先天性心脏病并重度肺动脉高压患者雾化吸入伊诺前列素的即刻效应

Immediately effects of inhaled aerosolised iloprost in adult patients with severe pulmonary hypertension secondary to congenital heart diseases

目的 评价雾化吸入伊洛前列素对重度成人先天性心脏病相关性肺动脉高压血液动力学的影响.方法 对165例经超声心动图诊断先天性心脏病合并重度肺动脉高压患者进行左右心导管检查获得基线血液动力学参数后雾化吸入伊洛前列素20 μg,10 min时再记录相关血液动力学资料,对比吸入伊诺前列素前后血液动力学的变化.结果 伊洛前列素吸入结束后即刻和吸入前比较,患者主动脉和肺动脉的压力略有下降（P＜0.05）,但心率、主动脉平均压、肺动脉收缩压与主动脉收缩压的比值（Pp/Ps）差异均无统计学意义（P＞0.05）.肺循环血量（Qp）明显增加[（7.2±4.8）L/min比（9.9±7.2）L/min,P＜0.01],肺血管阻力（PVR）明显下降[（13.4±8.7）Wood单位比（9.5±6.6）Wood单位,P＜0.01],左向右分流量明显增加[（3.2±4.4）L/min比（5.5±7.0）L/min,P＜0.01]及右向左分流量明显减少[（1.0±1. 0）L/min比（0.7±0.7）L/min,P＜0.01].亚组分析结果提示,不是所有先天性心脏病合并重度肺动脉高压患者血液动力学均呈相同的变换趋势,存在动脉导管未闭和（或）心室间隔缺损患者的肺动脉压力明显高于继发于心房间隔缺损患者,而且更易出现艾森曼格综合征（P＜0.05）.结论 伊洛前列素雾化吸入可快速降低先天性心脏病相关性肺动脉高压的压力、肺血管阻力,明显增加右心排血量,对体循环压力及心率影响较小,可以作为评估急性肺血管反应的肺血管扩张剂.

Objective To investigate the immediately effects of inhaled aerosolized iloprost in adult patients with severe pulmonary arterial hypertension（PAH）secondary to congenital heart diseases（CHD）.Methods Adult patients with severe PAH secondary to CHD（n = 165）were included in this study. Right heart catheterization was performed, Pulmonary and systemic blood flow, the oxygen consumption VO2（ml/min）were calculated using Fick＇s principle. Pulmonary vascular resistances（PVR）were calculated with standard formulas and indexed to body surface area. Hemodynamic parameters were measured before and after iloprost inhalation（20 μg）. Results Post iloprost inhalation, heart rate, mean aortic pressure, pulmonary systolic pressure to aortic systolic pressure ratio all remained unchanged（P 〉 0. 05）, while pulmonary artery pressure（PAP）were significantly reduced and Qp significantly increased from（7. 2 ±4. 8）L/min to（9. 9 ±7.2）L/min（P〈0.01）, PVR was also significantly reduced from（13.4 ±8.7）Wood units to（9.5 ±6. 6）Wood units（P 〈0. 01）,and left-to-right shunt volume increased from（3.2 ±4. 4）L/min to（5. 5 ±7.0）L/min（P 〈 0. 01）and right -to-left shunt volume decreased from（1.0 ± 1.0）L/min to（0. 7 ± 0. 7）L/min（P 〈 0. 01）. Subgroup analysis showed that adult patients with patent ductus arteriosus and/or ventricular septal defects are more likely to develop severe pulmonary arterial hypertension or Eisenmenger syndrome than patients with atrial septal defects. Conclusions Inhaled Aerosolised iloprost use is effective and safe for adult patients with severe pulmonary arterial hypertension secondary to congenital heart diseases.