Transposition of the great arteries {S, D, L}

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Transposition of the great arteries {S, D, L} Lucile Houyel, MDa, Richard Van Praagh, MDb, Francois Lacour-Gayet, MDa, Alain Serraf, MDa, Jérôme Petit, MDa, Jacqueline Bruniaux, MDa, Claude Planché, MDa The Journal of Thoracic and Cardiovascular Surgery Volume 110, Issue 3, Pages 613-624 (September 1995) DOI: 10.1016/S0022-5223(95)70092-7 Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 1 Exterior of heart specimen of case 18 (Tables II and III), 2.5-month-old boy, seen from front. Morphologically right atrium (RA) is right-sided, but right atrial appendage(RAA) and left atrial appendage (LAA) are left-sided, indicating visceroatrial situs solitus with left juxtaposition of atrial appendages. Morphologically right ventricle (RV) is right-sided and morphologically left ventricle (LV) is left-sided, denoting D-loop ventricles. Aorta (Ao) arises from right ventricle but is anterior and to left relative to large main pulmonary artery (MPA), which originates from left ventricle. Hence external inspection suggests correct diagnosis: TGA {S,D,L}, with left juxtaposition of atrial appendages and without pulmonary stenosis. The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 2 Heart specimen of case 26 (Tables II and III).A, Opened right ventricle (RV) and L-transposed aorta (Ao). Note that VSD is subaortic and that its inferior margins are formed by prominent right posterior division (RPD) and left anterior division (LAD) of septal band (SB). VSD is not paramembranous, that is, not confluent with tricuspid valve (TV) because of prominent right posterior division of septal band. Subsemilunar conal septum (parietal band) is not well seen. B, Opened left ventricle (LV), stenotic pulmonary outflow tract, and hypoplastic pulmonary artery (PA). Conal septum (CS) is markedly hypoplastic and malaligned leftward and posteriorly, "squeezing" subpulmonary outflow tract. Conal septum forms right angle relative to underlying ventricular septum (Table IV), with conal septum running into subpulmonary outflow tract. VSD is subaortic because of leftward and posterior malalignment of hypoplastic conal septum. Where conal septum abuts pulmonary outflow tract, fibrous tissue (FT) contributes to subpulmonary stenosis. Bicuspidpulmonary valve (PV) is in direct fibrous continuity with anterior leaflet of mitral valve (MV). The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 2 Heart specimen of case 26 (Tables II and III).A, Opened right ventricle (RV) and L-transposed aorta (Ao). Note that VSD is subaortic and that its inferior margins are formed by prominent right posterior division (RPD) and left anterior division (LAD) of septal band (SB). VSD is not paramembranous, that is, not confluent with tricuspid valve (TV) because of prominent right posterior division of septal band. Subsemilunar conal septum (parietal band) is not well seen. B, Opened left ventricle (LV), stenotic pulmonary outflow tract, and hypoplastic pulmonary artery (PA). Conal septum (CS) is markedly hypoplastic and malaligned leftward and posteriorly, "squeezing" subpulmonary outflow tract. Conal septum forms right angle relative to underlying ventricular septum (Table IV), with conal septum running into subpulmonary outflow tract. VSD is subaortic because of leftward and posterior malalignment of hypoplastic conal septum. Where conal septum abuts pulmonary outflow tract, fibrous tissue (FT) contributes to subpulmonary stenosis. Bicuspidpulmonary valve (PV) is in direct fibrous continuity with anterior leaflet of mitral valve (MV). The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 3 A, Opened right ventricle (RV) and L-transposed aorta (Ao) of case 20, 1.5-month-old boy (Tables II through IV). Conal septum (CS) is well developed, is not malaligned far to left, and hence is readily visible from right ventricular perspective. VSD is conoventricular in type, between conal septum above and ventricular septum and septal band (SB) below. Aortic valve (AoV) is widely separated from VSD by subaortic conus. B, Opened left ventricle (LV), showing inferior surface of conal septum (CS) that is angulated 55 degrees leftward relative to ventricular septum (VS) (Table IV) and that is displaced only mildly posteriorly (Table IV). Right surface of the conal septum is flush with left surface of ventricular septum. There is direct fibrous continuity between pulmonary valve (PV) and mitral valve (MV). VSD is closer to pulmonary valve than to aortic valve (A), but VSD is not truly subpulmonary because of leftward and posterior malalignment of conal septum; hence VSD may be described as "subpulmonary." Subpulmonary left ventricular outflow tract is widely patent because conal septal malalignment is mild. The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 3 A, Opened right ventricle (RV) and L-transposed aorta (Ao) of case 20, 1.5-month-old boy (Tables II through IV). Conal septum (CS) is well developed, is not malaligned far to left, and hence is readily visible from right ventricular perspective. VSD is conoventricular in type, between conal septum above and ventricular septum and septal band (SB) below. Aortic valve (AoV) is widely separated from VSD by subaortic conus. B, Opened left ventricle (LV), showing inferior surface of conal septum (CS) that is angulated 55 degrees leftward relative to ventricular septum (VS) (Table IV) and that is displaced only mildly posteriorly (Table IV). Right surface of the conal septum is flush with left surface of ventricular septum. There is direct fibrous continuity between pulmonary valve (PV) and mitral valve (MV). VSD is closer to pulmonary valve than to aortic valve (A), but VSD is not truly subpulmonary because of leftward and posterior malalignment of conal septum; hence VSD may be described as "subpulmonary." Subpulmonary left ventricular outflow tract is widely patent because conal septal malalignment is mild. The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 4 Two-dimensional echocardiographic study of case 15, 7-month-old girl (Tables I and III). Parasternal short-axis view shows anterior and leftward aorta (Ao) and posterior and rightward pulmonary artery (PA), typical of L-TGA. The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 5 Two-dimensional echocardiographic study of case 16, 4-year-old boy (Tables I and III). Parasternal long-axis view shows large subaortic VSD and posterior malalignment of conal septum (CS) with subpulmonary stenosis. LA, Left atrium; LV, left ventricle; PA, pulmonary artery; RV, right ventricle. The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 6 Selective right ventricular (RV) angiocardiogram in posteroanterior projection in case 12, 10-month-old boy (Tables I and III). Right ventricle is superior and small, left ventricle (LV) is inferior and large, and ventricular septum (VS) is approximately horizontal, indicating that ventricular malposition with superoinferior ventricles is present. Inferior vena cava (IVC) and right atrium (RA) are right-sided, indicating situs solitus of viscera and atria. Catheter passed from right atrium to small and superior right ventricle, suggesting concordant atrioventricular alignments. Aorta (Ao) arises from right ventricle to left of large and unobstructed pulmonary artery (PA) that originates from left ventricle. VSD is large and subpulmonary. (See Table III, case 12, for additional anatomic findings.) The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions

Fig. 7 Selective left ventricular (LV) angiocardiogram, left lateral projection, in case 9, 10-month-old boy (Tables I and III). Transposed pulmonary artery (PA) arises from well-developed left ventricle. Conal septum is mildly posteriorly displaced (arrow), without producing subpulmonary stenosis. Also seen are conoventricular type of VSD that is subpulmonary, right ventricle (RV), and transposed aorta (Ao). The Journal of Thoracic and Cardiovascular Surgery 1995 110, 613-624DOI: (10.1016/S0022-5223(95)70092-7) Copyright © 1995 Mosby, Inc. Terms and Conditions