儿茶酚胺敏感性多形性室性心动过速临床特征分析
发布时间：2019-03-20 21:20
【摘要】：目的观察6例汉族儿茶酚胺敏感性多形性室性心动过速(CPVT)患者的临床表现,为汉族人群CPVT的诊断提供参考。方法回顾分析2002年7月至2010年3月在我中心通过RYR2、CASQ2基因突变检测确诊为CPVT的6例患者的临床资料,包括基本资料、发病情况、心电图参数、动态心电图表现及平板运动试验结果。另外,我们对其中的5例患者进行了肾上腺素激发试验。结果6例患者(男4例)确诊时的平均年龄为13.0±4.2(7-20)岁,首发症状均为晕厥。静息状态下体表十二导联心电图(ECG)显示右胸导联T波切迹、双峰、双向以及明显的U波,无J点抬高、ST段偏移及QT间期异常,二维超声心动图(ECHO)未及心脏结构异常。此6例患者均记录到典型的双向形室性心动过速(bVT)和(或)多形性室性心动过速(pVT)：ECG(2/6)、Holter(5/6)、平板运动试验(3/6)、肾上腺素激发试验(5/5)。6例患者均给予β受体阻滞剂(BB)口服治疗,出院后3个月门诊随访,均未再发晕厥。结论：CPVT是一种遗传性心脏离子通道疾病,静息状态下ECG表现为右胸导联T波改变、明显的U波,发病时表现为bVT、pVT,儿茶酚胺类激素的分泌是其发病的主要触发因子,BB对其安全有效。
[Abstract]:Objective to observe the clinical manifestations of 6 (CPVT) patients with catecholamine-sensitive polymorphic ventricular tachycardia (VVT) in Han nationality, and to provide a reference for the diagnosis of CPVT in Han population. Methods from July 2002 to March 2010, the clinical data of 6 patients with CPVT diagnosed by RYR2,CASQ2 gene mutation detection were retrospectively analyzed, including basic data, incidence and ECG parameters. Dynamic electrocardiogram and results of treadmill exercise test. In addition, we performed an adrenaline challenge test in 5 of them. Results the mean age of 6 patients (4 males) was 13.0 卤4.2 (7 / 20) years old. The first symptom was syncope. At rest, the surface 12-lead electrocardiogram (ECG) showed T-wave notch, double-peak, bi-directional and obvious U-wave in the right chest lead, no J-point elevation, ST segment deviation and QT interval abnormality, and two-dimensional echocardiography (ECHO) did not reach the cardiac structure abnormality. Typical bi-directional ventricular tachycardia (bVT) and / or pleomorphic ventricular tachycardia (pVT): ECG (2 / 6), Holter (5 / 6) and treadmill exercise test (3 / 6) were recorded in all 6 patients. 6 patients were treated with 尾-blocker (BB) orally and were followed up for 3 months after discharge. No further syncope was found in all the 6 patients who were treated with 尾-adrenoceptor blocker (尾-adrenoceptor blocker) for 3 months after discharge. Conclusion: CPVT is a kind of hereditary heart ion channel disease. At rest, ECG shows the change of T wave in right chest lead and obvious U wave. The secretion of catecholamines in bVT,pVT, is the main trigger of the disease. BB is safe and effective.
【学位授予单位】：南京医科大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R541.7
本文编号：2444642
[Abstract]:Objective to observe the clinical manifestations of 6 (CPVT) patients with catecholamine-sensitive polymorphic ventricular tachycardia (VVT) in Han nationality, and to provide a reference for the diagnosis of CPVT in Han population. Methods from July 2002 to March 2010, the clinical data of 6 patients with CPVT diagnosed by RYR2,CASQ2 gene mutation detection were retrospectively analyzed, including basic data, incidence and ECG parameters. Dynamic electrocardiogram and results of treadmill exercise test. In addition, we performed an adrenaline challenge test in 5 of them. Results the mean age of 6 patients (4 males) was 13.0 卤4.2 (7 / 20) years old. The first symptom was syncope. At rest, the surface 12-lead electrocardiogram (ECG) showed T-wave notch, double-peak, bi-directional and obvious U-wave in the right chest lead, no J-point elevation, ST segment deviation and QT interval abnormality, and two-dimensional echocardiography (ECHO) did not reach the cardiac structure abnormality. Typical bi-directional ventricular tachycardia (bVT) and / or pleomorphic ventricular tachycardia (pVT): ECG (2 / 6), Holter (5 / 6) and treadmill exercise test (3 / 6) were recorded in all 6 patients. 6 patients were treated with 尾-blocker (BB) orally and were followed up for 3 months after discharge. No further syncope was found in all the 6 patients who were treated with 尾-adrenoceptor blocker (尾-adrenoceptor blocker) for 3 months after discharge. Conclusion: CPVT is a kind of hereditary heart ion channel disease. At rest, ECG shows the change of T wave in right chest lead and obvious U wave. The secretion of catecholamines in bVT,pVT, is the main trigger of the disease. BB is safe and effective.
【学位授予单位】：南京医科大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R541.7
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