左向右分流先天性心脏病和大动脉转位并室间隔缺损肺血管病变的对比研究

Comparison of pulmonary vascular disease in congenital heart disease with left-to-right shunts to transposition of great arteries with ventricular septal defect

目的比较伴有重度肺动脉高压左向右分流先天性心脏病和大动脉转位肺血管病变。方法用诊断性治疗所选患者进行根治手术,术中取肺活检。组I(n=38)包括伴有重度肺动脉高压的左向右分流先天性心脏病患者;组II(n=11)包括>6个月伴有重度肺动脉高压的大动脉转位并室间隔缺损患者;组III(n=6)从心肺循环正常的尸体取肺组织作对照。肺组织进行H-E分级及定量分析。结果组I(n=38)伴有重度肺动脉高压的左向右分流先天性心脏病患者,31例(31/38)为H-E I级,3例(3/38)为H-E II级,3例(3/38)为H-E III级,仅1例(1/38)为H-E IV级伴有丛样病变。组II(n=11)大龄完全性大动脉转位并室间隔缺损和Taussig-Bing畸形患者,7例(7/11)为H-E I级,2例(2/11)为H-E II级,1例(1/11)为H-E III级,仅1例(1/11)为H-E IV级伴有丛样病变。组I和组II的中膜厚度百分比(%MT)和中膜面积百分比(%MS)较对照组(组III)明显升高。组II的中膜面积百分比(%MS)较组I明显升高。中膜厚度百分比(%MT)和肺小动脉密度(APSC)在左向右分流先天性心脏病患者(组I)和完全性大动脉转位并室间隔缺损和Taussig-Bing畸形患者(组II)间差异无统计学意义。结论采用诊断性治疗-根治手术策略选择的大部分患者的肺血管病变通常仍是可逆的,伴有重度肺动脉高压的左向右分流先天性心脏病和大动脉转位肺血管病变相似。

[ Objective ] To examine the nature of the pulmonary arterial vessels in these selective patients and compare pulmonary vascular disease （PVD） in left-to-right shunt CHD to transposition of great arteries with ventricular septal defect. [Methods] Lung biopsy specimens were obtained from patients selected using a Diagnostic-treatment-and-Repair strategy. Group Ⅰ （n =38） comprised patients with left-to-right shunt CHD and severe PH; Group Ⅱ （n =11） consisted of older patients with transposition of great arteries with ventrieular septal defect and severe pH. Autopsy cases of normal cardiovascular system and pulmonary circulation were studied inGroup Ⅲ（n =6）. The nature of the pulmonary arterial vessel was determined by Heath-Edwards classification system. All specimens were quantitatively analyzed. [Results] In Group Ⅰ （n =38）, 31 showed gradeⅠ change, 3 showed grade Ⅱ change, 3 owed gradeⅢ change and only 1 showed grade Ⅳ change. In Group Ⅱ （n =11）, 7 showed grade Ⅰ change, 2 showed grade Ⅱ change, 1 showed grade Ⅲ change and only 1 showed grade Ⅳ change. Follow-up data showed the reversal of PVD in these 2 with plexiform lesions. Percentage media wall thickness, percentage media wall area and arteriole density in Group Ⅰ and Group Ⅱ were significantly higher than those in Group Ⅲ.Percentage media wall area in Group Ⅱ was significantly higher than that in Group Ⅰ; significant differences of percentage media wall thickness and arteriole density could not be found betweenGroup Ⅰ and Group Ⅱ. [ Conclusions ] PVD in these selected patients with CHD and severe PH using a Diagnostic-treatment-and-Repair strategy is generally reversible and the changes of PVD in patients with transposition of great arteries with ventricular septal defect are similar to those in left-to-right shunt CHD.