Aims: To investigate outcome and complications of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (CHD) and to identify predictors of (in-) appropriate shocks. Methods and results: Sixty-four CHD patients ≥ 18 years at first ICD implantation [63% tetralogy of Fallot (TOF) and age at implantation 37 ± 13 years] were identified using the Dutch adult CHD registry and a Belgian tertiary care centre database. Median follow-up duration was 3.7 years. Early complications included pocket haematoma (n = 3), lead failure (n = 2), and pneumothorax (n = 2). Late complications occurred in 11 (17%) patients, including lead failure (n = 6) and and electrical storm (n = 3). Overall, 30 device-related re-interventions were performed in 20 patients (31%), including four premature generator changes and seven lead replacements. Half of the patients received one or more shocks, and 46 shocks in 15 patients (23%) were classified as appropriate. One hundred and sixty shocks in 26 patients (41%) were classified as inappropriate. No predictors of (in-)appropriate shocks were identified, except TOF being associated with less appropriate shocks than patients with other CHD (HR 0.29, P = 0.02). Conclusion: The ICD provided effective therapy in a quarter of adults with CHD with low complication rates. The incidence of inappropriate shocks, however, appeared to be excessive and warrants further attention.

Adults, Congenital heart disease, Implantable defibrillators, Sudden cardiac death, Tetralogy of Fallot,
European Heart Journal
Department of Cardiology

Yap, S.C, Roos-Hesselink, J.W, Hoendermis, E.S, Budts, W, Vliegen, H.W, Mulder, B.J.M, … Drenthen, W. (2007). Outcome of implantable cardioverter defibrillators in adults with congenital heart disease: A multi-centre study. European Heart Journal, 28(15), 1854–1861. doi:10.1093/eurheartj/ehl306