Fibrosis-Related Gene Expression in Single Ventricle Heart Disease.
Fibrosis-Related Gene Expression in Single Ventricle Heart Disease.
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DOI：
10.1016/j.jpeds.2017.08.055
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发表时间：
2017-12
期刊：
影响因子：
--
通讯作者：
Miyamoto SD
中科院分区：
文献类型：
--
作者：
Nakano SJ;Siomos AK;Garcia AM;Nguyen H;SooHoo M;Galambos C;Nunley K;Stauffer BL;Sucharov CC;Miyamoto SD
目的探讨右室衰竭患儿心肌纤维化及纤维化相关基因的表达。用实时定量聚合酶链式反应技术对儿童SV受试者和无失败对照的RV移植心肌进行检测。受试者分为3组：SV衰竭(SV-F，右室衰竭在I期姑息治疗前或之后)、SV未衰竭(SV-NF，一次移植后RV功能正常的婴儿)和III期(III期后Fontan或RV衰竭)。为了评估年龄和右室容量负荷相近的受试者，比较了SV-F和SV-NF，并将III期与NFRV进行了比较。对所有心脏进行组织学纤维化评估。进行Mann-Whitney检验以确定基因表达的差异。SV-F组胶原蛋白(COL1、α、COL3)表达低于SV-NF组(分别为P=0.019和P=0.035)，III期与NFRV组相当。组织金属蛋白酶抑制物(TIMPs1、3和4)在III期较NFRV下调(p=0.0047、p=0.013和p=0.013)。基质金属蛋白酶(MMP2、MMP9)在SV-NF与SV-F、III期与NFRV之间表达相似。右室衰竭患者的右室纤维化发生率(18%)与右室功能正常者(38%)相比无显著差异。纤维化不是婴幼儿室性心动过速的主要原因。还需要更多的研究来了解抗纤维化疗法是否对这一人群有益。
To evaluate fibrosis and fibrosis-related gene expression in the myocardium of pediatric single ventricle (SV) subjects with right ventricular (RV) failure. Real-time quantitative polymerase chain reaction was performed on explanted RV myocardium of pediatric SV subjects and non-failing controls. Subjects were divided into 3 groups: SV failing (SV-F, RV failure prior to or following Stage I palliation), SV non-failing (SV-NF, infants listed for primary transplant with normal RV function), and Stage III (Fontan or RV failure following Stage III). In order to evaluate subjects of similar age and RV volume loading, SV-F was compared with SV-NF and Stage III was compared with NF RV. Histologic fibrosis was assessed in all hearts. Mann-Whitney tests were performed to identify differences in gene expression. Collagen (Col1α, Col3) expression is decreased in SV-F compared with SV-NF (P = .019 and p=0.035, respectively), and is equivalent in Stage III compared with NF RV. Tissue inhibitors of metalloproteinase (TIMPs1, 3 and 4) are down-regulated in Stage III compared with NF RV (p=0.0047, p=0.013 and p=0.013, respectively). Matrix metalloproteinases (MMP2, MMP9) are similar between SV-NF and SV-F, and between Stage III and NF RV. There is no difference in the prevalence of RV fibrosis by histology in SV subjects with RV failure (18%) compared with those with normal RV function (38%). Fibrosis is not a primary contributor to RV failure in infants and young children with SV. Additional studies are required to understand whether anti-fibrotic therapies are beneficial in this population.
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影响因子：
6.4
作者：
Herpel, E;Pritsch, M;Schnabel, PA
通讯作者：
Schnabel, PA
影响因子：
3.5
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影响因子：
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通讯作者：
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作者：
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通讯作者：
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