Harvard Vanguard Medical Associates, Harvard Medical School, Boston, MA 02215, USA.
Prog Cardiovasc Dis. 2010 Jan-Feb;52(4):347-61. doi: 10.1016/j.pcad.2009.11.007.
The systemic amyloidoses are an uncommon group of disorders characterized by the extracellular deposition of amyloid in one or more organs. Cardiac deposition, leading to an infiltrative/restrictive cardiomyopathy, is a common feature of amyloidosis. It may be the presenting feature of the disease or may be discovered while investigating a patient presenting with non-cardiac amyloidosis. In this article we review the features of cardiac amyloidosis and its varied manifestations. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is stressed in light of recent advances in therapy which can, when appropriately used, significantly improve prognosis.
系统性淀粉样变性是一组罕见的疾病，其特征是一种或多种器官中外源性淀粉样物质的沉积。心脏沉积导致浸润性/限制性心肌病，是淀粉样变性的常见特征。它可能是疾病的首发表现，也可能是在调查患有非心脏淀粉样变性的患者时发现的。本文回顾了心脏淀粉样变性的特征及其不同表现。鉴于最近在治疗方面取得了进展，如果适当地使用，这些进展可以显著改善预后，因此强调了对高度怀疑和精确生化分型淀粉样沉积物的重要性。
