Perioperative management of total anomalous pulmonary venous drainage

Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart disease involving abnormal drainage of all the four pulmonary veins in to systemic venous drainage or right atrium. TAPVD can occur in isolation or as an additional component of complex congenital heart disease with univentricular or biventricular physiology. If corrected at the right time it allows the baby to have essentially normal growth and development with good quality of life but delay in diagnosis and referral can result in grave consequences like severe pulmonary hypertension, cardiogenic shock and mortality. It can present in the neonatal period or infancy depending upon obstruction to the abnormal pulmonary venous drainage. Obstructed TAPVD when presenting in the neonatal period constitutes a significant pre operative challenge and the outcome is often determined by the positive synergy in the transport and referral hospital team. The current review is targeted to stream line perioperative management strategy based on the currently available evidence in literature.