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. The purpose of this study is to demonstrate that bosentan improves 6-minute walk distance, pulmonary function test (PTFs), dyspnea, and quality of life, and to demonstrate that bosentan is safe and well tolerated in this patient population. This study was 12 months in duration with patients performing PFTs, 6MWT test every 3 months.
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