title: Long-term follow-up in patients with arrhythmogenic right ventricular cardiomyopathy.
abstract: BACKGROUND
  Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of sudden cardiac death due to tachyarrhythmias. The purpose of this study was to investigate the long-term prognosis in patients with ARVC and the incidence of rapid ventricular arrhythmias during follow-up.
  
  
  METHODS
  Thirty ARVC patients (19 male, 63.3%, mean age 48 ± 15 years) fulfilling modified Task Force criteria 2010 were included. Of them, 13 patients (43.3%) received implantable cardioverter-defibrillator (ICD) implantation. Rapid ventricular arrhythmia was defined as electrical storm or the occurrence of ventricular tachycardia (VT) or ventricular fibrillation (VF) with a cycle length of 240 ms or less that necessitate shock delivery to 2 or more times within a 24-hour period.
  
  
  RESULTS
  With a mean follow-up of 68 ± 10 months, 6 patients (20%) with ICD implantation had recurrent rapid VT/VF. One (3.3%) of them died of multiple shocks and SCD, and 5 (16.7%) had multiple ICD therapies due to VT/VF and electrical storm. The interval between the diagnosis of ARVC and occurrence of rapid VT/VF was 13.4 ± 4.9 months. Most (5/6, 83.3%) events of recurrent rapid VT/VF occurred within 2 years. Ablated patients who did not receive an ICD implant were totally free of rapid VT/VF.
  
  
  CONCLUSIONS
  For patients with ARVC, long-term prognosis is favorable. During a long-term follow-up, patients meeting the criteria for ICD implantation have a higher rate of rapid and potentially life-threatening arrhythmias. However, early and clustered recurrence of rapid VT/VF in patients with an ICD is common, whereas late occurrence of rapid VT/VF is very rare.
authors.affiliation: Division of Cardiology, Taipei Veterans General Hospital, Yuanshan Branch, Yi-Lan, Taiwan.
authors.forename: Cheng-Hung
authors.initials: CH
authors.lastname: Li
authors.forename: Yenn-Jiang
authors.initials: YJ
authors.lastname: Lin
authors.forename: Jin-Long
authors.initials: JL
authors.lastname: Huang
authors.forename: Tsu-Juey
authors.initials: TJ
authors.lastname: Wu
authors.forename: Chen-Chuan
authors.initials: CC
authors.lastname: Cheng
authors.forename: Wei-Shiang
authors.initials: WS
authors.lastname: Lin
authors.forename: Ta-Chuan
authors.initials: TC
authors.lastname: Tuan
authors.forename: Shih-Lin
authors.initials: SL
authors.lastname: Chang
authors.forename: Li-Wei
authors.initials: LW
authors.lastname: Lo
authors.forename: Yu-Feng
authors.initials: YF
authors.lastname: Hu
authors.forename: Tze-Fan
authors.initials: TF
authors.lastname: Chao
authors.forename: Fa-Po
authors.initials: FP
authors.lastname: Chung
authors.forename: Chin-Feng
authors.initials: CF
authors.lastname: Tsai
authors.forename: Hsuan-Ming
authors.initials: HM
authors.lastname: Tsao
authors.forename: Shih-Ann
authors.initials: SA
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D017115:Catheter Ablation / Q000009:adverse effects / Q000401:mortality; D016009:Chi-Square Distribution; D016757:Death, Sudden, Cardiac / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D018572:Disease-Free Survival; D004554:Electric Countershock / Q000009:adverse effects / Q000295:instrumentation* / Q000401:mortality; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D013624:Taiwan; D013997:Time Factors; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D055815:Young Adult