心脏淀粉样变性的诊断进展

An update on diagnosis of cardiac amyloidosis

淀粉样变性是由于淀粉样蛋白沉积在细胞外基质,造成沉积部位组织和器官损伤的一组疾病,出现心脏受累时即为心脏淀粉样变性,该病病情重、进展快、治疗困难、病死率高,但临床少见且识别率低。本文着重叙述其诊断手段和相关进展,并对诊断流程进行了归纳,以协助临床了解并正确诊断该病,使患者获得及时治疗并从中获益。

Amyloidosis is a group of diseases caused by amyloid protein deposition in the extracellular matrix,causing damage of multiple tissues and organs,and the diagnosis of cardiac amyloidosis is established when heart is involved.The aim of this article is to review relevant concepts,classifications,pathophysiological features and clinical manifestations as well as diagnostic algorithm of cardiac amyloidosis,in order to improve the clinical recognition and correct diagnosis of this disease,so that patients receive timely treatment and benefit from it.