Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular cardiomyopathy: the course of electronic parameters, clinical features, and complications during long-term follow-up

J Interv Card Electrophysiol. 2014 Oct;41(1):23-9. doi: 10.1007/s10840-014-9920-0. Epub 2014 Jun 14.


Purpose: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive cardiomyopathy characterized by myocardial atrophy and fibro-fatty replacement of the right ventricle (RV) and ventricular tachyarrhythmias in young patients. Our aim was to evaluate clinical course and electronic parameters in patients with implantable cardioverter-defibrillator (ICD) and ARVC, during long-term follow-up.

Methods and results: We report on 12 patients with ARVC (mean age 40 ± 13 years) who were treated with ICD implantation in our center. Although several RV sites were tested for proper lead positions, the amplitude of R-wave at implantation was quite low (7.4 ± 3.0 mV). After a mean follow-up of 91 ± 28 months, R-wave amplitude significantly decreased to a mean value of 5.4 ± 2.5 mV (p=0.03). We also found a noticeable, nearly significant increase in pacing threshold (p=0.052) and a moderate increase in defibrillation impedance (p=0.07). Six patients (46 %) experienced at least one appropriate ICD therapy; three patients (23 %) experienced inappropriate ICD shocks secondary to the supraventricular tachycardia, T-wave oversensing, and electromagnetic interference.

Conclusions: ICD in patients with ARVC has been demonstrated to be feasible and safe. In our case series, we found low R-wave amplitudes at implantation and a significant R-wave decrease during follow-up; a considerable and nearly significant increase in pacing threshold was also observed. These findings may be related to the progressive fibro-fatty replacement of RV myocardium. Multiple sites should be tested in the right ventricle if sensing or pacing values are not optimal, and all the electronic parameters should be carefully monitored throughout the entire follow-up.

MeSH terms

  • Adolescent
  • Adult
  • Arrhythmogenic Right Ventricular Dysplasia / complications
  • Arrhythmogenic Right Ventricular Dysplasia / therapy*
  • Defibrillators, Implantable* / adverse effects
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Treatment Outcome