Catheter ablation of electrical storm in patients with arrhythmogenic right ventricular cardiomyopathy

BACKGROUND Therapeutic strategies for electrical storm (ES) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well defined. OBJECTIVE The purpose of this study was to report the acute and long-term results of ventricular tachycardia (VT) radiofrequency catheter ablation (RFCA) as a treatment of ES in patients with ARVC. METHODS This multicenter study retrospectively enrolled 23 consecutive patients with ARVC (mean age 43.6 6 +/- 16.7 years; all men) who underwent 24 RFCA procedures for ES between 2003 and 2015. RESULTS Thirteen patients (57%) had a previous VT RFCA procedure; 14 (61%) had right ventricular dysfunction and 7 (30%) left ventricular ejection fraction <= 50%. The clinical VT was inducible in 19 procedures (79%). Epicardial ablation was performed in 4 procedures (17%). The median number of targeted VTs was 1 (range 1-6). Complete acute success (no VT inducible) was achieved in 11 procedures (46%) and partial acute success (clinical VT nor inducible) in 11 (46%). After a median follow-up of 3.9 years (range 1 month-10 years), ES recurred in 2 patients and end-stage heart failure developed in 4 (17%), leading to 1 death and 3 heart transplantations. At 1-year follow-up, the probability of freedom from VT recurrence was 75% and did not significantly predict long-term survival. At the last evaluation, 8 patients (35%) were free of non-beta-blocker antiarrhythmic drugs as compared with 1 (4%) at baseline (P = .02). CONCLUSION Catheter ablation was efficient to prevent ES recurrence in patients with ARVC. However, these patients were at high risk of evolution toward ARVC-related heart failure that was not associated with VT recurrence.