title: High Arrhythmic Burden but Low Mortality during Long-term Follow-up in Arrhythmogenic Right Ventricular Cardiomyopathy.
abstract: BACKGROUND
  Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a high incidence of ventricular tachyarrhythmia and sudden death. The mainstay of management is the implantable cardioverter defibrillator (ICD). A small number of patient cohorts have generated a large number of reports.
  
  
  METHODS
  Prospective registry data supplemented with clinical and ICD records of 30 patients with ARVC fulfilling the 2010 modified Task Force Criteria. This cohort has not been reported on previously.
  
  
  RESULTS
  Median age at diagnosis: 46yrs (range 21-68); 20 (80%) male; six (19%) Maori. Duration of follow-up: 7.4yrs (range 1.7-23). Implantable cardioverter defibrillator implantation in 26; three (12%) for resuscitated sudden cardiac death; 17 (65%) for symptomatic ventricular tachyarrhythmia; three (12%) for syncope; and three (12%) for family history of sudden death attributable to ARVC. Two patients died during follow-up, one had an ICD, though died of a carcinoma. Thirteen (50%) experienced appropriate ICD therapy with median time to therapy 12 months, and four (15%) experienced inappropriate shock therapy. Male gender was an independent predictor of appropriate ICD therapy (HR 1.6, 95% CI 1.5-2.7, P=0.01).
  
  
  CONCLUSIONS
  The long-term prognosis of patients with ARVC is favourable although high proportions receive appropriate ICD therapy. Male gender is an independent predictor of appropriate ICD therapy.
authors.affiliation: Green Lane Cardiovascular Services, Auckland City Hospital, Auckland, New Zealand; Faculty of Medicine and Health Sciences, University of Auckland, Auckland, New Zealand.
authors.forename: Andrew
authors.initials: A
authors.lastname: Martin
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Jackie
authors.initials: J
authors.lastname: Crawford
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand; Department of Paediatrics, Child and Youth Health, University of Auckland. Electronic address: jskinner@adhb.govt.nz.
authors.forename: Jonathan R
authors.initials: JR
authors.lastname: Skinner
authors.affiliation: Green Lane Cardiovascular Services, Auckland City Hospital, Auckland, New Zealand.
authors.forename: Warren
authors.initials: W
authors.lastname: Smith
mesh_terms: D000328:Adult; D000368:Aged; D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D017281:Cost of Illness; D017147:Defibrillators, Implantable; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D012737:Sex Factors
keywords: Arrhythmogenic right ventricular cardiomyopathy/dysplasia; Cardiomyopathy; Death; Electrophysiology; Implantable cardioverter defibrillators; Sudden