目的:对于因肺血管阻力或者肺动脉压力较高患者,往往无法进行右侧心脏短路手术(Fontan手术)。本文主要研究了波生坦对此类患者血流动力学方面的改善作用。 方法:本组共8例患者(2例女,6例男,年龄7个月到5岁,平均年龄1岁)。所有患者都曾进行过姑息手术,其中4例行肺动脉环缩,2例行BT分流,5例行Glenn手术。因肺血管阻力或肺动脉压力较高,都不具备行Fontan手术的指征。 结果:通过波生坦治疗,肺动脉压力和肺血管阻力都成功得到控制。平均肺动脉压力在用药前后分别为21.1±7.2mmHg和11.9±4.1mmHg(P<0.01)。平均肺血管阻力指数用药前后分别为5.7±3.3U/m2和1.3±0.4U/m2(P<0.01).平均肺循环/体循环阻力之比用药前后分别为0.25±0.11和0.07±0.03(P<0.01)。所有患者临床症状得到缓解并最终成功完成Fontan手术。 结论:对于单心室生理合并肺血管阻力及肺动脉压力升高的患者,波生坦可以有效改善临床症状及血流动力学。波生坦可以增加外科手术适应症并改善Fontan手术的预后。 Congenital heart disease Bosentan induces clinical and hemodynamic improvement in candidates for right-sided heart bypass surgery Keiichi Hirono MDa, Naoki Yoshimura MDb, Masato Taguchi PhDc, Kazuhiro Watanabe MDa, Tsuneyuki Nakamura MDd, Fukiko Ichida MDa, Corresponding Author Contact Information, E-mail The Corresponding Author and Toshio Miyawaki MDa Objective To investigate the efficacy of bosentan in patients with single-ventricle physiology who were unable to undergo right-sided heart bypass surgery because of high pulmonary vascular resistance and pulmonary artery pressure. Methods Eight patients with single-ventricle physiology (2 male and 6 female; aged 7 months to 5 years, median 1 year) were enrolled. Prior surgical interventions included pulmonary artery banding in 4 patients, Blalock–Taussig shunt operation in 2 patients, and bidirectional Glenn operation in 5 patients. Right-sided heart bypass surgery was contraindicated for all patients because of high pulmonary vascular resistance and pulmonary artery pressure. Results Bosentan therapy successfully reduced pulmonary artery pressure and pulmonary vascular resistance in all patients. Mean pulmonary artery pressure at baseline and after bosentan therapy was 21.1 ± 7.2 mm Hg and 11.9 ± 4.1 mm Hg, respectively (P < .01). Mean pulmonary vascular resistance index at baseline and after bosentan therapy was 5.7 ± 3.3 U/m2 and 1.3 ± 0.4 U/m2, respectively (P < .01). Mean pulmonary vascular resistance/systemic vascular resistance at baseline and after bosentan therapy was 0.25 ± 0.11 and 0.07 ± 0.03, respectively (P < .01). All patients had improved clinical symptoms and underwent successful Fontan operations. Conclusion Bosentan induces mid-term clinical and hemodynamic improvement in patients with single-ventricle physiology and elevated pulmonary vascular resistance and pulmonary artery pressure. Bosentan therapy may increase the surgical options and improve outcomes in candidates for right-sided heart bypass surgery. The Journal of Thoracic and Cardiovascular Surgery Volume 140, Issue 2, August 2010, Pages 346-351 |