作者：钟锦珑1，2 ，马小静1 ，夏娟1 ，陈慧颖1

单位：

1.430022湖北省武汉市，武汉科技大学附属武汉亚洲心脏病医院超声科 武汉市心血管影像 临床医学研究中心　2.430065湖北省武汉市，武汉科技大学医学部医学院

Units：

1.Department of Ultrasound, Wuhan Asia Heart Hospital Affiliated to Wuhan University of Science and Technology/Wuhan Clinical Medical Research Center of Cardiovascular Imaging, Wuhan 430022, China 2.Wuhan University of Science and Technology School of Medicine, Wuhan 430065, China

关键词：

淀粉样变性；心肌淀粉样变性；病例报告

Keywords：

Amyloidosis; Cardiac amyloidosis; Case reports

CLC：

R 597.2

DOI：

10.12114/j.issn.1008-5971.2023.00.246

Funds：

武汉市科技计划项目（2019020701011422）；武汉市 医学科研项目（WX21Q30）

摘要：

心肌淀粉样变性（CA）指蛋白质出现错误折叠后沉积于心脏，引起以心脏舒张功能障碍为主的特异 性限制性心肌病，常可导致难治性心力衰竭或其他脏器衰竭甚至死亡。CA临床表现多样，因而其早期诊断仍较困难。 本文报道的2例CA患者均以胸闷、呼吸困难等非特异性症状就诊，根据患者临床症状、二维斑点追踪成像检查结果、 脂肪组织病理检查结果诊断为CA，后予以利尿、营养心肌、抗凝、维持电解质等对症治疗，患者症状好转。本文同时 通过文献复习分析了CA的发病机制、分型、临床表现、诊断、治疗及预后，以期为临床诊治CA提供参考。

Abstract：

Myocardial amyloidosis (CA) refers to the deposition of proteins in the heart after misfolding, leading to specific restrictive cardiomyopathy, mainly with diastolic dysfunction, often leading to refractory heart failure or other organ failure even death. The early diagnosis of CA is still difficult because of the diverse clinical manifestations. The two patients reported in this article were presented with non-specific symptoms such as chest tightness and dyspnea. CA diagnosis was confirmed by clinical symptoms, two-dimensional spot tracking imaging examination results, and pathological examination results of adipose tissue, symptomatic treatment such as diuresis, myocardial nutrition, anticoagulation and electrolyte maintenance was given, and the patient's, symptoms improved. This article also analyzed the pathogenesis, classification, clinical manifestations, diagnosis, treatment and prognosis of CA through literature review, in order to provide reference for clinical diagnosis and treatment of CA.

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