李德剑,徐超,冯建飞,王明权,宋东邦,曹勇,张大东,王伟民

• 1:无锡明慈心血管病医院心内科

摘要(Abstract)：

心脏淀粉样变是蛋白质分子异常折叠所致的淀粉样物质沉积在心肌细胞外所致，导致心肌细胞代谢、钙离子转运、受体调节的改变以及心肌细胞水肿；早期表现为心脏舒张功能障碍，后期可逐渐发展成为限制性心肌病，最终发展为心力衰竭。临床主要分为轻链型和转甲状腺素蛋白型，约占95%左右，前者预后差，进一步明确诊断和及时诊治至关重要。本文报道1例54岁女性患者，因“反复活动性气喘、气短1年余，加重3 d”入无锡明慈心血管病医院。超声心动图和心电图提示心脏淀粉样变可能，初步诊断心脏淀粉样变。后在心腔内超声引导下联合可调弯鞘管成功行心内膜下心肌活检，成功抓取不同部位心肌组织5块，未见并发症。心肌组织病理确诊为心脏淀粉样变、λ轻链型。

关键词(KeyWords)： 心脏淀粉样变;心内膜心肌活检;心腔内超声;可调弯鞘管

Abstract:

Keywords:

基金项目(Foundation):

作者(Author): 李德剑,徐超,冯建飞,王明权,宋东邦,曹勇,张大东,王伟民

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