儿茶酚胺介导的多形性室速研究进展

被引：7

作者：

刘茜蒨 ^{[1
,2
,3
]}

王擎 ^{[2
,4
]}

胡大一 ^{[3
]}

机构：

[1] 北京大学深圳医院心内科

[2] Department of Molecular Cardiology,Center for Cardi-ovascular Genetics,Lerner Research Institute,The Cleveland Clinic

[3] 北京大学人民医院心内科

[4] 华中科技大学生命科学与技术学院人类基因组研究中心

来源：

心血管病学进展 | 2009年 / 30卷 / 03期

关键词：

心律失常; 儿茶酚胺介导的多形性室速; 分子生物学; 电生理学;

D O I：

暂无

中图分类号：

R541.71 [];

学科分类号：

1002 ; 100201 ;

摘要：

儿茶酚胺介导的多形性室速是一种少见却严重的遗传性心律失常,表现为无器质性心脏病的个体在运动或激动时发生双向性、多形性室速导致发作性晕厥及进展为心室颤动导致猝死。心肌细胞肌浆网异常释放钙离子使细胞内钙离子超载引起的延迟后除极可能是儿茶酚胺介导的多形性室速发生的机制。目前已知的和儿茶酚胺介导的多形性室速相关的基因为常染色体显性遗传的RyR2(位于1q42.1-q43)和常染色体隐性遗传的CASQ2(位于1p13.3-p11)。治疗:β-阻断剂适用于所有临床症状的个体和可能有RyR2突变而没有心脏事件(晕厥)或运动试验诱发的室性心律失常等病史的个体。反复心脏骤停患者需植入式心律转复除颤器。每6至12个月随访以监测疗效。患者所有的一级亲属,都应予心脏评估。

引用

页码：409 / 412

页数：4

共 10 条

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