Abstract
Although persistent left superior vena cava (PLSVC) itself is a common venous anomaly in congenital heart disease, PLSVC with absent right superior vena cava (RSVC) is a rare venous congenital malformation. Due to the lack of symptoms, this malformation is often detected fortuitously when patients undergo central venous catheter placement, pacemaker implantation, or open cardiac surgery. This particular venous malformation is rare, but clinicians in many fields should be well aware of its variations and management techniques to avoid complications. Anesthesiologists should know that patients with PLSVC rarely have absent RSVC. TEE was helpful in the diagnosis of PLSVC with absent RSVC during emergent surgery.