Incidence and Clinical Predictors of Early and Late Complications of Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease

Background: The implantable cardioverter-defibrillator (ICD) has been proven to improve survival in adults with congenital heart disease (ACHD), but it is associated with a high rate of complications. We aimed to quantify the incidence of early (≤ 3 months; ECs) and late (> 3 months; LCs) complications in ACHD patients implanted with an ICD and to identify their clinical predictors. Methods: We retrospectively reviewed 207 patients who had ICD follow-up at Toronto General Hospital from 1996 to 2019. Results: The most common diagnoses were tetralogy of Fallot (32.4%), dextro-transposition of the great arteries (17.9%), and congenital corrected transposition of the great arteries (13%). No intraprocedural complications were observed. Median follow-up was 3.4 years (IQR 0.1-23). 24 patients (12%) developed EC (4 hematomas, 20 lead dislodgements). A total of 56 LCs occurred (46% lead failure, 21% infection, 11% prophylactic lead extraction, 9% neurologic pain, 9% erosion, 4% other) with an incidence rate of LCs of 18% per 5 person-years. Anatomic complexity (odds ratio 2.9; P = 0.02) and cardiac resynchronization therapy defibrillator implant (odds ratio 2.5; P = 0.04) were associated with ECs. Survival rates free from LCs were 92%, 86%, and 65%, respectively, after 1, 5, and 10 years. Presence of legacy leads (hazard ratio 2.9; P = 0.006) and subpulmonary ejection fraction (5% increase, hazard ratio 1.35; P = 0.031) were associated with LCs. Conclusions: ACHD patients at risk of sudden cardiac death continue to benefit from newer device technology. However, these patients, particularly those with greater anatomic and device complexity, remain at increased risk of developing complications over their lifetime. Given the life expectancy of this population, careful consideration needs to be given when a device for primary prevention is being contemplated.