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Notes: 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 WI and seven with DDD. All VVI 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. (PACE 1997;20[Pt. I]:1243-1246)

Notes: Patients with one of several varieties of malformation designated as “univentricular hearts” may be especially challenging when permanent pacing is required. Our objective was to review our experience in this subset of patients that had undergone permanent pacing and thus determine the optimal approach. A retrospective chart review was done of 32 patients with some variety of “univentricular” malformation who had required permanent pacing at our institution. Although technically challenging, permanent pacing in this group of patients can be successful through several approaches. The various approaches, as well as consideration of the differences that exist between patients undergoing septation and those undergoing a Fontan procedure are discussed. Although long-term permanent pacing is possible in this group of patients, before pacing begins, a thorough understanding of the anatomy and prior surgical procedures is necessary. Use of a combined atrial transvenous and ventricular epicardial pacing system may work well for some patients. With the development of newer and more reliable coronary sinus leads, dual chamber transvenous pacing with ventricular stimulation via the coronary sinus could become the approach of choice in some patients with “univentricular hearts.”