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心脏淀粉样变性:临床表现、诊断与治疗综述。

A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment.

机构信息

Department of Internal Medicine, Division of Cardiovascular Diseases, University of Tennessee Health Science Center, Memphis, TN.

Department of Internal Medicine, Division of Cardiovascular Diseases, University of Tennessee Health Science Center, Memphis, TN.

出版信息

Curr Probl Cardiol. 2022 Dec;47(12):101366. doi: 10.1016/j.cpcardiol.2022.101366. Epub 2022 Aug 20.

Abstract

Amyloidosis is a group of disorders that can affect almost any organ due to the misfolding of proteins with their subsequent deposition in various tissues, leading to various disease manifestations based on the location. When the heart is involved, amyloidosis can manifest with a multitude of presentations such as heart failure, arrhythmias, orthostatic hypotension, syncope, and pre-syncope. Diagnosis of cardiac amyloidosis can be difficult due to the non-specific nature of symptoms and the relative rarity of the disease. Amyloidosis can remain undiagnosed for years, leading to its high morbidity and mortality due to this delay in diagnosis. Newer imaging modalities, such as cardiac magnetic resonance imaging, advanced echocardiography, and biomarkers, make a timely cardiac amyloidosis diagnosis more feasible. Many treatment options are available, which have provided new hope for this patient population. This manuscript will review the pathology, diagnosis, and treatment options available for cardiac amyloidosis and provide a comprehensive overview of this complicated disease process.

摘要

淀粉样变性是一组疾病,由于蛋白质错误折叠及其随后在各种组织中的沉积,几乎可以影响任何器官,导致基于位置的各种疾病表现。当心脏受累时,淀粉样变性可表现为心力衰竭、心律失常、体位性低血压、晕厥和先兆晕厥等多种表现。由于症状的非特异性和疾病的相对罕见性,心脏淀粉样变性的诊断可能很困难。淀粉样变性可能多年未被诊断,由于这种诊断延迟,其发病率和死亡率很高。心脏磁共振成像、高级超声心动图和生物标志物等较新的成像方式使及时诊断心脏淀粉样变性变得更加可行。有许多治疗选择,为这一患者群体提供了新的希望。本文将回顾心脏淀粉样变性的病理学、诊断和治疗选择,并对这一复杂的疾病过程进行全面概述。

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