Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is one of the most typical congenital heart defects requiring emergent surgery soon after birth. Obstruction of the pulmonary venous drainage that can lead to profound cyanosis, respiratory failure and shock indicates emergent intervention. Prenatal diagnosis of TAPVC contributes to a smooth transition from delivery to surgery. However, prenatal ultrasound screening frequently fails to reveal isolated TAPVC. In neonates with isolated obstructive TAPVC, common pulmonary venous chamber-to-left atrial anastomosis is the intervention of choice except in patients with severe intraventricular hemorrhage, extremely low birth weight, or gestational age of 30 weeks or less. In neonates with right isomerism and functional single ventricle but severely obstructive TAPVC, prognosis after neonatal surgery is extremely poor. Catheter intervention to delay open heart surgery may be preferred in such neonates.