先天性心脏病伴肺动脉高压内皮素-1和内皮型一氧化氮合酶的表达  

Endothelin-1 and eNOS expression in pulmonary aterioles of patients with congenital heart diseases with pulmonary hypertension

作　　者：孔霞[1] 项如莲[2] 胡型锑[3] 高宝辉[4] 陈其[2] 方周溪[5] 

机构地区：[1]南京医科大学附属南京第一医院儿科,南京210006 [2]温州医学院附属育英儿童医院心血管科,浙江温州325027 [3]温州医学院附属二院心胸外科,浙江温州325027 [4]温州医学院附属二院病理科,浙江温州325027 [5]温州医学院电镜室,浙江温州325027

出　　处：《中国病理生理杂志》2005年第11期2137-2141,共5页Chinese Journal of Pathophysiology

基　　金：温州市科委资助项目(No.WKW01039)

摘　　要：目的研究内皮素-1(ET-1)及内皮型一氧化氮合酶(eNOS)对先天性心脏病伴肺动脉高压(PH)的调控作用。方法将40例先天性心脏病患儿按其肺动脉收缩压分为中重度PH组12例、轻度PH组14例、无PH组14例;另选取10例非心脏疾患手术病人10例作为对照组。采用放射免疫、硝酸还原酶、透射电镜、免疫组化等方法,观察各组患儿肺细小动脉超微结构、血ET-1、一氧化氮(NO)浓度及肺细小动脉ET-1、eNOS蛋白表达的变化。结果①血浆ET-1水平,中重度PH组、轻度PH组明显高于无PH组(P<0.01);血清NO浓度中重度PH组、轻度PH组明显低于无PH组(P<0.01)。②电镜下,中重度PH组肺细小动脉中膜平滑肌增生,外膜胶原纤维密集;轻度PH组肺细小动脉中膜平滑肌细胞和外膜胶原纤维增生轻于中重度PH组;无PH组肺细小动脉结构改变不明显。③免疫组化显示中重度PH组、轻度PH组肺细小动脉ET-1平均吸光度值显著高于无PH组(P<0.01);而中重度PH组、轻度PH组肺细小动脉eNOS平均吸光度值显著低于无PH组(P<0.01)。结论ET-1、eNOS可能参与了先天性心脏病PH的形成与肺血管结构重建。AIM： To clarify the role of eNOS and ET - 1 in development of pulmonary hypertension （PH） associated with congenital heart diseases. METHODS： 40 patients were randomly divided into three groups： severe or moderate PH group （group A, 12 cases）, slight PH group （group B, 14 cases） and normal group （group C, 14 cases）. ET- 1 and eNOS were examined by using the technique of immunohistochemistry. RESULTS： ① Plasma ET- 1 concentration was significandy higher in group A and B than that in group C （P 〈 0.01 ）. Serum NO concentration in group A and B was significantly lower than that in group C （ P 〈 0.01 ）. ② The proliferation of medial smooth muscle cells and collagenous fibers of pulmonary arteriole in patients with PH were observed by electron microscopy. ③ ET- 1 in pulmonary arteriole was significandy higher in group A and group B than that in group C （ P 〈 0.01 ）. eNOS in pulmonary, arteriole was significantly lower in group A and group B than that in group C （ P 〈 0.01）. CONCLUSION： ET- 1 and eNOS may play an important role in the pathogenesis of PH.

关 键 词：内皮缩血管肽-1 一氧化氮 一氧化氮合酶 心脏病 先天性 肺动脉高压 

分 类 号：R363[医药卫生—病理学]