本文报告一例致心律失常性右室发育不全。据笔者们所见的文献,本例可能系国内第三例报告。患者为中年女性,反复发作心动过速已20年,此次因持续发作20日而入院。入院描记心电图示左束支传导阻滞型室速,心电轴+130°。多次静注利多卡因及普鲁卡因酰胺均无效,直流电击一次复律成功。实性心律时,V_1～V_2导联的S—T段起始部位可见“E”波。二维超声心动图可见右室普遍扩张,收缩无力,右室流出道膨隆,右房亦示扩大。患者无家族史。 This article reports a case of arrhythmogenic right ventricular hypoplasia. According to the author’s literature, this case may be the third case of domestic report. The patient is a middle-aged woman, recurrent tachycardia has been 20 years, the hospital for sustained episodes of 20 and admission. Admission description ECG left bundle branch block type ventricular tachycardia, ECG axis + 130 °. Multiple intravenous lidocaine and procaine amide were invalid, a DC cardioversion success. In solid rhythm, the “E” wave can be seen at the start of S-T segment of V_1 ~ V_2 lead. Two-dimensional echocardiography shows the general expansion of the right ventricle, contracting weakness, right ventricular outflow tract bulging, right atrium also showed expansion. Patients have no family history.