早期复极综合征并反复晕厥两家系报告  

Early repolarization syndrome and recurrent syncope in two Chinese pedigrees

作　　者：谭珍妮[1] 邓伟[1] 罗苑苑[1] 李蘅[1] 孙少喜[1] 孟素荣[1] 许顶立[1] 彭健[1] 

机构地区：[1]南方医科大学南方医院心内科,广州510515

出　　处：《中华心血管病杂志》2010年第9期801-804,共4页Chinese Journal of Cardiology

基　　金：广东省科技计划项目（2008B030301142）

摘　　要：目的 探讨两个早期复极综合征（early repolarization syndrome,ERS）合并反复晕厥家系的临床特点及可能机制.方法 分别对两个先证者所在家系的所有家族成员进行常规临床检查和心电图、超声心动图、动态心电图、X线胸片、直立倾斜试验、生化等检查.结果 两家系中所有成员均无器质性心脏病的临床证据.先证者1反复心原性晕厥,静息心电图表现为ERS,反复发作尖端扭转型室性心动过速及心室颤动;其弟弟、儿子及侄儿均诊断为ERS,其他家族成员均未有心脏事件（晕厥、心脏骤停和心脏性猝死）.先证者2表现为反复不明原因晕厥,心电图示ERS,其中V1～V3导联ST段弓背向上抬高;其父于65岁时心脏性猝死,其一侄儿诊断为ERS,余家族成员均正常.结论 ERS可表现为反复晕厥、尖端扭转型室性心动过速和心室颤动,不一定总是良性的临床过程.ERS与Brugada综合征可以共存于同一患者,但尚不能充分证明两者有共同的发生机制.遗传性家系的研究为进一步基因研究打下了坚实的基础.Objective To investigate the clinical characteristics in two families with early repolarization syndrome（ERS） and recurrent syncope. Method All family members including the probands were screened with routine clinical examination, electrocardiography, echocardiography, Holter recording,chest x-ray, head-up tilt test and blood biochemistry. Results There was no clinical evidence of organic heart disease in all members from the two families. Proband 1 showed recurrent syncope, ERS and repeated torsade de pointes ventricular tachycardia and ventricular fibrillation were documented with resting ECG.ERS was detected in one brother, one nephew and one son from him and all were free of cardiac events including syncope, cardiac arrest and sudden cardiac death. Proband 2 showed recurrent syncope, ERS and ST segment arched upward elevation in V1 - V3 were documented by ECG. His father suffered sudden cardiac death at the age of 65 and asymptomatic ERS was detected in one of his nephew. Conclusions ERS is not always linked with benign clinical course and can sometimes lead to repeated synocope, torsade de pointes ventricular tachycardia and ventricular fibrillation. Pedigree research is of importance for ERS.

关 键 词：晕厥 心动过速 室性 心室颤动 早期复极综合征 

分 类 号：R541.7[医药卫生—心血管疾病]