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Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease.

机译:西地那非治疗对先天性心脏病成人肺动脉高压的影响。

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BACKGROUND: 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. OBJECTIVE: 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. RESULTS: 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. CONCLUSIONS: 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.
机译:背景:已证明西地那非对肺动脉高压(PAH)患者有效。但是,西地那非对患有先天性心脏病(CHD)的成年人PAH的影响的研究较少。目的:在这项前瞻性,开放标签,非对照和多中心研究中,60例冠心病相关的PAH患者接受口服西地那非(75 mg /天)治疗12周。入选患者在12周的开始和结束时进行了6分钟步行测试(SMWT)和心脏导管检查。主要终点是SMWT评估的运动能力的变化;次要终点包括功能等级评估,心肺血流动力学评估和临床恶化(定义为PAH的死亡,移植和再次住院)。还检查了药物安全性和耐受性。结果:口服西地那非显着增加了SMWT距离(422.94 +/- 76.95 m与371.99 +/- 78.73 m,P <0.0001)。博格呼吸困难评分也有显着改善(2.1 +/- 1.32比2.57 +/- 1.42,P = 0.0307)。此外,还发现了世界卫生组织(WHO)功能类别和心肺血流动力学的显着改善(平均肺动脉压,P = 0.0002;心脏指数,P <0.0001;肺血管阻力,P <0.0001)。该研究的副作用较轻,与已报道的研究一致。所有入选患者均未出现明显的临床恶化。结论:本研究证实并扩展了先前的研究。这提示口服西地那非对成人CHD相关PAH患者的治疗是安全有效的。

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