Background:Pulmonary vasodilators improve the functional capacity of some patients with pulmonary arterial hypertension. However, pulmonary vasodilators frequently fail to improve unequivocal endpoints of efficacy in patients with lower pulmonary arterial pressures who have been palliated with a Fontan procedure.Objective:Haemodynamic measurements and the results of acute vasodilator testing in a subset of patients were reviewed to determine whether some patients acutely respond more favourably to sildenafil and might be candidates for precision care with a phosphodiesterase V inhibitor long term.Materials and Methods:Heart catheterisation was performed in 11 patients with a Fontan procedure. Haemodynamic measurements were performed before and after treatment with intravenous sildenafil (mean 0.14, range 0.05–0.20 mg/kg). Results (mean ± standard deviation) were compared by paired and unpaired t-tests to identify statistically significant changes.Results:Sildenafil was acutely associated with changes in mean pulmonary arterial pressure, transpulmonary gradient, indexed blood flow, and indexed vascular resistance. Changes in mean pulmonary arterial pressure were greater for patients with a mean pulmonary arterial pressure greater than 14 mmHg compared to patients with a lower mean pulmonary arterial pressure. Changes in transpulmonary gradient were greater for patients with a transpulmonary gradient greater than 5 mmHg compared to patients with a lower transpulmonary gradient.Conclusion:Sildenafil acutely decreases mean pulmonary arterial pressure and transpulmonary gradient and causes greater acute changes in patients with higher mean pulmonary arterial pressures and transpulmonary gradients. Haemodynamic measurements and vasodilator testing might help to guide precision care following Fontan palliation.

中文翻译：

---

Fontan 姑息治疗后的急性血管扩张剂测试：指导精准护理的机会？

背景：肺血管扩张剂可改善部分肺动脉高压患者的功能能力。然而，肺血管扩张剂经常不能改善已通过 Fontan 手术缓解的低肺动脉压患者的明确疗效终点。患者对西地那非的急性反应更有利，可能适合长期使用磷酸二酯酶 V 抑制剂进行精准护理。材料和方法：对 11 名接受 Fontan 手术的患者进行了心脏导管插入术。在静脉注射西地那非治疗前后进行血流动力学测量（平均 0.14，范围 0.05-0.20 mg/kg）。通过配对和非配对 t 检验比较结果（平均值 ± 标准差），以确定具有统计学意义的变化。结果：西地那非与平均肺动脉压、跨肺压差、指数血流和指数血管阻力的变化密切相关。与平均肺动脉压较低的患者相比，平均肺动脉压大于 14 mmHg 的患者的平均肺动脉压变化更大。与跨肺梯度较低的患者相比，跨肺梯度大于 5 mmHg 的患者的跨肺梯度变化更大。结论：西地那非急剧降低平均肺动脉压和跨肺压差，并在平均肺动脉压和跨肺压差较高的患者中引起更大的急性变化。血流动力学测量和血管扩张剂测试可能有助于指导 Fontan 姑息治疗后的精准护理。