不伴体-肺动脉侧支的婴幼儿肺血减少型复杂先心病肺动脉发育程度的对比观察

The comparison between pathologic changes and imaging study of pulmonary arteries of complex congenital heart defect with diminished pulmonary blood flow and without aortopulmonary collateral artery in infants

目的分析不伴体-肺动脉侧支的婴幼儿肺血减少型复杂先心病肺细小动脉结构,与左、右肺动脉的发育进行对照。方法46例不伴体-肺动脉侧支的肺血减少型复杂先心病婴幼儿为病变组。对照组为同年龄组非心、肺源性疾病死亡患儿肺组织标本5例。形态半定量测量肺细小动脉平均中膜厚度百分比(MT%)、平均中膜面积百分比(MS%)和单位面积肺细小动脉数目(APSC)。结果二组APSC的差异有统计学意义(P=0.007)。心血管造影测量的左、右肺动脉直径之间以及APSC与McGoon比值、肺动脉指数之间呈正相关性。结论不伴体-肺动脉侧支的肺血减少型复杂先心病婴幼儿肺细小动脉的发育程度与中心肺动脉的发育程度具有良好的相关性。

Objective To assess the development of small pulmonary arteries in complex congenital heart defect （CHD）with diminished pulmonary blood flow without aortopulmonary collateral artery in infants, we studied the pre-operative diameters of central pulmonary artery, McGoon ratio, and pulmonary arterial index（PAI）with ultrasonic cardiogram（UCG） and cardioangiography（CAG）. Methods Biopsy specimens were obtained from 46 infants suffering complex congenital heart defect （CHD） with diminished pulmonary blood flow and without aortopulmonary collateral arteries. The patients were managed in our hospital between January 2002 to September 2004. The age of the patients ranged from 6 to 36 months. All of the patients underwent UCG and CAG. Autopsy specimens obtained from 5 infants, aged 4-18months, who died of non-cardiovascular diseases, were assigned as the normal control group. The tissue was fixed with formalin and embedded in wax. Sections were stained with hematoxylin-eosin and Weigert＇s elastic stain with van Gieoson＇s counterstain. The percentage of media thickness （%MT）, percentage of media section area （%MS） and numbers of small arterial per square centimeter（APSC） were analyzed with an image analyzer. The diameters of left pulmonary artery（LPA） and right pulmonary artery（RPA） were measured by UCG and CAG. McGoon ratio and pulmonary arterial index were calculated according to the CAG measurements. Results There was no significant difference in %MT and %MS between the two groups （P〉0. 05）. APSC were 408. 58/cm^2 and 217.15/cm^2 respectively in two groups, P value was 0. 007. The diameter of LPA correlated with the diameter of RPA. APSC correlated with MeGoon ratio and PAl applying the regression equation： LPA （mm） = 3. 992 ＋ 0. 352 × RPA （mm）（P = 0. 015, r = 0. 387）, APSC = 133. 995 ＋ 82. 212 × McGoon ratio（P = 0. 001, r = 0. 51 ）, APSC = 151.811 ＋ 0. 691 × PAI （P = 0. 004, r = 0. 448）. APSC correlated with PAl and RPA, P value were 0. 043 and 0. 026 respectively and correlation coefficients were 0. 528 and 0. 57. The regression equation： APSC = 140. 357 ＋ 0. 462 PAI, APSC = 90. 097 ＋ 17. 589 RPA. Conclusions The extent of development of small pulmonary arteries correlates with that of the central pulmonary artery in infants suffering from complex CHD with diminished pulmonary blood flow and without aortopulmonary collateral arteries.