A Single-arm, Open-label Phase IV Study to Evaluate the Effectiveness of Sotatercept in Improving Pulmonary Vascular Recruitment in Patients With Pulmonary Arterial Hypertension (PAH)
The goal of this clinical trial is to determine whether sotatercept is effective in improving diffusing capacity in patients with pulmonary arterial hypertension. Participants will be asked to: * Take Sotatercept every 21 days (±3 days) * Each participant will be enrolled in the study for 29 Weeks * Visit the clinic 15 times * Have a physical exam * Perform assessments of lung function and exercise tests * Have an ultrasound of their heart * Have blood draws done at regular intervals The main objectives of the study are: Primary objective: To assess whether sotatercept will improve recruitment of diffusing membrane capacity (DM) with exercise. Secondary objective: To identify components of the diffusing capacity that respond to treatment with sotatercept in pulmonary arterial hypertension.
• Age ≥ 18 years.
• Documented diagnostic right heart catheterization (RHC) at any time prior to screening confirming the diagnosis of PAH Group 1 in any of the following subtypes:
‣ Idiopathic PAH
⁃ Heritable PAH
⁃ Drug/toxin-induced PAH
⁃ PAH associated with CTD
⁃ PAH associated with simple, congenital systemic-to-pulmonary shunts at least 1 year following repair.
• Symptomatic PAH classified as WHO FC II or III.
• On stable doses of ≥2 background PAH therapies for at least 60 days prior to screening; for infusion prostacyclins, dose adjustment within 10% of the optimal dose is allowed per medical practice. Patients on 1 background PAH therapy are eligible if there is documented intolerance or contraindication to use of the other 2 classes (e.g. liver enzyme elevation while taking an ERA).
• Females of childbearing potential must:
‣ Have a negative urine or serum pregnancy tests as verified by the investigator prior to starting study therapy.
⁃ If sexually active, have used, and agree to use highly effective contraception without interruption during the study (including dose interruptions), and for 16 weeks (112 days) after discontinuation of study treatment.
⁃ Refrain from breastfeeding a child or donating blood, eggs, or ovum for the duration of the study and for at least 16 weeks (112 days) after the last dose of study treatment.
• Male participants must:
‣ Agree to use a condom, defined as a male latex condom or nonlatex condom NOT made out of natural (animal) membrane (e.g., polyurethane), during sexual contact with a pregnant female or a female of childbearing potential while participating in the study, during dose interruptions and for at least 16 weeks (112 days) following investigational product discontinuation, even if he has undergone a successful vasectomy.
⁃ Refrain from donating blood or sperm for the duration of the study and for 16 weeks (112 days) after the last dose of study treatment
• Ability to adhere to study visit schedule and understand and comply with all protocol requirements.
• Ability to understand and provide written informed consent.