致心律失常性右室心肌病的临床与电生理学特征  

The clinical and electrophysiologic characteristics of arrythmogenic right ventricular cardiomyopathy

作　　者：李广平[1] 张梅[1] 徐延敏[1] 陈欣[1] 董媛媛[1] 苏新华[1] 黄体钢[1] 

机构地区：[1]天津医科大学第二医院心脏科,天津心脏病学研究所300211

出　　处：《临床心电学杂志》2002年第4期193-196,共4页Journal of Clinical Electrocardiology

摘　　要：目的与方法　致心律失常性右室心肌病 (ARVC)可引起严重心律失常 ,本文对 6例ARVC患者进行了临床和电生理学特点观察和研究。结果　 6例患者有室性心动过速 (VT)的 5例 ,多形性VT2例。所有患者均表现右心室 (RV)扩大 ,可见到RV局部病变的 2例 ,均没有见到左心室受累和明显的心功能受损。接受电生理学检查的 4例患者中 ,有 3例可以由电刺激诱发和终止 ,电生理标测 2例源自RV心尖部 ,2例源自RV游离壁 ,与超声提示的RV病变部位有关。 3例患者发生过阿斯综合征 ,其中有 2例发生院外猝死 ,2例猝死病人均发作过多形性VT。结论　ARVC以RV进行性病变伴室性心律失常为特征。射频消融 (RFCA)和ICD植入治疗、药物治疗是基本治疗手段 ,药物治疗以胺碘达隆加 β-受体阻滞剂或索他洛尔 (Sotalol)为多选 ,但是单纯的药物治疗并不能防止病人发生心脏性猝死。对于有条件的病人应该考虑进行RFCA或ICD治疗。Objective and Mothods Arrythmogenic right ventricular cardiomyopathy (ARVC) may produce severe cardiac arrythmias.We studied the clinical and electrophysiologic features in 6 patients who were diagnosed as ARVC.Results 5 patients had episodes of ventricular tachycardia(VT)and 2 patients had multiform VT in these 6 patients with VT.Right ventricular enlargements were manifested in all patients diagnosed as ARVC and regional lesions could be found in 2 patients even there were not manifestations of depressed or impaired left ventricular function.VTs could be induced and terminated by programmed electrical stimulation in 3 of 4 patients undergone electrophysiologic study.Electrophysiologic mapping proved that ventricular arrythmias originated from right ventricular apex in 2 patients and free wall in other 2 patients.2 of 3 patients,who experienced sudden cardiac death before admission,had multiform VT.Conclusion ARVC is characterized by progressive right ventricular lesion and ventricular arrythmias.Radiofrequency catheter ablation,ICD implantation and antiarrythmic agents of class Ⅱ and Ⅲ are primary therapies.

关 键 词：致心律失常性右室心肌病 临床特点 电生理 

分 类 号：R542.2[医药卫生—心血管疾病]