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Implantable cardioverter–defibrillator shocks during long-term follow-up in arrhythmogenic right ventricular cardiomyopathy
  1. Steven A Muller1,2,
  2. Anneline S J M te Riele1,2
  1. 1Cardiology, UMC Utrecht, Utrecht, The Netherlands
  2. 2Netherlands Heart Institute, Utrecht, The Netherlands
  1. Correspondence to Dr Anneline S J M te Riele, Cardiology, UMC Utrecht, 3584 CX Utrecht, Netherlands; ariele{at}

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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 ( 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 al4 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) …

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  • Contributors Both authors were responsible for conceptualising, writing, editing and reviewing the article.

  • Funding ASJMtR is supported by the ZonMW Off Road Grant 2021.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

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