PO-04-190 LONG TERM OUTCOMES OF VENTRICULAR TACHYCARDIA ABLATION IN DESMOPLAKIN ARRHYTHMOGENIC CARDIOMYOPATHY – A MULTICENTER STUDY

Abstract

Desmoplakin (DSP) pathogenic/likely pathogenic (P/LP) variants have been associated with the development of highly arrhyhtmogenic phenotypes of the spectrum of the arrhythmogenic cardiomyopathy (DSP-ACM). Data addressing arrhythmic outcomes after ventricular tachycardia (VT) ablation in patients (pts) with DSP-ACM are scarce. To report on long-term outcomes of pts with DSP-ACM undergoing VT-ablation All pts with P/LP DSP variant at 8 tertiary academic institutions in North America and Europe undergoing VT ablation were included in the study. Demographic, clinical, and instrumental data, as well as all arrhythmic events including per patient rates of VA episodes/year were collected. Sustained ventricular arrhythmia (VA) episodes (defined as a combination of sustained VT, proper implantable cardioverter defibrillator (ICD) intervention, and ventricular fibrillation episodes) after index procedure were the primary outcome of the study and reported with Kaplan Meyer curves. A per-pt pre- and post- VT ablation paired comparison of VA episodes/year rates was performed as well. Nineteen DSP-ACM pts (29 [25–36] y.o.; 68.4% male; 94.7% probands) referred for VT ablation were included. At the time of index procedures, all patients had a highly arrhythmic profile with a high PVC burden and over 30 different VT morphologies (56.6% right bundle branch block (BBBB); 26.6% left BBB; 16.8% unknown)(Table1). VT ablation targets were located in the LV in 7/19 pts, the RV in 7/19 pts, and both ventricles in 5/19 pts. An epicardial ablation was needed in 14/19 pts. Over a median follow-up of 27 [20–72] months, 10/19 (52.6%) pts experienced recurrent VA episodes (Figure1-PanelA). A significant reduction in per-patient event/year pre- and post- VT ablation was observed (0.50 0.25–0.94] vs 0.14 [0–0.40]; Matched Pair Wilcoxon p=0.009) (Figure1-Panel B). VT ablation in DSP-ACM is effective in significantly reducing the VA burden of the disease. However, it is not uncommon for pts to experience VA recurrences during follow up.