12例心肌淀粉样变性的临床特点和误诊分析

Clinical manifestations and the reasons for misdiagnosis of 12 patients with cardiac amyloidosis

目的:分析心肌淀粉样变性患者临床特点及误诊原因。方法:对12例心肌淀粉样变性患者的临床资料进行回顾性分析。结果:①误诊率高,首诊误诊率为91.7%。最常误诊为肥厚型心肌病(33.3%),其次为冠心病(25%);②心肌淀粉样变性常联合肾脏和肝功能损害;③超声心动图显示心肌颗粒样闪光回声增强者占41.7%,室间隔增厚和房间隔增厚的检出率分别为83.3%和33.3%;④心电图改变以肢体导联低电压和胸前导联R波递增不良最为常见。结论:心肌淀粉样变性常联合多脏器损害,有超声心动图和心电图的特征性改变。首诊误诊率很高,需对此病提高认识。

Objective：To analyze clinical manifestations and misdiagnosis of the cardiac amyloidosis. Method：The clinical data of 12 patients with cardiac amyloidosis were analysed. Result：①The misdiagnosis rate was high （91.7%）.The cardiac amyloidosis was mostly miadiagonosesd as hypertrophic cardiomyopathy （33.3%）,and the second coronary artery disease（25%）. ②Renal and/or gastrointestinal involvement were very frequently. ③The echocardiography indicated that 41.7% of the patients were possessed of hyperechoic granular flash.The detaction rate of the increased thickness of the interventricular septums,interartricular septums were 83.3 % and 33.3%. ④The low voltage in the limb leads,combining with poor R wave progression in the precordial leads were the most frequently showed in the ECG.Conclusion：The cardiac amyloidosis was often combinded with dysfunction of several organs and tissues. It showed characteristic features in the echocardiography and electrocardiogram. The misdiagnosis rate of cardiac amyloidosis was high and we should raise the awareness of this disease.