title: Implantable cardioverter-defibrillators in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Medicine, Johns Hopkins Hospital, Baltimore, Maryland, USA.
authors.forename: Ariel
authors.initials: A
authors.lastname: Roguin
authors.forename: Chandra S
authors.initials: CS
authors.lastname: Bomma
authors.forename: Khurram
authors.initials: K
authors.lastname: Nasir
authors.forename: Harikrishna
authors.initials: H
authors.lastname: Tandri
authors.forename: Crystal
authors.initials: C
authors.lastname: Tichnell
authors.forename: Cynthia
authors.initials: C
authors.lastname: James
authors.forename: Julie
authors.initials: J
authors.lastname: Rutberg
authors.forename: Jane
authors.initials: J
authors.lastname: Crosson
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Spevak
authors.forename: Ronald D
authors.initials: RD
authors.lastname: Berger
authors.forename: Henry R
authors.initials: HR
authors.lastname: Halperin
authors.forename: Hugh
authors.initials: H
authors.lastname: Calkins
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000628:therapy*; D002648:Child; D017147:Defibrillators, Implantable; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D019919:Prosthesis Implantation / Q000379:methods; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome