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Original research
Implantable cardiac defibrillator events in patients with arrhythmogenic right ventricular cardiomyopathy
  1. Olgierd Woźniak1,
  2. Karolina Borowiec1,
  3. Marek Konka1,
  4. Alicja Cicha-Mikołajczyk2,
  5. Andrzej Przybylski3,
  6. Łukasz Szumowski3,
  7. Piotr Hoffman1,
  8. Krzysztof Poślednik1,
  9. Elżbieta Katarzyna Biernacka1
  1. 1Department of Congenital Heart Diseases, National Institute of Cardiology, Warsaw, Poland
  2. 2Department of Epidemiology, Cardiovascular Disease Prevention and Health Promotion, National Institute of Cardiology, Warsaw, Poland
  3. 3Arrhythmia Department, National Institute of Cardiology, Warsaw, Poland
  1. Correspondence to Dr Olgierd Woźniak, Department of Congenital Heart Diseases, National Institute of Cardiology, Warsaw, Poland; owozniak{at}


Objective Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a risk of sudden cardiac death. Optimal risk stratification is still under debate. The main purpose of this long-term, single-centre observation was to analyse predictors of appropriate and inappropriate implantable cardioverter-defibrillator (ICD) interventions in the population of patients with ARVC with a high risk of life-threatening arrhythmias.

Methods The study comprised 65 adult patients (median age 40 years, 48 men) with a definite diagnosis of ARVC who received ICD over a time span of 20 years in primary (40%) or secondary (60%) prevention of sudden cardiac death. The study endpoints were first appropriate and inappropriate ICD interventions (shock or antitachycardia pacing) after device implantation.

Results During a median follow-up of 7.75 years after ICD implantation, nine patients died and six individuals underwent heart transplantation. Appropriate ICD interventions occurred in 43 patients (66.2%) and inappropriate ICD interventions in 18 patients (27.7%). Multivariable analysis using cause-specific hazard model identified three predictors of appropriate ICD interventions: right ventricle dysfunction (cause-specific HR 2.85, 95% CI 1.56 to 5.21, p<0.001), age <40 years at ICD implantation (cause-specific HR 2.37, 95% CI 1.13 to 4.94, p=0.022) and a history of sustained ventricular tachycardia (cause-specific HR 2.55, 95% CI 1.16 to 5.63, p=0.020). Predictors of inappropriate ICD therapy were not found. Complications related to ICD implantation occurred in 12 patients.

Conclusions Right ventricle dysfunction, age <40 years and a history of sustained ventricular tachycardia were predictors of appropriate ICD interventions in patients with ARVC. The results may be used to improve risk stratification before ICD implantation.

  • arrhythmogenic right ventricular dysplasia
  • implanted cardiac defibrillators
  • ventricular tachycardia
  • ventricular fibrillation
  • cardiac arrest

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  • Contributors OW (guarantor): general planning, interpretation of results, discussion. KB: reporting and integration of data. MK: conduct of echocardiographic studies. AC-M: conduct of statistical analysis. AP: conduct of ICD interrogations. ŁS: interpretation of ECG results. PH: interpretation of clinical results. KP: collection of data. EKB: general planning and interpretation of data.

  • Funding This work was supported by the National Institute of Cardiology, Warsaw, Poland.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Patient consent for publication Not required.

  • Ethics approval The study was granted agreement by the local bioethics committee (approval 2.12/IV/2012) and conformed to the principles of the Helsinki Declaration.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.