title: Electrical Storm in ICD Recipients with Arrhythmogenic Right Ventricular Cardiomyopathy.
abstract: BACKGROUND
  Implantable cardioverter defibrillator (ICD) is the most important management for prevention of sudden cardiac death (SCD) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, some patients may receive multiple ICD therapies in a short period, a condition referred as "electrical storm" (ES).
  
  
  OBJECTIVE
  This study aimed to determine the prevalence, therapeutic options, and prognostic implications of ES in ARVC patients with an ICD.
  
  
  METHODS
  We retrospectively analyzed the baseline and follow-up data of 39 ARVC patients with an ICD. ES was defined as three or more separated episodes of ventricular tachycardia or ventricular fibrillation (VT/VF) within a 24-hour period.
  
  
  RESULTS
  During a median follow-up of 49 months (range 6-225), 12 of 39 (31%) patients suffered at least one episode of ES. The interval between the first ES and the initial ICD implantation ranged from 1 month to 109 months, and ES was the first ICD discharge in three patients. The median number of VT/VF events per ES was four (range 3-39). Five patients experienced 20 episodes of ES that were treated by antitachycardia pacing only, while the other seven patients suffered shock therapies during ES. In three patients, ES required emergency hospitalization, and the repeatedly occurred VT/VF was finally subsided by intravenous amiodarone. There was no significant difference in actual survival between patients with and without such an event.
  
  
  CONCLUSIONS
  ES is not rare in ARVC patients with an ICD for prevention of SCD, but it does not independently confer increased mortality. Intravenous amiodarone is effective in management of ES when VT/VF repeatedly occurred.
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Cardiac Arrhythmia Center, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, P. R. China.
authors.forename: Kang
authors.initials: K
authors.lastname: Yin
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Cardiac Arrhythmia Center, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, P. R. China.
authors.forename: Ligang
authors.initials: L
authors.lastname: Ding
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Cardiac Arrhythmia Center, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, P. R. China.
authors.forename: Wei
authors.initials: W
authors.lastname: Hua
authors.affiliation: State Key Laboratory of Cardiovascular Disease, Cardiac Arrhythmia Center, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, P. R. China.
authors.forename: Shu
authors.initials: S
authors.lastname: Zhang
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000401:mortality* / Q000517:prevention & control*; D015984:Causality; D002681:China / Q000453:epidemiology; D015897:Comorbidity; D017147:Defibrillators, Implantable / Q000706:statistics & numerical data*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000401:mortality*; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000401:mortality*
keywords: arrhythmogenic right ventricular cardiomyopathy; electrical storm; implantable cardioverter defibrillator; shock therapy; survival