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Part 2 book “Wilcox’s surgical anatomy of the heart” has contents: Analytical description of congenitally malformed hearts, lesions with normal segmental connections, lesions in hearts with abnormal segmental connections, abnormalities of the great vessels, positional anomalies of the heart.
6 Analytical description of congenitally malformed hearts Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:07 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts Systems for describing congenital cardiac malformations have frequently been based upon embryological concepts and theories As useful as these systems have been, they have often had the effect of confusing the clinician, rather than clarifying the basic anatomy of a given lesion As far as the surgeon is concerned, the essence of a particular malformation lies not in its presumed morphogenesis, but in the underlying anatomy An effective system for describing this anatomy must be based upon the morphology as it is observed At the same time, it must be capable of accounting for all congenital cardiac conditions, even those that, as yet, might not have been encountered To be useful clinically, the system must be not only broad and accurate, but also clear and consistent The terminology used, therefore, should be unambiguous It should be as simple as possible The sequential segmental approach provides such a system1, particularly when the emphasis is placed on its surgical applications2 The basis of the system is, in the first instance, to analyse individually the architectural make-up of the atrial chambers, the ventricular mass, and the arterial segment3 Emphasis is thus given to the nature of the junctional arrangements (Figure 6.1) Still further attention is devoted to the interrelationships of the cardiac structures within each of the individual segments This provides the icu lar Arterial trunks l ria rte loa icu iov Atr The first step in analysing any malformed heart is to determine the arrangement of the chambers within the atrial mass When distinction is based on the anatomy of the appendages, which are the most constant components of the atriums, and specifically on the extent of the pectinate muscles relative to the atrial vestibules5, atrial chambers can be of only morphologically right or morphologically left type The morphologically right appendage is broad and triangular (Figure 6.2), whereas the morphologically left appendage is fingerlike, and has a much narrower neck (Figure 6.3) In most instances, it is possible to identify the appendages simply on the basis of their shape (Figure 6.4) Only in circumstances of uncertainty will it prove necessary to inspect the extent of the pectinate muscles (Figures 6.5, 6.6) This feature, of course, is readily visible to the surgeon once the atrial chambers have been opened When judged on the extent of the pectinate muscles, there are only four topological ways in which the appendages can be arranged within the atrial mass (Figure 6.7) Almost always, the atrium ntr tr en ATRIAL ARRANGEMENT possessing the appendage in which the pectinate muscles extend to the crux is right-sided, while the one with a smooth inferoposterior vestibule is left-sided This usual arrangement is often called ‘situs solitus’ Rarely, the appendages can be disposed in mirror-image fashion, so-called ‘situs inversus’ More common than the mirror-imaged topological arrangement, but still relatively rare, is the situation in which the appendages of both chambers in the atrial mass have the same morphology This can occur in two forms, with either morphologically right (Figure 6.8) or morphologically left (Figure 6.9) appendages on both sides These bilaterally symmetrical topological patterns, or isomeric arrangements, have traditionally been named according to the arrangement of the abdominal organs, particularly the spleen This is because they usually exist with a jumbled up abdominal arrangement, an arrangement also termed visceral heterotaxy6 It is far more convenient, as well as more accurate, to designate them in terms of their own intrinsic morphology8, particularly as this can be determined readily by the surgeon in the operating room Isomerism of the right appendages is usually, but not always, found with absence of the spleen It is most often associated with right bronchial isomerism Isomerism of the left appendages is typically found, but again not always, with multiple spleens The Ve Atriums basic framework within which all other associated malformations can be catalogued4 129 Fig 6.1 The cartoon shows the three segments of the heart These Ventricles are the atriums, the ventricular mass, and the arterial trunks The segments are joined together at the atrioventricular and ventriculoarterial junctions Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:07 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 130 Wilcox’s Surgical Anatomy of the Heart Triangular appendage Sup Right Left Inf Fig 6.2 The computed tomogram shows the typical triangular Right coronary artery shape of the morphologically right atrial appendage Hooked and tubular appendage Sup Post Ant Fig 6.3 As shown in this computed tomogram, the Inf association with left bronchial isomerism is more constant The anticipated topological arrangement of the atrial appendages can be predicted preoperatively with a high morphologically left atrial appendage has a characteristic narrow and hooked shape (compare with Figure 6.2) degree of accuracy by studying the relationships of the abdominal great vessels as determined with cross-sectional ultrasonography10 When identified, knowledge of isomerism of the atrial appendages is of value in two additional ways Firstly, it alerts the surgeon to unusual dispositions of the sinus node11 In right isomerism, the sinus node, being a morphologically right atrial structure, is Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:07 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 131 Morphologically left appendage Left Sup Inf Right Fig 6.4 This operative view, taken through a median sternotomy, shows the differences between the broad triangular morphologically right atrial appendage, and the narrow finger-like morphologically left atrial appendage Morphologically right appendage ‘Septum spurium’ Sup Ant Post Inf Extensive pectinate muscles Fig 6.5 The computed tomogram shows the extensive pectinate muscles found in the roof of the morphologically right atrium When assessed relative to the vestibule of the atrioventricular junction, they extend to the crux of the heart duplicated A node is found laterally in each of the terminal grooves12 In left isomerism, there are no terminal grooves (Figure 6.9) In this situation, the sinus node is a poorly formed structure, without a constant site Usually the node is found in the anterior interatrial groove, close to the atrioventricular junction12 The second advantage of recognising isomeric appendages is that the arrangements are known to be harbingers of complex intracardiac lesions Hearts with isomerism of either type tend to have bilateral superior caval veins, an effectively common atrial chamber, albeit with two Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:07 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 132 Wilcox’s Surgical Anatomy of the Heart Pulmonary veins Sup Post Ant Fig 6.6 As shown in this computed tomogram, the pectinate Pectinate muscles Inf Mirror-imaged Usual Isomeric right Isomeric left isomeric appendages, and common atrioventricular valves Right isomerism is always associated with a totally anomalous pulmonary venous connection, even if the pulmonary veins are joined to one or other atrium It is also seen most frequently with pulmonary stenosis or atresia, and in association with a univentricular atrioventricular connection, typically a double-inlet ventricle through a common valve Left isomerism, in the majority of cases, is associated with interruption of the muscles of the morphologically left appendage are confined within the tubular component, which has a very narrow junction with the body of the atrium (black double-headed arrow) Fig 6.7 The cartoon shows the four possible arrangements of the atrial appendages, which cannot always be distinguished on the basis of their shape The best means of distinguishing between them is to establish the extent of the pectinate muscles These muscles extend all the way to the crux in the morphologically right atrial appendage, but are confined around the mouth of the appendage in the morphologically left atrial appendage, leaving a smooth posterior vestibule Using this criterion, all congenitally malformed hearts have appendages fitting within one of the four groups shown in the cartoon inferior caval vein, with continuation of the venous drainage from the abdomen through the azygos system of veins THE ATRIOVENTRICULAR JUNCTIONS Having established the arrangement of the atrial appendages, the next step in sequential analysis is to determine the morphology of the atrioventricular junctions For this, the surgeon needs to know how the atrial chambers are, or are not, connected to the chambers present within the ventricular mass Most often, there are two such chambers, which can be of only right or left morphology The morphological distinction is based on the nature of the apical trabeculations In the morphologically right ventricle, these trabeculations are coarse, in contrast to the fine criss-crossing trabeculations that characterise the morphologically left Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:07 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 Congenitally malformed hearts 133 Left-sided morphologically right appendage Fig 6.8 This surgical view through a median sternotomy Left Inf Sup Right shows a left-sided atrial appendage of morphologically right pattern The right-sided appendage is also of right morphology, so the patient has isomerism of the right atrial appendages Note the crest of the appendage in relation to the left superior caval vein and the terminal groove When examined internally, pectinate muscles encircled both atrioventricular junctions Left Sup Inf Right Fig 6.9 This surgical view through a median sternotomy shows a Right-sided morphologically left appendage ventricle (Figure 6.10) It is the interrelationships between the two ventricles that permit the description of ventricular topology When analysed according to the way that the palmar surfaces of the hands can be placed on the right-sided atrial appendage of morphologically left pattern The left-sided appendage is also of left morphology, so the patient has isomerism of the left atrial appendages Note the absence of any terminal groove Internal inspection confirmed the presence of smooth bilateral posterior vestibules septal surface of the morphologically right ventricle such that the thumb is within the inlet component, and the fingers in the ventricular outlet, the patterns reflect either right-hand or left-hand topology (Figure 6.11) Having assessed ventricular morphology, it is possible to determine the way in which the atrial chambers are connected, or not connected, to the ventricular mass In most instances, there are two atrioventricular junctions, although one of the junctions can be absent Also Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 04:33:07 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.007 Cambridge Books Online © Cambridge University Press, 2013 134 Wilcox’s Surgical Anatomy of the Heart Left Post Coarse right ventricular trabeculations Ant Right Fig 6.10 The apical part of the normal ventricular mass has been amputated, and is viewed from above It shows the marked difference between the fine apical trabeculations of the morphologically left ventricle when compared to the coarse right ventricular apical trabeculations Fine left ventricular trabeculations Fig 6.11 The cartoon shows how the Right-hand topology Left-hand topology patterns of ventricular topology can be described, figuratively speaking, in terms of the way that the palmar surface of the hands can be placed on the septal surface of the morphologically right ventricle The fingers point up the outlet, and the thumb lies in the inlet, giving right-hand and left-hand patterns In the arrangements shown, the atrioventricular connections are concordant, but the ventriculoarterial connections can also be discordant, with the aorta arising from the morphologically right ventricle (see Figure 6.29) important is the morphology of the valves that guard the atrioventricular junctions, because the paired junctions can be guarded by a common atrioventricular valve This shows that junctional and valvar morphology are separate and independent features There are five distinct and discrete ways in which the atrial chambers may be connected to the ventricular mass, the final one having two subtypes, along with an intriguing further variation Most often, the atrial chambers are connected to their morphologically appropriate ventricles This pattern is called concordant atrioventricular connections When each atrium is connected in this way to its own ventricle, there is rarely any difficulty in distinguishing the morphology of the ventricles, even when the ventricles themselves are unusually related one to the other In the second pattern, which represents discordant connections, each atrium is connected with a morphologically inappropriate ventricle Concordant and discordant connections can exist with either the usual or mirror-imaged arrangement of the atrial appendages (Figure 6.12), but not with isomeric appendages When the appendages are isomeric, and each atrium is connected to its own ventricle, then of necessity one junction will 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 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 368 Wilcox’s Surgical Anatomy of the Heart Fig 10.7 The cartoon, drawn in anatomical Morph left ventricle uppermost Morph right ventricle uppermost Usual side-by-side ventricles Aorta orientation, shows the tilting along the long axis of the ventricular mass in a heart with congenitally corrected transposition, which usually has side-by-side ventricles (central panel) The tilting produces a superoinferior relationship of the ventricles, almost always in association with twisting of the atrioventricular connections (see Figures 10.4 to 10.6) Depending on the direction of tilting, either the morphologically (Morph.) left ventricle (left-hand panel) or the morphologically right ventricle (right-hand panel) can be positioned uppermost Most usually it is the right ventricle that assumes the superior location As shown in Figure 10.6, superoinferior ventricular relationships can also be found when the atrioventricular connections are concordant Sup Pulm trunk Left Right Inf Tricuspid valve Fig 10.8 In the heart shown in Figure 10.6, the right ventricle Morphologically right ventricle ventricular mass is as anticipated for the atrioventricular connections Thus, when there is rotation of the ventricular mass sufficient to produce crossing of the atrioventricular connections, this does not disturb the intrinsic relationships of the two ventricles7 In exceedingly rare with a double outlet showed right-hand topology despite the twisted atrioventricular connections In this image, the morphologically right ventricle exhibits left-hand topology (black arrows), again with a double outlet from the ventricle The atrioventricular valve, however, is morphologically tricuspid, and connects the right ventricle to the usually positioned right atrium Despite the left-hand topology, the atrioventricular connections in this heart are concordant This is a very rare example of segmental disharmony5 Pulm., pulmonary circumstances, however, the ventricles can not only occupy unusual positions, but the topology is not as expected for the existing atrioventricular connections Hence, when the usual atrial arrangement coexists with concordant atrioventricular connections, the ventricular topology is usually right- handed (Figure 10.6) On occasion, nonetheless, the right atrium can be joined to a morphologically right ventricle that shows left-hand ventricular topology, with the right ventricle being additionally leftsided (Figure 10.8) This situation is one of the rare occasions when it is necessary to Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:01 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 Positional anomalies of the heart 369 Morphologically left atrial appendage Left Inf Sup Right Morphologically right atrial appendage describe ventricular topology in addition to accounting for the atrioventricular connections5 HEARTS WITH ISOMERIC ATRIAL APPENDAGES The problems occurring in hearts with isomeric atrial appendages have been mentioned already Hearts with this arrangement are not only found in unusual positions, but are almost always associated with an abnormal arrangement of the thoracoabdominal organs; hence the popular rubric of visceral heterotaxy Many will continue to classify these patients in terms of ‘asplenia’ and ‘polysplenia’8 It is of greater value for the surgeon to base any system of categorisation on the morphology of the atrial appendages Splenic morphology does not always correspond to the atrial anatomy, itself based on the extent of the pectinate muscles relative to the atrial vestibules There is a greater correspondence between the anatomy of the appendages as thus determined and what is expected of the ‘splenic syndromes’ than between these syndromes and splenic morphology9,10 More important, it is of little consequence to the cardiac surgeon at the time of operation whether his or her Fig 10.9 This operative view, taken through a median sternotomy, shows the different morphology of the morphologically right as opposed to the morphologically left atrial appendages in a patient with the usual atrial arrangement patient has one spleen, multiple spleens, or no spleen at all Determining the morphology of the appendages brings attention directly to the heart, enabling the surgeon working in the operating room to make the diagnosis of isomerism immediately, even if this had not been predicted by the preoperative studies The surgeon can readily distinguish the appendages as being either morphologically right or left (Figure 10.9) The surgeon should always confirm whether the patient possesses lateralised atrial appendages The finding of isomeric left (Figure 10.10) or right (Figure 10.11) appendages should immediately alert the surgeon to potential problems over and above those anticipated in the patient with the usual or a mirror-imaged atrial arrangement It is now also possible to distinguish directly the presence of isomeric atrial appendages using computed tomography (Figures 10.12–10.15), while use of other three-dimensional techniques such as magnetic resonance imaging will readily reveal the arrangement of the bronchial tree and the abdominal organs There should be no problem, therefore, in distinguishing those patients having isomeric right as opposed to isomeric left appendages In hearts from patients with isomeric right appendages (Figures 10.16– 10.18), it is the rule to find complex intracardiac anomalies, usually with the absence of the spleen The pulmonary veins will be connected in a totally anomalous fashion even when they are joined to the heart (Figure 10.19) Most frequently, there are major anomalies of systemic venous drainage A common atrioventricular valve is usually present (Figures 10.16 – 10.18), often with a double-inlet ventricle (Figure 10.19) Pulmonary stenosis or atresia is frequently found, and there are bilateral sinus nodes11–13 Increasing operative experience now shows that even the most complex combinations can be treated surgically Although isomerism of the morphologically right appendages is almost always accompanied by severe intracardiac malformations, this is not necessarily the case when there are isomeric left appendages (Figures 10.20–10.22) Thus, the surgeon is more likely to be confronted with an undiagnosed case in the setting of left isomerism Therefore, it becomes important to know that the sinus node is in an anomalous position It is usually hypoplastic If it can be found, it will be located close to the atrioventricular Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 370 Wilcox’s Surgical Anatomy of the Heart Left Sup Inf Morphologically left atrial appendage Right Aorta Fig 10.10 This operative view, taken through a median sternotomy, shows that the appendage on the right side of the heart has left morphology In this patient, the left-sided appendage was also morphologically left, so the patient had isomerism of the morphologically left atrial appendages Right superior caval vein Morphologically right atrial appendage Left Fig 10.11 In this patient, the left-sided atrial appendage is Inf Sup Left superior caval vein + terminal groove Right broad-based, with a wide junction to the body of the atrium Note that the left-sided superior caval vein joins to the atrial roof, and that there is a left-sided terminal groove The right-sided appendage was also of right morphology The patient has isomerism of the right atrial appendages Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 Positional anomalies of the heart 371 Morph right atrial appendage Morph right ventricle Sup Fig 10.12 The reconstructed computed tomographic angiogram shows a patient with a right-sided morphologically (Morph.) right atrial appendage, the right-sided atrium joining to a morphologically right ventricle Ant Post Inf Sup Morph right atrial appendage Ant Post Inf Aorta Fig 10.13 The image shows the left side of the heart in Morph right ventricle Figure 10.12 reconstructed The left-sided atrial appendage also has a broad base, and is morphologically (morph.) right The patient has isomerism of the morphologically right appendage Note the incomplete left ventricle in the posteroinferior position (star) The aorta arises from the dominant right ventricle Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 372 Wilcox’s Surgical Anatomy of the Heart Ant Left Right Aorta Post Fig 10.14 The cross-sectional image is from the same dataset as shown in Figures 10.12 and 10.13 Both appendages (stars) are broad-based (black double-headed arrows), with pectinate muscles extending to the crux (white arrows; see also Figure 10.18) There is obvious isomerism of the right atrial appendages (compare with Figure 10.15) Sup Left Right Aorta Inf Common atrium Fig 10.15 The computed tomogram in this patient, seen from the front, shows the presence of two narrow appendages (stars) with narrow necks (black double-headed arrows) This patient has isomerism of the morphologically left atrial appendages (compare with Figure 10.14) Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 Positional anomalies of the heart Morphologically right atrial appendage Sup Ant Post Inf Septal strand Fig 10.16 The heart has been opened through the right Pectinate muscles to crux atrioventricular junction close to the crux, and the parietal wall spread upwards The pectinate muscles encircle the right-sided vestibule, indicating that the right-sided appendage is morphologically right Note the common atrioventricular valve (star), and absence of the coronary sinus Morphologically right atrial appendage Sup Post Ant Inf Septal strand Pectinate muscles to crux Fig 10.17 The left side of the heart shown in Figure 10.16 has been opened through an incision close to the crux The pectinate muscles also encircle the left-sided vestibule, showing that there are morphologically right atrial appendages bilaterally The star shows the common atrioventricular valve Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 373 374 Wilcox’s Surgical Anatomy of the Heart Common atrioventricular junction Sup Right Left Inf Fig 10.18 In this heart, the atrial chambers have been Pectinate muscles to crux Pulmonary veins to atrial roof disconnected from the atrioventricular junctions inferiorly, and tilted superiorly The pectinate muscles encircle the entirety of the common atrioventricular junction, indicating the presence of isomeric right atrial appendages The common junction is guarded by a valve with separate right and left atrioventricular valvar orifices Sup Right Left Inf Fig 10.19 In this heart from a patient with isomeric right atrial appendages, the pulmonary veins drain in anatomically anomalous fashion to the atrial roof, even though returning directly to the heart Note that the common atrioventricular valve is connected exclusively to a dominant left ventricle, indicating the presence of a double-inlet connection Pectinate muscles to crux junction11–13 Interruption of the inferior caval vein, with return through the azygos venous system, is a frequent accompaniment In the more severely affected cases, there may be a common atrioventricular valve14 Pulmonary stenosis or atresia is not usually a feature, nor is the presence of univentricular atrioventricular connection, but there is frequently aortic coarctation Whatever the types of isomerism, when there is a common atrioventricular valve Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 Positional anomalies of the heart 375 Morphologically left atrial appendage Sup Ant Post Inf Fig 10.20 The right-sided atrium in this heart has a morphologically left appendage Morphologically left atrial appendage appendage Sup Ant Post Inf Fig 10.21 The image shows the opposite side of the heart illustrated in Figure 10.20 The left-sided appendage is also of left morphology and each atrium is connected to its own ventricle, it is frequent to find a left-hand pattern of ventricular topology In this setting, the patient may well have been diagnosed as having congenitally corrected transposition The presence of isomeric appendages, however, makes it highly likely that there will be a sling of ventricular conduction tissue joining dual atrioventricular nodes (Figure 10.22, righthand panel)11 This places the entire edge of the ventricular septum at risk should Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:02 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 376 Wilcox’s Surgical Anatomy of the Heart Left bundle branch in left-sided ventricle Anterior connection Fig 10.22 The cartoon, drawn in anatomical Sling Posterior connection Posterior connection surgical correction be attempted When isomerism of the appendages is found with biventricular atrioventricular connections and a right-hand pattern of ventricular topology, the atrioventricular conduction axis should be expected in its usual posterior position (Figure 10.22, left-hand panel) This entire discussion emphasises the significance of full sequential segmental analysis of any patient presented for cardiac surgery References Wilkinson JL, Acerete F Terminological pitfalls in congenital heart disease Reappraisal of some confusing terms, with an account of a simplified system of basic nomenclature Br Heart J 1973; 35: 1166–1177 Van Praagh R, Weinberg PM, Matsuoka R, Van Praagh S Malpositions of the heart Edited by Adams FH, Emmanouilides GC In: Moss’ Heart Disease in Infants, Children and Adolescents Baltimore, MD: Williams & Wilkins, 1983; pp 422–458 Byron F Ectopia cordis Report of a case with attempted operative correction J Thorac Surg 1948; 17: 717–722 Cantrell JR, Haller JA, Ravitch MM A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart Surg Gynecol Obstet 1958; 107: 602–614 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 Symons JC, Shinebourne EA, Joseph MC, et al Criss-cross heart with congenitally corrected transposition: report of a case with d-transposed aorta and ventricular preexcitation Eur J Cardiol 1977; 5: 493–505 Seo J-W, Yoo S-J, Ho SY, Lee HJ, Anderson RH Further morphological observations on hearts with twisted atrioventricular connections (criss-cross hearts) Cardiovasc Pathol 1992; 1: 211–217 Stanger P, Rudolph AM, Edwards JE Cardiac malpositions: an overview based on study of sixty-five necropsy specimens Circulation 1977; 56: 159–172 Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH Atrial appendages and venoatrial connections in hearts with orientation, shows the disposition of the axis of atrioventricular conduction tissue in hearts with isomerism of the atrial appendages, biventricular atrioventricular connections, and a common atrioventricular valve The arrangement varies depending on whether there is right-hand ventricular topology (lefthand panel), when there is only a posterior connection of the ventricular conduction tissues, or left-hand topology (right-hand panel), when the ventricular conduction tissues are connected by both posterior and anterior atrioventricular nodes, producing a sling of conduction tissue along the crest of the muscular ventricular septum 10 11 12 13 14 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:03 WEST 2013 http://dx.doi.org/10.1017/CBO9781139028561.011 Cambridge Books Online © Cambridge University Press, 2013 patients with visceral heterotaxy Ann Thorac Surg 1995; 60: 561–569 Uemura H, Ho SY, Devine WA, Anderson RH Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both Am J Cardiol 1995; 76: 846–849 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 Dickinson DF, Wilkinson JL, Anderson KR, et al The cardiac conduction system in situs ambiguous Circulation 1979; 59: 879–885 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 Uemura H, Anderson RH, Ho SY, et al Left ventricular structures in atrioventricular septal defect associated with isomerism of the atrial appendages compared with similar features with usual atrial arrangement J Thorac Cardiovasc Surg 1995; 110: 445–452 Index Numbers in italics refer only to illustrations A Abbott’s artery 337, 338 absent pulmonary valve syndrome 239 accessory pathways 114–19, 114 left-sided 116, 117 paraseptal 117–19, 118 right-sided 117, 118 aneurysms membranous septum 274 patent arterial duct 350 ansa subclavia 12 anterior septum 119 anterolateral muscle bundle hypertrophy 216–17 aorta anatomy 45–7 arch 45–6, 48 development 340–1 double 341–3 interrupted 300–1, 337–40 right-sided 343–4 ascending 45, 47 coarctation 211, 300–1, 334–7 intercostal arteries 46–7, 336 common arterial trunk 308–9, 312 congenitally corrected transposition 281–6, 286 coronary arteries, anomalous origins 91–6, 313, 354–5 descending 46–7 double-outlet ventricle 295–6, 297, 300–1 hypoplasia 334 parallel arterial trunks 313–19 pulmonary arteries arising from 345–6, 347 spiral arrangement of arterial trunks 313, 314 mirror-image 315 tetralogy of Fallot 231–2 transposition 275–8 vascular rings 340–5 see also common arterial trunk aortic atresia in congenitally corrected transposition 291 with mitral atresia 260, 263–6 aortic root 16, 52, 59, 91, 159 aortic sinuses 78, 91–4, 124, 211–12 in aortic stenosis 218 Leiden nomenclature 93 in transposition 278, 280 aortic stenosis subvalvar 210, 213–18 dynamic 218 fixed 214–17 supravalvar 218, 219–21 valvar 210–13 bicuspid 210–12, 213–14 tricuspid 213, 214 unicuspid 210, 212 aortic valve anatomy 45, 46, 53, 55–60, 73–81, 210 aortic–mitral fibrous curtain 45, 56, 58, 79 conduction axis and 79, 82, 85, 112, 218 in double-outlet ventricle 295–6, 297 endocarditis 218 imperforate 145 insufficiency 210, 218, 221–2 leaflets 45, 46, 73–5, 75–7, 78–9 left coronary 79, 80 non-adjacent 79 right coronary 79 overriding 145, 197, 198 prolapse 195, 197, 198, 218, 223 sinutubular junction 45, 47, 52, 75, 77 stenosis see aortic stenosis ventriculoarterial junction 52, 54, 77, 148, 149 zones of apposition 55, 79, 211 see also aortic atresia aortic–mitral fibrous curtain 45, 56, 58, 79 aortopulmonary collateral arteries 224, 232–4 aortopulmonary window 351–3 aortoventricular tunnels 359–61 arrhythmias postoperative 124–6, 260, 270 ventricular tachycardia 123 see also supraventricular tachycardia arterial duct in aortic coarctation 334, 336 in common arterial trunk 311 embryology 341 pulmonary arteries arising from 232, 233, 234, 345, 347 surgical closure 348–51 in tetralogy of Fallot 232, 233, 234 arterial ligament 49, 347 arterial switch procedure 278, 279–82 arterial trunk(s) 145 common see common arterial trunk parallel 313–17 discordant atrioventricular connections 317–19 relationship to arterial valves 148 solitary 145, 303, 304 spiral arrangement 313, 314 mirror-image arrangement 315 arterial valves 52–5, 73–8, 148, 210 attachments/support 55–60 malformations 145, 210–41 see also aortic valve; pulmonary valve atrial appendages 16–17, 129, 130, 369 isomerism 19, 129–32, 133, 143, 369–76 anomalous pulmonary venous connection 132, 330, 369 juxtaposition 17 left see left atrium, appendage right see right atrium, appendage situs inversus 129 atrial fibrillation 122–3, 126 atrial flutter 120–1 atrial maze operations 124–6 atrial morphology left 34–5 in malformed hearts 129–32 right 19–34 atrial septal defects 155–62 oval fossa 22, 156, 157–8 sinus venosus 156–9, 160–2, 332–3 unroofing of the coronary sinus 159–62, 163–4, 334 atrial septum 22–5, 27, 151, 152 atrio–Hisian tracts 114 atriopulmonary connection 250–2 atrioventricular bundle (bundle of His) 59, 112, 114–19, 189 in congenitally corrected transposition 280, 285–6 in double-inlet ventricle 252 valvar anatomy and 79, 85 in ventricular septal defects 187–91 atrioventricular conduction axis 25–31, 112–13 in atrial appendage isomerism 130–1, 375–6 in atrioventricular septal defects 167–70, 181–2, 184–5 in common arterial trunk 307 in congenitally corrected transposition 280–1, 285–6 when closing a ventricular septal defect 285–6 in double-inlet ventricle left dominant 252 right dominant 254 septation procedure 252–3 ventricular septal defect enlargement 247–8 in double-outlet ventricle 291, 294 Fontan procedure and 121, 124–5 in parallel arterial trunk 319 in tetralogy of Fallot 226, 228–9 in transposition 270 tricuspid valve 85 atresia (Fontan procedure) 125, 259–60, 261 straddling 200, 201 valvar relationships 68, 79, 84–5, 86, 218 ventricular pre-excitation 113–19 in ventricular septal defects 187–91 see also sinus node Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:16 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 378 Index atrioventricular groove 27, 118, 230 atrioventricular junction morphology in congenital malformations 132–9 biventricular and mixed atrioventricular 135, 143 concordant 134, 143 discordant 134, 143 uniatrial biventricular 136, 138, 143, 254, 258 univentricular 136–9, 145 valves 140–3 ventricular morphology 132–3, 134, 143 ventricular relationships 143–5 see also double-inlet ventricle atrioventricular nodal tachycardia 119 atrioventricular node 25–6, 112, 113, 118 aortic valve and 79, 82, 85 artery 28, 31, 70, 89, 105, 118 in atrioventricular septal defects 168, 169, 185 in congenitally corrected transposition 280 in double-inlet ventricle 252 mitral valve and 68, 69, 85 in transposition repair 270 atrioventricular septal defects 155–6, 166–85, 209 bridging leaflets morphology 170–6 relationship to septal structures 172–8 in common arterial trunk 306 complete 172 conduction pathways 167–70, 181–2, 184–5 Gerbode defects 34, 164–5, 165–7 hypoplasia of the ventricular septum 179–81 intermediate 172, 177 left ventricular dominance 184–5 left ventricular outflow tract is narrow 182–3 mural leaflets 171 partial 172, 178 right ventricular dominance 183–4 terminology 162–4 ventricular shunting only 175–6, 178–80 atrioventricular septum membranous see membranous septum muscular 27, 151, 153 atrioventricular valves 52, 53–4, 55–6, 61–6 common 141, 170–6, 174 atrial appendage isomerism 373–4 common arterial trunk 306 double-inlet ventricle 246, 255 double-outlet ventricle 301 in congenitally corrected transposition 289–91 malformations 199–208 morphology in malformed hearts 140–3 see also mitral valve; tricuspid valve azygous vein 11, 12 with inferior caval vein abnormalities 324 pulmonary venous drainage via 328–30 B Bachmann’s bundle 22 basal cords 63, 65, 68, 74 bicuspid valve see mitral valve Bland–White–Garland syndrome 353, 354–5 brachiocephalic artery 12, 46 mirror-imaged 344–5 brachiocephalic vein 2, 5, 109 pulmonary venous drainage via 328, 329 bronchial arteries 46, 47, 48, 350 bronchial obstruction 348 bundle of His see atrioventricular bundle bundles of Kent 115 C Cantrell’s syndrome 365–6 cardiac position/malposition see under heart cardiac veins 105–7, 161 great 86, 87, 106, 107 middle 106 small 106 see also coronary sinuses carotid artery, left 46 Carpentier’s classification of mural leaflet scallops 66, 68 caval veins inferior abnormalities 322, 323, 324 atrial appendage isomerism 132, 374 oval fossa and 156, 159 scimitar syndrome 330 sinus venosus and 156, 160 persistent left superior 8, 17, 18, 322–3, 325 unroofing of the coronary sinus 159–60, 161–2, 164 superior abnormalities 322 proximity of phrenic nerve 3, sinus venosus and 156–9, 161 in venous switch procedures 267 total cavopulmonary connection 248–50, 252, 259 cavoatrial junction 159 central fibrous body 31–3, 45, 57–9 ventricular septal defects 186 circumflex coronary artery 71, 96, 101–2, 105 valves and 70, 88–9 coarctation of the aorta 211, 300–1, 334–7 intercostal arteries 46–7, 336 commissural cords 63, 64 commissures 55, 63 common arterial trunk 145, 148, 303–13 arterial anatomy 307–13 definition 303, 304 interventricular communications 306–7 surgical repair 304–5 truncal valve 303, 307, 308–9 conduction axis see atrioventricular conduction axis congenital malformations cataloguing 148–9 morphological analysis 129 atrial arrangement 129–32 atrioventricular connections 132–9 cardiac position 148 valves 140–3, 145, 148 ventricular relationships 143–5 ventricular topology 132–3, 134, 143 ventriculoarterial junctions 145–8 surface anatomy 16, 17, 18, 19 see also specific conditions congenitally corrected transposition 144, 279–91, 366 complicating lesions 282–91 conoventricular perimembranous defects 191 coronary arteries 91–105 anomalies classification 353 epicardial course 94–6, 355–6, 358 fistulous communications 357–9 intramural course 355, 356 origins 91–6, 211–12, 313, 353–5 in common arterial trunk 310, 314 in congenitally corrected transposition 366 in double-outlet ventricle 301 interventricular anterior 88, 96, 100–1, 103, 366 inferior 89, 97–8, 100, 101, 105 left 19, 87–8, 99–101 anomalies 92, 94, 353, 354–5, 357, 360 atresia 355, 357 circumflex 70, 71, 88–9, 96, 101–2, 105 common arterial trunk 313, 314 dominance 70, 101, 105 intermediate 100, 103 main 99 myocardial bridging 102, 106 right 19, 39, 97–9 anomalies 92, 93, 94, 95, 353, 357, 359 dominance 97–8, 101 in tetralogy of Fallot 231 valves and 72, 89 in transposition 278, 279–82 valves and 87–9 mitral 70, 71, 88–9 tricuspid 72, 75, 89, 97, 98, 100 coronary sinuses 106–7, 322 atresia 325, 327 in atrioventricular septal defects 168–70 dilation 325 pulmonary venous drainage via 330, 331, 334 unroofing 159–62, 163–4, 334 valvar relationships 70, 86–7, 87–88 coronary veins 105–7, 161 see also coronary sinuses Cox maze III procedure 126 criss-cross heart 145, 366–9 cysterna chyli 107 D d-transposition 263, 276 Damus–Kaye–Stansel procedure 247 dead-end tract 112 dextrocardia 364 see also right-sided heart diverticulum of Kommerell 343, 344 double aortic arch 341–3 double-inlet ventricle 136, 245–54 conduction axis 247–8, 252–3, 254 left dominant 136, 245, 247–53 Fontan procedure 247, 248–52 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:16 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Index Holmes heart 248, 315, 316 parallel arterial trunks 314–17 septation procedure 252–3 right dominant 246, 254 solitary/indeterminate ventricle 136, 139, 140, 254, 256 double-outlet ventricle 145, 146, 147, 225, 261, 292–303 definition 292–4 discordant atrioventricular connections 291, 294 left 303 nature of the interventricular communication 294–9 surgical repair 297, 302–3 double switch procedure 291 ductus arteriosus see arterial duct E Ebstein’s malformation 117, 202–4, 262 in congenitally corrected transposition 289, 290 ectopia cordis 365–6 Eisenmenger’s syndrome 323 endocarditis of the aortic valve 218 epicardium 14–16 Eustachian ridge 23, 29 Eustachian valve 156, 260, 323 exteriorisation of the heart 365–6 hemitruncus 345 hepatic veins 322 Holmes heart 248, 315, 316 Horner’s syndrome 12 hypoplastic left heart syndrome 140, 260, 263–6 I indeterminate ventricle 140, 254, 256 inferior caval vein see caval veins, inferior infundibular artery 93, 99 infundibulum 38, 39, 41, 83, 151 absent in ventricular septal defect 196, 197 atretic 235–6, 237 in double-outlet ventricle 297, 298–9, 303 morphological analysis 145–8 stenosis 300 in transposition 278 interatrial groove see Waterston’s groove intercostal arteries 46–7, 336 intercostal spaces fourth 3, 12 fifth sixth 3, intercostal veins 6, 8, 9–10 interrupted aortic arch 300–1, 337–40 interventricular arteries anterior 88, 96, 100–1, 103, 366 inferior 89, 97–8, 100, 101, 105 F Fallot’s tetralogy see tetralogy of Fallot fibrous tissue aortic valve 79, 81 aortic–mitral fibrous curtain 45, 56, 58, 79 central fibrous body 31–3, 45, 57–9, 186 fibrous trigones 33, 56, 57 Fontan procedure combined mitral and aortic atresia 260–1 conduction axis and 121, 124–5 double-inlet ventricle left dominant 247, 248–52 right dominant 254 tricuspid atresia 125, 257–60, 261 foramen ovale see oval fossa G gastric veins 331, 333 Gerbode defects 34, 164–7 Glenn procedure 162, 250 H heart malposition 364 criss-cross 145, 366–9 exteriorisation 365–6 right-sided 347–8, 364, 366 position 2, 148 surface anatomy 14, 16–19 K Koch’s triangle 25–8, 29–30, 72, 112, 119, 152 Konno procedure 80, 83 L laryngeal nerve see recurrent laryngeal nerve lateral thoracotomy left 3–6 right 6–12 latissimus dorsi left atrium 34–5 access 15, 19, 34 appendage 17, 34, 36, 129, 130, 131 isomerism 19, 129–30, 131, 132, 133, 369–74 vestibule 35, 37 left bundle branch 45, 85, 112, 115 in congenitally corrected transposition 281 in double-inlet ventricle 252 left ventricle 43–5 double-outlet 303 incomplete 254, 255 in univentricular atrioventricular connections 137–8, 145 ventriculoarterial junction 52, 54 left ventricular dominance 136, 184–5, 247–53 Fontan procedure 247, 248–52 Holmes heart 248, 315, 316 parallel arterial trunks 314–17 septation procedure 252–3 left ventricular outflow tract (subaortic outflow tract) 45 obstruction/stenosis 80, 83, 210, 213–18 in atrioventricular septal defects 182–3 in transposition 274, 276 Leiden convention (aortic sinus nomenclature) 91, 93 levoatrial cardinal vein 323, 326 ligament of Marshall 49 linea alba lung agenesis of the right lung 347–8 arterial supply in tetralogy of Fallot 224, 232–4 lymphatic system 6, 10, 107–10, 337, 350 M macro-reentrant atrial tachycardia 125 Mahaim connections 114, 119 median sternotomy 2–3, 5, 16 mediastinum 2, 3, membranous septum 32–4, 45, 79, 151 aneurysm 274 ventricular septal defect 186 mitral atresia 137, 141, 199, 254, 258, 260–1, 323 mitral valve anatomy 53, 61–70, 177 clefts 206, 208 conduction axis and 68, 69, 85 coronary arteries and 70, 71, 88–9 coronary sinuses and 70, 86–7 dilation 201 fibrous support 45, 56, 58, 59, 61 imperforate 140–1, 261, 266 leaflets 44, 61–2, 63, 66–7, 66, 171, 173 aortic 56, 64, 65, 66 mural 62, 65, 66–7, 68 offset with respect to tricuspid valve 26, 30 overriding 142–3 papillary muscles 44, 68, 69, 200 parachute deformity 207–8, 209 prolapse 64, 66, 205, 206–8 straddling 142, 200 tendinous cords 62, 63–4, 67–8 zone of apposition 56, 66–7 see also atrioventricular valves; mitral atresia moderator band 41, 147, 148, 153, 155 in tetralogy of Fallot 231, 232 muscle of Lancisi (medial papillary muscle) 40, 70, 73 Mustard procedure 266, 269–70 N Nikaidoh procedure 275 nodal arteries atrioventricular 28, 31, 70, 89, 105, 118 sinus see sinus node, artery nodule of Arantius 74 non-branching bundle 181, 252, 280, 285–6 non-coronary sinus 78, 92 Norwood procedure 247, 260 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:16 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 379 380 Index O oblique ligament 16 oblique sinus 15–16 omphalocele 365–6 ostium primum defects 155–6, 166–85, 209 ostium secundum defects see oval fossa, defects outflow tract tachycardias 123 oval fossa 23, 27–28, 151, 152 defects 156 absent 158 deficient 22, 157 perforated 157 P papillary muscles mitral valve 44, 68, 69, 200 tricuspid valve anterior 41, 231 medial 40, 70, 73 parachute deformity mitral valve 207–8, 209 tricuspid valve 208 parallel arterial trunks (with concordant ventriculoarterial connections) 313–17 and discordant atrioventricular connections 317–19 patent arterial duct aortic coarctation 334, 336 common arterial trunk 311 surgical closure 348–51 tetralogy of Fallot 232, 233 pectinate muscles 19, 22 atrial arrangements 129, 131–132 penetrating atrioventricular bundle see atrioventricular bundle (bundle of His) pericardial cavity 14–16 oblique sinus 15–16 transverse sinus 14–15, 45, 59, 68, 99 pericardial fold 15 pericardium 14–16 phrenic nerve 2–3, 4, left 6, 8, 46 right 6, 7, portal vein 331, 332–3 primary atrial septum (septum primum) 151 pulmonary arteries aortic origin 345–6, 347 common arterial trunk 307–13 left 49 arising from right 346–7, 348–9 in pulmonary atresia with intact ventricular septum 238 in pulmonary valve insufficiency 239–41 right 12, 49–50, 347 spiral arrangement of arterial trunks 313, 314 mirror-image 315 in tetralogy of Fallot 224, 232–4 total cavopulmonary connection 248–50, 252, 259 trunk 45, 47–9, 145 aortopulmonary window 351–3 atriopulmonary connection 250–2 coronary artery arising from 353, 354–5 dilated, in pulmonary valve insufficiency 239–41 in double-outlet ventricle 295–6 parallel arterial trunks 313–19 solitary 145, 303, 304 see also common arterial trunk pulmonary atresia in congenitally corrected transposition 291, 292 with intact ventricular septum 235–8, 260, 261 in tetralogy of Fallot (with ventricular septal defect) 224, 232–4 pulmonary hypertension 346 pulmonary stenosis 224–6 in congenitally corrected transposition 288–9 subvalvar 210, 273 supravalvar 221–4 in tetralogy of Fallot 232 in transposition 273 valvar 219–21 pulmonary valve absent pulmonary valve syndrome 239 anatomy 55, 57, 73–8, 81–4 dysplasia 218, 224 imperforate 145, 236–7, 238 insufficiency 238–41 leaflets 38, 39, 73–5, 82–3 left adjacent 82 non-adjacent 82, 83 right adjacent 82, 83 overriding 145 ventriculoarterial junction 52, 54, 148, 149 see also pulmonary atresia; pulmonary stenosis pulmonary veins anomalous connection 157, 163, 323–34, 369, 374 atrial fibrillation and 122 pulmonary venous sinus 35 R Rastelli classification 171–2, 174 Type A 174 Type B 175 Type C 175 Rastelli procedure 275 recurrent laryngeal nerve closure of patent arterial duct 350 left 4–6, 9, 46 right 11, 12 right atrium 19–34 access 19 appendage 17, 19, 129, 130, 131 anomalous pulmonary venous connection 132, 330, 369 isomerism 129, 130, 132, 133, 143, 369, 370–2 pulmonary venous drainage to 330, 331 septal surface 22–5, 27 venous sinus 19, 20, 26 vestibule 34 right bundle branch 85, 112, 182, 191 in congenitally corrected transposition 281 right ventricle 19, 35–43 double-outlet see double-outlet ventricle incomplete double-inlet ventricle 247, 248, 248, 252 tricuspid atresia 257, 259 in univentricular atrioventricular connections 137–8, 145 ventriculoarterial junction 52, 54 right ventricular dominance 183–4, 254 right ventricular outflow tract obstruction/stenosis 210, 218 in double-outlet ventricle 300 in transposition 273, 275 in tetralogy of Fallot 227–31 see also infundibulum right-sided aortic arch 343–4 right-sided heart 347–8, 364, 366 Ross procedure 84 S scimitar syndrome 330 secondary atrial septum (septum secundum) 151 secundum defects see oval fossa, defects semilunar valves see aortic valve; pulmonary valve Senning procedure 266, 270 septal band see septomarginal trabeculations septal perforating arteries 42, 100, 104, 105 septation procedure 252–3 septomarginal trabeculations (septal band) 40–1, 42, 147–8, 153, 226 in tetralogy of Fallot 226, 231, 232 in ventricular septal defect 194–5 double-outlet ventricle 297 septoparietal trabeculation 41, 42 hypertrophy in tetralogy of Fallot 225, 229, 230–1 see also moderator band serratus anterior Shone’s syndrome 208 sinus node 19, 21 artery course 19, 20–2, 23–6, 36, 99, 101, 102–3 oval fossa and 156, 158 preservation 34, 159, 251, 259–60, 266–7 variants 21, 23, 102, 103–4, 106, 269 horseshoe 20, 21 isomerism of the atrial appendages 130–1, 369 sinus venosus defects 158–9, 163 transposition 266 sinus venosus defects 156–9, 160–2, 332–3 sinutubular junction 45, 47, 52, 75, 77 pulmonary stenosis 219, 221 supravalvar aortic stenosis 218, 219–21 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:16 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 Index situs inversus 129 situs solitus 129 solitary arterial trunk 145, 303, 304 solitary origin of the coronary arteries 94–6, 97, 313, 355, 356–357 solitary ventricle 136, 139, 140, 254, 256 Sondergaard’s groove see Waterston’s groove spleen 129, 369 strut cords 63, 65, 68 subaortic outflow tract see left ventricular outflow tract subclavian arteries 6, 10, 11, 46 development 341 interrupted aortic arch 339, 340 isolated 345, 346 subclavian sympathetic loop (ansa subclavia) 12 subpulmonary obstruction/stenosis 210, 218 in double-outlet ventricle 300 in tetralogy of Fallot 227–31 in transposition 273, 275 sudden cardiac death 94, 355, 356 sulcus terminalis (terminal groove) 19, 20 superior caval vein see caval veins, superior superoinferior ventricles 366–9 supraventricular crest 38–9, 40, 83, 152, 155, 226 supraventricular tachycardia atrial fibrillation 122–3, 126 atrial flutter 120–1 atrioventricular nodal tachycardia 119 postoperative 124–6, 260, 270 Wolff–Parkinson–White syndrome 114–19 surgical approaches 19 left-sided thoracotomy 3–6 median sternotomy 2–3, 5, 16 right-sided thoracotomy 6–12 ‘swiss-cheese’ ventricular septal defect 193 T tachycardia see supraventricular tachycardia; ventricular tachycardia Taussig–Bing malformation 200, 297, 299, 300–1 tendon of Todaro 25, 32, 59, 63, 112, 113 terminal crest 19, 22 terminal groove 19, 20 tetralogy of Fallot 146–147, 225–34 absent pulmonary valve syndrome 239 aortic connection 231–2 conduction axis 226, 228–9 left superior caval vein 323 pulmonary atresia 224, 232–4 subpulmonary obstruction 227–31 ventricular septal defects 226, 227, 228, 230 Thebesian valve 106–7, 108, 323 thoracic artery, internal 2, 3, thoracic duct 6, 10, 107–10, 337, 350 thoracotomy left-sided 3–6 median sternotomy 2–3, 5, 16 right-sided 6–12 thymus gland 2, 3, 4, 45 arterial supply venous drainage 2, thyroid artery, inferior tissue tags aortic stenosis 217 subpulmonary obstruction in congenitally corrected transposition 288, 289 in transposition 275 total cavopulmonary connection 248–50, 252, 259 trabeculations comparison of left and right ventricles 43, 132, 134 left ventricle 44–5 right ventricle 37–43 see also moderator band; septomarginal trabeculations; septoparietal trabeculation tracheo-oesophageal compression 342 transposition 261–78 aorta 275–8 with complicating lesions 270–5 coronary arteries and the arterial switch procedure 278, 279–82 similarities in parallel arterial trunk 318 terminology 261–4, 276 venous switch procedures 266–70 see also congenitally corrected transposition transverse sinus 14–15, 45, 59, 68, 99 triangle of Koch 25–8, 29–30, 72, 112, 119, 152 tricuspid atresia 137, 141, 199, 203, 251, 254–60 tricuspid valve anatomy 34, 61–6, 70–2 conduction axis and 85 Fontan procedure 125, 259–60, 261 straddling valve 200, 201 coronary arteries and 72, 75, 89, 97, 98, 100 dilation 201 dysplasia 205, 206 Ebstein’s malformation 117, 202–4, 262 in congenitally corrected transposition 289, 290 fibrous annulus rare/absent 59, 62, 72 imperforate 140–1, 142, 260, 261–2 leaflets 36–7, 38–9, 61–2, 63, 70–1 anteriosuperior 70 inferior 71 septal 70, 73, 190 offset with respect to mitral valve 26, 30 papillary muscles anterior 41, 231 medial 40, 70, 73 parachute deformity 208 straddling/overriding 142–3, 189, 199, 200, 201–3 in congenitally corrected transposition 290–1 in transposition 272–3 tendinous cords 62, 63–4, 70, 71, 74 in ventricular septal defect 189, 190, 199 transposition 270–1, 272–3 zones of apposition 37 see also atrioventricular valves; tricuspid atresia truncal valve 303, 307, 308–9 truncus arteriosus see common arterial trunk 381 U univentricular atrioventricular connections 136–9, 145 unroofing of the coronary sinus 159–62, 163–4, 334 V vagus nerve 2, 4, 5, 46 closure of patent arterial duct 350 valvar annulus, in echocardiography 55, 60, 77 valve of Vieussens 107 vascular pedicle 47 vascular rings 340–5 vascular sling 346–7, 348–9 venae cavae see caval veins venous duct 331 venous sinus pulmonary 35 systemic 19, 20, 26 venous switch procedures 266–70 ventricle(s) 17–19 margins 18–19 morphological analysis 132–3, 134, 143 univentricular atrioventricular connections 136–9, 145 ventricular relationships 143–5 solitary 136, 139, 140, 254, 256 superoinferior 366–9 see also left ventricle; right ventricle ventricular pre-excitation 113–19 ventricular septal defects 186–99 categorisation 186, 188 in common arterial trunk 306–7 conduction axis 187–91 in congenitally corrected transposition 283–8, 291 in double-inlet ventricle 247–8 large defects not to be confused with 247, 254, 257 in double-outlet ventricle 294–9 doubly committed and directly juxta-arterial 187, 195–8, 300 congenitally corrected transposition 286, 288 tetralogy of Fallot 226, 230 transposition 273, 274, 277 in interrupted aortic arch 339, 340 muscular 186, 191–5 tetralogy of Fallot 226, 228 transposition 270, 272, 272, 273 perimembranous 186–91, 199 congenitally corrected transposition 287 and doubly committed 198 tetralogy of Fallot 226, 227 transposition 270, 271, 272 in tetralogy of Fallot 226, 227, 228, 230 in transposition 270–3, 277 tricuspid valve and atresia 260 cleft 190 Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:16 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 382 Index ventricular septal defects (cont.) overriding/straddling 189, 199, 270–1, 272–3 ventricular septal hypoplasia 179–81 ventricular septum 151–5 ventricular tachycardia 123 ventriculoarterial junctions 52–3, 77 common see common arterial trunk morphological analysis 145–8 connections 145 infundibulum 145–8 valvar/truncal relationships 148, 149 valves 145 ventriculoinfundibular fold 83, 147, 152, 155 double-outlet ventricle 297 ventricular septal defect 194–5 vestibule of mitral orifice 35, 37 of tricuspid orifice 34 visceral heterotaxy 129, 369 W Warden operation 159 Waterston’s groove (interatrial groove) 19, 22, 26, 28, 34, 35, 251 Wolff–Parkinson–White syndrome 114–19 X xiphoid process Downloaded from Cambridge Books Online by IP 113.166.95.77 on Fri Sep 13 05:22:17 WEST 2013 http://ebooks.cambridge.org/ebook.jsf?bid=CBO9781139028561 Cambridge Books Online © Cambridge University Press, 2013 ... much of the septum delimited on the right ventricular aspect by the septal leaflet of the tricuspid valve separates the inlet of the right ventricle from the outlet of the left (Figure 7.6) The. .. the components of the right ventricle Apart from the apical part, however, these parts of the right ventricle not correlate with comparable components on the left side of the heart Inlet component... 04: 42: 20 WEST 20 13 http://dx.doi.org/10.1017/CBO9781139 028 561.008 Cambridge Books Online © Cambridge University Press, 20 13 158 Wilcox’s Surgical Anatomy of the Heart Apex Inf Sup Fig 7.13 The surgical