OBJECTIVE: This review evaluates the outcome of a treatment strategy using dual-chamber pacemakers for neonates with congenital heart block. METHODS: From 1989 to 2006, 10 neonates had dual-chamber epicardial pacemaker placement for congenital heart block. Mean age and weight were 4.4 +/- 5.6 days and 2.8 +/- 0.6 kg. Four patients had heterotaxy syndrome and required concomitant cardiac procedures. One patient had fetal hydrops, myocarditis, and cardiomyopathy. Five patients had structurally normal hearts. Sternotomy (2 partial, 8 full) and unipolar leads (2 non-steroid eluting, 18 steroid-eluting) were used in all. Generators were placed in submuscular bilateral rectus sheath pockets. RESULTS: Successful atrioventricular synchrony was established in all patients. Mean P-wave sensing was 4.7 +/- 2.6 mV and atrial voltage threshold was 0.8 +/- 0.3 V. Mean R-wave sensing was 13.0 +/- 5.7 mV and ventricular voltage threshold 0.9 +/- 0.5 V. There were no wound complications or acute lead failures. Median postoperative stay was 14 days. Three of the 4 patients with heterotaxy died at 3 days, 14 days, and 15 months postoperatively. The patient with cardiomyopathy died suddenly at 6 months of acute myocarditis. No patient with a structurally normal heart died (P < .05). Mean follow-up interval in survivors is 6.1 +/- 7.1 years with 1 patient lost to follow-up. CONCLUSIONS: Implantation of a dual-chamber epicardial pacemaker in neonates with congenital heart block is technically feasible and results in excellent outcomes in patients with structurally normal hearts. System longevity at 6 years is excellent. Patients with congenital heart block and heterotaxy syndrome have a poor prognosis despite dual-chamber pacing.