先心病合并肺高压者"移行段"肺小动脉内膜的vWF定量表达分析

目的观察左向右分流型先天性心脏病(先心病)合并肺高压病人,肺活检"移行段"小动脉内膜vWF表达,对比病理分级、术前外周静脉血vWF水平和围术期结果.方法连续观察左向右分流型先心病病人70例,年龄4个月至63岁.术前Swan-Ganz漂浮导管监测计算肺动脉与体动脉压之比(Pp/Ps)并分级肺高压62例,其中轻度16例、中度24例、重度22例;无肺高压8例;以良性胸部疾病手术26例为对照.术中肺活检作常规病理检查和定量检测"移行段"小动脉内膜vWF含量值,同时检测术前外周静脉血vWF水平;观察术后心功能和肺部并发症.结果肺高压组"移行段"小动脉内膜vWF表达显著高于对照组和无肺高压先心病组,随Pp/Ps升高,vWF含量值逐步减少(=61.88-33.30x,r＝-0.701,P＜0.05);自高而低为无肺高压、轻度、中度和重度,与肺活检病变定性分级呈负相关(r=-0.697,P＜0.05).术前外周静脉血浆vWF水平与"移行段"小动脉内膜vWF表达呈负相关(=86.511-0.26x,r＝-0.649,P＜0.01).术后心肺并发症多在中、重度肺高压组.结论左向右分流型先心病合并肺高压愈重;"移行段"小动脉内膜vWF的表达趋向降低,与术前外周静脉血浆vWF水平呈负相关关系.

Quantitative vWF expression in the endothelium of pulmonary transitional arterioles in pulmonary hypertension secondary to congenital heart defect

Objective To correlate vWF expression in the endothelium of transitional pulmonary arterioles from lung biopsy in patients with pulmonary hypertension (PH) secondary to left-to-right shunt congenital heart defects (CHD). The results will be compared to vWF expression in peripheral venous plasma pre-operatively and the early post-operative outcome. Methods Consecutive 70 cases of left-to-right shunt congenital heart defects were involved in the study, the age ranged from 4 months to 63 years. Pulmonary systolic pressure/systemic systolic pressure (Pp/Ps) was calculated from Swan-Ganz floating catheter monitoring before corrective operation. PH was found in 62 cases, with slight PH in 16, moderated PH in 24 and severe PH in 22. Non-PH in 8 cases. Twenty-six cases of non-heart and lung disease were involved in as control. Lung biopsy tissue was stained for routine pathologic classification and quantitative vWF expression, with testing vWF in the peripheral venous blood. Post-operative complication was noted. Results There was a statistically difference of vWF expression in the endothelium of pulmonary transitional arterioles between PH group and non-PH group, with reducing intensity in order of non-PH, slight Ph, moderate PH and severe PH. A negative relationship was statistically noted between Pp/Ps and vWF expression in the arteriole endothelium (=61.88-33.30x, r=-0.701, P<0.01). It had negative relationship with the qualitative pathology analysis(r=-0.697,P<0.05)and vWF expression in the peripheral venous plasma (=86.511-0.26x, r=-0.649, P<0.01). Complication of during and post-operation was more common in moderate and severe PH cases. Conclusion vWF expression in the endothelium of pulmonary transitional arteriole of PH secondary to left-to-right shunt CHD tends to reduce gradually with the severity of Pp/Ps classification. It shows a negative relationship with that expression in the peripheral venous plasma pre-operatively.