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扩张型心肌病的原因不明,绝大多数病人左室严重受累。本文报告的14例病人均以严重的右心功能障碍为特征,其中女性5例,男性9例,平均年龄28(9～62)岁。病初表现为心律失常者10例,心衰者开始有4例,以后有3例,6例室性心动过速引起晕厥,4例室上性心律失常导致心悸。8例无右室衰竭的临床体征。至今追踪观察12例,平均时间4.1(0.5～14)年。在追踪期间11例死于症状开始后6个月～12年。其中猝死5例,但仅2例有心衰,有2例是同胞兄弟,临床所见几乎完全相同,均有室性心动过速所致的晕厥,并分别于2和3年后突然死亡。所有病人不但有右室搏动,且还有第3或第4心音,有右心衰者仅7例颈静脉压升高。心电图检查9例有右室异常,其中右束支传导阻滞2例,右室肥大2例、电轴右偏(平均额面电轴>
The cause of dilated cardiomyopathy is unknown, and the vast majority of patients have severe left ventricular involvement. All 14 patients reported here are characterized by severe right heart dysfunction, including 5 females and 9 males, with an average age of 28 (9 to 62) years. Early symptoms of arrhythmia in 10 cases, heart failure began in 4 cases, after 3 cases, 6 cases of ventricular tachycardia caused syncope, 4 cases of supraventricular arrhythmias lead to palpitations. Eight patients had no clinical signs of right ventricular failure. Up to now, 12 cases have been followed up and the average time is 4.1 (0.5-14) years. During the follow-up period, 11 patients died 6 months to 12 years after the onset of symptoms. Among them, 5 cases were sudden death, but only 2 cases had heart failure and 2 cases were siblings. The clinical findings were almost the same. Both had syncope caused by ventricular tachycardia and died suddenly after 2 and 3 years respectively. All patients not only had right ventricular beatings, but also third or fourth heart sounds, and only seven patients with right heart failure had elevated jugular venous pressure. There were 9 cases of right ventricular anomalies in ECG examination, including 2 cases of right bundle branch block, 2 cases of right ventricular hypertrophy, right partial axial deviation (average frontal axis>