Bernard Belhassen, Vincent Probst, Ruben Casado-Arroyo, Jean-Sylvain Hermida, Guy Zahavi, Laurens P Bosman, Anne Rollin, Giulio Conte, Esther Zorio, Rob W Roudijk, Carla Giustetto, Josef Kautzner, Firat Duru, Xavier Waintraub, Gabriele Paglino, J. Peter van Tintelen, Elijah R. Behr, Jacob Tfelt-Hansen, Philippe Maury, Francisco Bermúdez-Jiménez, Sandro Ninni, Stepan Havranek, Estelle Gandjbakhch, Alessio Gasperetti, Simone Sala, Josep Brugada, Dominique Lacroix, Chris Miles, Frederic Sacher, Laurent Fauchier, Paolo Della Bella, Christian de Chillou, Anneline S.J.M. te Riele, Elena Arbelo, Petr Peichl, Srijita Sen-Chowdhry, Alexandros Protonotarios, Mikael Laredo, Giovanni Peretto, Anat Milman, Richard N.W. Hauer, Leonardo Calò, Guillaume Duthoit, Antoine Andorin, Jean-Marc Sellal, Eyal Nof, Nicolas Badenco, Konstantinos P. Letsas, Roy Beinart, Bertrand Pierre, Faculty of Medicine and Pharmacy, and Clinical sciences
Aims In arrhythmogenic cardiomyopathy (ACM), sustained ventricular tachycardia (VT) typically displays a left bundle branch block (LBBB) morphology while a right bundle branch block (RBBB) morphology is rare. The present study assesses the VT morphology in ACM patients with sustained VT and their clinical and genetic characteristics. Methods and results Twenty-six centres from 11 European countries provided information on 954 ACM patients who had ≥1 episode of sustained VT spontaneously documented during patients’ clinical course. Arrhythmogenic cardiomyopathy was defined according to the 2010 Task Force Criteria, and VT morphology according to the QRS pattern in V1. Overall, 882 (92.5%) patients displayed LBBB-VT alone and 72 (7.5%) RBBB-VT [alone in 42 (4.4%) or in combination with LBBB-VT in 30 (3.1%)]. Male sex prevalence was 79.3%, 88.1%, and 56.7% in the LBBB-VT, RBBB-VT, and LBBB + RBBB-VT groups, respectively (P = 0.007). First RBBB-VT occurred 5 years after the first LBBB-VT (46.5 ± 14.4 vs 41.1 ± 15.8 years, P = 0.011). An implanted cardioverter-defibrillator was more frequently implanted in the RBBB-VT (92.9%) and the LBBB + RBBB-VT groups (90%) than in the LBBB-VT group (68.1%) (P Conclusion RBBB-VT accounts for a significant proportion of sustained VTs in ACM. Sex and type of pathogenic mutations were associated with VT type, female sex with LBBB + RBBB-VT, and DSP mutation with RBBB-VT.