摘要目的 评价吸入伊洛前列素的急性肺血管扩张试验在先天性心脏病(CHD)肺动脉高压(PH)患者心脏外科手术适应证选择中的作用.方法 对2006年6月至2008年12月46例CHD合并重度PH患者的临床资料进行回顾性分析.其中男性15例,女性31例,平均年龄(12±9)岁.所有患者术前均接受心导管检查和吸入伊洛前列索试验,患者平均肺动脉压(mPAP)(80±13)mm Hg(1 mill Hg=0.133 kPa),平均肺小动脉阻力指数(PVRI)(17±10)wood·m2.将吸入伊洛前列素试验肺血管阳性反应定义为在体循环压力不变或上升的情况下,PVRI下降≥20%,并作为选择手术适应证的重要条件.药物试验阳性患者在心脏外科修补术后均放置肺动脉漂浮导管,监测术后肺动脉压力、阻力以及心功能状况.结果 46例患者中,药物试验阳性29例(63.1%),吸药后PVRI由(15±6)wood·m2降至(9-4-4)wood·m2,肺循环体循环阻力比(Rp/Rs)由0.7±0.2降至0.4±0.2(P值均<0.05).药物试验反应阴性者17例(36.9%),吸药后PVRI由(21±10)wood·m2降至(19±9)wood·m2(P<0.05),Rp/Rs由1.0±0.5降至0.9±0.5(P>0.05).23例患者接受了心脏外科手术治疗,全部存活.其中药物试验阳性组21例,术后mPAP降至(27±10)mm Hg.药物试验阴性组仅2例接受外科修补术,术后mPAP均>45 mm Hg.结论 吸入伊洛前列素试验阳性患者术后肺动脉压力和PVRI明显降低,可作为评价合并PH的CHD手术适应证的一种蕈要手段.

abstractsObjective To evaluate the efficacy of iloprost in acute vasodilatation test during cardiac catheterization and to explore a useful hemodynamic indication regarding operability in the patients with severe pulmonary hypertension (PH) related to congenital heart disease (CHD). Methods The clinical data of 46 patients [mean age (12 ±9) years] with severe PH related to CHD from June 2006 to December 2008 was retrospectively analyzed. All patients underwent standard right and left cardiac catheterization and a trial of inhaled iloprost test during cardiac catheterization. The mean pulmonary arterial pressure was (80 ±13) mm Hg ( 1 mm Hg = 0.133 kPa) and pulmonary vascular resistance index was ( 17 ± 10 ) wood · m2.A positive response to inhaled iloprost was defined as a decrease of at least 20% in pulmonary vascular resistance index ( PVRI) without changes on systemic artery pressure. Patients with positive response to iloprost underwent cardiac surgical repair. The pulmonary artery pressure and PVRI was monitored by Swan- Ganz catheter postoperatively. Results Of the 46 patients, 29 (63.1% ) showed a positive response after iloprost inhalation, defined by a significant reduction in PVRI from (15 ±6) wood · m2 at baseline to(9 ±4) wood · m2 in response to iloprost inhalation therapy (P < 0. 05). The ratio of pulmonary to systemic resistance ( Rp/Rs) decreased from 0. 7 ± 0. 2 to 0.4 ± 0. 2 (P < 0. 05 ). Seventeen patients (36. 9% ) didn't respond to iloprost displayed only little changes in PVRI [from (21 ±10) wood · m2 to (19 ±9) wood · m2] and Rp/Rs (from 1. 0 ± 0. 5 to 0. 9 ± 0. 5). Out of 29 positive patients, 21 (72% ) underwent successful cardiac surgical repair with a reduction of mean pulmonary arterial pressure ( mPAP) to an average of (27 ± 10) mm Hg after the operation. Only 2 patients out of the 17 patients from the negative group were referred to surgery. Their mPAP was greater than 45 mm Hg. Conclusions A significant reduction in pulmonary artery pressure after cardiac surgery was observed in patients with positive response to inhaled iloprost. Inhaled iloprost may be a valuable tool in the preoperative evaluation of patients with severe PH related to CHD.