Systemic venous anatomy in congenital heart disease: implications for electrophysiologic testing and catheter ablation.

INTRODUCTION: Cardiac arrhythmias are a significant problem in patients with congenital heart disease. Many patients with congenital heart disease have abnormal systemic venous anatomy which can complicate electrophysiologic testing, catheter ablation and pacemaker and defibrillator implantation. We reviewed the systemic venous anatomy in a cohort of patients undergoing electrophysiologic testing and catheter ablation. METHODS AND
RESULTS: We reviewed all electrophysiologic studies performed in patients with adult congenital heart disease (n = 80) at our institution between January 1998 and October 2009. Ten patients (13%) had a congenital systemic venous anomaly. Of these, seven (9%) had a left superior vena cava and four (5%) had infrahepatic interruption of the inferior vena cava (two had both anomalies). One patient's inferior vena cava was connected to a left-sided atrium; she had right atrial isomerism. In four patients (40%), systemic venous abnormalities were discovered at the time of electrophysiologic testing.
CONCLUSIONS: Systemic venous anomalies occur frequently in the congenital heart disease population and may complicate electrophysiologic testing and catheter ablation. Pre-procedural imaging may assist in facilitating a successful procedure.