The goal of this study is to learn about the safety of sotatercept and how well Japanese children tolerate it, when taken along with standard (usual) pulmonary arterial hypertension (PAH) treatment. Researchers also want to learn what happens to it in a person's body over time and whether it lowers resistance in blood vessels in the lungs.
Has pulmonary arterial hypertension (PAH, World Health Organization Group 1) confirmed by a prior right heart catheterization
Has idiopathic, heritable, drug- or toxin-induced PAH, connective tissue disease-associated PAH, repaired congenital heart disease-associated PAH, or PAH with coincidental shunt
Has PAH classified as World Health Organization Functional Class I, or symptomatic World Health Organization Functional Class II to IV
Has been receiving stable standard-of-care background therapy for PAH for at least 90 days
History of left-sided heart disease
Has severe congenital or developmental abnormalities of the lung, thorax, and/or diaphragm
History of Eisenmenger syndrome, Potts shunt, or recent atrial septostomy within 180 days
Has unrepaired or residual cardiac shunt with Qp/Qs >1.5
Single group
Sotatercept
Biological/VaccineSotatercept subcutaneous injection every 3 weeks
Number of Participants With One or More Adverse Events
An adverse event is any untoward medical occurrence in a clinical study participant, temporally associated with the use of study intervention, whether or not considered related to the study intervention.
Number of Participants Who Discontinue Study Intervention Due to an Adverse Event
An adverse event is any untoward medical occurrence in a clinical study participant, temporally associated with the use of study intervention, whether or not considered related to the study intervention.
Laboratory Parameter (Hematology): Concentration of Hemoglobin
Hematological parameters will be investigated in blood samples from participants by means of clinical laboratory assays and evaluated by the investigator. The concentration of hemoglobin will be presented.
Laboratory Parameter (Hematology): Hematocrit
Hematological parameters will be investigated in blood samples from participants by means of clinical laboratory assays and evaluated by the investigator. The hematocrit will be presented.
Mean Change from Baseline in 6-Minute Walk Distance (6MWD)
6MWD will be assessed using the 6-minute walk test (6MWT).
Mean Change from Baseline in N-terminal Prohormone B-type Natriuretic Peptide (NT-proBNP)
The change from baseline in plasma NT-proBNP levels will be reported.
Percentage of Participants Who Either Improved or Maintained Their World Health Organization Functional Class (WHO FC)
The severity of an individual's PAH symptoms will be graded using the WHO FC system. WHO functional classification for PAH range from Class I (no limitation in physical activity, no dyspnea with normal activity), Class II (slight limitation of physical activity), Class III (marked limitation of physical activity) and Class IV (cannot perform a physical activity without any symptoms, dyspnea at rest). The change from baseline in WHO FC will be classified into "Improved", "No change" and "Worsened". Improvement = reduction in FC, worsened = increase in FC and no change = no change in FC.
Number of Participants With One or More Adverse Events
An adverse event is any untoward medical occurrence in a clinical study participant, temporally associated with the use of study intervention, whether or not considered related to the study intervention.
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