诊断轻链型心脏淀粉样变的超声心动图新进展.pdfVIP

中国实验诊断学2025年3月第29卷第3期ChinJLabDiagn,March,2025,Vol.29,No.3

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诊断轻链型心脏淀粉样变的超声心动图新进展

张赫展，付启航，张冬雪，徐卉*

（吉林大学第一医院心脏超声科，吉林长春130021)

摘要：轻链型心脏淀粉样变（Amyioidlight一chaincardiacamyloidosis，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。

关键词：轻链型心脏淀粉样变；超声心动图；诊断

D0I:10.3969/j.issn.1007-4287.2025.03.018

Newprogressinechocardiographyforthediagnosisofamyioidlight-chaincardiacamyloidosisZHANGHezhan,FU

Qihang,ZHANGDongzue,XUHui*.(DepartmentofCardiacUltrasound,TheFirstHospitalofJilinUniversi-

ty,Changchun130021,China)

*Correspondingauthor:XUHui,x_hui@jlu.edu.cn

Abstract:Amyloidlight-chaincardiacamyloidosis(AL-CA)isaninfiltrativerestrictivecardiomyopathy.Itis

causedbytheabnormalproliferationofplasmacellsthatproducemonoclonalimmunoglobulinlightchains.Thesemis-

foldedlightchainsforminsolubleamyloidfibrilsanddepositintheinterstitiumofmyocardialcellsandaroundblood

vessels,leadingtodisordersofcardiacstructureandfunction.Itsclinicalmanifestationsarenon-specific,thedisease

progressesrapidly,andtheprognosisisextremelypoor.Therefore,earlydiagnosisandpromptinitiationoftreatment

areofgreatsignificance.Echocardiography,asthepreferredimagingmethodfordiagnosingAL-CA,providesmoresen-

sitiveindicatorsforclinicalpracticethroughtheassistanceofspeckletrackingtechnology,pressure-strainloopanalysis,

multi-parameterassessment,derivativeparameters,andartificialintelligence,significantlyimprovingthe