Surgical correction of total anomalous pulmonary venous connection to persistent left-sided superior vena cava: a case report

Abstract Background Total anomalous pulmonary venous connection (TAPVC) to left superior vena cava (LSVC) is an extremely rare congenital heart disease, and its surgical management is very challenging. Case summary We report one such case of a 5-year-old south Asian male with double outlet right ventricle and unbalanced atrioventricular canal defect, where all the pulmonary veins were found opening into LSVC, which was then opening into the left side of the common atrium. Intraoperatively, the LSVC was transected just below the left internal jugular vein and left subclavian vein junction and left-sided bidirectional Glenn shunt done using 8 mm Dacron tube graft. Pulmonary veins were left draining through the LSVC into the common atrium. Right-sided Glenn shunt was completed as usual. Currently, the patient is year and half post-surgery and is doing well; school going on par with the peer group maintaining a room air saturation of 87%. Discussion Here, we report a successful surgical correction of TAPVC to LSVC in a child with univentricular physiology, however due to the paucity of data and rarity of such cases, optimal surgical management is yet to be defined.