儿茶酚胺敏感性多形性室性心动过速1例

发布时间：2018-06-21 08:14

本文选题：儿茶酚胺敏感性多形性室性心动过速 + 离子通道病　；参考：《浙江大学》2013年博士论文

【摘要】：儿茶酚胺敏感性多形性室性心动过速(CPVT)是因一类因肌浆网(SR)钙离子通道基因突变导致的遗传性心律失常疾病(IADs)/离子通道病,其临床特征为运动或情绪激动诱发的双向性或多形性室性心动过速。CPVT常常于儿童或青年时期发病,发病率估计在1/10000;CPVT致死率极高,8年的心脏事件发生率在30%以上。目前CPVT最佳的诊断试验是在运动负荷试验中诱发出双向性或多形性室性心律失常。β受体阻滞剂是CPVT的治疗基石,其他的治疗选择包括钙离子受体阻滞剂、氟卡尼和左心交感神经切除术(LCSD),而植入型心律转复除颤器(ICD)对于预防心脏性猝死(SCD)具有重要意义。本文报道了1例具有猝死家族史的年轻女性病人,因反复活动或情绪激动后心悸、晕厥10余年入院,通过详细的病史询问分析和阳性的运动负荷试验结果临床诊断为CPVT,予以β受体阻滞剂治疗获得良好的效果。本文同时对CPVT的发病基础、基因突变、病理生理学机制、临床特征和治疗策略进行了总结讨论。
[Abstract]:Catecholamine-sensitive pleomorphic ventricular tachycardia (CPVT) is an inherited arrhythmia disease caused by mutations in the calcium channel gene of sarcoplasmic reticulum (SRS). The clinical characteristics of CPVT are bidirectional or pleomorphic ventricular tachycardia induced by exercise or emotion. CPVT often occurs in children or young people. The incidence of CPVT is estimated to be very high at 1 / 10 000 of CPVT, and the incidence of cardiac events in 8 years is over 30%. The best diagnostic test for CPVT is to induce bidirectional or pleomorphic ventricular arrhythmias in exercise stress tests. Beta blockers are the cornerstone of CPVT, and other treatment options include calcium receptor blockers. Flukanil and left cardiac sympathetic neurotomy (LCSD) and implantable cardioverter defibrillator (ICD) are important for the prevention of sudden cardiac death (SCD). A young female patient with a family history of sudden death was admitted to hospital for more than 10 years because of repeated activity or emotion and palpitation. CPVT was clinically diagnosed as CPVT by detailed history inquiry and positive exercise load test, and a good effect was obtained by 尾 -blocker therapy. The pathogenesis, gene mutation, pathophysiological mechanism, clinical features and therapeutic strategies of CPVT were summarized and discussed.
【学位授予单位】：浙江大学
【学位级别】：博士
【学位授予年份】：2013
【分类号】：R541.71

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