title: Use of implantable cardioverter-defibrillator in inherited arrhythmogenic diseases: data from Italian ICD Registry for the years 2001-6.
abstract: BACKGROUND
  Previous studies have demonstrated the lifesaving role of an implantable cardioverter-defibrillator (ICD) in high-risk patients with genetic arrhythmogenic diseases.
  
  
  OBJECTIVE
  To evaluate the clinical data of patients with hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVD/C), idiopathic ventricular arrhythmia (IDIO-VA) including Brugada syndrome, short QT syndrome, and long QT syndrome (LQT) enrolled in the Italian ICD Registry in the years 2001-6.
  
  
  METHODS
  The survey collected prospectively clinical data and technical characteristics of ICD implants on the basis of EURID form.
  
  
  RESULTS
  The number of patients was 1,274 in the HCM group, 520 in the ARVD/C group, 460 in the IDIO-VA group, and 245 in the LQT group. Primary prevention involved 29.5% of patients in the HCM group, 15.9% in the ARVD/C group, 16.9% in the IDIO-VA group, and 16.6% in the LQT group, respectively. Ventricular tachycardia and ventricular fibrillation were reported in 49.0% and 24.8% in the HCM group, 72.9% and 20.0% in the ARVD/C group, 48.2% and 42.9% in the IDIO-VA group, and 21.2% and 61.6% in the LQT group, respectively. Single-, dual-, and triple-chamber ICDs were implanted in 41.5%, 47.6%, and 10.9% in the HCM group; 57.9%, 36.7%, and 5.4% in the ARVD/C group; 55.0%, 45.0%, and 0% in the IDIO-VA group; and 57.4%, 42.6%, and 0% in LQT group, respectively.
  
  
  CONCLUSIONS
  ICD therapies in the years 2001-6 for patients with inherited arrhythmia syndromes were utilized in Italy in a still-limited number of patients. Secondary prevention represented the major indication for ICD implant and the majority of patients were treated by single-chamber ICDs.
authors.affiliation: Department of Cardiopulmonary Sciences, Azienda Ospedaliero-Universitaria S. Maria della Misericordia, Udine, Italy. ap.ircab@gmail.com
authors.forename: Alessandro
authors.initials: A
authors.lastname: Proclemer
authors.forename: Marco
authors.initials: M
authors.lastname: Ghidina
authors.forename: Domenico
authors.initials: D
authors.lastname: Facchin
authors.forename: Luca
authors.initials: L
authors.lastname: Rebellato
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
authors.forename: Maurizio
authors.initials: M
authors.lastname: Gasparini
authors.forename: Dario
authors.initials: D
authors.lastname: Gregori