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Emery–Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure
dc.contributor.author | Martínez-Veira, Cristina | |
dc.contributor.author | Cannie, Douglas E. | |
dc.contributor.author | Syrris, Petros | |
dc.contributor.author | Protonotarios, Alexandros | |
dc.contributor.author | Bakalakos, Athanasios | |
dc.contributor.author | Pruny, Jean-François | |
dc.contributor.author | Ditaranto, Rafaello | |
dc.contributor.author | Larrañaga-Moreira, José María | |
dc.contributor.author | Medo, Kristen | |
dc.contributor.author | Bermúdez-Jiménez, Francisco J. | |
dc.contributor.author | Ben Yaou, Rabah | |
dc.contributor.author | Leturcq, France | |
dc.contributor.author | Robles-Mezcua, Ainhoa | |
dc.contributor.author | Marini-Betolo, Chiara | |
dc.contributor.author | Cabrera, Eva | |
dc.contributor.author | Reuter, Chloe | |
dc.contributor.author | Limeres-Freire, Javier | |
dc.contributor.author | Rodríguez-Palomares, José Fernando | |
dc.contributor.author | Mestroni, Luisa | |
dc.contributor.author | Taylor, Matthew R. G. | |
dc.contributor.author | Parikh, Victoria N. | |
dc.contributor.author | Ashley, Euan A. | |
dc.contributor.author | Barriales-Villa, Roberto | |
dc.contributor.author | Jiménez-Jáimez, Juan | |
dc.contributor.author | García-Pavía, Pablo | |
dc.contributor.author | Charron, Philippe | |
dc.contributor.author | Biagini, Elena | |
dc.contributor.author | García-Pinilla, José Manuel | |
dc.contributor.author | Bourke, John | |
dc.contributor.author | Savvatis, Konstantinos | |
dc.contributor.author | Wahbi, Karim | |
dc.contributor.author | Elliott, Perry M. | |
dc.date.accessioned | 2023-10-11T06:55:29Z | |
dc.date.available | 2023-10-11T06:55:29Z | |
dc.date.issued | 2023-08-28 | |
dc.identifier.citation | Douglas E Cannie, Petros Syrris, Alexandros Protonotarios, Athanasios Bakalakos, Jean-François Pruny, Raffaello Ditaranto, Cristina Martinez-Veira, Jose M Larrañaga-Moreira, Kristen Medo, Francisco José Bermúdez-Jiménez, Rabah Ben Yaou, France Leturcq, Ainhoa Robles Mezcua, Chiara Marini-Bettolo, Eva Cabrera, Chloe Reuter, Javier Limeres Freire, José F Rodríguez-Palomares, Luisa Mestroni, Matthew R G Taylor, Victoria N Parikh, Euan A Ashley, Roberto Barriales-Villa, Juan Jiménez-Jáimez, Pablo Garcia-Pavia, Philippe Charron, Elena Biagini, José M García Pinilla, John Bourke, Konstantinos Savvatis, Karim Wahbi, Perry M Elliott, Emery–Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure, European Heart Journal, 2023;, ehad561, https://doi.org/10.1093/eurheartj/ehad561 | es_ES |
dc.identifier.uri | http://hdl.handle.net/2183/33718 | |
dc.description.abstract | [Abstract] Emery–Dreifuss muscular dystrophy (EDMD) is caused by variants in EMD (EDMD1) and LMNA (EDMD2). Cardiac conduction defects and atrial arrhythmia are common to both, but LMNA variants also cause end-stage heart failure (ESHF) and malignant ventricular arrhythmia (MVA). This study aimed to better characterize the cardiac complications of EMD variants. Methods Consecutively referred EMD variant-carriers were retrospectively recruited from 12 international cardiomyopathy units. MVA and ESHF incidences in male and female variant-carriers were determined. Male EMD variant-carriers with a cardiac phenotype at baseline (EMDCARDIAC) were compared with consecutively recruited male LMNA variant-carriers with a cardiac phenotype at baseline (LMNACARDIAC). Results Longitudinal follow-up data were available for 38 male and 21 female EMD variant-carriers [mean (SD) ages 33.4 (13.3) and 43.3 (16.8) years, respectively]. Nine (23.7%) males developed MVA and five (13.2%) developed ESHF during a median (inter-quartile range) follow-up of 65.0 (24.3–109.5) months. No female EMD variant-carrier had MVA or ESHF, but nine (42.8%) developed a cardiac phenotype at a median (inter-quartile range) age of 58.6 (53.2–60.4) years. Incidence rates for MVA were similar for EMDCARDIAC and LMNACARDIAC (4.8 and 6.6 per 100 person-years, respectively; log-rank P = .49). Incidence rates for ESHF were 2.4 and 5.9 per 100 person-years for EMDCARDIAC and LMNACARDIAC, respectively (log-rank P = .09). Conclusions Male EMD variant-carriers have a risk of progressive heart failure and ventricular arrhythmias similar to that of male LMNA variant-carriers. Early implantable cardioverter defibrillator implantation and heart failure drug therapy should be considered in male EMD variant-carriers with cardiac disease. | es_ES |
dc.language.iso | eng | es_ES |
dc.publisher | Oxford Academic | es_ES |
dc.relation.uri | https://doi.org/10.1093/eurheartj/ehad561 | es_ES |
dc.rights | Atribución-NoComercial 3.0 España | es_ES |
dc.rights.uri | http://creativecommons.org/licenses/by-nc/3.0/es/ | * |
dc.subject | Emery–Dreifuss muscular dystrophy | es_ES |
dc.subject | Distrofia muscular de Emery-Dreifuss | es_ES |
dc.subject | Emerin | es_ES |
dc.subject | Emerina | es_ES |
dc.subject | Cardiomyopathy | es_ES |
dc.subject | Cardiomiopatía | es_ES |
dc.subject | Sudden death | es_ES |
dc.subject | Muerte súbita | es_ES |
dc.subject | Ventricular arrhythmia | es_ES |
dc.subject | Arritmia ventricular | es_ES |
dc.subject | Heart failure | es_ES |
dc.subject | Infarto | es_ES |
dc.title | Emery–Dreifuss muscular dystrophy Type 1 is associated with a high risk of malignant ventricular arrhythmias and end-stage heart failure | es_ES |
dc.type | info:eu-repo/semantics/article | es_ES |
dc.rights.access | info:eu-repo/semantics/openAccess | es_ES |
UDC.journalTitle | European Heart Journal | es_ES |
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