Sotatercept in Pulmonary Arterial Hypertension
brief summary
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 18 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.
detailed description
Potential participants must provide their written informed consent before any study-specific procedures.
All screening procedures will be completed over two weeks during three separate visits. For participants from the Calgary site (site 2), all Visit 1 procedures except for PFT and CPET may be performed on a separate day in Calgary. Visits 2 and 3 will be performed in Edmonton (site 1). According to patient preference, visits 2 and 3 can be performed on the same day, with a minimum 3-hour break between visits. For participants from Calgary (site 2), the first drug administration will occur in Calgary within seven days of completion of Visit 3 procedures.
Screening will include reviewing the participant's medical, surgical, and family history, collecting demographics, race, and ethnicity, and requesting medical records for relevant external procedures.
Screening procedures include:
Visit 1:
* Informed consent * Inclusion/exclusion criteria * Medical history review * Physical examination * 12-lead ECG * Vital signs * Weight * WHO FC assessment * 6MWT * Pregnancy test * Hematology (complete blood count) * Urinalysis * Hemoglobin * Serum chemistry * NT-proBNP * PFT * CPET * QoL assessments * AE/SAE Review * Concomitant medication review
Visit 2:
* Hemoglobin * Rest/exercise DLCO, VC, DM * Rest/exercise CW-NIRS * AE/SAE Review * Concomitant medication review
Visit 3:
* Pregnancy test * Rest/exercise Echocardiography * AE/SAE Review * Concomitant Medication review * Study drug administration
Measurements/assessments taken during the Screening Period will be recorded as the baseline values for the study assessment of endpoints.
Treatment Period (Visit 4-14) All study procedures should be performed prior to the study drug administration. For participants at the Calgary site, Visits 4,8-14 occur at their home site. For participants from the Calgary site (site 2), Visit 5-7 will be performed in Edmonton (site 1). Visits 5-7 are to be performed before the administration of the 3rd dose of the study drug. According to the patient's preference, visits 6 and 7 may be combined.
official title
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)