先天性心脏病肺动脉高压肺组织超微结构的改变及临床意义  

Ultrastructural changes of pulmonary tissue in congenital heart disease with pulmonary hypertension

作　　者：莫绪明[1] 谷兴琳[1] 王敏生[2] 江家婉[3] 蔡红星[4] 王春生[2] 王振喜[1] 

机构地区：[1]南京医科大学附属南京儿童医院心胸外科,210008 [2]上海医科大学中山医院心脏外科 [3]上海医科大学电镜室 [4]徐州医学院法医教研室

出　　处：《中华小儿外科杂志》2000年第5期278-281,共4页Chinese Journal of Pediatric Surgery

基　　金：江苏省青年基金!(BQ980 0 5);江苏省卫生厅科研基金!(H9741 )

摘　　要：目的　探讨先心病肺动脉高压 (PH)患儿肺组织超微结构的改变及其临床意义。方法　 2 1例先心病患儿为 1997年 11月～ 1999年 2月间住院行心血管手术者 ,全组平均肺动脉压为 (5 8± 2 1)mmHg ,肺血管阻力 (760± 2 0 6)dyn·s·cm-5。除 4例动脉导管未闭 (PDA)行导管结扎外 ,其余患儿均在体外循环下行心内直视手术 ,于开胸后立即取肺组织在电镜下观察肺组织超微结构的变化。结果　 2 1例PH患儿肺组织均有不同程度的纤维化 ,且在毛细血管两侧不对称 ,主要集中于非气血交换侧 ,以胶原纤维增生最显著 ,弹力纤维增生次之。肺泡Ⅱ型细胞显著增多 ,其胞质内可见大量的线粒体、粗面内质网 ,板层小体增多并可见其内容物丢失。肺泡Ⅰ型细胞基膜显著增厚 ,肺小动脉平滑肌细胞增生 ,毛细血管基膜增厚。结论　重症先心病PH患儿肺组织超微结构发生明显改变 ,结合临床 ,提示对于先心病患儿宜尽早手术纠治 ,以从根本上消除形成PH的条件。Objective To study the pathological changes of pulmonary tissue of congenital heart disease (CHD) with pulmonary hypertension (PH). Methods Twenty one patients (12 males and 9 females) with CHD and PH underwent operation between November 1997 and February 1999. Their mPAP was 58±21?mmHg and mPVP was 760±206?dyn·s·cm -5 . There were 14 cases with severe PH and 7 cases with medium PH. All the patients underwent cardiac repairing with cardiopulmonary bypass (CPB) except for 4 cases of PDA ligations. Ultrastructural changes of pulmonary tissue were examined. Results Fibrosis of pulmonary tissue was asymmetrical on both sides of capillary vessels. It was more pronounced on non gas blood exchange side. The histological hyperplasia was more prominent in collagenous fiber than the elastic fiber. There was general thickening of alveolo capillary membrane. The intimal proliferation of capillary vessel and pulmonary arteries caused the narrowing of vessel lumen. Type Ⅱ alveolar cells increased considerably. There were abundant mitochondria, rough surfaced endoplasmic recticulum, and significantly increased ply bodies. The contents of most ply bodies were lost in cytoplasm of type Ⅱ alveolar cells. Basal lamina of type Ⅰ alveolar cells was thickened. Conclusions The results indicate that the ultrastructure of pulmonary tissue has changed considerably in the patients with congenital heart disease and pulmonary hypertension. To minimize PH, surgical intervention should be carried out as early as possible.

关 键 词：先天性心脏病 肺动脉高压 超微结构 肺 外科手术 

分 类 号：R725.4[医药卫生—儿科]