Lammers Astrid E, Haworth Sheila G, Pierce Christine M
Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
Cardiol Young. 2006 Feb;16(1):84-6. doi: 10.1017/S1047951105002155.
Oral sildenafil has been demonstrated to be an effective treatment for pulmonary hypertension, and is increasingly used in children. We report an infant with pulmonary hypertension, stable on regular treatment with oral sildenafil, who presented in acute respiratory failure after aspiration, requiring ventilation and intensive care. The course of the stay in intensive care was difficult, with recurrent pulmonary hypertensive crises despite use of oral sildenafil, use of 100% oxygen, high frequency oscillatory ventilation, and inhaled nitric oxide. In view of his instability, and the presumed inability to absorb the sildenafil orally due to gastrointestinal malabsorption, sildenafil was administered as a continuous intravenous infusion. With this therapy, it proved possible to wean from oxygen, nitric oxide, and ventilatory support. Intravenous sildenafil, therefore, might be an effective alternative for children with pulmonary hypertension during episodes of acute deterioration and malabsorption, preventing life-threatening pulmonary hypertensive crises. Its pharmacokinetics, efficacy, and safety, nonetheless, need to be validated in randomized controlled trials.
口服西地那非已被证明是治疗肺动脉高压的有效方法，并且越来越多地用于儿童。我们报告了一名患有肺动脉高压的婴儿，在口服西地那非常规治疗下病情稳定，但在误吸后出现急性呼吸衰竭，需要通气和重症监护。在重症监护期间病程艰难，尽管使用了口服西地那非、100%氧气、高频振荡通气和吸入一氧化氮，仍反复出现肺动脉高压危象。鉴于其病情不稳定，且由于胃肠道吸收不良推测无法口服吸收西地那非，故将西地那非作为持续静脉输注给药。采用这种治疗方法后，成功实现了脱机吸氧、停用一氧化氮和撤机。因此，静脉注射西地那非可能是肺动脉高压患儿在急性病情恶化和吸收不良发作期间的一种有效替代方法，可预防危及生命的肺动脉高压危象。然而，其药代动力学、疗效和安全性仍需在随机对照试验中得到验证。
