Safety and Efficacy of Inhaled Treprostinil in Adult PH With ILD Including CPFE

Inclusion Criteria:

1. Subject voluntarily gave informed consent to participate in the study.
2. Males and females aged 18 years or older at the time of informed consent. a. Females of reproductive potential were non-pregnant (as confirmed by a urinepregnancy test at screening) and non-lactating, and: i. Abstained from intercourse (when in line with their preferred and usual lifestyle), or ii. Used 2 medicallyacceptable, highly effective forms of contraception for the duration of study, and atleast 30 days after discontinuing study drug. b. Males with a partner of childbearing potential used condoms for the duration oftreatment and for at least 48 hours after discontinuing study drug.
3. The subject had a confirmed diagnosis of WHO Group 3 PH based on computed tomography (CT) imaging which was performed within 6 months prior to randomization anddemonstrated evidence of diffuse parenchymal lung disease. Subjects had any form ofILD or CPFE.
4. Subjects were required to have a right heart catheterization (RHC) within 1 year priorto randomization with the following documented parameters:
5. Pulmonary vascular resistance (PVR) >3 Wood Units (WU) and
6. A pulmonary capillary wedge pressure (PCWP) of <15 mmHg and
7. A mean pulmonary arterial pressure (mPAP) of >25 mmHg
8. Baseline 6MWD ≥100 m.
9. Subjects on a chronic medication for underlying lung disease (ie, pirfenidone,nintedanib, etc) were on a stable and optimized dose for ≥30 days prior torandomization.
10. In the opinion of the Investigator, the subject was able to communicate effectivelywith study personnel, and was considered reliable, willing and likely to becooperative with protocol requirements, including attending all study visits.
11. Subjects with connective tissue disease (CTD) had a Baseline forced vital capacity (FVC) of <70%.

Exclusion criteria:

1. The subject had a diagnosis of PAH or PH for reasons other than WHO Group 3 PH ILD asoutlined in Inclusion Criterion 3.
2. The subject showed intolerance or significant lack of efficacy to a prostacyclin orprostacyclin analogue that resulted in discontinuation or inability to effectivelytitrate that therapy.
3. The subject received any PAH-approved therapy including: prostacyclin therapy (ie,epoprostenol, treprostinil, iloprost, or beraprost; except for acute vasoreactivitytesting), prostacyclin (IP) receptor agonist (selexipag), endothelin receptorantagonist (ERA), phosphodiesterase type 5 inhibitor (PDE5-I), or soluble guanylatecyclase (sGC) stimulator within 60 days of randomization.
4. The subject had evidence of clinically significant left-sided heart disease as definedby:
5. PCWP >15 mmHg
6. Left ventricular ejection fraction <40%. Note: Subjects with abnormal leftventricular function attributable entirely to impaired left ventricular fillingdue to the effects of right ventricular overload (ie, right ventricularhypertrophy and/or dilatation) were not excluded.
7. The subject was receiving >10 L/min of oxygen supplementation by any mode of deliveryat rest at Baseline.
8. Current use of any inhaled tobacco/marijuana products or significant history of drugabuse at the time of informed consent.
9. Exacerbation of underlying lung disease or active pulmonary or upper respiratoryinfection within 30 days of randomization.
10. Initiation of pulmonary rehabilitation within 12 weeks prior to randomization.
11. In the opinion of the Investigator, the subject had any condition that would interferewith the interpretation of study assessments or has any disease or condition (ie,peripheral vascular disease, musculoskeletal disorder, morbid obesity) that wouldlikely be the primary limit to ambulation (as opposed to PH).
12. Use of any investigational drug/device, or participation in any investigational studywith therapeutic intent within 30 days prior to randomization.
13. Severe concomitant illness limiting life expectancy (<6 months).
14. Acute pulmonary embolism within 90 days of randomization.