淀粉样心肌病的诊断

The diagnostic approach to amyloid cardiomyopathy

目的探讨淀粉样心肌病的诊断方法。方法分析2例表现为难治性右心衰竭患者,分别采用右心导管和心内膜活检等方法明确诊断。结果 2例患者心脏超声和MRI均提示室间隔增厚、心肌回声和延迟扫描增强,右心导管检查提示右心压力增高,心内膜活检刚果红染色阳性。结论心脏症状通常是系统性淀粉样变性的第一表现,部分患者表现为难治性心力衰竭,右心导管和心内膜活检可以明确诊断。

Objective To investigate the diagnostic approach to amyloid cardiomyopathy. Methods The clinical data of two patients with amyloid cardiomyopathy, which presented clinically as refractory right heart failure, were retrospectively analyzed. The diagnosis of both patients was confirmed by means of right heart catheterization and endomyocardial biopsy. Results Thickened ventricular septum was demonstrated on echocardiograph and delayed myocardial enhancement was seen on enhanced MRI in both patients. Right heart catheterization examination revealed a higher right heart pressure, and endomyocardial biopsy showed that Congo red staining was positive. Conclusion Usually, cardiac symptom is the first manifestation in systemic amyloidosis. In some patients the disease is manifested as refractory heart failure. Right heart catheterization and endomyocardial biopsy can confirm the diagnosis.（J Intervent Radiol, 2012, 21： 766-769）