Objective: To study if there is an aryl-hydrocarbon receptor (AHR) expression in patients of pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) and to explore if the amount of AHR expression related to pulmonary vascular remodeling. Methods:A total of 32 CHD-PAH patients diagnosed by echocardiography and right heart catheterization for surgical repair were enrolled, and the lung tissue biopsy was performed during the operation. The pulmonaryAHR was detected by immunolfuorescence assay, the ratios of vessel wall area/total area (WA/TA) and vessel wall thickness/vessel external diameter (WD/TD) of small pulmonary arteries were calculated with the imaging software, the mRNA expression of AHR, hypoxia-inducible factor-1α (HIF-1α), aryl-hydrocarbon receptor nuclear translocator (ARNT) and vascular endothelial growth factor (VEGF) were examined by RT-PCR. In addition, blood level of AHR was measured by ELISA. Results: There was AHR expression in pulmonary tissue in all 32 patients. And AHR mRNA expressions were positively related to mPAP (r=0.809,P<0.001), WA/TA (r=0.723,P<0.001), WD/TD (r=0.746,P<0.001); and positively related to mRNA expressions of HIF-1α (r=0.889,P<0.001), ARNT (r=0.738,P<0.001), VEGF (r=0.822,P<0.001). Pulmonary tissue VEGF mRNA expressions were positively related to mPAP (r=0.739,P<0.001), WD/TD (r=0.702,P<0.001) and WA/TA (r=0.657,P<0.001). Blood levels of AHR were positively related to mPAP (r=0.754,P<0.001), WD/TD (r=0.754, P<0.001) and WA/TA (r=0.739,P<0.001). Conclusion: AHR might be involved in pulmonary vascular remodeling in CHD-PAHpatients.%目的：了解先天性心脏病相关性肺动脉高压（CHD-PAH）患者肺组织是否有芳香烃受体（AHR）表达，同时探讨AHR表达量与肺血管重构是否相关。 方法：入选超声心动图和右心导管检查确诊的预行外科修补术的CHD-PAH患者32例。术中行肺组织活检。采用组织免疫荧光检测肺组织标本AHR表达情况，运用图像分析软件计算肺小血管的管壁面积/管总面积（WA/TA）和管壁厚/管外径（WD/TD）2个相对比值，采用实时荧光定量多聚酶链反应（Real-Time PCR）方法检测AHR mRNA、缺氧诱导因子-1α（HIF-1α）mRNA、芳香烃受体核转位蛋白（ARNT）mRNA和血管内皮生长因子（VEGF）mRNA表达情况。此外，术前采集患者外周血，采用酶联免疫吸附法（ELISA）测定血清AHR浓度。 结果：32例患者肺组织中均有AHR表达。且肺组织AHR mRNA表达量与平均肺动脉压（mPAP）（r=0.809， P<0.001）、肺小血管WA/TA（r=0.723，P<0.001）和WD/TD（r=0.746，P<0.001）、HIF-1αmRNA表达量（r=0.889， P<0.001）、ARNT mRNA表达量（r=0.738，P<0.001）、VEGF mRNA表达量（r=0.822，P<0.001）呈正相关。肺组织VEGF mRNA表达量与mPAP（r=0.739，P<0.001）、WD/TD （r=0.702，P<0.001）和WA/TA （r=0.657，P<0.001）呈正相关。血清AHR浓度与mPAP（r=0.754，P<0.001）、WD/TD（r=0.754，P<0.001）和WA/TA （r=0.739，P<0.001）呈正相关。 结论：AHR可能参与CHD-PAH患者的肺血管重构。
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