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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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
Tipo de documento: Artículo
Estado:Published version
Fecha de publicación:2022
País:España
Institución: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
Acceso en línea:http://hdl.handle.net/10256/21569
Nivel de acceso:Acceso abierto
Palabra clave:Mort sobtada
Sudden death
Brugada, Síndrome de -- Diagnòstic
Brugada syndrome -- Diagnosi
Patologia forense
Forensic pathology
Cor -- Histopatologia
Heart -- Histopathology
Sumario: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

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