title: Predictors of appropriate ICD therapy in patients with arrhythmogenic right ventricular cardiomyopathy: long term experience of a tertiary care center.
abstract: BACKGROUND
  Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically transmitted disease prone to ventricular arrhythmias. We therefore investigated the clinical, echocardiographical and electrophysiological predictors of appropriate implantable cardioverter defibrillator (ICD) therapy in patients with ARVC.
  
  
  METHODS
  A retrospective analysis was performed in 26 patients (median age of 40 years at diagnosis, 21 males and 5 females) with ARVC who underwent ICD implantation.
  
  
  RESULTS
  Over a median (range) follow-up period of 10 (2.7, 37) years, appropriate ICD therapy for ventricular arrhythmias was documented in 12 (46%) out of 26 patients. In all patients with appropriate ICD therapy the ICD was originally inserted for secondary prevention. Median time from ICD implantation to ICD therapy was 9 months (range 3.6, 54 months). History of heart failure was a significant predictor of appropriate ICD therapy (p = 0.033). Left ventricular disease involvement (p = 0.059) and age at implantation (p = 0.063) were borderline significant predictors. Patients with syncope at time of diagnosis were significantly less likely to receive ICD therapy (p = 0.02). Invasive electrophysiological testing was not significantly associated with appropriate ICD therapy.
  
  
  CONCLUSIONS
  In our cohort of patients with ARVC, history of heart failure was a significant predictor of appropriate ICD therapy, whereas left ventricular involvement and age at time of ICD implantation were of borderline significance. These predictors should be tested in larger prospective cohorts to optimize ICD therapy in this rare cardiomyopathy.
authors.affiliation: Clinic for Cardiology, Cardiovascular Center, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: Pia K
authors.initials: PK
authors.lastname: Schuler
authors.forename: Laurent M
authors.initials: LM
authors.lastname: Haegeli
authors.forename: Ardan M
authors.initials: AM
authors.lastname: Saguner
authors.forename: Thomas
authors.initials: T
authors.lastname: Wolber
authors.forename: Felix C
authors.initials: FC
authors.lastname: Tanner
authors.forename: Rolf
authors.initials: R
authors.lastname: Jenni
authors.forename: Natascia
authors.initials: N
authors.lastname: Corti
authors.forename: Thomas F
authors.initials: TF
authors.lastname: Lüscher
authors.forename: Corinna
authors.initials: C
authors.lastname: Brunckhorst
authors.forename: Firat
authors.initials: F
authors.lastname: Duru
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D016896:Treatment Outcome