Abstract
Systemic venous anomalies without other congenital heart defects are usually asymptomatic and often found incidentally during a vascular intervention or other surgery. A 60-year-old man with DDD cardiac permanent pacemaker was admitted to the emergency department with syncope and total atrioventricular block due to end-of-life of the permanent pacemaker. The lead of the transient pacemaker could not be advanced via transfemoral access to the right ventricle. Venography revealed that the left-sided inferior vena cava drained into the persistent left superior vena cava, and both continued with the coronary sinus. To avoid unexpected events, venography should be performed to detect venous congenital anomalies during transient or persistent pacemaker implantation.