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(BQ) Part 1 book Wilcox’s surgical anatomy of the heart presents the following contents: Surgical approaches to the heart, anatomy of the cardiac chambers, surgical anatomy of the valves of the heart, surgical anatomy of the coronary circulation, surgical anatomy of the conduction system,...
z.f Wilcox’s Surgical Anatomy of the Heart Fourth edition Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:17 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:17 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Wilcox’s Surgical Anatomy of the Heart Fourth edition Robert H Anderson, BSc, MD, FRCPath Visiting Professor, Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, UK; Visiting Professor of Pediatrics, Medical University of South Carolina, Charleston, SC, USA Diane E Spicer, BS, PA(ASCP) Pathologists’ Assistant, University of Florida – Pediatric Cardiology, Gainesville, Florida, and Congenital Heart Institute of Florida, St Petersburg, FL, USA Anthony M Hlavacek, MD Associate Professor, Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA Andrew C Cook, BSc, PhD Senior Lecturer, Cardiac Unit, Institute of Child Health, University College London, London, UK Carl L Backer, MD A C Buehler Professor of Surgery, Northwestern University Feinberg School of Medicine, Ann & Robert H Lurie Children’s Hospital of Chicago, Chicago, IL, USA Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:17 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 University Printing House, Cambridge CB2 8BS, United Kingdom Cambridge University Press is part of the University of Cambridge It furthers the University’s mission by disseminating knowledge in the pursuit of education, learning, and research at the highest international levels of excellence www.cambridge.org Information on this title: www.cambridge.org/9781107014480 Fourth edition © Robert H Anderson, Diane E Spicer, Anthony M Hlavacek, Andrew C Cook, and Carl L Backer 2013 This publication is in copyright Subject to statutory exception and to the provisions of relevant collective licensing agreements, no reproduction of any part may take place without the written permission of Cambridge University Press Fourth edition first published 2013 Third edition first published 2004 Printed and bound by Grafos SA, Arte sobre papel, Barcelona, Spain A catalogue record for this publication is available from the British Library Library of Congress Cataloguing in Publication data Anderson, Robert H (Robert Henry), 1942– Wilcox’s surgical anatomy of the heart – Fourth edition / Robert H Anderson, BSc, MD, FRCPath, Diane E Spicer, BS, Anthony M Hlavacek, MD, Andrew C Cook, BSc, PhD, Carl L Backer, MD pages cm ISBN 978-1-107-01448-0 (hardback) Heart – Anatomy Heart – Surgery I Title II Title: Wilcox’s Surgical Anatomy of the Heart QM181.W55 2013 6110 12–dc23 2012051614 ISBN 978-1-107-01448-0 Hardback Cambridge University Press has no responsibility for the persistence or accuracy of URLs for external or third-party internet websites referred to in this publication, and does not guarantee that any content on such websites is, or will remain, accurate or appropriate Every effort has been made in preparing this book to provide accurate and up-to-date information which is in accord with accepted standards and practice at the time of publication Although case histories are drawn from actual cases, every effort has been made to disguise the identities of the individuals involved Nevertheless, the authors, editors and publishers can make no warranties that the information contained herein is totally free from error, not least because clinical standards are constantly changing through research and regulation The authors, editors and publishers therefore disclaim all liability for direct or consequential damages resulting from the use of material contained in this book Readers are strongly advised to pay careful attention to information provided by the manufacturer of any drugs or equipment that they plan to use Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:17 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Contents Preface page vii Acknowledgements viii Surgical approaches to the heart Anatomy of the cardiac chambers 13 Surgical anatomy of the valves of the heart 51 Surgical anatomy of the coronary circulation 90 Surgical anatomy of the conduction system 111 Analytical description of congenitally malformed hearts 128 Lesions with normal segmental connections 150 Lesions in hearts with abnormal segmental connections 244 Abnormalities of the great vessels 321 Positional anomalies of the heart 363 Index 377 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:38 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Preface The books and articles devoted to technique in cardiac surgery are legion This is most appropriate, as the success of cardiac surgery is greatly dependent upon excellent operative technique But excellence of technique can be dissipated without a firm knowledge of the underlying cardiac morphology This is just as true for the normal heart as for those hearts with complex congenital lesions It is the feasibility of operating upon such complex malformations that has highlighted the need for a more detailed understanding of the basic anatomy in itself Thus, in recent years surgeons have come to appreciate the necessity of avoiding damage to the coronary vessels, often invisible when working within the cardiac chambers, and particularly to avoid the vital conduction tissues, invisible at all times Although detailed and accurate descriptions of the conduction system have been available since the time of their discovery, only rarely has its position been described with the cardiac surgeon in mind At the time the first edition of this volume was published, to the best of our knowledge there had been no other books that specifically displayed the anatomy of normal and abnormal hearts as perceived at the time of operation We tried to satisfy this need in the first volume by combining the experience of a practising cardiac surgeon with that of a professional cardiac anatomist We added significantly to the illustrations in the second edition, while seeking to retain the overall concept, as feedback from those who had used the first edition was very positive In the third edition, we sought to expand and improve still further on the changes made in the second edition In the second edition, we had added an entirely new chapter on cardiac valvar anatomy, and greatly expanded our treatment of coronary vascular anatomy We retained this format in the third edition, as we were gratified that, as hoped, readers were able to find a particular subject more easily The third edition also contained still more new illustrations, retaining the approach of orientating these illustrations, where appropriate, as seen by the surgeon working in the operating room, but reverting to anatomical orientation for most of the pictures of specimens So as to clarify the various orientations of each individual illustration, we continued to include a set of axes showing, when appropriate, the directions of superior, inferior, anterior, posterior, left, right, apex, and base All accounts were based on the anatomy as it is observed and, except in the case of malformations involving the aortic arch and its branches, they owe nothing to speculative embryology A major change was forced upon us as we prepared this fourth edition, as our original surgical author, Benson Wilcox, died in May of 2010 It is very difficult to replace such a pioneer and champion of surgical education, but we are gratified that Carl Backer has assumed the role of surgical editor We are also pleased to add Diane Spicer to our anatomical team She has contributed enormously by providing many new and better illustrations of the anatomy as seen in the autopsied heart These advances are complimented by the contributions of our other new editor, Tony Hlavacek Tony has provided quite remarkable images obtained using computed tomography and magnetic resonance imaging, which show that the heart can be imaged with just as much accuracy during life as when we hold the specimens in our hands on the autopsy bench Recognising the huge contributions of Ben Wilcox, we are also pleased to rename this fourth edition ‘Wilcox’s Surgical Anatomy of the Heart’ As with the previous editions, it is our hope that the new edition will continue to be of interest not only to the surgeon, but also to the cardiologist, anaesthesiologist, and surgical pathologist All of these practitioners ideally should have some knowledge of cardiac structures and their exquisite intricacies, particularly those cardiologists who increasingly treat lesions that previously were the province of the surgeon Our senior anatomist remains active, and has been fortunate to be granted access to several archives of autopsied hearts held in the United States of America subsequent to his retirement from the Institute of Child Health in London We remain confident that, in the hands of this new team, and if supply demands, the book will pass through still further editions, hopefully continuing to improve with each version Robert H Anderson, Diane E Spicer, Anthony M Hlavacek, Andrew C Cook, and Carl L Backer, London, Tampa, Charleston and Chicago November, 2012 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:08:45 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.001 Cambridge Books Online © Cambridge University Press, 2013 Acknowledgements A good deal of the material displayed in these pages, and the concepts espoused, are due in no small part to the help of our friends and collaborators As indicated in our preface, the major change since we produced the third edition has been the sad passing of our founding surgical editor, Benson R Wilcox We have renamed this fourth edition ‘Wilcox’s Surgical Anatomy of the Heart’ We dedicate this edition to his eternal memory A further change has been the retirement of Robert H Anderson from the Institute of Child Health at Great Ormond Street Children’s Hospital, London Retirement, however, has permitted him to establish new connections, not least with the newest additions to our team of authors This has permitted many new hearts to be specifically photographed for this new edition, not only of autopsy specimens, but also in the operating room In addition, it has created the collaboration that permits the inclusion of wonderful images obtained using computed tomography and magnetic resonance imaging We continue, nonetheless, to owe a particular debt to Anton Becker of the University of Amsterdam, Bob Zuberbuhler of Children’s Hospital of Pittsburgh, Pennsylvania, United States of America, and F Jay Fricker of University of Florida, Gainesville, Florida, United States of America, all of whom permitted us to use material from the extensive collections of normal and pathological specimens held in their centres We also continue to acknowledge the debt owed to Siew Yen Ho, of the National Heart and Lung Institute, part of Imperial College in London Yen produced many of the original drawings from which we prepared our artwork, and photographed many of the hearts in the Brompton archive The initial photographs and surgical artwork could not have been produced without the considerable help given by the Department of Medical Illustrations and Photography, University of North Carolina As with the third edition, we owe an equal debt of gratitude to Gemma Price, who has continued to improve our series of cartoons For both the third edition and this edition, she has worked over and above the call of duty We also thank Vi Hue Tran, who helped photograph the hearts from Great Ormond Street We are again indebted to Christine Anderson for her help during the preparation of the manuscript, and thank the team supporting Carl Backer at Lurie Children’s of Chicago, in particular Pat Heraty and Anne E Sarwark Finally, it is a pleasure to acknowledge the support provided by Cambridge University Press, who have ensured that all the good parts of the previous editions were retained In particular, we thank Nicholas Dunton and Joanna Chamberlin for all their help during the preparation of the book for publication Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:09:22 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Surgical approaches to the heart Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:09:30 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.002 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts Usual arrangement Mirror-imaged pattern Concordant Concordant 135 Fig 6.12 The cartoon shows how the atrial chambers can be Discordant Discordant Right isomerism Left isomerism Right-hand topology Right-hand topology connected to the ventricles in concordant or discordant fashion, with each pattern existing in usual and mirror-imaged variants Fig 6.13 The cartoon demonstrates the mixed and biventricular Left-hand topology Left-hand topology concordantly connected, but the other junction will be discordantly connected (Figure 6.13) This will occur irrespective of the topological pattern of the atrioventricular connections found when there are isomeric atrial appendages, and each atrium is connected to its own ventricle In each pattern, half of the heart is concordantly connected, and the other half is discordant It is essential in these settings, therefore, to describe both the type of isomerism, and the specific ventricular topology ventricular mass (see later) The arrangement produces a third discrete pattern, namely biventricular and mixed atrioventricular connections In the three connections described thus far, each atrium is connected to its own ventricle This means that the atrioventricular connections themselves Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 136 Wilcox’s Surgical Anatomy of the Heart are biventricular The essential feature in the remaining two types of atrioventricular connection is that the atrial chambers, with one exception, connect to only one ventricle In one of these patterns, both atrial chambers connect to the same ventricle This is a double-inlet atrioventricular connection (Figure 6.14) In the other variant, one of the atrial chambers is connected to a ventricle, but the other atrium has no connection with the ventricular mass This latter arrangement can be divided into two subtypes, depending on whether absence of the connection is right-sided (Figure 6.15) or left-sided (Figure 6.16) An intriguing variation is seen when one of the atrioventricular connections is absent, be it right-sided or left-sided, namely when the atrioventricular valve guarding the solitary connection straddles the septum, being attached in both ventricles The end result is a uniatrial, but biventricular, atrioventricular connection (Figure 6.17) There has been much controversy concerning the description of the hearts in which the atrial chambers connect to only one ventricle It became conventional to describe them in terms of single ventricle, common ventricle, or univentricular hearts It is exceedingly rare, however, to find patients with solitary ventricles Almost always, in patients described as having univentricular hearts, the ventricular mass contains more than one chamber By focusing on the fact that the atrioventricular connection is, in reality, joined to only one ventricle, we are able to achieve a satisfactory solution for this dilemma Thus, the hearts can logically and accurately be described in terms of being functionally univentricular It follows that, in some patients with biventricular atrioventricular connections, imbalance between the ventricles can again produce a functionally univentricular arrangement13 In those with univentricular atrioventricular connections, however, one of the ventricles must be incomplete, while the other Left atrium Right atrium Dominant left ventricle Sup Right Left Fig 6.14 When both atriums are connected to only one ventricle, Inf the atrioventricular connection is univentricular In this heart, showing a four-chamber section in anatomical orientation, there is a double inlet to a dominant left ventricle The black braces show the segments of atrial vestibular myocardium, connected to the dominant left ventricle through separate atrioventricular valves Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 137 Sup Right Left atrium Left Inf Fig 6.15 This anatomical specimen, seen in Right atrium Dominant left ventricle Right AV groove four-chamber orientation, shows the absence of the right atrioventricular connection, with the fibroadipose tissue of the right atrioventricular (AV) groove interposing between the right atrial floor and the base of the ventricular mass This arrangement produces another form of univentricular atrioventricular connection In this case, the morphology is that of classical tricuspid atresia, with the left atrium connected to a dominant left ventricle Note the presence of the base of the incomplete right ventricle (arrow), which has no connection with the atrial chambers Left atrium Right atrium Dominant left ventricle Sup Fig 6.16 This anatomical specimen, again seen in four-chamber Right Left orientation (compare with Figures 6.14 and 6.15), shows the absence of the left atrioventricular connection (red dotted line), giving the third variant of univentricular atrioventricular connection In this example, the right atrium is connected to a dominant left ventricle through a right-sided atrioventricular valve Inf ventricle is dominant The dominant ventricle, which supports the atrioventricular junction or junctions, can take one of three morphologies: right, left, or indeterminate (Figure 6.18) Most frequently, as judged from the pattern of its apical trabecular component, the dominant ventricle will be morphologically left There will be a complementary right ventricle, perforce incomplete because it will lack its atrioventricular connection, and hence its inlet component Such incomplete right ventricles are always found anterosuperiorly relative to the dominant left ventricle, irrespective of whether there is a double inlet, or an absent right or absent left atrioventricular connection They can, however, be Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 138 Wilcox’s Surgical Anatomy of the Heart Straddling & overriding AV valve LA RA RV Fig 6.17 This cartoon illustrates the arrangement when there is an absence of the right atrioventricular (AV) connection, but with the solitary atrioventricular valve straddling the ventricular septum This produces a uniatrial but biventricular atrioventricular connection, shown here in the setting of a usual atrial arrangement with righthand ventricular topology RA, LA, right and left atriums; RV, LV, right and left ventricles LV Absent AV connection Usual Mirror-imaged Right isomerism Left isomerism Fig 6.18 This cartoon shows the multiple possibilities for Dominant left with incomplete RV Solitary and indeterminate ventricle positioned either to the right (Figure 6.19), or to the left (Figure 6.20) relative to the dominant ventricle More rarely, the atrial chambers can be connected to a dominant right ventricle This happens most frequently in the absence of the left atrioventricular Dominant right with incomplete LV univentricular atrioventricular connection that can be produced by combining the variations in atrial morphology, atrioventricular connection, and ventricular morphology It takes no account of the further variations possible according to ventricular relationships, ventriculoarterial connections, etc The arrows in the middle row emphasise the absence of one of the atrioventricular connections LV, RV, left and right ventricles connection (Figure 6.21), but can be found with a double inlet or, rarely, with an absence of the right atrioventricular connection When only the right ventricle is connected to the atrial chambers, and hence dominant, it is the left ventricle that is incomplete, lacking its atrioventricular connection and its inlet portion The incomplete left ventricle will always be found in a posteroinferior position Usually it is left-sided, although rarely it can be right-sided The third morphological configuration found with a double inlet Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 139 Sup Aorta Left Right Inf VSD Fig 6.19 In this anatomical specimen with a double-inlet left ventricle, the incomplete right ventricle is positioned anterosuperiorly and to the right of the dominant left ventricle VSD, ventricular septal defect Incomplete right ventricle Sup Right Left Aorta Inf VSD Fig 6.20 This anatomical specimen, again with a double-inlet left ventricle, has the incomplete right ventricle in an anterosuperior and leftward position relative to the dominant left ventricle VSD, ventricular septal defect Incomplete right ventricle or, exceedingly rarely, with an absence of either atrioventricular connection, is when the atrial chambers connect to a solitary ventricle Such solitary ventricles have indeterminate apical trabecular morphology (Figure 6.22) Incomplete second ventricles are never found in this variant of univentricular atrioventricular connection, in which the only septal structure in the ventricle is that separating the outflow tracts Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 140 Wilcox’s Surgical Anatomy of the Heart Incomplete left ventricle Left atrium Right atrium Dominant right ventricle Fig 6.21 This section through a specimen, Sup shown in four-chamber orientation, illustrates the absence of the left atrioventricular connection (red dotted line) with the right atrium connected to a dominant right ventricle This produces one of the variants of the hypoplastic left heart syndrome Left Right Inf To pulmonary trunk Coarse apical trabeculations Left AV valve To aorta Sup Right AV valve Fig 6.22 This heart, opened in Left Right Inf VALVAR MORPHOLOGY The atrioventricular valvar morphology is independent of the way in which the atrial chambers connect with the ventricles Valvar morphology, therefore, constitutes a separate feature of the atrioventricular junctions When there are concordant, discordant, mixed, or double-inlet connections, then both atrial chambers are connected, actually or potentially, to the ventricular mass The two atrioventricular junctions can be clam-shell-like fashion, has a double inlet to, and double outlet from, a solitary and indeterminate ventricle, the ventricle itself having very coarse apical trabeculations AV, atrioventricular guarded by two separate atrioventricular valves (Figure 6.23), or by a common valve (Figure 6.24) When there are two valves, either of them can be imperforate, blocking a potential atrioventricular connection An imperforate valve, therefore, needs to be Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 141 Base Left Right Apex Fig 6.23 This normal heart, sectioned in four-chamber orientation, shows the right and left atrioventricular junctions (black dotted lines with double-headed arrows) guarded by separate atrioventricular valves Left atrium Right atrium Left ventricle Right ventricle Sup Left Right Fig 6.24 This section, again in four-chamber orientation (compare with Figure 6.23), shows that the right and left atrioventricular junctions (black dotted line with double-headed arrow) are guarded by a common atrioventricular valve in the setting of an atrioventricular septal defect Inf distinguished from absence of an atrioventricular connection, as either can produce atrioventricular valvar atresia The essence of the imperforate valve is that the atrioventricular connection has formed, but is blocked by the conjoined valvar leaflets (Figure 6.25) In the setting of absence of the connection, the floor of the atrium involved is completely separated from the ventricular mass by the fibroadipose tissue of the atrioventricular groove (see Figures 6.15, 6.16) Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 142 Wilcox’s Surgical Anatomy of the Heart Sup Left Right Inf Left atrium Dominant left ventricle Right atrium Fig 6.25 This section of a heart, cut in four-chamber orientation, shows an imperforate right atrioventricular valve connecting to a hypoplastic right ventricle The atrioventricular connections remain concordant, even though the right valve (arrow) is imperforate Hypoplastic right ventricle Left atrium Right atrium Base Fig 6.26 This section of a heart, seen in four-chamber orientation, shows how the tension apparatus of the right atrioventricular valve (arrows) is attached to both sides of the ventricular septum (star), while the orifice of the right atrioventricular junction (white brace) is overriding the septal crest (star) Left Right Apex Either of two valves, or a common valve, can also straddle a septum within the ventricular mass Straddling of the tension apparatus of a valve should be distinguished from overriding of its supporting atrioventricular junction Straddling exists when the valvar tension apparatus is attached to both sides of the ventricular septum (Figure 6.26) Overriding is present when the junction is connected to both ventricles The degree of override, which usually coexists with straddling, determines the precise atrioventricular connection present So as to adjudicate the connection in the presence of overriding, the overriding valve is assigned to the ventricle connected to its greater part (Figure 6.27) The possible arrangements are much more limited when one atrioventricular connection is absent In this situation, the solitary valve can either be committed in its Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:08 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 143 Fig 6.27 In the presence of an RA LA RA LA RV RV LV LV Straddling tricuspid valve with doubleinlet left ventricle Straddling tricuspid valve concordant AV connections entirety to one ventricle, or it can straddle and override When the valve straddles and overrides in this setting, then the atrioventricular connection itself is uniatrial but biventricular (Figure 6.17) VENTRICULAR MORPHOLOGY AND TOPOLOGY The nature of the atrioventricular connections is inextricably linked with the architectural arrangement of the ventricular mass Biventricular atrioventricular connections, for example, cannot be diagnosed without knowledge of ventricular morphology Double-inlet and absent connections can all be identified without mention of ventricular morphology, although in this setting it is always necessary to give more information concerning the arrangement of the ventricular mass In the case of mixed and biventricular atrioventricular connections, it is important to describe the pattern in which the morphologically right ventricle is structured relative to the morphologically left ventricle This feature can take only one of two topological arrangements This is because, when the connections are mixed, the right-sided atrium, with either a morphologically right or left appendage, can be connected to either a morphologically right or a morphologically left ventricle (see Figure 6.13) When there is right isomerism, and the right-sided atrium is connected to a morphologically right ventricle, the ventricular mass typically is seen as in hearts with concordant atrioventricular connections and the usual atrial arrangement In contrast, when there is right isomerism, and the right-sided atrium is connected to a morphologically left ventricle, the ventricular mass is usually found in the presence of discordant atrioventricular connections and the usual atrial arrangement As we have discussed already, these two basic patterns of ventricular topology can conveniently be described according to the way in which the hands, figuratively speaking, can be placed palm downwards upon the septal surface of the morphologically right ventricle The other hand will then fit in the morphologically left ventricle in similar fashion, but it is the arrangement of the morphologically right ventricle that is chosen for the purposes of description (Figure 6.11) In the hearts with biventricular and mixed atrioventricular connections, it is this ventricular topology that determines the disposition of the atrioventricular overriding junction, whether it is a straddling tricuspid valve as shown in Figure 6.26 or ventriculoarterial, the overriding junction (black braces) is assigned to the ventricle supporting its greater part In the situation illustrated with the straddling tricuspid valve, the atrioventricular connections are defined accordingly There is a spectrum between the illustrated extremes (doubleheaded arrow) The left-hand panel shows the situation with the junction connected primarily to the right ventricle (large arrow), the lesser part joining the left ventricle (small arrow) Hence, the atrioventricular connections are deemed to be concordant In the right-hand panel, the larger part of the overriding junction is committed to the left ventricle, so that the connection is deemed to be a double inlet This is the essence of the 50% rule RA, LA, right and left atriums; RV, LV, right and left ventricles conduction tissues11 When the atrial chambers connect to only one ventricle, the morphology of that ventricle must always be described This is because the dominant ventricle can be of left ventricular, right ventricular, or solitary and indeterminate pattern It is also necessary to describe the relationships of the dominant and incomplete ventricles VENTRICULAR RELATIONSHIPS Ventricular relationships, as opposed to ventricular topology, generally should be described as a separate feature of the heart Where each atrium is connected to its own ventricle, the relationships are almost always in harmony with both the connection and topology present When the atrial chambers are in their usual position with concordant atrioventricular connections, the relationships described in the setting of the heart within the chest are almost always for the morphologically right ventricle to be right-sided, anterior, and inferior to the morphologically left ventricle In mirror-imaged atrial arrangement, with concordant atrioventricular connections, the morphologically right ventricle is almost invariably left-sided and relatively anterior, Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:09 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 144 Wilcox’s Surgical Anatomy of the Heart Sup Aorta Post Ant Inf Outlet Fig 6.28 The left side of the heart is shown from a patient with Inlet discordant atrioventricular and ventriculoarterial connections; in other words, congenitally corrected transposition It is the palmar surface of the left hand only that can be placed on the septal surface of the morphologically right ventricle so that the fingers occupy the ventricular outlet, and the thumb goes in the tricuspid valve guarding the inlet component Morph right apical trabeculations Morph left atrium Aorta Outlet Inlet Fig 6.29 The computed tomogram shows the right side of the Apical trabecular component Sup Ant Post Inf but frequently more side-by-side relative to its neighbour With the atrial chambers in their usual arrangement, and discordant atrioventricular connections, there is almost always the lefthand pattern of ventricular topology The usual relationship is for the morphologically heart from a patient having congenitally corrected transposition in the setting of a mirror-imaged atrial arrangement The morphologically left atrium is right-sided, but connects to a morphologically right ventricle with right-hand topology, the palmar surface of the right hand fitting on the septal surface so that the fingers are in the subaortic outlet and the thumb in the inlet component right ventricle to be left-sided (Figure 6.28) When discordant atrioventricular connections accompany mirror-imaged atrial arrangement, there is usually right-hand topology (Figure 6.29) The ventricular relationships are thus similar to those seen in the normal heart, although the two chambers tend to be more side-by-side When the relationships are as anticipated, it is unnecessary to describe them Very occasionally, the relationships of the ventricles are not as anticipated for the connections present This disharmony between connections and relationships Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:09 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 145 Fig 6.30 This cartoon shows the patterns Aorta Pulmonary trunk Common arterial trunk underscores the anomaly known as the crisscross heart14 With these hearts, and also those with superoinferior ventricles, connections and relationships must be described separately, using as much detail as is necessary to achieve unambiguous categorisation The essence of the criss-cross heart, and those with superoinferior ventricles, is that the ventricular relationships are not as expected for the atrioventricular connection present Even more rarely, the ventricular topology may be disharmonious with the atrioventricular connection15 All features must then be described In hearts with a univentricular atrioventricular connection, it is the relationship of the incomplete ventricle to the dominant ventricle that must be described When the left ventricle is dominant, the incomplete right ventricle is always anterosuperior, but can be right- or left-sided The sidedness of the ventricle does not affect the basic disposition of the atrioventricular conduction tissues in these hearts With a dominant right ventricle, the incomplete and rudimentary left ventricle, if present, is always posteroinferior, but again can be right- or left-sided In this case, the sidedness of the incomplete ventricle will affect the disposition of the atrioventricular conduction tissue When considering the atrioventricular junctions, therefore, there are four different features to take into account These are, firstly, the way the atrial myocardium is connected to the ventricular mass; secondly, the morphology of the atrioventricular valves guarding the junctions; thirdly, the ventricular morphology and topology; and finally, the Solitary arterial trunk ventricular relationships All are of importance to the surgeon because they influence the disposition of the atrioventricular conduction axis VENTRICULOARTERIAL JUNCTIONS Analysis of the ventriculoarterial junctions proceeds as described for the atrioventricular junctions, with the morphology of the connections, the valvar morphology, and the relationships of the arterial trunks being different facets requiring separate description in mutually exclusive terms It is also necessary to take account of infundibular morphology Ventriculoarterial connections There are four discrete ways in which the arterial trunks can take their origin from the ventricular mass; namely, in concordant, discordant, double-outlet, and single-outlet fashion Concordant ventriculoarterial connections exist when the arterial trunks arise from morphologically appropriate ventricles Discordant connections account for the trunks being connected with morphologically inappropriate ventricles Double-outlet connections exist when both great arteries take origin from the same ventricle, which may be of right, left, or indeterminate morphology A single-outlet arrangement is seen when only one arterial trunk is connected to the heart This may be a common trunk, directly supplying the systemic, pulmonary, and coronary that identify the morphology of the arterial trunks When there is absence of the intrapericardial pulmonary arteries, there is no way of knowing if, had there been an atretic pulmonary trunk, it would have originated from the base of the heart or from the aorta This pattern, therefore, is best described as a solitary arterial trunk arteries, or it may be an aortic or pulmonary trunk when the complementary arterial trunk is atretic, and its connection to a known ventricle cannot be established (Figure 6.30) Rarely, in the absence of intrapericardial pulmonary arteries, it may be more accurate to describe an arterial trunk as solitary rather than common (Figure 6.30) Arterial valvar morphology The morphological arrangement of the arterial valves is limited because they have no tension apparatus Furthermore, a common valve can exist only with a common trunk The different patterns, therefore, involve one or two arterial valves Usually both valves are perforate, but either or both may override the ventricular septum When a valvar orifice is overriding, the valve is assigned to the ventricle supporting its greater part, thus avoiding the need for intermediate categories The other pattern of valvar morphology is when one of the arterial valves is imperforate As with the atrioventricular junctions, an imperforate arterial valve must be distinguished from absence of one ventriculoarterial connection, as both can produce arterial valvar atresia Infundibular morphology Describing the morphology at the ventriculoarterial junctions also involves the arrangement of the musculature within the ventricular outflow tracts This is infundibular morphology Although the outlet regions are integral parts of the Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:09 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 146 Wilcox’s Surgical Anatomy of the Heart Pulmonary valve Outlet septum Aortic valve SMT Sup Left Right Ventriculoinfundibular fold Inf Interventricular communication Outlet septum Fig 6.31 This view of a heart with a double-outlet right ventricle, seen in anatomical orientation, and with a non-committed interventricular communication, shows the muscular components of the ventricular outflow tracts The hinges of both arterial valves are completely surrounded by outflow musculature SMT, septomarginal trabeculation Pulmonary valve Parietal attachment Septal attachment Sup Ant Post Ventriculoinfundibular fold Inf ventricular mass, it is traditional to consider them in concert with the great arteries The two outflow tracts together make a complete cone of musculature, Fig 6.32 The image shows the outflow tracts in a heart with tetralogy of Fallot, viewed from the apex of the right ventricle, with the aortic valve (star) overriding the crest of the muscular ventricular septum, which is reinforced by the septomarginal trabeculation, or septal band (white Y) The septal and parietal attachments of the muscular outlet septum are well seen which has parietal and septal components, along with a component adjacent to the atrioventricular junction (Figure 6.31) The parietal components make up the anterior free wall of the outflow tracts The septal component is the outlet, or infundibular, septum This has a body, with septal and parietal insertions, best Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:09 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts Outlet septum Aortic valve Sup Post Fig 6.33 In this heart with tetralogy of Fallot, the Ant Ventriculoinfundibular fold Inf Aortic valve SMT Outlet septum ventriculoinfundibular fold (red dotted line) interposes between the leaflets of the aortic and mitral valves in the roof of the interventricular communication (star), producing a completely muscular subaortic infundibulum SMT, septomarginal trabeculation Pulmonary valve Tricuspid valve Sup Post Ant Inf seen in the setting of tetralogy of Fallot (Figure 6.32) The component adjacent to the atrioventricular junction is the ventriculoinfundibular fold It separates the leaflets of arterial from atrioventricular valves (Figure 6.33) It is the inner heart curvature16 The fold may be attenuated to Fig 6.34 This heart has the ventriculoarterial connection of a double-outlet right ventricle, but with fibrous continuity between the leaflets of the aortic and tricuspid valves (red double-headed arrow) give arterial-atrioventricular valvar fibrous continuity (Figure 6.34) The infundibular structures never contain or overlie conduction tissue17 They should be distinguished from another structure, namely the septomarginal trabeculation, or septal band This latter structure is part of the ventricular septum, reinforcing its right ventricular aspect It has a body that continues apically as the moderator band, and two limbs (Figure 6.35) The anterocephalad limb, in the normal heart, extends to the pulmonary valve, Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:10 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 147 148 Wilcox’s Surgical Anatomy of the Heart Supraventricular crest Pulmonary valve Tricuspid valve Sup Fig 6.35 The septal surface of the normal right ventricle is photographed to show the extent of the septomarginal trabeculation, or septal band, as shown by the white Y In the normal heart, the supraventricular crest inserts between the limbs of the trabeculation The star shows the moderator band, one of the series of septoparietal trabeculations that take origin from the anterior surface of the marginal trabeculation Left Right Inf overlying the outlet septum The posterocaudal limb runs beneath the interventricular membranous septum This posterocaudal limb usually overlies the branching part of the atrioventricular bundle, with the right bundle branch passing down to the apex of the right ventricle within the body of the trabeculation Although each outflow tract is potentially a complete muscular structure, in most hearts it is only the outflow tract of the right ventricle that is a complete muscular cone This is because, within the left ventricle, part of the ventriculoinfundibular fold is usually attenuated to permit fibrous continuity between the leaflets of the arterial and atrioventricular valves In the normal heart, therefore, there is a muscular subpulmonary infundibulum in the right ventricle, and fibrous valvar continuity in the roof of the left ventricle In congenitally malformed hearts, three other patterns may be found These are, firstly, a muscular subaortic infundibulum with pulmonary-atrioventricular continuity; secondly, a bilaterally muscular infundibulum; and thirdly, bilateral deficiency of the infundibulums In the presence of a common arterial trunk, there may be a complete muscular subtruncal infundibulum, but more often there is truncal-atrioventricular valvar continuity Arterial valvar and truncal relationships The final feature of consideration at the ventriculoarterial junctions is the relationship of the arterial valves and arterial trunks Valvar relationships are independent of both ventriculoarterial connections and infundibular morphology Of the many methods of description, our preference is to describe the aortic relative to the pulmonary valve as viewed from below in right–left, anteroposterior and, when necessary, superoinferior coordinates This can be done as precisely as required In our experience, eight coordinates combining lateral and anterior to posterior positions suffice (Figure 6.36) When describing the relationship of the arterial trunks, it is sufficient to account for trunks that spiral around each other as they ascend, and to distinguish them from trunks that ascend in parallel fashion18 POSITION OF THE HEART The system discussed in this chapter establishes the cardiac template Irrespective of the internal architecture, it is well known that the heart itself can occupy many and varied positions (see Chapter 10), particularly when there are complex intracardiac malformations To describe the position of the heart in unambiguous fashion, account should be taken separately of its site within the thorax, and the orientation of its apex We describe the heart as being in the left or right side of the chest, or in the midline Apical orientation is described as being to the left, to the middle, or to the right CATALOGUE OF MALFORMATIONS Having described the template of the heart, and its position, finally it is necessary to catalogue all intracardiac malformations In most cases, it is these lesions which will require surgical attention Any lesion, nonetheless, cannot be presumed to be the only lesion present until the rest of the Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:10 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 149 anterior right anterior left anterior left side-by-side right side-by-side Pulmonary trunk right posterior ‘normal’ left posterior Fig 6.36 The cartoon shows the combination of anterior to posterior, and right to left coordinates that are used to describe the interrelationships of the aortic and pulmonary valves at their origins from the ventricular mass posterior heart has been established as normal It is these associated lesions that will be emphasised in subsequent chapters, taking particular note, as before, of the features of surgical significance References cited Shinebourne EA, Macartney FJ, Anderson RH Sequential chamber localization: the logical approach to diagnosis in congenital heart disease Br Heart J 1976; 38: 327–340 Anderson RH, Wilcox BR Understanding cardiac anatomy: the prerequisite for optimal cardiac surgery Ann Thorac Surg 1995; 59: 1366–1375 Van Praagh R The segmental approach to diagnosis in congenital heart disease In: Bergsma D (ed) Birth defects original article series, Vol VIII, No The Fourth Conference on the Clinical Delineation of Birth Defects Part XV The Cardiovascular System The National Foundation March of Dimes Baltimore, MD: Williams and Wilkins, 1972; pp 4–23 Anderson RH, Ho SY Continuing Medical Education Sequential segmental analysis – description and categorization for the millennium Cardiol Young 1997; 7: 98–116 Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH Atrial appendages and 10 venoatrial connections in hearts with patients with visceral heterotaxy Ann Thorac Surg 1995; 60: 561–569 Van Mierop LHS, Gessner IH, Schiebler GL Asplenia and polysplenia syndromes In: Bergsma D (ed) Birth Defects: Original Article Series, Vol VIII, No The Fourth Conference on the Clinical Delineation of Birth Defects Part XV The Cardiovascular System The National Foundation March of Dimes Baltimore, MD: Williams and Wilkins, 1972: 36–44 Ivemark BI Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood An analysis of the heart; malformations in the splenic agenesis syndrome, with 14 new cases Acta Paediatr Suppl 1955; 44: 7–110 Macartney FJ, Zuberbuhler JR, Anderson RH Morphological considerations pertaining to recognition of atrial isomerism Consequences for sequential chamber localisation Br Heart J 1980; 44: 657–667 Sharma S, Devine W, Anderson RH, Zuberbuhler JR The determination of atrial arrangement by examination of appendage morphology in 1842 heart specimens Br Heart J 1988; 60: 227–231 Huhta JC, Smallhorn JF, Macartney FJ Two dimensional echocardiographic diagnosis of situs Br Heart J 1982; 48: 97–108 11 Smith A, Ho SY, Anderson RH, et al The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialized conduction system Cardiol Young 2006; 16: 437–454 12 Ho SY, Seo J-W, Brown NA, et al Morphology of the sinus node in human and mouse hearts with isomerism of the atrial appendages Br Heart J 1995; 74: 437–442 13 Jacobs ML, Anderson RH Nomenclature of the functionally univentricular heart Cardiol Young 2006; 16(Suppl 1): 3–8 14 Anderson RH Criss-cross hearts revisited Pediatr Cardiol 1982; 3: 305–313 15 Anderson RH, Smith A, Wilkinson JL Disharmony between atrioventricular connections and segmental combinations – unusual variants of “criss-cross” hearts J Am Coll Cardiol 1987; 10: 1274–1277 16 Anderson RH, Becker AE, Van Mierop LHS What should we call the “crista”? Br Heart J 1977; 39: 856–859 17 Hosseinpour A-R, Jones TJ, Barron DJ, Brawn WJ, Anderson RH An appreciation of the structural variability in the components of the ventricular outlets in congenitally malformed hearts Eur J Cardiothorac Surg 2007; 31: 888–893 18 Cavalle-Garrido T, Bernasconi A, Perrin D, Anderson RH Hearts with concordant ventriculoarterial connections but parallel arterial trunks Heart 2007; 93: 100–106 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:10 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 ... cm ISBN 978 -1- 107- 014 48-0 (hardback) Heart – Anatomy Heart – Surgery I Title II Title: Wilcox’s Surgical Anatomy of the Heart QM1 81. W55 2 013 611 0 12 –dc23 2 012 0 516 14 ISBN 978 -1- 107- 014 48-0 Hardback... viii Surgical approaches to the heart Anatomy of the cardiac chambers 13 Surgical anatomy of the valves of the heart 51 Surgical anatomy of the coronary circulation 90 Surgical anatomy of the. .. IP 11 3 .16 6.95.77 on Fri Sep 13 04:09: 31 WEST 2 013 http://dx.doi.org /10 .10 17/CBO97 811 390285 61. 002 Cambridge Books Online © Cambridge University Press, 2 013 11 12 Wilcox’s Surgical Anatomy of the