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Brugada综合征是特发性室颤的一种特殊类型,是常染色体不完全性遗传疾病,其发病相关联SCN5A基因编码心肌钠通道。本文综述了国内外关于Brugada综合征中SCN5A基因突变的电生理报道,探讨了Brugada综合征与SCN5A基因突变编码的通道电生理特性之间的联系。
Brugada syndrome is a special type of idiopathic ventricular fibrillation, is an autosomal recessive genetic disease, the incidence associated with SCN5A gene encoding cardiac sodium channel. This review summarizes the electrophysiologic reports of SCN5A gene mutation in Brugada syndrome at home and abroad, and discusses the relationship between Brugada syndrome and electrophysiological characteristics of the channel encoded by SCN5A mutation.