Total anomalous pulmonary venous connection to the portal and splenic vein associated with unilateral hypoplasia of pulmonary veins.

Total anomalous pulmonary venous return (TAPVR) represents a rare congenital anomaly with wide anatomical and physiological variability. We report a case of a newborn with a challenging form of obstructed infracardiac TAPVR, in whom left and right pulmonary veins drained separately into the portal system. The right pulmonary venous sinus connected to the left branch of the portal vein, whereas the left venous sinus connected to the splenic vein. Surgical repair consisted of the creation of a common retrocardiac venous trunk which was anastomosed to the left atrium. The postoperative course was characterized by persisting congestion of the right lung. Two months later, right pulmonary vein hypoplasia was successfully enlarged with autologous pericardium.