Total anomalous pulmonary venous connection with persistent left superior vena cava: A retrospective surgical series

Abstract

Introduction: Total anomalous pulmonary venous connection (TAPVC) is a common cyanotic congenital heart disease seen in developing countries at tertiary referral centers. Association with persistent left superior vena cava (LSVC) without innominate vein (H-connection) is an extremely rare condition which makes the surgical correction extremely challenging. Materials and Methods: This was a retrospective case record review of 18 patients with TAPVC in the presence of LSVC without innominate vein (without H-connection) at our institution from January 2007 to February 2021. We detail our experience of our modified surgical technique for this complex anatomy using various modifications for each type of TAPVC with LSVC in the absence of innominate vein. Results: Out of 18 patients of TAPVC with LSVC, 17 patients survived and are being followed up regularly. There was one mortality (5.5%) in a 5-month-old infant with double-outlet right ventricle with ventricular septal defect with severe pulmonary arterial hypertension with infracardiac TAPVC who died in intensive care unit on the 5th postoperative day. Conclusion: In our experience, the modified surgical technique for treating this complex variant of TAPVC is especially useful for favorable long-term outcomes with minimal incidence of recurrent pulmonary venous obstruction.