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轻链型（AL）心脏淀粉样变性表现为射血分数降低的心力衰竭。

Light-Chain (AL) Cardiac Amyloidosis Presenting as Heart Failure With Reduced Ejection Fraction.

作者信息

Santiago Luis E, Alvi Ali Tariq, Melnychuk Veniamin, Mesquita Philip, Aneja Pallavi

机构信息

Internal Medicine, HCA Florida Northwest Hospital, Margate, USA.

Internal Medicine, HCA Florida Westside Hospital, Plantation, USA.

出版信息

Cureus. 2024 Feb 29;16(2):e55271. doi: 10.7759/cureus.55271. eCollection 2024 Feb.

DOI:10.7759/cureus.55271
PMID:38558722
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10981542/
Abstract

Systemic amyloidosis is caused by the extracellular deposition of misfolded proteins in various organs and usually leads to organ dysfunction. The two common subtypes include light-chain amyloidosis and transthyretin amyloidosis. Deposition of these proteins in the heart can lead to infiltrative and restrictive cardiomyopathy, commonly manifesting as heart failure with preserved ejection fraction. However, systolic heart failure with reduced ejection fraction is mainly seen in the advanced stages of the disease. Here, we present the case of a 53-year-old female who presented with new-onset heart failure with reduced ejection fraction with no prior symptoms or diagnosis of amyloidosis and diastolic dysfunction.

摘要

系统性淀粉样变性是由错误折叠的蛋白质在各器官细胞外沉积引起的，通常会导致器官功能障碍。两种常见的亚型包括轻链淀粉样变性和转甲状腺素蛋白淀粉样变性。这些蛋白质在心脏中的沉积可导致浸润性和限制性心肌病，通常表现为射血分数保留的心力衰竭。然而，射血分数降低的收缩性心力衰竭主要见于疾病晚期。在此，我们报告一例53岁女性病例，该患者出现新发射血分数降低的心力衰竭，既往无淀粉样变性和舒张功能障碍的症状或诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/746cea8980b5/cureus-0016-00000055271-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/97caebd61e14/cureus-0016-00000055271-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/a9f6912dc4f9/cureus-0016-00000055271-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/81069fd5892d/cureus-0016-00000055271-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/b9499df2ed44/cureus-0016-00000055271-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/e5e046f3732e/cureus-0016-00000055271-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/746cea8980b5/cureus-0016-00000055271-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/97caebd61e14/cureus-0016-00000055271-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/a9f6912dc4f9/cureus-0016-00000055271-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/81069fd5892d/cureus-0016-00000055271-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/b9499df2ed44/cureus-0016-00000055271-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/e5e046f3732e/cureus-0016-00000055271-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b1d/10981542/746cea8980b5/cureus-0016-00000055271-i06.jpg

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本文引用的文献

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Cureus. 2022 Dec 7;14(12):e32281. doi: 10.7759/cureus.32281. eCollection 2022 Dec.
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Cardiac Amyloidosis Presenting as Biventricular Systolic Heart Failure.表现为双心室收缩性心力衰竭的心脏淀粉样变性
Case Rep Cardiol. 2021 Apr 1;2021:6671469. doi: 10.1155/2021/6671469. eCollection 2021.
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Cardiac Amyloidosis: Overlooked, Underappreciated, and Treatable.心脏淀粉样变：被忽视、被低估且可治疗。
Annu Rev Med. 2020 Jan 27;71:203-219. doi: 10.1146/annurev-med-052918-020140.
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Contemporary Imaging Diagnosis of Cardiac Amyloidosis.心脏淀粉样变性的当代影像学诊断
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State-of-the-art radionuclide imaging in cardiac transthyretin amyloidosis.心脏转甲状腺素蛋白淀粉样变性的前沿放射性核素成像
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Cardiac Structural and Functional Consequences of Amyloid Deposition by Cardiac Magnetic Resonance and Echocardiography and Their Prognostic Roles.心脏磁共振和超声心动图检查发现的淀粉样变性所致的心脏结构和功能改变及其预后价值。
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Eur Heart J. 2017 Oct 7;38(38):2879-2887. doi: 10.1093/eurheartj/ehx350.
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