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Wojciech Mazur · Marilyn J. Siegel Tomasz Miszalski-Jamka · Robert Pelberg CT Atlas of Adult Congenital Heart Disease 123 CT Atlas of Adult Congenital Heart Disease Wojciech M azur (cid:129) M arilyn J. Siegel Tomasz Miszalski-Jamka (cid:129) Robert Pelberg CT Atlas of Adult Congenital Heart Disease Wojciech Mazur Robert Pelberg The Christ Hospital Heart and Vascular Center The Christ Hospital Heart and Vascular Center of Greater Cincinnati of Greater Cincinnati The Lindner Center for Research and Education The Lindner Center for Research and Education Cincinnati Cincinnati OH OH USA USA Marilyn J. Siegel Mallinckrodt Institute of Radiology Washington University School of Medicine St. Louis Missouri USA Tomasz Miszalski-Jamka Department of Clinical Radiology and Imaging Diagnostics 4th Military Hospital Wrocław Poland Center for Diagnosis Prevention and Telemedicine John Paul II Hospital Kraków Poland Illustrations by: Michał Bratko, Kraków, Poland ISBN 978-1-4471-5087-9 ISBN 978-1-4471-5088-6 (eBook) DOI 10.1007/978-1-4471-5088-6 Springer London Heidelberg New York Dordrecht Library of Congress Control Number: 2013941382 © Springer-Verlag London 2013 This work is subject to copyright. 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Printed on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com) To my wife, Eugenia, and my children, Andre and Jan Paul, for their love and support during the countless hours it took to create this text. Wojciech Mazur To my husband, Barry, my mentor, colleague, friend and loving spouse for his unwavering support and encouragement to make this project a reality. Marilyn J. Siegel To my marvelous parents, my beloved wife, Ania, and splendid daughters, Ania and Ewa for their tremendous love and constant support in my whole life. I would like also to express my deepest and sincere gratitude to my mentors and tutors Professor Jacek Musiał, Professor Piotr Podolec, Professor Andrzej Szczeklik, and Professor Anetta Undas for their invaluable help and passion to medicine and science. Tomasz Miszalski-Jamka To my wonderful wife, Wendy, and my fantastic children, Josh, Adam and Noah for their never ending devotion and love and for their constant support without which projects like this would never come to fruition. Robert Pelberg In Appreciation We extend a heart felt thank you to Lisa Ambach whose contributions to the editing and orga- nization of this work were immense and without whom this book would not be possible. Thank you, also, to David Collins for his efforts in helping us complete this challenging project. Thank you to Chris Thomson and The Christ Hospital for their fi nancial support of this work. The authors would like to extend a sincere thank you to Mr Robert Banyś and Drs. Frandics Chan, Joao Carlos Costa, Kelly Han, Anthony Hlavacek, Eric Kimura-Hayama, Małgorzata Irzyk, Piotr Klimeczek, Maciej Krupiński, Bartosz Laskowicz, R. Rajesh Kannan, Vasco Silva, and Małgorzata Urbańczyk for their generous contributions in providing images for this work. vii Preface Congenital heart disease (CHD) with a worldwide incidence of 0.8 % represents the most com- mon class of inborn birth defects. Improvements in surgical techniques and medical manage- ment result in an increased life expectancy and lead to a greater number of children with CHD surviving into adulthood. It is now estimated that at least 85 % of children with CHD will survive to adulthood, and many of these patients will require follow-up medical care as an adult [1, 2]. Consequently, adult cardiologists will have the opportunity to care for increasing numbers of patients with congenital heart defects. In many instances, these patients have undergone pallia- tive but not corrective procedures and may be at higher risk for the development of congestive heart failure, cardiac arrhythmia, and overall cardiovascular morbidity and mortality. Furthermore, as this adult congenital heart disease population ages, their risk of developing coronary artery disease is similar or in certain instances higher than the age- and risk-matched population. The amount of time devoted to congenital heart disease during adult cardiovascular fellowships and radiology residencies is rather modest. Furthermore, most cardiologists and radiologists have limited experience in imaging adult patients with complex CHD. In addition, patient care is not infrequently transferred from pediatric to adult cardiologists who may not be adequately prepared to provide care for these patients. Moreover, the cardiologist or radi- ologist may encounter an adult patient presenting with unknown, corrected, or palliated CHD. Therefore, it is critical that they be familiar with the imaging appearances of corrected and uncorrected CHD classes and of their potential long-term complications. Traditionally, invasive angiography was used to evaluate these patients. As imaging tech- nology has advanced, noninvasive techniques such as echocardiography, magnetic resonance imaging (MRI), and multidetector computed tomographic angiography (CTA) are applicable. Despite its relatively low cost, portability, accessibility, and lack of radiation exposure, echocardiography is often limited in its ability to completely evaluate complex native and palliated CHD due to its operator dependency and potential lack of adequate acoustic windows. In addition, extracardiac structures such as the pulmonary arteries, pulmonary veins, and aortic arch are dif fi cult to evaluate with echocardiography. For these reasons, MRI is superior to echocardiography but may be challenging due to prolonged imaging times and to the presence of image degrading metal implants (embolization coils and intravascular stents). Moreover, MRI is contraindicated in patients with pacemakers and de fi brillators, which are frequently implanted in long-term survivors of CHD. Furthermore, the diagnostic capabilities of MRI are limited for the evaluation of the airways and lungs. In the past several years, we have witnessed a rapid increase in the use of CTA, driven mainly by technical improvements that allow shorter imaging times and high spatial and tem- poral resolution. Thus, CTA combines widespread availability with the advantages of volumet- ric data acquisition, short acquisition times, and the possibility of simultaneous evaluation of myocardial and extracardiac structures, ventricular function, and coronary anatomy. The American College of Cardiology (ACC), the American Heart Association (AHA), and the Society of Cardiothoracic Computed Tomography (SCCT) have recognized CT as a reason- able and state-of-the-art imaging technique for the assessment of CHD, and this indication is listed as appropriate in the 2010 appropriateness criteria document [3]. ix