摘要
目的 探讨左向右分流型先天性心脏病患儿肺组织的病理形态学改变、超微结构改变与肺动脉高压 (pulmonaryhy pertension ,PH)形成的关系。方法 应用光镜、电镜、组织化学染色 ,对 4 1例先心病患儿肺组织及肺各级小动脉进行研究、分析。结果 伴有PH的肺组织各级小动脉 (包括部分肌型及肌型动脉 )数量增加、管壁增厚、管腔狭窄 ,尤以中重度PH患者显著 ,主要表现血管中膜平滑肌 (SMC)和内皮细胞 (EC)增生 ,内弹力膜增厚 ,外膜胶原纤维增多。先天性心脏病患儿肺组织内无肌型小动脉肌化、部分肌型及肌型小动脉数量增加 ,随着PH的增高 ,差异有显著性 (P <0 0 1)。超微结构改变包括 :①肺泡隔纤维化逐渐明显 ,胶原纤维增生 ;②小动脉中膜SMC层数明显增加 ,SMC细胞面积增大 ,核染色质增粗 ,细胞间间隙明显增宽 ,血管外膜胶原纤维高度密集 ;③小动脉血管内皮细胞增生呈高柱状 ,细胞线粒体肿胀、空泡化 ,吞饮小泡多见 ;④毛细血管充血、瘀血 ,基膜明显增厚。结论 先心病引起缺氧导致肺血管重建 ,使肺血管阻力增加 ,是PH形成的关键因素 ,肺毛细血管的超微结构病变所致微循环障碍更加促进了PH的发生、发展。
Purpose To investigate the relationship among the formation of pulmonary hypertension (PH) and the pathomorphologic and ultrastructural changes in the lung biopsy of child congenital heart defects (CHD). Methods Optical microscope, electronic microscope and histochemistry staining were used in 41 cases of CHD to investigate and analyze the pathological changes of the pulmonary tissues and pulmonary arterioles of all levels. Results In the PH group, the number of the pulmonary arterioles of all levels (including partial myocytic arterioles and myocytic one) increased, the arteriolar walls thickened and the arteriolar lumen strictured, especially in the moderate-severe PH patients. Its main changes were proliferation of the smooth muscular cells (SMC) and the endothelial cells (EC) of the tunica media of the blood vessels, thickening of the internal elastic membrane and increasing of the collagenous fibers in the adventitia. No muscularization of the partial-myocytic arterioles was seen, but the numbers of the partial-myocytic arterioles and the myocytic one increased in the pulmonary tissue of CHD children. The difference was very significant (P<0.01) with the increasing of PH. The ultrastructural changes were as follows: ① Fibrosis of the alveolar septum was gradually conspicuous and collagen fibers were proliferated; ② The layers of SMC in the tunica media of arterioles were predominantly increased, the area of SMC augmented, nuclear chromatin widened, intercellular space obviously broadened and collagen fibers of the adventitia were highly dense; ③ Endothelial cells of the arterioles proliferated as tall column, swelling, vacuolization and pinocytosis vesicles in the cellular mitochondria were found; ④ Blood capillaries were hyperemia and congestion, and the thickness of the basilar membrane increased. Conclusions The reconstruction of the pulmonary vessels resulted from hypoxia caused by CHD and leading to an increase pulmonary arterial pressure are the key factors of the formation of PH. The disturbance of microcirculation resulted from the ultrastructural changes of the pulmonary capillaries further promotes the development of PH.
出处
《临床与实验病理学杂志》
CAS
CSCD
2004年第5期599-603,共5页
Chinese Journal of Clinical and Experimental Pathology
基金
温州市科技局基金资助 (NoS2 0 0 1A3 2 )
