The aim of the present study was to investigate outcomes of implantable cardioverter-defibrillator (ICD) treatment in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We reviewed baseline/follow-up data of 15 consecutive ARVC patients (mean age 55 +/- 15 years) and 30 randomly drawn patients with coronary artery disease (CAD) (mean age 60 +/- 10 years) with matching durations of follow-up (all implanted with ICDs for primary/secondary prevention of sudden death). At implant, appropriate placement of the RV lead was more difficult in ARVC patients. During follow-up (median 41 months), appropriate interventions for any ventricular tachyarrhythmias occurred in 8 (53%) ARVC patients and 17 (57%) CAD patients, but the occurrence of high rate (>240 beats/min) ventricular tachyarrhythmias was higher in ARVC patients. Inappropriate ICD interventions occurred in 5 (33%) ARVC patients and 10 (33%) CAD patients. Lead-related adverse events requiring surgical revision occurred in 7 (47%) ARVC patients as compared with 4 (13%) CAD patients (P = 0.0004). While ICD implantation is highly effective for prevention of sudden death in ARVC, it does carry elevated burdens of long-term lead-related adverse events. These findings underline the need of careful follow-up in ARVC aimed at early recognition of complications that can impair ICD function.