Ventricular Cardiomyopathy: Rare Late Occurrence of Rapid Ventricular Tachycardia/Fibrillation

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of sudden cardiac death (SCD), usually due to tachyarrhythmias, for which implantable cardioverter-defibrillator (ICD) shock may be effective. Objective To investigate the long-term prognosis in patients with ARVC. Methods: Thirty ARVC patients (19 male, mean age 48 years) fulfilling modified Task Force criteria 2010 were included. Of them, 13 patients had a history of SCD, unexplained syncope with VT/VF and received ICD implantation. Results: With a mean follow-up of 68 ± 10 months, 6 patients with ICD had recurrent rapid VT/VF. One of them died of cardiac disease, and 5 had multiple ICD therapies due to VT/VF and electrical storm. The interval between diagnosis of ARVC and recurrent rapid VT/VF was 13.4 ± 4.9 months. Most rapid VT/VF occurred within 2 years, except one occurred 30 months later. Ablated patients not qualifying for ICD implant were free of any rapid VT/VF, although recurrence of VT were still noted. Conclusions: For patients with ARVC, long-term prognosis is favorable. During a very long-term follow-up, patients with ICD have a higher rate of rapid and potentially life-threatening arrhythmias. Early recurrence of rapid VT/VF in patients with ICD is common, however, late recurrence of rapid VT/VF very rare.