The prevalence of left and right bundle branch block morphology ventricular tachycardia amongst patients with arrhythmogenic cardiomyopathy and sustained ventricular tachycardia: insights from the European Survey on Arrhythmogenic Cardiomyopathy
Por:
Belhassen B, Laredo M, Roudijk RW, Peretto G, Zahavi G, Sen-Chowdhry S, Badenco N, Te Riele ASJM, Sala S, Duthoit G, van Tintelen JP, Paglino G, Sellal JM, Gasperetti A, Arbelo E, Andorin A, Ninni S, Rollin A, Peichl P, Waintraub X, Bosman LP, Pierre B, Nof E, Miles C, Tfelt-Hansen J, Protonotarios A, Giustetto C, Sacher F, Hermida JS, Havranek S, Calo L, Casado-Arroyo R, Conte G, Letsas KP, Zorio E, Bermúdez-Jiménez FJ, Behr ER, Beinart R, Fauchier L, Kautzner J, Maury P, Lacroix D, Probst V, Brugada-Terradellas J, Duru F, Chillou C, Bella PD, Gandjbakhch E, Hauer R and Milman A
Publicada:
2 feb 2022
Ahead of Print:
1 ene 2022
Resumen:
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.
Filiaciones:
Belhassen B:
Sackler School of Medicine, Tel Aviv University, P.O. Box 39040, Tel Aviv 6997801, Israel
Heart Institute, Hadassah University Hospital, Kalman Ya'Akov Man Street, 9112001, Jerusalem, Israel
Laredo M:
Sorbonne Université, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Institut de Cardiologie, 47-83 Boulevard de l'Hôpital, 75013 Paris, France
Roudijk RW:
Netherlands Heart Institute, Moreelsepark 1 3511 EP Utrecht, The Netherlands
Peretto G:
IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132 Milano, Italy
Zahavi G:
Department of Anesthesiology, Sheba Medical Center, Tel Hashomer, 5265601, Israel
Sackler School of Medicine, Tel Aviv University, P.O. Box 39040, Tel Aviv 6997801, Israel
Sen-Chowdhry S:
Institute of Cardiovascular Science University College London, 62 Huntley St, London WC1E 6DD, UK
Badenco N:
Sorbonne Université, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Institut de Cardiologie, 47-83 Boulevard de l'Hôpital, 75013 Paris, France
Te Riele ASJM:
Department of Cardiology, University Medical Center, 62 Huntley St, London WC1E 6DD, The Netherlands
Netherlands Heart Institute, Moreelsepark 1 3511 EP Utrecht, The Netherlands
Sala S:
IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132 Milano, Italy
Duthoit G:
Sorbonne Université, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Institut de Cardiologie, 47-83 Boulevard de l'Hôpital, 75013 Paris, France
van Tintelen JP:
Department of Genetics, University Medical Center, Moreelsepark 1 3511 EP Utrecht, The Netherlands
Netherlands Heart Institute, Moreelsepark 1 3511 EP Utrecht, The Netherlands
Paglino G:
IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132 Milano, Italy
Sellal JM:
Département de Cardiologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre lès-Nancy, Rue du Morvan, 54500 France
Gasperetti A:
Department of Cardiology, University Heart Center Zurich, Hottingerstrasse 14 CH-8032 Zürich, Switzerland
Arbelo E:
Cardiovascular Institute, Hospital Clinic and IDIBAPS, Calle Villarroel, 170 08036 Barcelona, Catalonia, Spain
Andorin A:
Service de Cardiologie, CHU de Nantes, Bd Jacques Monod - 44800 Saint-Herblain, Nantes, France
Ninni S:
Université de Lille et Institut Cœur-Poumon, CHRU, Boulevard du Professeur Jules Leclercq, 59000 Lille, France
Rollin A:
Cardiology, University Hospital Rangueil, 1 Avenue du Professeur Jean Poulhès, 31400 Toulouse, France
Peichl P:
Department of Cardiology, Institute for Clinical and Experimental Medicine (IKEM), Vídenská 1958, 140 21 Praha 4, Prague, Czech Republic
Waintraub X:
Sorbonne Université, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Institut de Cardiologie, 47-83 Boulevard de l'Hôpital, 75013 Paris, France
Bosman LP:
Netherlands Heart Institute, Moreelsepark 1 3511 EP Utrecht, The Netherlands
Department of Cardiology, University Medical Center, 62 Huntley St, London WC1E 6DD, The Netherlands
Pierre B:
Service de Cardiologie, Centre Hospitalier Universitaire Trousseau, 2 Boulevard Tonnellé, 37000 Tours, France
Nof E:
Sackler School of Medicine, Tel Aviv University, P.O. Box 39040, Tel Aviv 6997801, Israel
Leviev Heart Institute, Sheba Medical Center, 5265601 Tel Hashomer, Israel
Miles C:
Cardiovascular Sciences and Cardiology Clinical Academic Group St. George's University Hospitals NHS Foundation Trust, Cranmer Terrace London SW17 0RE, UK
Tfelt-Hansen J:
The Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen 2100, Denmark
Department of Forensic Medicine, Faculty of Medical Sciences, University of Copenhagen, Blegdamsvej 3B, 2200, Copenhagen, Denmark
Protonotarios A:
Nikos Protonotarios Medical Centre, ?e??fe?e?a???, 843 00, Naxos, Greece
UCL Institute of Cardiovascular Science, 62 Huntley St, London WC1E 6DD, UK
Giustetto C:
Division of Cardiology, Department of Medical Sciences, University of Torino, Città della Salute e della Scienza Hospital, Corso Bramante, 88, 10126 Torino TO, Italy
Sacher F:
Hôpital Cardiologique du Haut-Lévêque & Université Bordeaux, LIRYC Institute, Avenue du Haut Lévêque, 33600 Pessac, Bordeaux, France
Hermida JS:
Centre Hospitalier Universitaire d'Amiens-Picardie, 2 Place Victor Pauchet, 80080 Amiens, France
Havranek S:
Second Department of Medicine-Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital in Prague, Katerinská 1660/32, 121 08 Nové Mesto, Prague, Czech Republic
Calo L:
Division of Cardiology, Policlinico Casilino, Via Casilina, 1049, 00169 Roma RM, Italy
Casado-Arroyo R:
Department of Cardiology, Erasme University Hospital, Université Libre de Bruxelles, Route de Lennik 808, 1070 Bruxelles, Belgium
Conte G:
Electrophysiology Unit, Department of Cardiology, Fondazione Cardiocentro Ticino, Via Tesserete 48, 6900 Lugano, Switzerland
Letsas KP:
Arrhythmia Unit, Second Department of Cardiology, "Evangelismos" General Hospital of Athens, Ipsilantou 45-47, Athina 106 76, Athens, Greece
Zorio E:
Cardiology Department at Hospital Universitario y Politecnico La Fe and Research Group on Inherited Heart Diseases, Sudden Death and Mechanisms of Disease (CaFaMuSMe) from the Instituto de Investigación Sanitaria (IIS) La Fe, Avenida Fernando Abril Martorell, Torre 106 A 7planta, Valencia, Spain
Center for Biomedical Network Research on Cardiovascular Diseases (CIBERCV), Av. Monforte de Lemos, 3-5. Pabellón 11. Planta 0 28029, Madrid, Spain
Bermúdez-Jiménez FJ:
Cardiology Department, Hospital Universitario Virgen de las Nieves, Av. de las Fuerzas Armadas, 2, 18014 Granada, Spain
Behr ER:
Cardiovascular Sciences and Cardiology Clinical Academic Group St. George's University Hospitals NHS Foundation Trust, Cranmer Terrace London SW17 0RE, UK
Beinart R:
Sackler School of Medicine, Tel Aviv University, P.O. Box 39040, Tel Aviv 6997801, Israel
Leviev Heart Institute, Sheba Medical Center, 5265601 Tel Hashomer, Israel
Fauchier L:
Service de Cardiologie, Centre Hospitalier Universitaire Trousseau, 2 Boulevard Tonnellé, 37000 Tours, France
Université François Rabelais, 60 rue du Plat D'Etain 37020 Tours cedex 1, France
Kautzner J:
Department of Cardiology, Institute for Clinical and Experimental Medicine (IKEM), Vídenská 1958, 140 21 Praha 4, Prague, Czech Republic
Maury P:
Cardiology, University Hospital Rangueil, 1 Avenue du Professeur Jean Poulhès, 31400 Toulouse, France
Lacroix D:
Université de Lille et Institut Cœur-Poumon, CHRU, Boulevard du Professeur Jules Leclercq, 59000 Lille, France
Probst V:
Service de Cardiologie, CHU de Nantes, Bd Jacques Monod - 44800 Saint-Herblain, Nantes, France
Brugada-Terradellas J:
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
Duru F:
Department of Cardiology, University Heart Center Zurich, Hottingerstrasse 14 CH-8032 Zürich, Switzerland
Chillou C:
Département de Cardiologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre lès-Nancy, Rue du Morvan, 54500 France
Bella PD:
IRCCS San Raffaele Scientific Institute, Via Olgettina, 60, 20132 Milano, Italy
Gandjbakhch E:
Sorbonne Université, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Institut de Cardiologie, 47-83 Boulevard de l'Hôpital, 75013 Paris, France
Hauer R:
Netherlands Heart Institute, Moreelsepark 1 3511 EP Utrecht, The Netherlands
Department of Cardiology, University Medical Center, 62 Huntley St, London WC1E 6DD, The Netherlands
Milman A:
Sackler School of Medicine, Tel Aviv University, P.O. Box 39040, Tel Aviv 6997801, Israel
Leviev Heart Institute, Sheba Medical Center, 5265601 Tel Hashomer, Israel
Green Submitted, Bronze
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