title: Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular cardiomyopathy: the course of electronic parameters, clinical features, and complications during long-term follow-up.
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Division of Cardiology, Azienda Ospedaliera Universitaria Integrata, Piazzale Stefani 1, 37126, Verona, Italy, mugnai.giacomo@gmail.com.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Mugnai
authors.forename: Ruggero
authors.initials: R
authors.lastname: Tomei
authors.forename: Clementina
authors.initials: C
authors.lastname: Dugo
authors.forename: Luca
authors.initials: L
authors.lastname: Tomasi
authors.forename: Giovanni
authors.initials: G
authors.lastname: Morani
authors.forename: Corrado
authors.initials: C
authors.lastname: Vassanelli
mesh_terms: D000293:Adolescent; D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000628:therapy*; D017147:Defibrillators, Implantable / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome