Isolated critical epicardial arrhythmogenic substrate abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy and ventricular tachycardia

Ventricular tachycardia (VT) substrate abnormalities in arrhythmogenic right ventricular cardiomyopathy (ARVC) typically involve both the right ventricular (RV) endocardium (ENDO) and epicardium (EPI).The purpose of this study was to examine the prevalence, electrophysiological features, and outcomes of catheter ablation of VT in patients with isolated epicardial substrate (IES) abnormalities.We studied 71 consecutive patients with VT who met Task Force criteria for ARVC and underwent detailed ENDO and EPI mapping. Patients with critical IES demonstrated (1) confluent EPI bipolar abnormal electrograms (EGMs) and (2) no or minor (<5.0 cm2) RV ENDO low bipolar voltage. Induced VTs were localized using activation mapping, entrainment mapping, and/or pacemapping.Twelve patients (17%) had IES. Extensive EPI bipolar low-voltage area (Bi-LVA; 74 ± 40 cm2) and EGM abnormalities were identified in all patients. Uni-ENDO LVA (<5.5 mV) was seen in 11 of 12 patients (92%) (41 ± 25 cm2) and corresponded to EPI RV bipolar abnormalities. A median of 2 VTs (range 1-7; cycle length 288 ± 68 ms) were induced and localized to the EPI. EPI ablation resulted in noninducibility of all targeted VTs. Preablation cardiac magnetic resonance (CMR) imaging was performed in 10 of 12 patients with RV dyskinesis and/or late gadolinium enhancement in only 4 of 10 patients. During follow-up of 56 ± 46 months, 9 of 12 patients (75%) remained VT-free.In patients with ARVC and VT, substrate abnormalities can uncommonly be isolated to the RV EPI. Detection of critical IES may be limited with CMR imaging but suggested by ENDO unipolar EGM abnormalities. EPI ablation eliminates VT in these patients and typically results in long-term VT-free survival.