Long-term follow-up in patients with arrhythmogenic right ventricular cardiomyopathy

Cheng Hung Li, Yenn Jiang Lin, Jin Long Huang, Tsu Juey Wu, Chen Chuan Cheng, Wei Shiang Lin, Ta Chuan Tuan, Shih Lin Chang, Li Wei Lo, Yu Feng Hu, Tze Fan Chao, Fa Po Chung, Chin Feng Tsai, Hsuan Ming Tsao, Shih Ann Chen*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

25 Scopus citations


Long-Term Prognosis in Patients with ARVC. Introduction: 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.

Original languageEnglish
Pages (from-to)750-756
Number of pages7
JournalJournal of cardiovascular electrophysiology
Issue number7
StatePublished - Jul 2012


  • arrhythmogenic right ventricular cardiomyopathy/dysplasia
  • catheter ablation
  • implantable cardioverter-defibrillator
  • ventricular tachycardia/ventricular fibrillation


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