Lead Sponsor

United Therapeutics

Age

18 - 79 years

Sex

ALL

Healthy Volunteers

No

•Voluntarily gives informed consent
•Must meet criteria for lung transplant referral but is not required to be on an active lung transplant list
•Stable and significant diffuse parenchymal lung disease with a diagnosis of interstitial lung disease
•6MWD greater than or equal to 75 meters
•Right heart catheterization with a mean pulmonary arterial pressure \>= 30 mgHg, pulmonary capillary wedge pressure \<= 15 mmHg and pulmonary vascular resistance \> 240 dynes
•Echo-Doppler examination showing evidence of right ventricular dysfunction and normal left diastolic ventricular function
•Either not receiving any PAH-approved oral therapy, or is receiving monotherapy (ERA, PDE-5I, or riociguat) for \> 60 days and receiving a stable dose for \> 30 days prior to enrollment
•Able to communicate effectively with study personnel and will to be cooperative with protocol requirements

•History of repaired or unrepaired congenital heart disease
•Received prostanoid therapy in the past 30 days, or has shown an intolerance or lack of efficacy to prostanoid therapy resulting in discontinuation or inability to titrate prostacyclins
•Diagnosis of sarcoidosis
•History of thromboembolic disease
•Chronic renal insufficiency
•Pregnancy or lactating
•Currently receiving an investigational drug, has an investigational device in place, or has participated in an investigational drug or device study within 30 days prior to Screening

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