目的：对儿茶酚胺敏感性多形性室性心动过速（CPVT）临床表现进行观察分析，为CPVT的诊断提供参考依据。方法：回顾性分析2007年1月至2014年1月间我单位通过RYR2、贮钙蛋白2（CASQ2）基因突变检测确诊为CPVT的5例汉族患者的临床资料以及肾上腺素激发试验等结果。结果：患者心电图表现为静息状态下，右胸导联T波切迹、双向、双峰和明显U波，Q T间期正常。其中1例因情绪激动诱发晕厥，此时动态心电图提示：室早诱发pVT，进而恶化成为室扑、室颤，患者在持续晕厥数分钟后自行缓解，室颤转为室速、室早二联律，最终恢复窦律。5例患者中，有3例患者通过平板运动试验诱发出其CPVT的室性心动过速（VT），在终止运动之后，VT转化为PVC，随后逐渐恢复窦律。虽然另外2例未激发出VT，但PVC均出现频发。且4例患者在停用肾上腺素之后，自行恢复窦律，1例则经静脉应用BB后转复窦律。结论：CPVT属遗传性心脏离子通道疾病，儿茶酚胺激素的分泌则是诱发其发病的重要触发因子，β受体阻滞剂对CPVT的治疗具有较好有效性与安全性。%Objective:To catecholaminergic polymorphic ventricular tachycardia (CPVT) clinical manifestations were observed and analyzed to provide the appropriate reference for CPVT diagnosis. Methods:A retrospective analysis of clinical data from January 2007 to January 2014 my unit through RYR2, calcium storage protein 2 (CASQ2) mutations detected in ifve cases diagnosed CPVT patients and Han adrenaline challenge test results, etc. Review analysis.Results:The ECG showed the resting state, ECG showing its right precordial T Rinpoche track, two-way, and obviously bimodal U wave; and normal QT interval. 1 case of emotion -induced syncope, at which point the Holter Tip : PVCs induced pVT, and then deteriorated into ventricular lfutter, ventricular ifbrillation; patients with syncope minutes after sustained spontaneous remission; into ventricular tachycardia and ventricular ifbrillation, also, PVCs bigeminy; until eventually restored sinus rhythm. Five patients, three patients out of its CPVT induced ventricular tachycardia (VT) by treadmill exercise test, after the termination of the movement, VT converted to PVC, then gradually restored sinus rhythm. While the other two patients did not inspire VT, but both appear PVC frequent. And 4 patients after disabling adrenaline, self- restore sinus rhythm; one case the BB after intravenous cardioversion to sinus rhythm.Conclusion:CPVT is inherited cardiac ion channel diseases, secrete catecholamine hormones induce its incidence is an important trigger factor; whereas β-blocker therapy for CPVT has better efifcacy and safety.
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