心脏淀粉样变性

Cardiac Amyloidosis

淀粉样变性是以不可溶性的淀粉样物质沉积于器官或组织的细胞外区,导致相应的器官或组织功能障碍为特征的一组疾病。心脏是淀粉样变性常累及的器官。心脏淀粉样变性临床上通常按其病因分为原发性、家族性、老年性、继发性及透析相关性五种类型。其最常见的临床表现为限制性心肌病和顽固性心力衰竭,有时伴有传导阻滞。心肌活检可以确定诊断,近年来随着心外组织取样和显像技术的发展已大大减少了心内膜心肌活检的必要。尽管治疗上有所改进,但心脏淀粉样变性患者预后仍然较差,主要取决于原有疾病类型。

Amyloidosis is a group of diseases all characterized by deposition of insoluble amyloid fibrils in the extracellular spaces of organs and tissues, with resulting dysfunction of organs. The cardiovascular system is a common target of amyloidosis. In clinical practice, cardiac amyloidosis is classified as primary, familial, senile, secondary and hemodialysis-associated. The most common presentations are restrictive cardiomyopathy, and clinically intractable heart failure with or without conduction system disturbances. However, endomyocardial biopsy is the definitive diagnostic, recent developments in imaging techniques and extracardiac tissue sampling have minimized the need for invasive endomyocardial biopsy for amyloidosis. Despite advances in treatment, the prognosis for patients with amyloidosis is still poor and depends on the underlying disease type. Herein, we present new insights and recent advances in cardiovascular amyloidosis.