Structural Heart Alterations in Brugada Syndrome: Is it Really a Channelopathy? A Systematic Review

Brugada syndrome (BrS) is classified as an inherited cardiac channelopathy attributed to dysfunctional ion channels and/or associated proteins in cardiomyocytes rather than to structural heart alterations. However, hearts of some BrS patients exhibit slight histologic abnormalities, suggesting that...

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Detalhes bibliográficos
Autores: Oliva, Antonio, Grassi, Simone, Pinchi, Vilma, Cazzato, Francesca, Coll Vidal, Mònica, Alcalde Masegu, Mireia, Vallverdú-Prats, Marta, Perez-Serra, Alexandra, Martínez-Barrios, Estefanía, Cesar, Sergi, Iglesias, Anna, Cruzalegui, José, Hernández Cera, Clara, Fiol, Victoria, Arbelo, Elena, Díez-Escuté, Nuria, Arena, Vincenzo, Brugada Terradellas, Josep, Sarquella Brugada, Geòrgia, Brugada, Ramon, Campuzano Larrea, Oscar
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Recursos:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:10256/21569
Acesso em linha:http://hdl.handle.net/10256/21569
Access Level:acceso abierto
Palavra-chave:Mort sobtada
Sudden death
Brugada, Síndrome de -- Diagnòstic
Brugada syndrome -- Diagnosi
Patologia forense
Forensic pathology
Cor -- Histopatologia
Heart -- Histopathology
Descrição
Resumo:Brugada syndrome (BrS) is classified as an inherited cardiac channelopathy attributed to dysfunctional ion channels and/or associated proteins in cardiomyocytes rather than to structural heart alterations. However, hearts of some BrS patients exhibit slight histologic abnormalities, suggesting that BrS could be a phenotypic variant of arrhythmogenic cardiomyopathy. We performed a systematic review of the literature following Preferred Reporting Items for Systematic Reviews and Meta-Analyses Statement (PRISMA) criteria. Our comprehensive analysis of structural findings did not reveal enough definitive evidence for reclassification of BrS as a cardiomyopathy. The collection and comprehensive analysis of new cases with a definitive BrS diagnosis are needed to clarify whether some of these structural features may have key roles in the pathophysiological pathways associated with malignant arrhythmogenic episodes