国人Brugada综合征特点探讨(附三例报道)

目的：探讨国人Brugada综合征的特点。方法 报道3例Brugada综合征，并复习国内文献报道的20例，分析其特点。结果 (1)共23例Brugada综合征中男20例，女3例，发病年龄为9—73岁，平均40岁。(2)临床表现为晕厥14例，其中4例死亡，单纯阵发性心悸、胸闷3例，无症状6例；18个家系中有5个家系有明确或可疑家族史。(3)所有病例心电图Vl—V3导联ST段呈尖峰状和／或马鞍状抬高，大多ST段抬高程度多变，同时15例伴典型右束支传导阻滞，11例发生室速或室颤，其他还可见阵发性房颤、早博、房室传导阻滞、窦房传导阻滞等心律失常。结论：(1)国人Brugada综合征临床和心电图特点与国外文献报道相似。(2)诊断Brugada综合征主要应根据Vl—V3导联ST段尖峰状和／或马鞍状抬高的特征性改变，晕厥及典型右束支传导阻滞并非必需。(3)Vl—V3导联段抬高幅度多变是Brugada综合征的一个重要特点，ST段抬高程度可能与晕厥发作有关。

The characteristics of Brugada Syndrome in Chinese:a report of 3 cases

Objective To investigate the characteristics of Brugada Syndrome in China.Method 3 cases of Brugada Syndrome are reported,and 20 cases reported in Chinese Journal are reviewed.Results (1)Males(n=20) outnumber females(n=3) by far,and age distribution reveals a peak around the fourth decade (range 9 to 73 years).(2)14 patients present with syncope,4 out of which suffer from sudden death;3 patients present with palpitation or chest closed;while 6 patients are asymptomatic.Of 18 family,5 family histories are suspected or confirmed.(3)The electrocardiogram of all patients is abnormal,displaying right precordial (leads V 1 to V 3)convex curved or/and saddle shaped-type ST-segment elevation in all patients,and the magnitude of the ST-segment shift in most patients;typical right bundle-branch block in 15 patients;paroxysmal ventricular tachycardia or ventricular fibrillation in 11 patients;and paroxysmal atrial fibrillation,premature beat,atrioventricular block,sino-auricular block individual.Conclusions (1)The characteristics of Brugada Syndrome in Chinese is similar to that in foreigner.(2)The diagnosis of Brugada Syndrome is based on the special pattern of ST-segment elevation in leads V 1 to V 3,but is not syncope or right bundle blanch block.(3)The variability of the magnitude of the ST-segment elevation is the important character of Brugada Syndrome,and the magnitude of the ST-segment shift may relate to the occurrence of syncope.