Documented diagnostic right heart catheterization (RHC) within 12 months of screening documenting a minimum PVR of ≥ 4 Wood units and pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) of ≤ 15 mmHg, with the diagnosis of WHO PAH Group 1 in any of the following subtypes:
* Idiopathic PAH
* Heritable PAH
* Drug/toxine-induced PAH
* PAH associated with connective tissue disease
* PAH associated with simple, congenital systemic-to- pulmonary shunts at least 1year following repair
Patients under bi or tri-background-therapy
Symptomatic PAH classified WHO FC II or III
Patients will be started on Sotatercept
Ability to adhere to study visit schedule and understand and comply with all the protocol requirement.
Ability to understand and provide written informed consent
You may not be if
Diagnosis of PH WHO Groups 2, 3, 4, or 5
Diagnosis of the following PAH Group 1 subtypes: human immunodeficiency virus (HIV)-associated PAH, PAH associated with portal hypertension, schistosomiasis associated PAH, pulmonary veno occlusive disease and pulmonary capillary hemangiomatosis.
Hemoglobin at screening above gender-specific ULN, per local laboratory test
Pregnant or breastfeeding women
Any of the following clinical laboratory values at the Screening visit:
* eGFR \< 30 mL/min/1.73 m2 (as defined by MDRD equation)
* Serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), or total bilirubin levels \> 3 × ULN
* Platelet count \< 50,000/mm3 (\< 50.0 × 109/L)
Known allergic reaction to sotatercept (ACE-011), its excipients, or luspatercept
Clareo Health | Impact of Sotatercept on Pulmonary Artery and Right Ventricle Remodeling Imaging Assessed With 68Ga-FAPI PET/CT in Patients With PAH
