Echocardiography in Adult Congenital Heart Disease Wei Li, Michael Henein, and Michael A. Gatzoulis Echocardiography in Adult Congenital Heart Disease Wei Li, MD, PhD, FESC, FACC Michael Henein, MSc, PhD, FESC, FACC, FAHA Michael A. Gatzoulis, MD, PhD, FESC, FACC Royal Brompton & Harefi eld NHS Trust Imperial College School of Medicine London UK British Library Cataloguing in Publication Data Li, Wei Echocardiography in adult congenital heart disease 1. Congenital heart disease—Diagnosis 2. Echocardiography I. Title II. Henein, Michael Y., 1959– III. Gatzoulis, Michael A. 616.1′2′043 ISBN-13: 9781846288159 Library of Congress Control Number: 2007921870 ISBN: 978-1-84628-815-9 e-ISBN: 978-1-84628-816-6 Printed on acid-free paper © Springer-Verlag London Limited 2007 Apart from any fair dealing for the purposes of research or private study, or criticism or review, as permitted under the Copyright, Designs and Patents Act 1988, this publication may only be reproduced, stored or transmitted, in any form or by any means, with the prior permission in writing of the publishers, or in the case of reprographic reproduction in accordance with the terms of licences issued by the Copyright Licensing Agency. Enquiries concerning reproduction outside those terms should be sent to the publishers. The use of registered names, trademarks, etc. in this publication does not imply, even in the absence of a specifi c statement, that such names are exempt from the relevant laws and regulations and therefore free for general use. Product liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature. 9 8 7 6 5 4 3 2 1 Springer Science+Business Media springer.com Foreword Due to the success of surgical repair of congenital cardiac defects, the popula- tion of adults with congenital heart defects is increasing rapidly. In fact, it is estimated that there are now more adults than children with congenital heart disease in the United States. Although surgical repair is now relatively safe and survival into adulthood is assured for the majority of infants born with con- genital heart disease, such individuals are not “cured” and usually have a variety of residua and sequelae of the original defect and the surgical repair. Late complications are common and life expectancy remains limited for a large number of these patients. With an increasing population and persistent clinical problems, it is inevi- table that these patients require ongoing cardiac care throughout their life- times. Despite the fact that some regional centers of excellence for the care of the adult with congenital heart disease exist, many such patients present in community hospital and primary care settings. Echocardiography is the defi nitive imaging tool for evaluation of infants and children with congenital heart disease, and remains the starting point for initial evaluation of the adult with congenital heart disease. In conjunction with a physical examination and a complete history, including initial diagnosis and subsequent surgical interventions, an echocardiographic examination is essen- tial in the evaluation of any patient with congenital heart disease. However the professional who performs the echocardiographic study and the physician who interprets the images must understand the terminology of congenital heart disease and recognize the characteristic images and fl ow patterns associated with the various lesions encountered in this patient population. Drs. Li, Henein, and Gatzoulis are internationally renown experts from the Royal Brompton National Heart and Lung Hospital, arguably the premier adult congenital cardiac center in the world. They have produced a volume that is neither an encyclopedic compendium nor a massive echocardiographic atlas. It is, rather, a thorough overview of the important aspects of echocardiographic evaluation of adults with congenital heart disease. Nearly every page contains echo images illustrating various views and fi ndings for each type of congenital defect. Echocardiography critically infl uences patient management and this v vi Foreword book puts the echo fi ndings into an appropriate clinical perspective. The authors have also included information from other imaging modalities, such as MRI, which serve as reminders of the complementary role of various techniques in the evaluation and management of this complex patient population. Echocardiography in Adult Congenital Heart Disease is an indispensable reference for anyone involved in imaging patients with congenital heart defects. It will introduce some readers to the subject of adult congenital heart disease and serve as a refresher for others. I hope that it will engage you and encourage you to read more, seek out original sources and increase your knowledge of this important fi eld. This book will serve each of us in our role as lifelong learners and compel us to answer questions, produce new knowledge and teach others. Daniel J. Murphy, Jr., MD Stanford, California May 2007 Preface Doppler echocardiography has become the mainstay of adult cardiology prac- tice worldwide. It provides essential information on cardiac anatomy and phys- iology. Its ease of use and complementary role to the bed-side examination has even given it the name of the “imaging stethoscope.” In Echocardiography in Adult Congenital Heart Disease, we present our clin- ical experience in applying echocardiographic techniques specifi cally to the diagnosis and management of adult patients with congenital heart disease. Wherever possible, we have tried to provide suggestions for the adaptation of standard imaging protocols to accommodate specifi c congenital heart condi- tions. The extremely varied morphologic spectrum of congenital heart disease, however, reinforces the need for describing individual components of anatomy, that is, the atrioventricular and ventriculoarterial connections, and this cannot be overemphasized for complex lesions. Our practical textbook provides an anatomical basis for these malformed hearts, together with a discussion of the physiological patterns seen in different conditions. Our focus has been on contemporary clinical practice. We have, thus, related imaging to the clinical status of the patient, and provided informa- tion on disease progression and the effects of further intervention whether surgical or catheter. We hope that we have given a suffi cient number of examples of the many different conditions and variations on physiology to have written a useful guide for the diagnosis and management of your patients with congenital heart disease. We have made this book a focused manual, which can be kept by the echo machine for easy reference, rather than a large textbook, which might gather dust on a shelf. This means that we have, to some extent, sacrifi ced additional information, which would have had less impact on day-to-day prac- tice and would have made it more diffi cult to follow. We have also used termi- nology and descriptions with which both paediatric and adult congenital heart disease practitioners will be acquainted. We are indebted to our patients who gave us such an opportunity to improve our understanding of congenital heart disease; clearly, without them this book would not have been possible. vii viii Preface We would value a great deal of your feedback as to what else you would like to see included in the second edition and any comments on how we can make this focused textbook better for your day-to-day echocardiographic practice. Wei Li Michael Henein Michael A. Gatzoulis London, UK Contents Foreword by Daniel J. Murphy, Jr. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii 1 Septal Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 1.1. Interatrial Communication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 1.1.1. Secundum Atrial Septal Defect . . . . . . . . . . . . . . . . . . . . . . 1 1.1.2. Primum Atrial Septal Defect . . . . . . . . . . . . . . . . . . . . . . . . 8 1.1.3. Superior Vena Cava Defect . . . . . . . . . . . . . . . . . . . . . . . . . 10 1.1.4. Inferior Vena Cava Defect . . . . . . . . . . . . . . . . . . . . . . . . . . 13 1.1.5. Coronary Sinus Defect . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 1.1.6. Confl uent or Common Atrium . . . . . . . . . . . . . . . . . . . . . . 13 1.1.7. Management of Atrial Septal Defects . . . . . . . . . . . . . . . . 14 1.2. Ventricular Septal Defect . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 1.2.1. Classifi cation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 1.2.2. Complications of Ventricular Septal Defect . . . . . . . . . . . 17 1.2.3. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 1.2.4. Intraoperative Echocardiography . . . . . . . . . . . . . . . . . . . 21 1.3. Atrioventricular Septal Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 1.3.1. Echocardiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 1.3.2. Follow Up . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 2 Left Ventricular Infl ow Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . 25 2.1. Cor Triatriatum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25 2.1.1. Echocardiographic Examination . . . . . . . . . . . . . . . . . . . . 25 2.2. Supramitral Valve Membrane . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 2.2.1. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 2.3. Mitral Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 2.3.1. Double Orifi ce Mitral Valve . . . . . . . . . . . . . . . . . . . . . . . . 27 2.3.2. Parachute Mitral Valve . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 2.4. Mitral Valve Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 2.4.1. Assessment of Mitral Valve Regurgitation . . . . . . . . . . . . 29 ix x Contents 2.5. Partial Anomalous Pulmonary Venous Connections . . . . . . . . 37 2.5.1. Subcostal Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37 2.5.2. Parasternal Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 2.5.3. Suprasternal Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 2.6. Seimitar Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 2.6.1. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 3 Left Ventricular Outfl ow Tract Lesions . . . . . . . . . . . . . . . . . . . . . . . . 39 3.1. Valvar Aortic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 3.1.1. Echocardiographic Examination . . . . . . . . . . . . . . . . . . . . 39 3.1.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 3.1.3. Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 3.2. Subvalvar Aortic Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 3.2.1. Echocardiographic Examination . . . . . . . . . . . . . . . . . . . . 45 3.2.2. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45 3.3. Small Aortic Root . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47 3.3.1. Associated Abnormalities . . . . . . . . . . . . . . . . . . . . . . . . . . 47 3.4. Supravalvar Aortic Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49 3.4.1. Echocardiographic Examination . . . . . . . . . . . . . . . . . . . . 49 3.5. Marfan Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50 3.5.1. Echocardiographic Examination . . . . . . . . . . . . . . . . . . . . 50 3.6. Aneurysmal Aortic Root . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52 3.6.1. Sinus of Valsalva Aneurysms and Ruptured Sinus of Valsalva . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53 3.7. Postoperative Aortic Valve . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 3.7.1. Xenograft . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 3.7.2. Aortic Homograft . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56 3.7.3. Aortic Autograft (Ross Procedure) . . . . . . . . . . . . . . . . . . 57 3.7.4. Mechanical Valves . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58 4 Disease of the Aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 4.1. Patent Ductus Arteriosus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 4.1.1. Echocardiographic Examination . . . . . . . . . . . . . . . . . . . . 59 4.2. Coarctation of Aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 4.2.1. Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61 4.3. Interruption of the Aortic Arch . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 4.4. Common Arterial Trunk . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 5 Tricuspid Valve Abnormalities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65 5.1. Ebstein Anomaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65 5.2. Tricuspid Valve Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 5.3. Tricuspid Valve Stenosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 5.4. Double Orifi ce Tricuspid Valve . . . . . . . . . . . . . . . . . . . . . . . . . . . 71