肺血减少型先天性心脏病远端肺血管发育的研究

目的：探讨肺血减少型先天性心脏病患者远端肺血管发育的评估手段及指标。方法：对47例肺血减少型先天性心脏病患者（A组）进行3DCE—MRA及超声心动和术中测量，将MRA测量结果与术前心动超声及术中测量值进行组内比较；选择其中15例（B组）和另外5例同年龄组原发病为非心源性且对心脏及肺血管发育无直接影响的开胸手术患者（C组）进行肺组织活检，形态半定量测量肺细小动脉平均中膜厚度百分比（MT％）和中膜面积百分比（MS％）、单位面积肺细小动脉数目（APSC）、单位面积平均肺泡数（MAN）、单位面积平均肺泡数／肺细小动脉数（AAR）。将B组与C组病理学检测数据进行比对研究。在B组中将MRA可显示的肺血管分枝级数与病理学检测数据进行相关性分析。结果：A组内比较，3DCE-MRA测量值与术中测量值及超声心动测得值之间差异统计学意义（P〉0．05）；B组与C组比较，MT％和MS％差异没有统计学意义（P〉0．05），而APSC、MAN、AAR差异明显（P〈0．01）；B组内肺动脉分枝级数与APSC、MAN呈正相关（P〈0．01），与AAR呈负相关（P〈0．01），与Mc Goon比值和PAI呈正相关（P〈0.05）。结论．3DCE—MRA对于肺血管发育的评估具有较高的实用性和准确性，基本可以替代有创的心导管检查，其所测量的Mc Goon比值和PAI指数和显示的肺动脉分枝级数对手术方式的选择，围术期并发症的预判有一定的指导意义。

The Study on the Development of Distal Pulmonary Vessels in Congenital Hypoplasia of Pulmonary Artery

Objective ：To investigate the means and index for evaluating the development of distal pulmonary vessels in congenital hypoplasia of pulmonary artery. Methods：Forty seven patients with congenital hypoplasia of pulmonary artery （group A） underwent 3D contrast-enhanced magnetic resonance angiography （3D CE-MRA） and two-dimensional echocardiography. Lung tissues were taken from 5 patients who suffered from thoracotomy owing to other diseases with normal heart or lung（ group C） and 15 patients （group B ） in group A, and prepared for pathological examination. The semi-quantitative analysis was employed to detect the values of percentage of media thickness（ MT% ） , percentage of media section area（ MS% ） , numbers of pulmonary small artery per square centimeter（ APSC ） , mean alveolar numbers （MAN） and alveolar/small arterial ratio per unit area（AAR）. The correlation between the grading of pulmonary artery showed by magnetic resonance angiography （MRA） and the pathological findings of lung biopsies was analyzed in group B. The two-sample t-test wasapplied to compare the pathological findings between group B and group C. Results：There was not obvious difference in the findings measured by 3D CE-MRA, two-dimensional echocardiography and pathological examination, in group A（P 〉0.05）. The values of MT% and MS% in group B were similar to those in group C（P 〉0.05）, however, the levels of APSC, MAN and AAR were obviously different between the two groups （P 〈 0.01 ）. The grading numbers of pulmonary artery was positively related to the levels of APSC and MAN （ P 〈 0.01 ）, MCI and PAl （ P 〈 0. 05 ）, however negatively related to the level of AAR （P 〈 0. 01 ）. Conelusions：3D CE-MRA is a very accurate and practical method for evaluating the condition of pulmonary vessels, could substitute the traumatic cardiac catheterization. The McI and PAI detected by 3D CE-MRA and grading numbers of pulmonary artery showed by 3D CE-MRA could provide evidence for choosing modus operandi and predicting the perioperative complications.