心脏淀粉样变性 - health120yearscom.pdfVIP

# 896# 2007 28 6 A dv Cardiovasc D is, N ovem ber 2007, Vol. 28, N o. 6 刘紫燕 综述 邱原刚 审校 ( 浙江大学医学院附属第一医院心血管内科, 浙江 杭州 310003) Cardiac Am yloidosis L IU Z -i yan, Q IU Y uan-gang (D ep artmen t of Card iovascularD iseases, The F irs tAff iliated H osp ita ,l M edical school of Zhej iang Un iversity, H ang hou 3 10003, China ) : 1004-3934 ( 2007) 06-0 896-04 : R364. 2 : A : 淀粉样变 是以不可溶 的淀粉样物质沉积于器官或组织的细胞外区, 导致相应的器官或组织功能障碍为特征的一 组疾病心脏是淀粉样变 常累及的器官心脏淀粉样变 临床上通常按其病因分为原发 家族 老年 继发 及透析相关 五种类型其最常见的临床表现为限制 心肌病和顽固 心力衰竭, 有时伴有传导阻滞心肌活检可以确定诊断, 近年来随着 心外组织取样和显像技术的发展已大大减少了心内膜心肌活检的必要尽管治疗上有所改进, 但心脏淀粉样变 患者预后仍然较 差, 主要取决于原有疾病类型 : ; A bstrac t: Am y o idosis is a group of d iseases a character ized by depos ition o f inso ub e am y o id f ibri s in the extrace u ar spaces of o rgans and tissues, w ith resu ting dysfunction of o rgans. T he card iovascu ar system is a comm on target o f amy o idos is. In c in ica practice, ca rdiac am y o idosis is c assif ied as prmi ary, fam i ia, sen i e, secondary and hem odia ysis- assoc ia ted. T he most common presentations are re- strictive cardiom yopathy, and c inica y intractab e heart fa iure w ith or w ithou t conduction system d isturbances. H ow ever, endom yoca rd ia b iop sy is the de finitive d iagnostic, recent deve opm ents in mi ag ing techniques and ex tracard iac tissue sam p ing have m in mi ized the need fo r invasive endomyo cardia b iop sy fo r amy o ido sis. D esp ite advances in treatm ent, the prognosis for patients w ith am y o idosis is sti poo r and depends on the under y ing d isease type. H erein, w e present new ins ights and recen t advances in ca rdiovascu ar am y o idosis. K ey w ord s: amy o idasis; cardiom yopathy ( am y oidosis ) , ,