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一.临床特征Brugada综合征(Br S)是一种家族性常染色体显性遗传疾病,于1992年由西班牙Brugada兄弟首先报道。心电图表现为正常QT间期,右束支阻滞(RBBB)和右胸前导联(V1~V3)ST段持续性抬高,经心脏超声、心室造影甚至右室心肌活检检查,心脏无异常改变。对Br S准确发病率尚不清楚,全球约每10000人中有5~20人发病,而在东南亚国家,其发病率可能更高。根据目前的临床资料提示,Br S主要发病年龄段为成人,平均猝死年龄大约40岁,目前
A clinical features Brugada syndrome (Br S) is a familial autosomal dominant genetic disease, first reported in 1992 by the Spanish brothers Brugada. ECG showed normal QT interval, right bundle branch block (RBBB) and right chest lead (V1 ~ V3) ST segment sustained elevation, by echocardiography, ventriculography and even right ventricular myocardial biopsy examination, no abnormal heart change. The exact incidence of Br S is unknown, with about 5 to 20 out of 10,000 people worldwide, and in Southeast Asia, the incidence is likely to be higher. According to the current clinical data suggest that the main onset of Br S adult age, the average age of sudden death about 40 years old, at present