Zhang Xiao-Tong, Liu Ying-Long, Ruan Ying-Mao, Yu Cun-Tao, Liu Lei
Center of Pediatric Cardiac Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences Peking Union Medical College, Beijing 100037, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2008 Jun;10(3):311-4.
The development of pulmonary vascular bed is strongly flow-dependent. Abnormal pulmonary blood flow leads to pulmonary pathological changes. This study aimed to observe the pathological changes of small pulmonary arteries and alveoli in complex congenital heart defect with diminished pulmonary blood flow but without aortopulmonary collateral artery (APCA) and patent ductus arteriosus (PDA) in infants and young children.
Autopsy pulmonary specimens obtained from 5 children who died of non-cardiovascular diseases were used as the control group (age: 4-18 months). Fifty-six children (age: 4-36 months) with complex congenital heart defect with diminished pulmonary blood flow but without APCA and PDA served as the study group, including 34 cases of tetralogy of Fallot, 7 cases of double outlet right ventricle with pulmonary stenosis, 9 cases of single ventricle with pulmonary stenosis, 4 cases of tricuspid atresia with pulmonary stenosis and 2 cases of complete atrioventricular septal defect with pulmonary stenosis. Pulmonary specimen sections were stained by hematoxylin-eosin and Weigert-Van Gieson. Percentage of media thickness (MT%), percentage of media section area (MS%), number of small arterial per square centimeter (APSC), mean alveolar number (MAN), mean linear intercept (MLI), proportion of parenchyma area in total area (PPA%) and alveolar to small arterial ratio per unit area (AAR) were measured by morphologic quantitative analysis.
MT% (10.93+/-2.87% vs 15.08+/-2.51%), MS% (18.97+/-5.56% vs 25.04+/-3.87%) and APSC (202.43+/-67.45 vs 441.69+/-65.29) decreased significantly in the study group compared with the control group (P<0.01). The internal diameter of small pulmonary artery (80.26+/-21.57 microm vs 58.53+/-10.29 microm; P<0.05), AAR (46.59+/-14.43 vs 34.46+/-4.98; P<0.01) and MLI (144.98+/-44.87 microm vs 108.39+/-20.76 microm; P<0.05) increased significantly compared with the control group.
The media of small pulmonary arteries becomes thinner, the lumen of small pulmonary arteries becomes larger, and the number of small arterial per square centimeter and the mean alveolar number are reduced in infants and young children with complex congenital heart defect with diminished pulmonary blood flow but without APCA and PDA.
肺血管床的发育强烈依赖血流。异常的肺血流会导致肺部病理改变。本研究旨在观察婴幼儿中肺血流量减少但无主肺动脉侧支动脉（APCA）及动脉导管未闭（PDA）的复杂先天性心脏病患儿的小肺动脉及肺泡的病理变化。
选取5例死于非心血管疾病的儿童的尸检肺标本作为对照组（年龄：4 - 18个月）。56例（年龄：4 - 36个月）肺血流量减少但无APCA及PDA的复杂先天性心脏病患儿作为研究组，包括34例法洛四联症、7例右心室双出口伴肺动脉狭窄、9例单心室伴肺动脉狭窄、4例三尖瓣闭锁伴肺动脉狭窄及2例完全性房室间隔缺损伴肺动脉狭窄。肺标本切片进行苏木精 - 伊红及魏格特 - 范吉森染色。通过形态学定量分析测量中膜厚度百分比（MT%）、中膜截面积百分比（MS%）、每平方厘米小动脉数量（APSC）、平均肺泡数（MAN）、平均线性截距（MLI）、实质面积占总面积的比例（PPA%）及单位面积肺泡与小动脉比值（AAR）。
与对照组相比，研究组的MT%（10.93±2.87% 对 15.08±2.51%）、MS%（18.97±5.56% 对 25.04±3.87%）及APSC（202.43±67.45 对 441.69±65.29）显著降低（P<0.01）。小肺动脉内径（80.26±21.57微米 对 58.53±10.29微米；P<0.05）、AAR（46.59±14.43 对 34.46±4.98；P<0.01）及MLI（144.98±44.87微米 对 108.39±20.76微米；P<0.05）与对照组相比显著增加。
肺血流量减少但无APCA及PDA的复杂先天性心脏病婴幼儿的小肺动脉中膜变薄，小肺动脉管腔增大，每平方厘米小动脉数量及平均肺泡数减少。
