A Rare Combination of Transposition of Great Arteries and Total Anomalous Pulmonary Venous Connection

Abstract

The association of total anomalous pulmonary venous connection (TAPVC) with transposition of great arteries (TGA) is a very rare combination of 2 congenital cyanotic heart diseases. TAPVC, in the case of TGA with the intact interventricular septum (IVS), is a cause of early regression of the left ventricle (LV). Very few cases of this rare combination (TAPVC and TGA with or without VSD) have been reported in the world of literature. We found 10 case reports in the literature. The minimum age was 6 days and the maximum age of 16 years. 5 of them, which were operated before 1990, underwent atrial switch with TAPVC repair. Other 4 of them, operated after 1995, underwent arterial switch with TAPVC repair. In this case report we are reporting 2 such rare cases. In both cases, large VSD prevented LV regression.