Background
Despite frequent left ventricular (LV) involvement in Arrhythmogenic Cardiomyopathy (ACM), the immense majority of sustained monomorphic ventricular tachycardias (VT) occurring in the setting of ACM exhibit a left bundle branch block (LBBB) configuration suggesting a right ventricular (RV) arrhythmia origin. To our best knowledge, only case reports of sustained VT with a right bundle branch block (RBBB) morphology suggesting an LV origin have been reported.
Objective
The present study sought to assess the prevalence of spontaneous sustained VT of LBBB and RBBB morphology in a large cohort of ACM patients.
Methods
Twenty-six centers from 11 European countries provided information on a total of 952 patients with ACM and >1 episode of sustained VT. Patients were classified into 3 groups according to the morphology of the VT observed during their clinical course: LBBB-VT; RBBB-VT or LBBB+RBBB-VT.
Results
Among 952 patients, 881 (92.5%) had LBBB-VT, and 71 (7.5%) had RBBB-VT alone (n=42, 4.4%) or in combination with LBBB-VT (n=29, n=3.1%). A significant difference was observed in the ability to define ACM according to 2010 Task Force Criteria between the LBBB-VT group vs. the RBBB-VT group (P<0.001). Male prevalence was 90.5%, 79.2% and 55.9% in the RBBB-VT, the LBBB-VT and the LBBB+RBBB-VT groups, respectively (P=0.001). Patients’ age at first VT was not different amongst the 3 VT groups. ICD implantation was more frequent for the RBBB-VT and the LBBB+RBBB groups (≈ 90% each) vs. 67.9% for the LBBB-VT group (P=0.001). Death rates did not differ between the 3 groups (11%, 9.5% and 17.2% in the LBBB, RBBB and LBBB+RBBB-VT groups, respectively, P=0.425).
Conclusion
This large European survey demonstrates 2 novel findings: a) Sustained RBBB-VT is documented in a non-negligible proportion (7.5%) of patients with ACM; b) Males markedly predominate in the RBBB-VT and LBBB-VT groups but not in the LBBB+RBBB VT group.