BOSENTAN VS PLACEBO IN NYHA CLASS L/LL SCLERODERMA PATIENTSW/PULMON HYPERTENSION

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 这是一项博生坦治疗运动性肺动脉高压系统性硬化症患者的前瞻性、随机、安慰剂对照试验。受试者将被随机接受波生坦62.5 mg PO，2次/1个月，然后125 mg PO，2次/d，与安慰剂对照，为期16周。主要结果变量是超声心动图检查标准Bruce负荷方案期间的总运动时间。 需要评估的次要结果变量包括：6分钟步行距离、BMP水平、ET-1水平、简明36问卷、Shaq、UCDs呼吸困难指数。 在16周结束时，受试者将有资格获得长期的博森坦。运动耐力、无创血流动力学和生活质量的测量将每6个月重复一次，直到36个月内完成试验，这是PHRAOS长期(观察性)研究的一部分，如果参与者还没有参与，将被敦促参加。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This is a prospective, randomized, placebo-controlled trial of Bosentan in systemic sclerosis patients with exercise-induced pulmonary hypertension. Subjects will be randomized to receive Bosentan 62.5 mg PO Bid x 1 month followed by 125 mg PO Bid thereafter versus placebo for 16 weeks. The primary outcome variable is total exercise time during the standard Bruce stress protocol with echocardiogram. The secondary outcome variables to be evaluated include: 6 minute walk distance, BMP levels, ET-1 levels, Short-Form 36 questionnaire, sHAQ, UCDS Dyspnea index. At the completion of 16 weeks, subjects will be eligible to receive long term Bosentan. Measurements of exercise endurance, non-invasive hemodynamics, and quality of life will be repeated every 6 months until the completion of the trial in 36 months as part of the PHRAOS long term (observational) study in which participants will be urged to enter, if they are not already a part of it.