title: Implantable cardioverter defibrillators in arrhythmogenic right ventricular dysplasia/cardiomyopathy: patient outcomes, incidence of appropriate and inappropriate interventions, and complications.
abstract: BACKGROUND
  Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiomyopathy characterized by ventricular arrhythmias and an abnormal right ventricle. Implantable cardioverter defibrillator (ICD) therapy may prevent sudden cardiac death in patients with ARVD/C. Currently, an overview of outcomes, appropriate and inappropriate interventions, and complications of ICD therapy in ARVD/C is lacking.
  
  
  RESULTS
  A literature search was performed to identify studies reporting outcome and complications in patients with ARVD/C who underwent ICD implantation. Of 641 articles screened, 24 studies on 18 cohorts were eligible for inclusion. In case of multiple publications on a cohort, the most recent publication was included in the meta-analysis. There were 610 patients (mean age, 40.4 years; 42% women), who had an ICD for primary or secondary prevention of sudden cardiac death. Risk factors for sudden cardiac death were presyncope (61%), syncope (31%), previous cardiac arrest (14%), ventricular tachycardia (58%), and ventricular fibrillation (6%). Antiarrhythmic medication consisted mostly of β-blockers (38%), amiodarone (14%), or sotalol (30%). During the 3.8-year follow-up, annualized cardiac mortality rate was 0.9%, annualized noncardiac mortality rate was 0.8%, and annualized heart transplant rate was 0.9%. The annualized appropriate and inappropriate ICD intervention rates were 9.5% and 3.7%, respectively. ICD-related complications consisted of difficult lead placement (18.4%), lead malfunction (9.8%), infection (1.4%), lead displacement (3.3%), and any complication (20.3%).
  
  
  CONCLUSIONS
  Cardiac and noncardiac mortality rates after ICD implantation in patients with ARVD/C are low. Appropriate ICD interventions occur at a rate of 9.5%/y. Inappropriate ICD interventions and complications lead to considerable ICD-related morbidity.
authors.affiliation: Department of Cardiology, Thoraxcenter, Erasmus MC, Rotterdam, The Netherlands. a.schinkel@erasmusmc.nl
authors.forename: Arend F L
authors.initials: AF
authors.lastname: Schinkel
mesh_terms: D000328:Adult; D000368:Aged; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D016757:Death, Sudden, Cardiac; D017147:Defibrillators, Implantable; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D018570:Risk Assessment; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality
chemical_list: D000889:Anti-Arrhythmia Agents
keywords: arrhythmogenic right ventricular dysplasia/cardiomyopathy; complication; implantable cardioverter defibrillator; prognosis; sudden cardiac death