Oral Treprostinil in Subjects With Pulmonary Hypertension Associated With Pulmonary Fibrosis

This study will evaluate how oral treprostinil can affect pulmonary vascular resistance, as
assessed by right heart catheterization, and exercise capacity, as assessed by the
Six-Minute Walk Test. This is a 16-week study that will involve at least 10 clinical trial
centers. The expected enrollment period is around 24 months and 52 subjects will be entered
into the study.

Inclusion Criteria:

- 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

Exclusion Criteria:

- 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