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Brugada综合征为一遗传性致心律失常性疾病，心电图显示右心室导联ST段抬高2 mV与T波倒置，而心脏结构正常，易罹心室颤动和心脏性猝死。 Brugada综合征为常染色体显性遗传伴发不完全性外显，SCN5A基因突变已被确认为Brugada综合征主要致病原因，占接近30％患者；其他16个基因突变也连结到Brugada综合征只占5％，剩下65％Brugada综合征患者无肯定遗传学背景。现综述Brugada综合征遗传学若干进展。“,”Brugada syndome( BrS) is an inherited arrhythogensic disorder that exhibits ECG ST-segment elevation≥2 mV with a neg-ative T-wave in the right precordial leads( V1 ~V2 ) ,with normal heart structure,predisposing to ventricular fibrillation and sudden cardiac death.Genetically BrS is autosome dominant accompanied by incomplete penetrance,and mutation in SCN5A gene had been identified as the main pathogenic cause of BrS.Besides,other 16 gene mutation also links to BrS,but mutation in SCN5A accounts for approximately 30%and those in other genes 5%,leaving no definitive genetic background in 65%of BrS patients.Some advances on genetics related to BrS were re-viewed in this paper.