Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Hypertension With Bosentan: A Single Center Pilot Study
试验速览
- 阶段
- 4 期
- 状态
- 撤回
- 发起方
- 试验地点
- 2
- 主要终点
- 6 minute walk distance
研究概览
简要总结
Pulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently there is no significant data to support the use of pulmonary vasodilators for PAH in the setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH either at rest or during exercise. The study hypothesis is that bosentan may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.
详细描述
The purpose of this study was to evaluate bosentan in the setting of exercise or resting pulmonary hypertension in patients with underlying pulmonary fibrosis.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Parallel
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Idiopathic Pulmonary Fibrosis referred for lung transplantation
- •Minimum 50 meter 6 minute walk distance
- •No significant underlying liver disease
排除标准
- •Significant liver disease or cirrhosis
- •non ambulatory
- •previous adverse reaction/allergy to Bosentan
研究组 & 干预措施
treatment with bosentan
patients with resting or exercise induced PAH receive bosentan in a randomized open label fashion
干预措施: bosentan (Drug)
结局指标
主要结局
6 minute walk distance
时间窗: monthly assessement until date of lung transplantation
ATS Guideline 6MW distance before and after intervention
次要结局
- chemokine peripheral blood analysis(monthly)
- right heart catheterization hemodynamics(variable based on time between listing and actual lung transplantation)
研究者
Rajan Saggar
Principal Investigator
University of California, Los Angeles