Congenitally Corrected Transposition of the Great Arteries

Congenitally corrected transposition of the great arteries (ccTGA) is a rare defect combining atrioventricular discordance with ventriculoarterial discordance. The atria are connected to the opposite ventricle (left atrium to right ventricle via a tricuspid valve) and the ventricles are connected to the incorrect great artery (right ventricle to aorta). Thus oxygenated blood is circulated systemically by the morphologic right ventricle (RV) and deoxygenated blood returns to the right atrium to be pumped out the left ventricle (LV) to the lungs (Figure 1). The defect is therefore “corrected” because of the physiologic flow of blood through the body. For the purposes of this review, univentricular hearts, those with common atrioventricular (AV) valves and those with aortic atresia will not be discussed.