Oliva Antonio, Grassi Simone, Pinchi Vilma, Cazzato Francesca, Coll Mónica, Alcalde Mireia, Vallverdú-Prats Marta, Perez-Serra Alexandra, Martínez-Barrios Estefanía, Cesar Sergi, Iglesias Anna, Cruzalegui José, Hernández Clara, Fiol Victoria, Arbelo Elena, Díez-Escuté Nuria, Arena Vincenzo, Brugada Josep, Sarquella-Brugada Georgia, Brugada Ramon, Campuzano Oscar
Department of Health Surveillance and Bioethics, Section of Legal Medicine, Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.
Department of Health Sciences, Section of Forensic Medical Sciences, University of Florence, Largo Brambilla 3, 50134 Florence, Italy.
J Clin Med. 2022 Jul 28;11(15):4406. doi: 10.3390/jcm11154406.
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.
Brugada综合征（BrS）被归类为一种遗传性心脏离子通道病，其病因是心肌细胞中的离子通道和/或相关蛋白功能异常，而非结构性心脏改变。然而，一些BrS患者的心脏表现出轻微的组织学异常，这表明BrS可能是致心律失常性心肌病的一种表型变异。我们按照系统评价和Meta分析的首选报告项目声明（PRISMA）标准对文献进行了系统评价。我们对结构研究结果的综合分析未发现足够的确凿证据支持将BrS重新归类为心肌病。需要收集并综合分析确诊为BrS的新病例，以明确这些结构特征中的某些是否可能在与恶性心律失常发作相关的病理生理途径中起关键作用。
