Can Isolated Discordant Atrioventricular Connections Survive Without Interatrial or Interventricular Shunt?

Clinical presentation of isolated discordant atrioventricular connections is akin to transposition of the great arteries. In the absence of a significant intracardiac shunt, profound cyanosis is expected at birth. We report one such 5-month-old infant who had only mild cyanosis. The left-sided tricuspid valve straddled the interventricular septum with a closed interventricular communication, a type of “Double Outlet Left Atrium with three atrioventricular valves,” which provided the necessary “left to right” shunt while severe regurgitation through the straddling part and a patent ductus arteriosus provided the effective pulmonary blood flow.