Brugada综合征是一种有遗传倾向的以恶性心律失常为主要表现的综合征,由于编码心脏Na离子通道的基因(SCN5A)突变而发病。1992年Brugada兄弟描述其特征性心电图表现,之后发现该疾病又有夜间猝死这一凶险的预后,因此一直是心血管疾病基础和临床研究领域内的一大前沿和焦点。本文就BS的流行病学、遗传基础及细胞学机制和心电图特征等方面最新研究进展作一综述。 Brugada syndrome is a genetically predisposed syndrome characterized mainly by malignant arrhythmias, which is caused by a mutation in the gene encoding the cardiac Na ion channel (SCN5A). In 1992, Brugada brothers described its characteristic electrocardiographic findings, and later found that the disease has the dangerous prognosis of sudden death at night, it has been a major front and focus of the field of cardiovascular disease basic and clinical research. This article reviews the recent progress of BS in epidemiology, genetic basis, cytology and ECG features.