Li Qiangqiang, Dimopoulos Konstantinos, Zhang Chen, Zhu Yan, Liu Qian, Gu Hong
Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, 4th Floor, Cardiac Surgery Building, No. 2 Anzhen Road, Chaoyang District, Beijing, 100029, China.
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College, London, UK.
Pediatr Cardiol. 2018 Apr;39(4):757-762. doi: 10.1007/s00246-018-1818-7. Epub 2018 Feb 9.
Inhaled prostacyclin analogue iloprost is currently utilized in adult patients with pulmonary arterial hypertension (PAH), but little information is available on its use in the pediatric population. This study evaluated the safety and acute haemodynamic effects of inhaled iloprost in children with PAH associated with congenital heart disease (CHD). Children with PAH-CHD who underwent cardiac catheterization and iloprost administration in our catheter laboratory between June 2007 and October 2015 were included. Iloprost was administered by atomization inhalation and changes in hemodynamic parameters were recorded. In total, 100 children with PAH-CHD were enrolled. Median age was 13 [1.5-18.0] years and 34% were male. A ventricular septal defect was present in 84%, a patent duct in 12%, a complete atrioventricular septal defect in 2%, and an isolated atrial septal defect in 2%. Pulmonary vascular resistance indexed (PVRI) was above 8 WU m in 96% and was above a third systemic (R/R > 0.33) in 97%. Iloprost was well tolerated in all patients. Following iloprost inhalation, mean pulmonary arterial pressure decreased from 78.4 ± 9.2 to 72.8 ± 10.8 mmHg (p < 0.01) and pulmonary-to-systemic blood flow ratio (Q/Q) increased from 1.12 ± 0.48 to 1.37 ± 0.63 (p < 0.01), with no change in cardiac index (Q). PVRI decreased from 21.0 ± 9.0 to 16.9 ± 8.0 WU m (p < 0.01) following inhalation but 92% patients still had a PVRI > 8 WU m and 93% an R/R > 0.33. Acute inhalation of iloprost in children with PAH associated with CHD resulted in a significant improvement in hemodynamic parameters. Despite this, few patients achieve strict criteria of operability, underscoring the importance of early screening and timely repair of CHD.
吸入性前列环素类似物伊洛前列素目前用于治疗成人肺动脉高压（PAH），但关于其在儿科患者中的应用信息较少。本研究评估了吸入伊洛前列素对患有先天性心脏病（CHD）相关PAH儿童的安全性和急性血流动力学影响。纳入2007年6月至2015年10月期间在我们导管实验室接受心脏导管插入术并给予伊洛前列素的PAH-CHD儿童。通过雾化吸入给予伊洛前列素，并记录血流动力学参数的变化。总共纳入100例PAH-CHD儿童。中位年龄为13[1.5 - 18.0]岁，34%为男性。84%存在室间隔缺损，12%存在动脉导管未闭，2%存在完全性房室间隔缺损，2%存在孤立性房间隔缺损。96%的患者肺血管阻力指数（PVRI）高于8 WU m²，97%的患者肺血管阻力指数高于三分之一体循环阻力（R/R＞0.33）。所有患者对伊洛前列素耐受性良好。吸入伊洛前列素后，平均肺动脉压从78.4±9.2降至72.8±10.8 mmHg（p＜0.01），肺循环与体循环血流量比值（Qp/Qs）从1.12±0.48增至1.37±0.63（p＜0.01），心指数（Q）无变化。吸入后PVRI从21.0±9.0降至16.9±8.0 WU m²（p＜0.01），但92%的患者PVRI仍＞8 WU m²，93%的患者R/R＞0.33。对患有CHD相关PAH的儿童急性吸入伊洛前列素可显著改善血流动力学参数。尽管如此，很少有患者达到严格的手术标准，这突出了早期筛查和及时修复CHD的重要性。
