• Arrhythmogenic Right Ventricular Dysplasia
• Defibrillators, Implantable

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy predisposing patients to potentially life-threatening ventricular arrhythmias (VAs) and sudden cardiac death. As a result, most patients will receive an implantable cardioverter–defibrillator (ICD) for primary or secondary prevention.

Over the years, many authors have evaluated risk factors for VA and sudden cardiac death in ARVC to optimise patient selection for ICD implantation. This has culminated in the development of a risk calculator for individualised prediction of ‘any sustained VA’ and ‘fast VA’, the latter being defined as ventricular fibrillation or VA ≥250 beats/min (www.arvcrisk.com).1 This tool has subsequently been validated as well as further refined by enabling longitudinal risk prediction2 and adding programmed ventricular stimulation as an optional risk stratification tool.3 Despite these advances, the majority of prior studies only focus on the first VA in this patient population, which is notoriously known for the recurrent risk of potentially life-threatening VA.

In their Heart paper, Molitor et al 4 describe the cumulative burden, evolution and triggers of ICD shocks in both primary and secondary prevention in patients with ARVC during follow-up. Out of the 53 included patients, the majority (n=41/53, 78%) received their ICD for secondary prevention. During a median follow-up of 7.9 (IQR 10) years, 54.7% (n=29/53) of patients suffered a total of 71 appropriate shock episodes. Time to first ICD shock was 2.8 (IQR 3.6) …