Congenitally Corrected Transposition of the Great Arteries

Abstract

Congenitally corrected transposition of the great arteries (ccTGA) is a complex congenital heart defect that is defined by atrioventricular and ventriculoarterial discordance. Figure 10.1 shows a coronal view of a heart with ccTGA and ventricular septal defect (VSD). The morphologic right atrium (systemic collecting chamber) is connected to the morphologic left ventricle through a mitral valve, which is connected to the pulmonary artery. The morphologic left atrium (pulmonary venous collecting chamber) is connected to the morphologic right ventricle through a tricuspid valve, which is connected to the aorta. This arrangement results in physiologically corrected but not anatomically corrected circulation. Circulatory aberrations are generally owing to associated lesions that include VSDs, pulmonary stenosis (PS) or atresia, and tricuspid valve abnormalities. Congenitally corrected transposition of the great arteries may also occur in association with situs solitus (normal atrial situs) or situs inversus. In addition, the conduction system in patients with ccTGA is notably atypical, and the incidence of spontaneous heart block is high even without surgical correction.

Figure Credits

• Figs. 10.1–10.5: Devaney EJ, Bove EL. Congenitally corrected transposition of the great arteries. In: Mavroudis C, Backer CL, editors. Pediatric Cardiac Surgery. 4th ed. Hoboken: John Wiley & Sons; 2013. p. 530–41. (Reproduced with permission from John Wiley & Sons).

• Fig. 10.6: Mavroudis C, Backer CL, Kohr LM, Deal BJ, Stinios J, Muster AJ, Wax DF. Bidirectional Glenn shunt in association with congenital heart repairs: the 1½ ventricular repair. Ann Thorac Surg. 1999;68:976–81. (Reproduced with permission of Elsevier).

• Figs. 10.11–10.13: Ilbawi MN, Ocamp CB, Allen BS, Barth MJ, Roberson DA, Chiemmongkoltip P, et al. Intermediate results of the anatomic repair for congenitally corrected transposition. Ann Thorac Surg. 2002;73:594–600. (Reproduced with permission of Elsevier).

• Figs. 10.7–10.10, 10.14–10.35: Mavroudis C. Congenitally corrected transposition of the great arteries. In: Mavroudis C, Backer CL, editors. Atlas of Pediatric Cardiac Surgery. London: Springer-Verlag; 2015. p. 211–24. (Reproduced with permission from Springer Nature).