Abstract

BackgroundMost patients with isolated congenital atrioventricular block will eventually require a pacemaker implantation at an early age. AimsTo document a single-center experience of all patients with pacemaker implantation secondary to isolated congenital atrioventricular block. MethodsA retrospective review of all patients with isolated congenital atrioventricular block and pacemaker implantation over a period of 11 years was conducted. All patients were identified by using a surgical database. Descriptive statistics were mainly used. ResultsEighteen patients met the inclusion criteria. The median follow-up time, gestational age, and birth weight were 3.8 years, 35 weeks, and 2.1 kg, respectively. Eleven patients were born to mothers with autoimmune disease (61%). The median age at the time of initial implantation, initial generator longevity, and hospital stay per intervention were 5 days, 5 years, and 7 days, respectively. All patients had epicardial pacing except for one patient, who had a transvenous system. Among the 18 patients, initial implants consisted of 13 single-chamber, three dual-chamber, and two with temporary pacing only. Two patients underwent an upgrade to a biventricular system due to associated dilated cardiomyopathy without response. To date, all initially implanted leads are functional except for four leads. Four patients died due to extreme prematurity and from complications of dilated cardiomyopathy. ConclusionIsolated congenital atrioventricular block is associated with significant mortality, especially when associated with dilated cardiomyopathy. Baseline left bundle branch block escape rhythm was noted to be present in most patients with dilated cardiomyopathy. Overall, lead performance and battery longevity were excellent. However, transvenous-type temporary pacing was associated with significant complications.

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