中文摘要：

目的探讨以ET-1、NO变化为代表的内皮细胞功能改变及Survivin在左向右分流型先天性心脏病肺动脉高压发病及肺血管重构中的作用。方法49例先心病室间隔缺损患者根据肺动脉平均压的情况分为4组。放射免疫法或比色法测定血浆内皮素（ET-1）和一氧化氮（NO）含量;检测血管管壁厚度占外径的百分比（MT%）以反映肺小血管的形态学改变。用原位缺口末端DNA标记技术,检测肺组织细胞凋亡情况。用免疫组化及Western blot检测调亡相关蛋白Survivinx的表达。结果先心病患者伴随肺动脉压力的增高,血浆ET-1水平、组织Survivinx的表达呈上升趋势,MT%逐渐增高,二者呈显著正相关;而NO水平下降、细胞凋亡减少并与MT%呈显著负相关。结论左向右分流所致肺血管内皮细胞功能及Survivin表达的变化在肺动脉高压的发生及肺血管重构中起重要作用。

英文摘要：

Objective To discuss the role of the change of endothelial function by endothelin-1 （ ET-1 ）. nitric oxide（NO） and Survivin in the development of pulmonary hypertension（PH） and vascular remodeling of congenital heart disease（CHD）. Methods 47 ventrieular septal defect（VSD） cases were divided into 4 groups according to raean pulmonary pressure.Plasma levels of ET-1 and NO were measured by radioinanunoassay and commercial available kit.The TdT-mediated dUTP-biotin end labeling staining（TUNEL） technique was used to detect nuclear DNA fragment of apoptotic cells and immunohistochemical analysis. Western blot were used to detect the expression of survlvin. Percentage media wall thickness （MT%） was measured to represent vasculare remodeling. Results The plasma levels of ET-l,expresslon of Survlvin and MT% significantly PH. ET-1,Survivin were positive correlation with MT% ,but NO and apoptotic index were negative correlation with in. Conclusion The change of endothelial function and Survivin with left to right shunt play and important role in the development of PH and pulmonary artery remodeling.