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
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 cl...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2022 |
| País: | España |
| Institución: | Fundació Sant Joan de Déu |
| Repositorio: | r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
| OAI Identifier: | oai:fsjd.fundanetsuite.com:p20080 |
| Acceso en línea: | https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20080 |
| Access Level: | acceso abierto |
| Palabra clave: | Arrhythmogenic cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy dysplasia Arrhythmogenic left ventricular cardiomyopathy Ventricular tachycardia Genetics European survey |
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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 CardiomyopathyBelhassen BLaredo MRoudijk RWPeretto GZahavi GSen-Chowdhry SBadenco NTe Riele ASJMSala SDuthoit Gvan Tintelen JPPaglino GSellal JMGasperetti AArbelo EAndorin ANinni SRollin APeichl PWaintraub XBosman LPPierre BNof EMiles CTfelt-Hansen JProtonotarios AGiustetto CSacher FHermida JSHavranek SCalo LCasado-Arroyo RConte GLetsas KPZorio EBermúdez-Jiménez FJBehr ERBeinart RFauchier LKautzner JMaury PLacroix DProbst VBrugada JDuru FChillou CBella PDGandjbakhch EHauer RMilman AArrhythmogenic cardiomyopathyArrhythmogenic right ventricular cardiomyopathydysplasiaArrhythmogenic left ventricular cardiomyopathyVentricular tachycardiaGeneticsEuropean surveyAims 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.OXFORD UNIV PRESS2022info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20080EUROPACEISSN: 10995129ISSNe: 15322092reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déuinstname:Fundació Sant Joan de DéuInglésinfo:eu-repo/semantics/openAccessoai:fsjd.fundanetsuite.com:p200802026-05-27T12:37:41Z |
| dc.title.none.fl_str_mv |
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 |
| title |
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 |
| spellingShingle |
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 Belhassen B Arrhythmogenic cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy dysplasia Arrhythmogenic left ventricular cardiomyopathy Ventricular tachycardia Genetics European survey |
| title_short |
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 |
| title_full |
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 |
| title_fullStr |
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 |
| title_full_unstemmed |
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 |
| title_sort |
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 |
| dc.creator.none.fl_str_mv |
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 J Duru F Chillou C Bella PD Gandjbakhch E Hauer R Milman A |
| author |
Belhassen B |
| author_facet |
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 J Duru F Chillou C Bella PD Gandjbakhch E Hauer R Milman A |
| author_role |
author |
| author2 |
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 J Duru F Chillou C Bella PD Gandjbakhch E Hauer R Milman A |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Arrhythmogenic cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy dysplasia Arrhythmogenic left ventricular cardiomyopathy Ventricular tachycardia Genetics European survey |
| topic |
Arrhythmogenic cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy dysplasia Arrhythmogenic left ventricular cardiomyopathy Ventricular tachycardia Genetics European survey |
| description |
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. |
| publishDate |
2022 |
| dc.date.none.fl_str_mv |
2022 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
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article |
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publishedVersion |
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https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20080 |
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https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=20080 |
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Inglés |
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Inglés |
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info:eu-repo/semantics/openAccess |
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openAccess |
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OXFORD UNIV PRESS |
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OXFORD UNIV PRESS |
| dc.source.none.fl_str_mv |
EUROPACE ISSN: 10995129 ISSNe: 15322092 reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu instname:Fundació Sant Joan de Déu |
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Fundació Sant Joan de Déu |
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r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
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r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
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