Pulmonary Hypertension Clinical Trials

∙ Participants must meet all of the following criteria to be included in the trial:

• Ability to provide written informed consent prior to participation in the trial, which includes the ability to comply with the requirements and restrictions listed in the informed consent form (ICF). Participants must be able to read, comprehend, and write at a level sufficient to complete trial-related materials

• Adult male or female participants 18 (19 years in South Korea) to 80 years of age at the time the ICF is signed

• A confirmed diagnosis of the following WHO Group 3 PH based on HRCT imaging, which demonstrates the following evidence of ILD performed within 6 months prior to starting the Treatment Period:

‣ ILD

⁃ CPFE: only allowed for participants with 15% or less emphysema, and the extent of the ILD must be greater than that of the emphysema. Participants may have any form of ILD (including IIP, IPF, and connective tissue disease) or CPFE

• Results of the RHC within 35 days prior to Day 1 and meet all of the following criteria:

‣ Participants receiving treatment with endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogs or prostacyclin receptor agonists, and/or calcium channel blockers are eligible only if on a stable dose for at least 90 days prior to RHC at baseline and throughout the Screening Period. For infusion prostacyclin analogs, dose adjustment based on the participant's body weight is allowed per medical practice.

⁃ Participants on chronic medication for underlying lung disease (ie, pirfenidone, etc.) are eligible only if on a stable dose for at least 90 days prior to RHC at baseline and throughout the Screening Period

⁃ Participants receiving treatment with oxygen therapy are eligible only if beginning for at least 30 days prior to RHC at baseline and throughout the Screening Period, and on a stable dose of oxygen for 30 minutes before RHC

• Valid 6MWD

• Baseline FVC \<70% (only for participants with connective tissue disease).

• Women of childbearing potential (WOCBP) must have a negative pregnancy test before receiving the trial treatment and must agree to use contraception from the Screening Visit to at least 30 days after the last dose of the trial treatment. Male participants who could potentially cause pregnancy must agree to use contraception from the Screening Visit to at least 90 days after the last dose of the trial treatment to avoid pregnancy in their partners

• Able to complete the scheduled visits and follow the instructions of the investigator