Atrial remodeling and metabolic dysfunction in idiopathic isolated fibrotic atrial cardiomyopathy.
Atrial remodeling and metabolic dysfunction in idiopathic isolated fibrotic atrial cardiomyopathy.
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特发性孤立性纤维化心房心肌病的心房重塑和代谢功能障碍
DOI：
10.1016/j.ijcard.2018.04.080
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发表时间：
2018-08-15
影响因子：
3.5
通讯作者：
Chen M
中科院分区：
文献类型：
--
作者：
Cui C;Jiang X;Ju W;Wang J;Wang D;Sun Z;Chen M
特发性孤立性纤维化性心房心肌病（IIF-ACM）是一种以心房纤维化为特征的新型心肌病亚型，不累及心室心肌，并与显著的心房性心动过速相关。其发病机制尚不清楚。3例IIF-ACM患者通过手术干预获得心房样本。对照样本为先天性心脏病和正常窦性心律患者的3例心房活检，性别、年龄和基本临床特征相匹配。通过比较组织学、免疫荧光染色、电镜和蛋白质组学分析，探讨IIF-ACM独特的发病机制。与对照心房相比，IIF-ACM心房显示肌原纤维紊乱、深度纤维化和线粒体损伤。蛋白质组学分析确定代谢途径是IIF-ACM最深刻的变化。我们的研究表明，心房心肌代谢变化引起线粒体氧化应激和潜在的细胞损伤，从而进一步导致心房纤维化和肌原纤维紊乱，这是IIF-ACM的特征表型。
Idiopathic isolated fibrotic atrial cardiomyopathy (IIF-ACM) is a novel subtype of cardiomyopathy characterized by atrial fibrosis that does not involve the ventricular myocardium and is associated with significant atrial tachyarrhythmia. The mechanisms underlying its pathogenesis are unknown. Atrium samples were obtained from 3 patients with IIF-ACM via surgical intervention. Control samples were consisted of 3 atrium biopsies from patients with congenital heart disease and normal sinus rhythm, matched for gender, age and basic clinical characteristics. Comparative histology, immunofluorescence staining, electron microscopy and proteomics analyses were carried out to explore the unique pathogenesis of IIF-ACM. IIF-ACM atria displayed disordered myofibrils, profound fibrosis and mitochondrial damages compared to the control atria. Proteomics profiling identified metabolic pathways as the most profound changes in IIF-ACM. Our study suggested that metabolic changes in the atrial myocardium caused mitochondrial oxidative stress and potential cell damage, which further led to atrial fibrosis and myofibril disorganization, the characteristic phenotype of IIF-ACM.
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影响因子：
--
作者：
Li, Qing;Cui, He-He;Xu, Jun
通讯作者：
Xu, Jun
影响因子：
10.8
作者：
Ausma, J;Coumans, WA;Borgers, M
通讯作者：
Borgers, M
影响因子：
24
作者：
Mayr, Manuel;Yusuf, Shamil;Camm, A. John
通讯作者：
Camm, A. John
影响因子：
5.3
作者：
Kim SM;Hwang IK;Yoo DY;Eum WS;Kim DW;Shin MJ;Ahn EH;Jo HS;Ryu EJ;Yong JI;Cho SW;Kwon OS;Lee KW;Cho YS;Han KH;Park J;Choi SY
通讯作者：
Choi SY
影响因子：
20.1
作者：
Li, JF;Patel, VV;Radice, GL
通讯作者：
Radice, GL