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日常实践中诊断心脏淀粉样变性:心脏病专家实用指南

Diagnosing cardiac amyloidosis in every-day practice: A practical guide for the cardiologist.

作者信息

Papathanasiou Maria, Carpinteiro Alexander, Rischpler Christoph, Hagenacker Tim, Rassaf Tienush, Luedike Peter

机构信息

Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Hufelandstrasse 55, 45147 Essen, Germany.

West German Amyloidosis Center, University Hospital Essen, Hufelandstrasse 55, 45147 Essen, Germany.

出版信息

Int J Cardiol Heart Vasc. 2020 Apr 27;28:100519. doi: 10.1016/j.ijcha.2020.100519. eCollection 2020 Jun.

Abstract

Cardiac amyloidosis (CA) has emerged as a previously underestimated cause of heart failure and mortality. Underdiagnosis resulted mainly from unawareness of the true disease prevalence and the non-specific symptoms of the disease. CA results from extracellular deposition of misfolded protein fibrils, commonly derived from transthyretin (ATTR) or immunoglobulin light chains (AL). A significant proportion of older patients with heart failure and other extracardiac manifestations suffer from ATTR-CA, whereas AL-CA is still considered a rare disease. This article provides an overview of CA with a special focus on current and emerging diagnostic modalities. Furthermore, we provide a diagnostic algorithm for the evaluation of patients with suspected CA in every-day practice.

摘要

心脏淀粉样变性(CA)已成为一种先前被低估的心力衰竭和死亡原因。诊断不足主要是由于对该疾病的真实患病率以及非特异性症状认识不足。CA是由错误折叠的蛋白原纤维在细胞外沉积所致,这些原纤维通常来源于转甲状腺素蛋白(ATTR)或免疫球蛋白轻链(AL)。相当一部分患有心力衰竭和其他心外表现的老年患者患有ATTR-CA,而AL-CA仍被认为是一种罕见疾病。本文概述了CA,特别关注当前和新兴的诊断方式。此外,我们提供了一种诊断算法,用于在日常实践中评估疑似CA的患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9bcb/7191222/f60cd1db2502/gr1.jpg

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