrasound... 1 14 Abnormalities of Left Ventricular Outflow ............................. 272 Robert Young and Patrick W. O’Leary Leo Lopez and Joseph Mahgerefteh 2 Practical Issues Related to the Examination, 15 Tetralogy of Fallot .................... 305 Anatomic Image Orientation, and Segmental Himesh Vyas, Jennifer Johnson and Benjamin W. Eidem Cardiovascular Analysis ................ 12 Edmund Gillis, Ronald Springer, and Patrick W. O’Leary 16 d-Transposition of the Great Arteries ....320 3 Quantitative Methods in Echocardiography— Amy H. Schultz and Brian D. Soriano Basic Techniques ...................... 38 17 Double-Outlet Right and Benjamin W. Eidem and Patrick W. O’Leary Left Ventricles ....................... 344 4 Quantitative Methods in Echocardiography— Donald J. Hagler Advanced Techniques for the Assessment of 18 Truncus Arteriosus ................... 356 Ventricular Function................... 62 Bernadette Richards, Frederick D. Jones, and John P. Kovalchin Javier Ganame and Luc Mertens 19 Patent Ductus Arteriosus and 5 Anomalies of the Pulmonary and Systemic Aortopulmonary Window ............. 364 Venous Connections................... 91 Ritu Sachdeva Frank Cetta and Naser M. Ammash 20 Abnormalities of the Aortic Arch ....... 374 6 Abnormalities of Atria and Angira Patel and Luciana T. Young Atrial Septation ...................... 111 21 Marfan Syndrome: Aortic Aneurysm Allison K. Cabalka and Dissection ....................... 388 7 Atrioventricular Septal Defects ......... 131 Crystal R. Bonnichsen and Heidi M. Connolly Joseph T. Poterucha, Joseph J. Maleszewski, Patrick W. O’Leary, and 22 Hypertrophic Cardiomyopathy......... 405 Frank Cetta Patrick W. O’Leary 8 Ebstein’s Malformation and Tricuspid Valve 23 Additional Cardiomyopathies.......... 417 Diseases............................. 146 Colin J. McMahon and Ricardo H. Pignatelli Patrick W. O’Leary 24 Pericardial Disorders.................. 431 9 Echocardiographic Assessment of Mitral Martha Grogan and Jae K. Oh Valve Abnormalities .................. 166 25 Systemic Diseases..................... 446 Timothy Colen and Jeffrey F. Smallhorn Muhammad Yasir Qureshi and Steven E. Lipshultz 10 Congenitally Corrected Transposition of the 26 Vascular Abnormalities ............... 470 Great Arteries........................ 185 Michele A. Frommelt and Peter C. Frommelt Michael G. Earing, Nancy A. Ayres, and Frank Cetta 27 Cardiac Tumors...................... 486 11 Ventricular Septal Defects ............. 198 Joseph J. Maleszewski and Frank Cetta Sarah Gelehrter and Gregory Ensing 28 Evaluation of the Transplanted Heart ... 495 12 Univentricular Atrioventricular Jonathan N. Johnson Connections ......................... 220 29 Pulmonary Hypertension.............. 505 Shaji C. Menon and Allison K. Cabalka Peter Bartz and Stuart Berger ix 91226_fm_pi-x.indd 9 08/08/14 3:36 PM x n CONTENTS 30 Echocardiography in the Diagnosis and 38 Cardiac Magnetic Resonance and Computed Management of Endocarditis........... 515 Tomographic Imaging in Congenital Heart Dongngan T. Truong, L. LuAnn Minich and Lloyd Y. Tani Disease.............................. 628 31 Evaluation of Prosthetic Valves......... 528 Muhammad Yasir Qureshi, Crystal R. Bonnichsen, and Nandan S. Anavekar Sabrina Phillips, Fletcher Miller and Frank Cetta 39 Evaluation of the Adult with Transposition 32 Fetal Echocardiography ............... 537 after Atrial or Arterial Switch Operations 655 John M. Simpson and Lindsey E. Hunter Frank Cetta, David J. Driscoll and Benjamin W. Eidem 33 Three-Dimensional Echocardiography in 40 Tetralogy of Fallot with Pulmonary Congenital Heart Diseases ............. 554 Regurgitation ........................ 666 Girish S. Shirali Naser M. Ammash 34 Stress Echocardiography............... 566 41 Echocardiographic Evaluation of the Thomas R. Kimball Functionally Univentricular Heart after 35 Intracardiac and Intraoperative Fontan “Operation”................... 677 Transesophageal Echocardiography..... 578 Sabrina D. Phillips and Patrick W. O’Leary Donald J. Hagler 42 Eisenmenger Syndrome ............... 697 36 Interventional Echocardiography in Robert C. Lichtenberg and Frank Cetta Congenital Heart Disease.............. 604 Index 707 Nathaniel W. Taggart 37 Echocardiographic Assessment of Mechanical Circulatory Support...... 620 Jonathan N. Johnson and Meryl S. Cohen 91226_fm_pi-x.indd 10 08/08/14 3:36 PM

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Most books are stored in the elastic cloud where traffic is expensive. For this reason, we have a limit on daily download.