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心脏淀粉样变性

Cardiac Amyloidosis.

作者信息

Bloom Michelle Weisfelner, Gorevic Peter D

机构信息

Division of Cardiology, Renaissance School of Medicine, State University of New York at Stony Brook, Stony Brook, New York (M.W.B.).

Division of Rheumatology, Renaissance School of Medicine, State University of New York at Stony Brook, Stony Brook, New York (P.D.G.).

出版信息

Ann Intern Med. 2023 Mar;176(3):ITC33-ITC48. doi: 10.7326/AITC202303210. Epub 2023 Mar 14.

Abstract

Amyloidosis is a pathologic and clinical condition resulting from the accumulation of insoluble aggregates of misfolded proteins in tissues. Extracellular deposition of amyloid fibrils in the myocardium leads to cardiac amyloidosis, which is often overlooked as a cause of diastolic heart failure. Although cardiac amyloidosis was previously believed to have a poor prognosis, recent advances in diagnosis and treatment have emphasized the importance of early recognition and changed management of this condition. This article provides an overview of cardiac amyloidosis and summarizes current screening, diagnosis, evaluation, and treatment options.

摘要

淀粉样变性是一种病理和临床状况,由错误折叠的蛋白质在组织中形成不溶性聚集体堆积所致。淀粉样纤维在心肌中的细胞外沉积导致心脏淀粉样变性,它常被忽视作为舒张性心力衰竭的一个病因。尽管心脏淀粉样变性以前被认为预后较差,但近期在诊断和治疗方面的进展强调了早期识别的重要性,并改变了对这种疾病的管理。本文概述了心脏淀粉样变性,并总结了当前的筛查、诊断、评估和治疗选择。

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