致心律失常性右心室心肌病一家系报告

目的 探讨家族性致心律失常性右心室心肌病的心电图特点。方法报道致心律失常性右心室心肌病患者一家系并结合文献分析其特点。结果本家系中右心室心肌病的发病率很高，男女患病比例相仿，疾病的表现度有较大的可变性，有时可无症状。先证者心电图发现Epsilon波且以V3R最为明显，所有患者V1～V3QRS波群终末部均有挫折，QRS时间均〉110ms，V3～V4均有T波倒置现象。结论致心律失常性右心室心肌病存在明显的遗传倾向，临床上以室性心律失常及猝死为主要表现，心电图具有相对特征性改变。

Arrhythmogenic Right Ventricular Cardiomyopathy: A Pedigree Report

Objective To investigate electrocardiographic （ECG） characteristics of familial arrhythmogenic right ventricular cardiomyopathy （ARVC）. Methods The family pedigree of the index patient with ARVC was investigated and its features, combined with literature, were analyzed. Results The incidence of right ventricular cardiomyopathy was high in male as well as female in the family. The clinical expressivity was highly variable, including asymptomatic presentation. Epsilon wave appeared on ECG of the proband and was most obvious in lead V3R. A terminal deflection within the end of QRS wave in leads V1to V3, ORS duration 〉 110ms, and T wave inversion in leads V1 to V4 were seen in all patients. Conclusion ARVC is of higher hereditary susceptibility. The main clinical manifestations are ventricular arrhythmia and sudden cardiac death. There are specific changes on ECG.