Absent Right Superior Vena Cava and Persistent Left Superior Vena Cava in a Pediatric Patient With Double Outlet Right Ventricle: A Case Report

Abstract

Background: Persistent left superior vena cava (PLSVC) with absent right superior vena cava (RSVC) is a rare and mostly asymptomatic congenital anomaly. Its presence may complicate cardiovascular surgical procedures and is associated with an increased risk of perioperative complications. Case: We report a pediatric case where absent right superior vena cava with persistent left superior vena cava was found incidentally before the surgery of a 1-year-old patient with double outlet right ventricle. Intraoperative exploration confirmed the venous pathway, with the right superior vena cava draining into the left via the innominate vein, and subsequently into the coronary sinus in the right atrium. There was no evidence of an unroofed coronary sinus. Rastelli procedure, as the biventricular corrective strategy, was performed successfully and no surgical intervention was required for the venous anomaly. Conclusion: This case emphasizes the importance of thorough preoperative assessment to identify venous anomalies. Awareness of such anatomical variations is crucial for surgical planning and may help reduce the risk of perioperative arrhythmia and other complications.