Background: Long-term outcomes of cardiac implantable electronic devices (CIEDs), including permanent pacemakers (PPM) and implantable cardioverter defibrillators (ICD), are ill-defined in adult congenital heart disease (ACHD).

Objective: To assess outcomes of transvenous (TV) and epicardial (EPI) CIEDs in ACHD.

Methods: A retrospective review of CIEDs implanted in patients >18 years-old followed at the Ahmanson/UCLA ACHD Center was performed. Patients were grouped by implant approach (TV vs. EPI). Primary outcomes included time to CIED dysfunction, lead dysfunction and unplanned CIED reintervention.

Results: Over a 27-year period, 283 CIEDs (208 TV, 75 EPI) were implanted in 260 ACHD patients. Dysfunction developed in 77 CIEDs (50 TV, 27 EPI) for which 62 underwent unplanned reintervention (47 TV, 15 EPI). Time to CIED dysfunction and unplanned reintervention did not differ by implant approach; however, lead dysfunction was greater for EPI vs TV (HR 2.0, 95% CI 1.2–3.2, p = 0.01). Independent predictors of lead failure included cyanosis (HR 2.6, 95% CI 1.1–6.3; p = 0.03), implant indication other than bradycardia (HR 3.3, 95% CI 1.6–6.5; p < 0.01), right-sided Maze operation (HR 2.5, 95% CI 1.3–5.0; p = 0.01), and unipolar lead design (HR 4.5, 95% CI 1.8–11.5; p < 0.01). Importantly, EPI vs TV approach was not associated with lead dysfunction after adjusting for baseline covariates (HR 0.6, 95% CI 0.6–4.3; p = 0.3).

Conclusion: Overall, CIED system dysfunction and reinterventions are similar, whereas lead dysfunction is greater among EPI than TV devices. Patient and procedural differences, rather than EPI vs TV implant approach alone, appear to drive long-term CIED lead outcomes in the ACHD population.