Center for Diagnosis and Management of Pulmonary Vascular Diseases, Department of Cardiology, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
Cardiovasc Ther. 2010 Dec;28(6):350-5. doi: 10.1111/j.1755-5922.2010.00213.x.
It has been demonstrated that sildenafil is effective in patients with pulmonary arterial hypertension (PAH). However, the impact of sildenafil on PAH in adults with congenital heart disease (CHD) has been less investigated.
In this prospective, open-label, uncontrolled and multicenter study, 60 patients with PAH related to CHD received oral sildenafil (75 mg/day) for 12 weeks. The enrolled patients underwent six-minute walk test (SMWT) and cardiac catheterization at the beginning and the end of the 12 weeks. The primary end point was the changes in exercise capacity assessed by SMWT; the secondary end point included assessment of functional class, evaluation of cardiopulmonary hemodynamics, and clinical worsening (defined as death, transplantation, and rehospitalization for PAH). Drug safety and tolerability were also examined.
Oral sidenafil significantly increased SMWT distances (422.94 ± 76.95 m vs. 371.99 ± 78.73 m, P < 0.0001). There was also remarkable improvement in Borg dyspnea score (2.1 ± 1.32 vs. 2.57 ± 1.42, P = 0.0307). Moreover, significant improvements in World Healthy Organization (WHO) functional class and cardiopulmonary hemodynamics were also discovered (mean pulmonary artery pressure, P = 0.0002; cardiac index, P < 0.0001; pulmonary vascular resistance, P < 0.0001). Side effects in this study were mild and consistent with reported studies. None of the enrolled patients experienced significant clinical worsening.
This study confirmed and extended previous studies. It suggested that oral sildenafil was safe and effective for the treatment of adult patients with CHD-related PAH.
已有研究表明，西地那非对肺动脉高压（PAH）患者有效。然而，西地那非对先天性心脏病（CHD）相关 PAH 患者的影响研究较少。
在这项前瞻性、开放标签、非对照和多中心研究中，60 例 CHD 相关 PAH 患者接受口服西地那非（75 mg/天）治疗 12 周。纳入患者在治疗前和治疗 12 周后分别进行 6 分钟步行试验（SMWT）和心导管检查。主要终点是 SMWT 评估的运动能力变化；次要终点包括功能分级评估、心肺血液动力学评估和临床恶化（定义为死亡、移植和 PAH 再住院）。还检查了药物安全性和耐受性。
口服西地那非显著增加了 SMWT 距离（422.94 ± 76.95 m 比 371.99 ± 78.73 m，P < 0.0001）。Borg 呼吸困难评分也有显著改善（2.1 ± 1.32 比 2.57 ± 1.42，P = 0.0307）。此外，还发现世界卫生组织（WHO）功能分级和心肺血液动力学显著改善（平均肺动脉压，P = 0.0002；心指数，P < 0.0001；肺血管阻力，P < 0.0001）。本研究中的副作用较轻，与已报道的研究一致。所有纳入患者均未出现明显的临床恶化。
本研究证实并扩展了以往的研究，表明口服西地那非治疗 CHD 相关 PAH 成人患者安全有效。
