Total anomalous pulmonary venous connection (TAPVC) constitutes a heterogeneous group of lesions in which all of the pulmonary veins are connected to the right atrium or its systemic venous tributaries. A concomitant right-to-left shunt, typically through an interatrial communication, is required for survival. Significant obstruction to pulmonary venous drainage results in pulmonary edema and cardiogenic shock which is rapidly lethal if untreated. Surgical results for the treatment of TAPVC have greatly improved over the last three decades, reducing operative mortality to less than 10% in most centers. The main complication resulting from surgical repair is the development of postrepair pulmonary vein stenosis (PR-PVS) which is associated with high morbidity and mortality.