诊断轻链型心脏淀粉样变的超声心动图新进展  

New progress in echocardiography for the diagnosis of amyioid light-chain cardiac amyloidosis

作　　者：张赫展 付启航 张冬雪 徐卉[1] ZHANG Hezhan;FU Qihang;ZHANG Dongzue;XU Hui(Department of Cardiac Ultrasound,The First Hospital of Jilin University,Changchun 130021,China)

机构地区：[1]吉林大学第一医院心脏超声科,吉林长春130021

出　　处：《中国实验诊断学》2025年第3期363-369,共7页Chinese Journal of Laboratory Diagnosis

摘　　要：轻链型心脏淀粉样变(Amyioid light-chain cardiac amyloidosis,AL-CA)是一种浸润性限制型心肌病,是由浆细胞异常增殖产生单克隆免疫球蛋白轻链,其错误折叠形成不可溶的淀粉样蛋白纤维沉积于心肌细胞间质和血管周围,导致心脏结构和功能的素乱。其临床表现无特异性,病程进展迅速,预后极差。因此,早期诊断并及时启动治疗意义重大。超声心动图作为诊断AL-CA的首选影像学手段,通过斑点追踪技术、压力-应变环分析、多参数评估、衍生参数及人工智能的辅助,为临床提供了更多敏感指标,显著提高了AL-CA的早期检出率和诊断准确性。本文主要就超声心动图对AL-CA诊断及鉴别诊断中的应用进展作一综述。Amyloid light-chain cardiac amyloidosis(AL-CA)is an infiltrative restrictive cardiomyopathy.It is caused by the abnormal proliferation of plasma cells that produce monoclonal immunoglobulin light chains.These mis-folded light chains form insoluble amyloid fibrils and deposit in the interstitium of myocardial cells and around blood vessels,leading to disorders of cardiac structure and function.Its clinical manifestations are non-specific,the disease progresses rapidly,and the prognosis is extremely poor.Therefore,early diagnosis and prompt initiation of treatment are of great significance.Echocardiography,as the preferred imaging method for diagnosing AL-CA,provides more sen-sitive indicators for clinical practice through the assistance of speckle tracking technology,pressure-strain loop analysis,multi-parameter assessment,derivative parameters,and artificial intelligence,significantly improving the early detection rate and diagnostic accuracy of AL-CA.This article mainly reviews the application progress of echocardiography in the diagnosis and differential diagnosis of AL-CA.

关 键 词：轻链型心脏淀粉样变 超声心动图 诊断 

分 类 号：R54[医药卫生—心血管疾病]