Abstract 16513: Very Long-Term Follow-up in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy and an Implantable Cardioverter/Defibrillator
Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of ventricular tachycardia (VT) and sudden cardiac arrest (SCA) in young patients (pts). Treatment with implantable cardioverter/defibrillators (ICD) has proven to be effective in preventing arrhythmic death. However, data on the very long-term follow-up (FU; >5 years) in these pts are missing.
Methods and Results: Sixty pts with ARVC (age 44±17 years) were treated with a transvenous ICD system. Among these were 3 pts (5%) who received an ICD for primary prophylaxis, 3 pts (5%) with prior syncope, 36 pts (60%) with sustained VT, and 18 pts (30%) with survived SCA. During the long-term follow-up period of up to 18 years (mean FU: 11±5 years), event-free survival was 49%, 23%, 15%, and 8% for appropriate ICD therapies and 78%, 50%, 32%, and 15% for potentially fatal fast VT (fVT; >240 bpm) after 1, 5, 10, and 15 years, respectively. fVT occurred in 2/3 pts (67%) with primary prophylactic ICD, in 1/3 pts with prior syncope (33%), in 16/36 pts (44%) with sustained VT, and in 7/18 pts (39%) after SCA (P =ns). During FU, 12/60 pts (20%) died, 5/12 pts (42%) due to progressive heart failure. Three patients (5%) underwent heart transplantation 3.5±1.3 years after ICD implantation. Freedom from adverse events was 86%, 60%, 32%, and 10% after 1, 5, 10, and 15 years, respectively. Multivariate analysis identified inducible VT during programmed ventricular stimulation as the only independent predictor of appropriate ICD discharge (P <0.03).
Conclusions: These results strongly suggest an improvement in long-term prognosis by ICD therapy in high-risk patients with ARVC. However, meticulous placement and long-term observation of transvenous lead performance with focus on sensing function are required for the prevention and/or early recognition of disease progression and lead-related morbidity during the long-term follow-up of ICD therapy in ARVC.
- © 2010 by American Heart Association, Inc.