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 < 0.001). Mutations in PKP2 predominated in the LBBB-VT (65.2%) and the LBBB + RBBB-VT (41.7%) groups while DSP mutations predominated in the RBBB-VT group (45.5%). By multivariable analysis, female sex was associated with LBBB + RBBB-VT (P = 0.011) while DSP mutations were associated with RBBB-VT (P < 0.001). After a median follow-up of 103 (51-185) months, death occurred in 106 (11.1%) patients with no intergroup difference (P = 0.176). 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.

2nd Department of Medicine Department of Cardiovascular Medicine 1st Faculty of Medicine Charles University and General University Hospital Prague Kateřinská 1660 32 121 08 Nové Město Prague Czech Republic

Arrhythmia Unit 2nd Department of Cardiology Evangelismos General Hospital of Athens Ipsilantou 45 47 Athina 106 76 Athens Greece

Cardiology Department at Hospital Universitario y Politecnico La Fe and Research Group on Inherited Heart Diseases Sudden Death and Mechanisms of Disease La Fe Avenida Fernando Abril Martorell Torre 106 A 7planta Valencia Spain

Cardiology Department Hospital Universitario Virgen de las Nieves Av de las Fuerzas Armadas 2 18014 Granada Spain

Cardiology University Hospital Rangueil 1 Avenue du Professeur Jean Poulhès 31400 Toulouse France

Cardiovascular Institute Hospital Clinic and IDIBAPS Calle Villarroel 170 08036 Barcelona Catalonia Spain

Cardiovascular Institute Hospital Clínic Pediatric Arrhythmia Unit Hospital Sant Joan de Déu University of Barcelona Passeig de Sant Joan de Déu 2 08950 Esplugues de Llobregat Barcelona Spain

Cardiovascular Sciences and Cardiology Clinical Academic Group St George's University Hospitals NHS Foundation Trust Cranmer Terrace London SW17 0RE UK

Center for Biomedical Network Research on Cardiovascular Diseases Av Monforte de Lemos 3 5 Pabellón 11 Planta 0 28029 Madrid Spain

Centre Hospitalier Universitaire d'Amiens Picardie 2 Place Victor Pauchet 80080 Amiens France

Département de Cardiologie Centre Hospitalier Universitaire de Nancy Vandœuvre lès Nancy Rue du Morvan 54500 France

Department of Anesthesiology Sheba Medical Center Tel Hashomer 5265601 Israel

Department of Cardiology Erasme University Hospital Université Libre de Bruxelles Route de Lennik 808 1070 Bruxelles Belgium

Department of Cardiology Institute for Clinical and Experimental Medicine Vídeňská 1958 140 21 Praha 4 Prague Czech Republic

Department of Cardiology University Heart Center Zurich Hottingerstrasse 14 CH 8032 Zürich Switzerland

Department of Cardiology University Medical Center 62 Huntley St London WC1E 6DD The Netherlands

Department of Forensic Medicine Faculty of Medical Sciences University of Copenhagen Blegdamsvej 3B 2200 Copenhagen Denmark

Department of Genetics University Medical Center Moreelsepark 1 3511 EP Utrecht The Netherlands

Division of Cardiology Department of Medical Sciences University of Torino Città della Salute e della Scienza Hospital Corso Bramante 88 10126 Torino TO Italy

Division of Cardiology Policlinico Casilino Via Casilina 1049 00169 Roma RM Italy

Electrophysiology Unit Department of Cardiology Fondazione Cardiocentro Ticino Via Tesserete 48 6900 Lugano Switzerland

Heart Institute Hadassah University Hospital Kalman Ya'Akov Man Street 9112001 Jerusalem Israel

Hôpital Cardiologique du Haut Lévêque and Université Bordeaux LIRYC Institute Avenue du Haut Lévêque 33600 Pessac Bordeaux France

Institute of Cardiovascular Science University College London 62 Huntley St London WC1E 6DD UK

IRCCS San Raffaele Scientific Institute Via Olgettina 60 20132 Milano Italy

Leviev Heart Institute Sheba Medical Center 5265601 Tel Hashomer Israel

Netherlands Heart Institute Moreelsepark 1 3511 EP Utrecht The Netherlands

Nikos Protonotarios Medical Centre Περιφερειακός 843 00 Naxos Greece

Sackler School of Medicine Tel Aviv University P O Box 39040 Tel Aviv 6997801 Israel

Service de Cardiologie Centre Hospitalier Universitaire Trousseau 2 Boulevard Tonnellé 37000 Tours France

Service de Cardiologie CHU de Nantes Bd Jacques Monod 44800 Saint Herblain Nantes France

Sorbonne Université AP HP Groupe Hospitalier Pitié Salpêtrière Institut de Cardiologie 47 83 Boulevard de l'Hôpital 75013 Paris France

The Department of Cardiology The Heart Centre Copenhagen University Hospital Rigshospitalet Copenhagen 2100 Denmark

UCL Institute of Cardiovascular Science 62 Huntley St London WC1E 6DD UK

Université de Lille et Institut Cœur Poumon CHRU Boulevard du Professeur Jules Leclercq 59000 Lille France

Université François Rabelais 60 rue du Plat D'Etain 37020 Tours cedex 1 France

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Electrocardiographic findings in patients with arrhythmogenic cardiomyopathy and right bundle branch block ventricular tachycardia