OBJECTIVE:To assess the pathologic markers for evaluation of reversibility in pulmonary hypertension (PAH) related to congenital heart disease.METHODS:Twenty-eight patients with congenital heart disease complicated by PAH were subclassified into reversible pulmonary hypertension (RPAH) and irreversible pulmonary hypertension (IPAH), according to post-operative mean pulmonary artery pressure (MPAP). Pulmonary vascular lesion was analyzed according to Ruan's method. Mean medium thickness percent, mean medium area percent and pulmonary arteriolar density were measured by quantitative morphometry. Immunohistochemical study for transgelin and filamin A was carried out.RESULTS:Amongst the 28 cases studied, 24 were RPAH and 4 were IPAH. Of the 24 patients with RPAH, 13 (54.2%, 13/24) had pulmonary vascular lesion of grade 0, 9 (37.5%, 9/24) of grade 1 and 2 (8.3%, 2/24) of grade 2. Of the 4 patients with IPAH, 1 had lesion of grade 1, 1 of grade 2 and 2 of grade 3. Both preoperative and postoperative MPAP were higher in IPAH patients than that in RPAH patients[(53.3±23.4) mmHg versus (34.1±12.7) mmHg, P=0.020 and (35.0±8.8) mmHg versus (17.8±3.9) mmHg, P<0.01]. Compared to patients with pulmonary vascular lesion of grades 0 and 1, the preoperative MPAP in patients with grades 2 and 3 showed no significant difference, but the postoperative MPAP was higher (P<0.05 or 0.01). Compared to control group, mean medium thickness percent and mean medium area percent were significantly higher in RPAH and IPAH categories (12.0±3.5, 8.5±2.0 versus 5.7±1.0, P<0.01 and 55.8±11.1, 49.0±9.4 versus 34.0±5.5, P<0.01). Mean medium thickness percent was significantly higher in IPAP group than that in RPAH group (12.0±3.5 versus 8.5±2.0, P=0.001). Correlation analysis demonstrated that mean medium thickness percent and mean medium area percent had positive correlation with preoperative and postoperative MPAP. There was no correlation between grading of pulmonary vascular lesion and reversibility. Transgelin and filamin A had stronger staining in pulmonary vascular smooth muscle cells in IPAH than those in RPAH and controls(P<0.05).CONCLUSIONS:Pathologic assessment of lung biopsy remains the gold standard for evaluation of the reversibility in PAH related to congenital heart disease. Mean medium thickness percent, mean medium area percent and immunoreactivity for transgelin and filamin A are useful parameters.

目的：通过观察先天性心脏病相关性肺动脉高压肺活检组织内肺血管组织学改变，探讨肺血管病理学变化在判断肺动脉高压可逆性中的应用价值。

Objective: To analyze the demography,clinical features and hemodynamics in patients with different types of pulmonary arterial hypertension(PAH).Methods: We retrospectively studied 551 PAH patients who were treated at 31 medical centers in China from May 2007 to October 2010.The diagnosis was confirmed by right heart catheterization,and 386(70.1%) patients with female gender.According to the standard of American Heart Association,there were 4 types of PAH including idiopathic PAH(IPAH group),n=150,PAH associated with congenital heart disease(CHD-PAH group),n=273,PAH associated with connective tissue disease(CTD-PAH group),n=64,and chronic thromboembolic PAH(CTEPH),n=64.The demography,WHO functional classification(WHO-FC) and hemodynamics were collected and compared among different PAH groups.Results: CHD-PAH group had the largest number of patients(273/551,49.5%),with the youngest age and lowest BMI.While CTEPH group was opposite from CHD-PAH group in age and BMI,it had the highest ratio of female/male gender.Overall,fatigue(76.4%) and breathlessness(72.2%) were the most common symptoms in PAH patients.CTD-PAH group had the shortest 6 minutes walking distance(6MWD),and CHD-PAH group had the longest 6MWD.There were more patients with WHO-FC II in CHD-PAH group,and the mean pulmonary arterial pressure(mPAP),pulmonary vascular resistance(PVR) in CHD-PAH group was obviously higher than those in the other 3 groups,P < 0.01 respectively.Conclusion: With confirmed diagnosis,more IPAH patients were already at the severe condition.CHD-PAH was the most common type and it more happened in young and middle aged female gender,it had the highest mPAP and PVP,while rather good WHO-FC,which implies that the cardiac function may not be parallel to mPAP and PVR in those patients.

Left to right shunt congenital heart disease results in a consistent increase of blood in pulmonary vascular bed,thus leading to the constriction and remodeling of pulmonary vasculature and an increased pulmonary pressure,which may affect the surgical effect of patients.At early stage,pulmonary vascular disease is still reversible,which guarantees a good prognosis of patients.However,when the pulmonary vascular disease becomes severe,surgical treatment can not benefit patients and may even lead to a higher mortality.The severity and reversibility of pulmonary vasculature is not correlated with the pulmonary resistance or the pulmonary pressure.How to select patients for surgeries remains a clinical controversy confronted with many doctors.This review will provides the latest progress in basic and clinical researches to determine the operative indications for selecting patients with pulmonary hypertension associated with congenital heart disease for surgical treatment.

目的：探讨特发性肺动脉高压患者生长分化因子15（Growth differentiation factor 15，GDF-15）、干扰素γ诱导蛋白10(IP-10/CXCL10)、间质细胞源性因子(SDF-1a/CXCL12)、CXC趋化因子配体16（CXCL16）的水平以及与患者临床特征的相关性。<br>　　方法：连续入选2010-11至2012-05入住阜外心血管病医院的特发性肺动脉高压的患者以及与之年龄、性别相匹配的健康人作为对照。检测两组患者血浆各因子水平，比较两组间各因子水平差异；记录患者组右心导管检查、心脏超声检查、常规实验室检查等基线数据，对基线资料进行相关性分析。