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
Bernard Belhassen
(1, 2)
,
Mikael Laredo
(3, 4)
,
Rob Roudijk
(5)
,
Giovanni Peretto
(6)
,
Guy Zahavi
(2, 7)
,
Srijita Sen-Chowdhry
(8)
,
Nicolas Badenco
(3, 4)
,
Anneline Te Riele
(5)
,
Simone Sala
(6)
,
Guillaume Duthoit
(3, 4)
,
J Peter van Tintelen
(5, 9)
,
Gabriele Paglino
(6)
,
Jean-Marc Sellal
(10)
,
Alessio Gasperetti
(11)
,
Elena Arbelo
(12)
,
Antoine Andorin
(13)
,
Sandro Ninni
(14)
,
Anne Rollin
(15)
,
Petr Peichl
(16)
,
Xavier Waintraub
(3, 4)
,
Laurens Bosman
(5)
,
Bertrand Pierre
(17)
,
Eyal Nof
(2, 7)
,
Chris Miles
(18)
,
Jacob Tfelt-Hansen
(19, 20)
,
Alexandros Protonotarios
(8)
,
Carla Giustetto
(21)
,
Frederic Sacher
(22, 23)
,
Jean-Sylvain Hermida
(24)
,
Stepan Havranek
(25)
,
Leonardo Calo
(26)
,
Ruben Casado-Arroyo
(27)
,
Giulio Conte
(28)
,
Konstantinos Letsas
(29)
,
Esther Zorio
(30)
,
Francisco Bermúdez-Jiménez
(31)
,
Elijah Behr
(18)
,
Roy Beinart
(2, 7)
,
Laurent Fauchier
(17, 32)
,
Josef Kautzner
(16)
,
Philippe Maury
(15)
,
Dominique Lacroix
(14)
,
Vincent Probst
(13)
,
Josep Brugada
(33)
,
Firat Duru
(11)
,
Christian de Chillou
(10)
,
Paolo Della Bella
(6)
,
Estelle Gandjbakhch
(3)
,
Richard Hauer
(5)
,
Anat Milman
(2, 7)
1
Hadassah University Hospital
2 TAU - Tel Aviv University
3 Institut de cardiologie [CHU Pitié-Salpêtrière]
4 SU - Sorbonne Université
5 Netherlands Heart Institute
6 IRCCS San Raffaele Scientific Institute [Milan, Italie]
7 Chaim Sheba Medical Center
8 UCL - University College of London [London]
9 UMCU - University Medical Center [Utrecht]
10 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
11 USZ - Universitätsspital Zürich
12 IDIBAPS - Institut d'Investigacions Biomèdiques August Pi i Sunyer
13 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
14 Institut Coeur Poumon [CHU Lille]
15 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
16 IKEM - Institute for Clinical and Experimental Medicine
17 Hôpital Trousseau
18 St George’s University Hospitals
19 Copenhagen University Hospital
20 UCPH - University of Copenhagen = Københavns Universitet
21 UNITO - Università degli studi di Torino = University of Turin
22 Hôpital Haut-Lévêque [CHU Bordeaux]
23 IHU Liryc - Institut de rythmologie et modélisation cardiaque [Pessac]
24 CHU Amiens-Picardie
25 UK - Univerzita Karlova [Praha, Česká republika] = Charles University [Prague, Czech Republic]
26 Ospedale Policlinico Casilino - Policlinico Casilino
27 ULB - Université libre de Bruxelles
28 Fondazione Cardiocentro Ticino
29 Evangelismos Athens General Hospital
30 CIBERCV - Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares
31 HUVN - Hospital Universitario Virgen de las Nieves [Granada, Spain]
32 UT - Université de Tours
33 UB - Universitat de Barcelona
2 TAU - Tel Aviv University
3 Institut de cardiologie [CHU Pitié-Salpêtrière]
4 SU - Sorbonne Université
5 Netherlands Heart Institute
6 IRCCS San Raffaele Scientific Institute [Milan, Italie]
7 Chaim Sheba Medical Center
8 UCL - University College of London [London]
9 UMCU - University Medical Center [Utrecht]
10 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
11 USZ - Universitätsspital Zürich
12 IDIBAPS - Institut d'Investigacions Biomèdiques August Pi i Sunyer
13 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
14 Institut Coeur Poumon [CHU Lille]
15 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
16 IKEM - Institute for Clinical and Experimental Medicine
17 Hôpital Trousseau
18 St George’s University Hospitals
19 Copenhagen University Hospital
20 UCPH - University of Copenhagen = Københavns Universitet
21 UNITO - Università degli studi di Torino = University of Turin
22 Hôpital Haut-Lévêque [CHU Bordeaux]
23 IHU Liryc - Institut de rythmologie et modélisation cardiaque [Pessac]
24 CHU Amiens-Picardie
25 UK - Univerzita Karlova [Praha, Česká republika] = Charles University [Prague, Czech Republic]
26 Ospedale Policlinico Casilino - Policlinico Casilino
27 ULB - Université libre de Bruxelles
28 Fondazione Cardiocentro Ticino
29 Evangelismos Athens General Hospital
30 CIBERCV - Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares
31 HUVN - Hospital Universitario Virgen de las Nieves [Granada, Spain]
32 UT - Université de Tours
33 UB - Universitat de Barcelona
Bernard Belhassen
- Fonction : Auteur
- PersonId : 1226170
- ORCID : 0000-0002-7800-2546
Mikael Laredo
- Fonction : Auteur
- PersonId : 799578
- ORCID : 0000-0002-7326-2656
- IdRef : 227342860
Simone Sala
- Fonction : Auteur
- PersonId : 1226171
- ORCID : 0000-0003-3303-8892
J Peter van Tintelen
- Fonction : Auteur
- PersonId : 1217537
- ORCID : 0000-0003-3854-6749
Alessio Gasperetti
- Fonction : Auteur
- PersonId : 1226059
- ORCID : 0000-0003-3432-070X
Petr Peichl
- Fonction : Auteur
- PersonId : 1226172
- ORCID : 0000-0002-5625-0071
Eyal Nof
- Fonction : Auteur
- PersonId : 1226173
- ORCID : 0000-0002-1304-6070
Jean-Sylvain Hermida
- Fonction : Auteur
- PersonId : 1151137
- ORCID : 0000-0001-9276-2886
- IdRef : 07600211X
Giulio Conte
- Fonction : Auteur
- PersonId : 1226174
- ORCID : 0000-0003-2248-3456
Roy Beinart
- Fonction : Auteur
- PersonId : 1226175
- ORCID : 0000-0002-9268-4766
Laurent Fauchier
- Fonction : Auteur
- PersonId : 1091686
- ORCID : 0000-0002-9267-1658
- IdRef : 05973843X
Philippe Maury
- Fonction : Auteur
- PersonId : 764669
- ORCID : 0000-0002-4800-1841
- IdRef : 174165412
Firat Duru
- Fonction : Auteur
- PersonId : 1226176
- ORCID : 0000-0002-4748-0158
Estelle Gandjbakhch
- Fonction : Auteur
- PersonId : 1228986
- ORCID : 0000-0002-4846-5021
- IdRef : 070471495
Richard Hauer
- Fonction : Auteur
- PersonId : 1226177
- ORCID : 0000-0003-1467-7234
Anat Milman
- Fonction : Auteur
- PersonId : 1226178
- ORCID : 0000-0002-8551-4101
Résumé
Abstract 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.