This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.This is a randomized, placebo-controlled, multicenter, Phase IIIb study designed to assess the safety and efficacy of Bosentan (125 mg BID) in sickle cell disease patients with pulmonary arterial hypertension confirmed by right-heart catheterization. A total of 78 patients will be enrolled. The study will be conducted in approximately 24 centers in the US, the UK, France, and the Netherlands.
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