【摘要】：目的觀察6例漢族兒茶酚胺敏感性多形性室性心動過速(CPVT)患者的臨床表現(xiàn),為漢族人群CPVT的診斷提供參考。方法回顧分析2002年7月至2010年3月在我中心通過RYR2、CASQ2基因突變檢測確診為CPVT的6例患者的臨床資料,包括基本資料、發(fā)病情況、心電圖參數(shù)、動態(tài)心電圖表現(xiàn)及平板運動試驗結(jié)果。另外,我們對其中的5例患者進行了腎上腺素激發(fā)試驗。結(jié)果6例患者(男4例)確診時的平均年齡為13.0±4.2(7-20)歲,首發(fā)癥狀均為暈厥。靜息狀態(tài)下體表十二導聯(lián)心電圖(ECG)顯示右胸導聯(lián)T波切跡、雙峰、雙向以及明顯的U波,無J點抬高、ST段偏移及QT間期異常,二維超聲心動圖(ECHO)未及心臟結(jié)構(gòu)異常。此6例患者均記錄到典型的雙向形室性心動過速(bVT)和(或)多形性室性心動過速(pVT)：ECG(2/6)、Holter(5/6)、平板運動試驗(3/6)、腎上腺素激發(fā)試驗(5/5)。6例患者均給予β受體阻滯劑(BB)口服治療,出院后3個月門診隨訪,均未再發(fā)暈厥。結(jié)論：CPVT是一種遺傳性心臟離子通道疾病,靜息狀態(tài)下ECG表現(xiàn)為右胸導聯(lián)T波改變、明顯的U波,發(fā)病時表現(xiàn)為bVT、pVT,兒茶酚胺類激素的分泌是其發(fā)病的主要觸發(fā)因子,BB對其安全有效。
[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 鹵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.
【學位授予單位】：南京醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2012
【分類號】：R541.7

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