Long-term follow-up of arrhythmogenic right ventricular cardiomyopathy patients with an implantable cardioverter-defibrillator for prevention of sudden cardiac death.

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited cardiomyopathy with a high burden of ventricular arrhythmia, which is an important cause of sudden cardiac death (SCD). Implantable cardioverter-defibrillator (ICD) is believed to be the most reliable management against SCD.

HYPOTHESIS: Ventricular arrhythmia does not necessarily confer a poor prognosis in ARVC patients with an ICD.

METHODS: A total of 39 ARVC patients (34 male) implanted with an ICD at our electrophysiology center and followed up continuously were included in this study. The mean age at diagnosis was 42.1 ± 14.8 years.

RESULTS: Thirty-three patients (84.6%) had suffered ventricular arrhythmia with hemodynamic compromise before ICD implantation. During a median follow-up of 48.6 months (interquartile range, 32.3-73.3), 3 patients (7.7%) died, 1 of sudden death, 1 of heart failure, and 1 of cerebral infarction. Twenty-eight patients (71.8%) experienced 540 appropriate ICD interventions. The first appropriate ICD intervention occurred more than 2 years after initial ICD implantation in 5 patients (12.8%). Twelve patients (30.8%) suffered from electrical storm. The event-free period was significantly shorter in patients who did not have broad precordial T wave inversion ≥V1-V3 (hazard ratio = 0.39, 95% confidence interval: 0.16-0.96). No significant difference was shown in antiarrhythmic drugs and radiofrequency catheter ablation before ICD implantation between patients with and without appropriate ICD therapies (P > 0.05).

CONCLUSIONS: Recurrence of sustained ventricular tachycardia/ventricular fibrillation is frequent in high-risk patients with ARVC. The prognosis is favorable for ARVC patients treated with an ICD for prevention of SCD.