Permanent pacing in Ebstein's anomaly

Patients with Ebstein's anomaly present unique challenges to permanent pacing due to anatomical variations and tricuspid valve replacement. We retrospectively reviewed our experience with permanent pacing in patients with Ebstein's anomaly between 1976 and 1993. We identified 401 patients with Ebstein's anomaly, of whom 15 (3.7%) required permanent pacing (1 of the 15 was implanted elsewhere). Of the 15, there were 8 females and 7 males (mean age 32 years [range 7-74]); the indications for pacing were AV block in 11 and sinus node dysfunction in 4. Eight patients were programmed with VVI and seven with DDD. All WI patients were paced epicardially. Two patients with DDD pacemakers had transvenous atrial and ventricular leads, 4 DDD patients had transvenous atrial leads and epicardial ventricular leads, and 1 patient had both epicardial and transvenous systems. Associated surgical procedures included tricuspid valve replacement in 14 of 15, atrial septal defect repair in 10 of 15, atrioplasty in 7 of 15, prior tricuspid annuloplasty in 4 of 15, pulmonary vein dilation in 1 of 15, and conduction system ablation in 2 of 15. Patients had a mean follow-up of 35 months (range 1-168 months). Complications requiring operative intervention occurred in four patients. One patient had displacement of a transvenous ventricular lead. A second patient had an epicardial lead failure. A third patient had a nonfunctioning atrial lead that displaced across the tricuspid valve, causing severe tricuspid regurgitation. The fourth patient had multiple epicardial and endocardial leads exit block with secondary diaphragmatic stimulation. Permanent pacemakers were required in 3.7% of patients with Ebstein's anomaly, with the indication being intrinsic conduction disease in the majority of patients. Ninety-three percent of patients required tricuspid valve replacement, suggesting more severe manifestation of Ebstein's anomaly. Twenty-seven percent had complications requiring surgical intervention. Thus, permanent pacing in patients with Ebstein's anomaly can be challenging and should be approached by an experienced physician.