先天性心脏病合并肺高压患者血循环中vWF相关抗原的表达

The Expression of von Willebrand Factor Related Antigen in the Circulation of Patients with Congenital Heart Defect with Pulmonary Hypertension

【目的】观察先天性心脏病 (先心病 )合并肺高压患者血浆vonWillebrand(vWF)相关抗原的表达水平及其与肺动脉压力的相关性。【方法】连续观察左向右分流型先心病 32例 ,按肺动脉收缩压 /体循环动脉收缩压比值 (pp/ ps)分肺高压组(PH ,再依轻、中、重分为PH1和PH2组 )和非肺高压组 (NPH) ,分别为 19例和 13例。男 18例 ,女 14例 ,年龄 (13± 12 )岁。选择同期良性胸部疾病手术 13例为对照组。心内矫治术前采外周静脉、肺动脉和肺静脉血 ,以双抗体夹心酶联免疫吸附法检测血浆vWF水平。对照组只检测外周静脉血。【结果】①PH组血浆vWF表达水平 (PH1组 119%± 2 2 % ,PH2组 14 8%±2 9 % )明显高于NPH组 (96 %± 2 2 % )和对照组 (87%± 17% ) (P <0 0 1)。外周静脉血浆vWF表达水平的增高与pp/ps 呈正相关关系 (r =0 75 4 )。②血循环不同部位的血浆vWF表达水平有显著性差异 ,其强度沿肺静脉、外周静脉和肺动脉的位置顺序从高而低。【结论】①在无其它干扰因素影响下 ,左向右分流型先心病患者的术前外周静脉血浆vWF表达与 pp/ ps 呈正相关关系。②先心病合并肺高压患者血浆vWF表达在肺循环明显高于体循环。

To estimate the expression level of von Willebrand Factor related antigen (vWF) in plasma and the relationship with the degree of pulmonary hypertension in patients with congenital heart defects Thirty two consecutive cases with left to right shunt congenital heart defect were involved in this study They were divided into pulmonary hypertension (PH) group (regrouped PH1 for mild hypertension PH2 for middle or severe hypertension) and non pulmonary hypertension (NPH) group according to the pre operative hemodynamic parameter p p/p s (systolic pulmonary arterial pressure p p, systemic systolic blood pressure p s). There were 19 cases in PH group and 13 in NPH group, with totally 18 in male and 14 in female The mean age was (13±12) years At the same time, 13 patients suffering from benign thoracic surgical disease without heart lung problem were included in the study as a control group Pre operative peripheral venous blood sample, pulmonary artery blood sample and pulmonary vein blood sample were taken at the time just before the intra cardiac correction Only peripheral vein blood sample was collected in the control group Enzyme linked immunosorbent assay (ELISA) was used for assessing the expression of vWF in the plasma ①The expression level of vWF was significantly higher in PH group than that in NPH group and control group(P<0 01), with PH1 group in 119%±22%,PH2 group in 148%±29%,NPH group in 96%±22% and the control group in 87%±17% There was a positive relationship(r=0 754) between the expression of vWF in the peripheral venous plasma and p p/p s. ②There was a significant vWF expression difference among the blood sample from different circulation places The vWF expression concentration in the blood samples went down in a sequence of pulmonary venous plasma, peripheral venous plasma and pulmonary arterial plasma [Conclusion] ①A positive relationship between vWF expression in the pre operative peripheral venous plasma with the hemodynamic parameter p p/p s is found in patients suffering from the left to right shunt congenital heart defect in the circumstances without interfering factors. ②The expression of vWF in pulmonary circulation is higher than that in systemic circulation of patients with the left to right shunt congenital heart defect