心脏淀粉样变性诊断和治疗的研究进展  

Advances in Diagnosis and Treatment of Cardiac Amyloidosis

作　　者：刘燕雨 耿晨雨 李木源 王健[2] LIU Yanyu;GENG Chenyu;LI Muyuan;WANG Jian(School of Clinical Medicine,Weifang Medical University,Weifang 261041,China;Department of Cardiology,Weifang People′s Hospital,Weifang 261044,China)

机构地区：[1]潍坊医学院临床医学院,山东潍坊261041 [2]潍坊市人民医院心内科,山东潍坊261044

出　　处：《医学综述》2023年第24期5696-5703,共8页Medical Recapitulate

摘　　要：心脏淀粉样变性(CA)是一种由淀粉样蛋白堆积形成的限制性心肌病,其主要表现为心脏细胞外间隙错误折叠的蛋白质片段沉积,可导致心力衰竭、生活质量下降甚至死亡。根据浸润的蛋白性质不同,CA分为免疫球蛋白轻链型CA和转甲状腺素蛋白型CA(ATTR-CA)两种亚型。而根据是否存在转甲状腺素基因的突变,ATTR-CA又分为遗传型和野生型两种。既往关于CA的诊断明显不足,且越来越多的研究认为其是射血分数保留性心力衰竭的病因。随着诊断技术的进步,使用核素显像技术可以准确诊断ATTR-CA而无须心脏活检,而新型有效治疗方法的提出,包括转甲状腺素稳定剂和沉默剂等,改变了该疾病的治疗现状。Cardiac amyloidosis(CA)is a restrictive cardiomyopathy formed by amylo-id accumulation that manifests primarily as deposition of protein fragments that are misfolded in the heart′s extracellular space,which can lead to heart failure,reduced quality of life,and even death.According to the nature of the infiltrated proteins,CA can be categorized into two subtypes:immunoglobulin light chain CA and transthyretin CA(ATTR-CA).And based on whether there is a mutation in the thyroxine gene,ATTR-CA can be categorized into two types:genetic type and wild-type.The diagnosis of CA in the past has been significantly insufficient,and more and more people believe that it is the cause of ejaculatory fraction preserving heart failure.With the advancement of diagnostic technology,the use of nuclear imaging can accurately diagnose ATTR-CA without cardiac biopsy,and the introduction of new effective treatments,including transthyroxine stabilizers and silencing agents,have changed the treatment status quo of the disease.

关 键 词：心脏淀粉样变性 限制性心肌病 心力衰竭 

分 类 号：R541[医药卫生—心血管疾病]