Pulmonary Fibrosis, Pulmonary Hypertension
Conditions
Brief summary
This multicenter, randomized, open-label study will assess the safety and efficacy of oral treprostinil in subjects diagnosed with pulmonary hypertension associated with pulmonary fibrosis.
Detailed description
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.
Interventions
Oral treprostinil will be administered as TID dosing for up to 16 Weeks.
Sponsors
United Therapeutics
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 79 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change in pulmonary vascular resistance (PVR) | Baseline to Week 16 | Change in PVR between iMTD and fixed dose groups |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Change in Six-minute walk distance (6MWD) | Baseline to Week 16 | Change in 6MWD between iMTD and fixed dose groups |
| Change in N-terminal pro-brain natriuretic peptide (NT-ProBNP) | Baseline to Week 16 | Change in NT-ProBNP between iMTD and fixed dose groups |
Countries
United States
Outcome results
None listed