摘要
本文报道我院10年间经尸解和组织活检证实的心脏淀粉样变性5例,原发性者4例,伴多发性骨髓瘤浸者1例,4例因浮肿,诊断肾病综合征,1例因厌食,呕吐住院。5例均有不同程度的心功能障碍,但胸片仅1例心脏增大。超声心动图表现为左室对称性肥厚,4例心室腔正常,但左室重量增重,平均384克,FS％,EF不同程度下降。仅2例室壁有粗颗粒状亮回声。因此,我们认为:(1)心脏淀粉样变性主要表现是心律失常,传导障碍和药物难以控制的心力衰竭。(2)UCG可以表现心脏受累、病变严重程度、病情演变和预后。(3)室壁粗颗粒状亮回声不是本病特异征象。(4)本病确诊依活组织检查。
Cardiac Amyloidosis is anextremely rare disease in China During Past 10 years, thereeare 5 cases which diagnosis was made by tissu biopsy ora utopsy in our hospital.Main clinical featuies of circulatory system were arrrhythmia conduction abnormalities and ccrdiac insufficiency,Im-oortant Echocardio-graphic manifestatio were left ventirchlar hypertrophy (LVM:X=384g),FS% and LVEF were all lower, but the cavity of the heart were no dilatati-on in 4 Patients. 'Granular Sparkling' on 2-D Echo was found in 2 cases.Diagnosis was discused
