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Total anomalous pulmonary venous connection (TAPVC) is a potentially devastating form of congenital heart disease in which all pulmonary blood flow returns to the systemic venous circulation rather than the left atrium. Anomalous pulmonary venous flow may be obstructed at birth, and affected infants present with severe cyanosis and poor cardiac output unresponsive to standard resuscitation with prostaglandin. Obstructed TAPVC remains one of the few indications for emergent neonatal cardiac surgery. This review will discuss the physiology and perioperative management of isolated TAPVC without associated cardiac lesions.