Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy

Ariel Roguin, Chandra S. Bomma, Khurram Nasir, Harikrishna Tandri, Crystal Tichnell, Cynthia James, Julie Rutberg, Jane Crosson, Philip J. Spevak, Ronald D. Berger, Henry R. Halperin, Hugh Calkins

Research output: Contribution to journalArticlepeer-review

193 Scopus citations


Objectives The aim of this study was to assess the outcome of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) patients treated with an implantable cardioverter-defibrillator (ICD). Background Arrhythmogenic right ventricular dysplasia/cardiomyopathy is associated with tachyarrhythmia and an increased risk of sudden death. Methods This study included 42 ARVD/C patients with ICDs (52% male, age 6 to 69 years, median 37 years) followed at our center. Results Mean follow-up was 42 ± 26 months (range 4 to 135 months). Complications associated with ICD implantation included need for lead repositioning (n = 3) and system infection (n = 2). During follow-up, one patient died of a brain malignancy and one had heart transplantation. Lead replacement was required in six patients as a result of lead fracture and insulation damage (n = 4) or change in thresholds (n = 2). During this period, 33 of 42 (78%) patients received a median of 4 (range 1 to 75) appropriate ICD interventions. The median period between ICD implantation and the first firing was 9 months (range 0.1 to 66 months). The ICD firing storms were observed in five patients. Inappropriate interventions were seen in 10 patients. Predictors of appropriate firing were induction of ventricular tachycardia (VT) during electrophysiologic study (EPS) (84% vs. 44%, p = 0.024), detection of spontaneous VT (70% vs. 15%, p = 0.001), male versus female gender (91% vs. 65%, p = 0.04), and severe right ventricular dilation (39% vs. 0%, p = 0.013). Using multivariate analysis, VT induction during EPS was associated with increased risk for firing in ARVD/C patients; odds ratio 11.2 (95% confidence interval 1.23 to 101.24, p = 0.031). Conclusions Patients with ARVD/C have a high arrhythmia rate requiring appropriate ICD interventions. The ICD therapy appears to be well tolerated and important in the management of patients with ARVD/C.

Original languageEnglish (US)
Pages (from-to)1843-1852
Number of pages10
JournalJournal of the American College of Cardiology
Issue number10
StatePublished - May 19 2004


  • ARVD/C
  • CI
  • EPS
  • ICD
  • LV
  • OR
  • RV
  • SCD
  • arrhythmogenic right ventricular dysplasia/cardiomyopathy
  • confidence interval
  • electrophysiologic study
  • implantable cardioverter-defibrillators
  • left ventricle/ventricular
  • odds ratio
  • right ventricle/ventricular

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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