HAL is a multi-disciplinary open access archive for the deposit and dissemination of scientific research documents, whether they are published or not. The documents may come from teaching and research institutions in France or abroad, or from public or private research centers. L'archive ouverte pluridisciplinaire HAL, est destinée au dépôt et à la diffusion de documents scientifiques de niveau recherche, publiés ou non, émanant des établissements d'enseignement et de recherche français ou étrangers, des laboratoires publics ou privés.
Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital malformation which associates discordant atrioventricular and ventriculo‐arterial connections. Although frequently associated with a ventricular septal defect (VSD), its anatomy remains controversial. This could be due in hearts with usual atrial arrangement to the apparently different anatomy of the left‐sided right ventricle compared with a right‐sided right ventricle. We wanted to compare the RV septal anatomy between ccTGA, transposition of the great arteries and normal heart and to determine the anatomy of the VSD in ccTGA. We analysed 102 human heart specimens: 31 ccTGA, 36 transpositions of the great arteries, 35 normal hearts. According to the last classification of VSD (ICD‐11), VSD were classified as outlet if located above the superoseptal commissure of the tricuspid valve and inlet if underneath. We measured the lengths of the superior and inferior limbs of the septal band and the angle between the two limbs. To assess the orientation of the septal band, we also measured the angle between superior limb and the arterial valve above. A VSD was present in 26 ccTGA (84%) and was an outlet VSD in 16 cases (61%). The mean angle between the two limbs of the septal band was 76.4° for ccTGA compared with 90.6° for transposition of the great arteries (P = 0.011) and 76.1° for normal hearts (P= NS). The mean angle between the superior limb of the septal band and the arterial valve above was 70.6° for ccTGA compared with 90.6° for transposition of the great arteries (P = 0.0004) and 69.1° for normal hearts (P= NS). The inferior limb of the septal band was significantly shorter in ccTGA (P < 0.0003): SL/IL length ratio was 21.4 for ccTGA, 2.2 for transposition of the great arteries and 1.5 for normal hearts. The typical VSD in ccTGA is an outlet VSD. Its frequent misdiagnosis as an inlet VSD might be explained by the shortness of the inferior limb, which creates the illusion of a posterior VSD, and by the fact that the VSD is usually assessed from the left ventricular aspect. Surprisingly, the orientation of the septal band is similar in ccTGA and normal heart, despite the discordant atrioventricular connections, and different in ccTGA and transposition of the great arteries, despite the discordant ventriculo‐arterial connections. These findings suggest that the mechanism leading to transposition in ccTGA and in TGA probably is different. The term ‘double discordance’ might therefore be more appropriate as a description of this complex anomaly.
Background Congenitally corrected transposition of the great arteries (ccTGA) or double discordance is a rare congenital heart defect associating discordant atrioventricular and ventriculoarterial connections. Late prognosis depends on the progressive failure of the systemic right ventricle (RV). A possible cause for systemic RV dysfunction could be ventricular asynchrony. Cardiac resynchronization therapy (CRT) may thus be indicated in some cases. However, the cardiologists may experience some problems with the coronary sinus (CS) when implanting a 3-lead CRT transvenous system in these patients. Aim of the study To evaluate the anatomy of the CS and cardiac veins in specimens with ccTGA, in order to assess the feasibility of transvenous CRT. Material and methods Among the anatomic collection of the French Centre of Reference for complex CHD, 51 heart specimens had ccTGA with 2 ventricles. There were 33 post-natal and 18 fetal hearts. Hearts were reviewed with special attention paid to the course and drainage of the CS and cardiac veins. Segmental anatomy, location of the ventricular septal defect (VSD), status of the pulmonary outflow tract and anomalies of the atrioventricular valves were reviewed. Results Segmental anatomy was {S,L,L} in 46/51 hearts, {S,L,D} in 2 and {I,D,D} in 3. There was a VSD in 40 (outlet in 25, inlet in 11, both in 2, muscular in 2), pulmonary atresia in 13, subpulmonary stenosis in 6, abnormal tricuspid valve in 20/48 including Ebstein anomaly in 6, straddling in 9 (3 had replacement). The CS was always located behind the morphologically left atrium (LA). However, its anatomy was normal, with normal drainage into the morphologically right atrium, in only 25/51 (49% of cases). The CS was of reduced length with normal orifice in 17. Orifice was atretic with normal size CS in 2, and CS was completely absent in 6 with direct drainage of coronary veins into the LA. At least 1 available vein was found in all cases with patent CS orifice. Conclusion CS in ccTGA is always located behind the morphologically LA. However, its anatomy is abnormal in half of cases. The most frequent anomalies are reduced length (33%) and absent CS or atretic orifice (15.5%). The anatomy of CS should therefore be assessed by imaging techniques (multislice CT imaging or CS venography) before considering transvenous CRT in these patients.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.