Sotatercept

In non-CHD adult PAH trials, sotatercept improved exercise capacity, pulmonary vascular resistance, NT-proBNP, and WHO functional class. Prior pivotal sotatercept trials excluded patients with unrepaired CHD and Eisenmenger syndrome, leaving uncertainty in cyanotic and erythrocytotic patients. Sotatercept is an activin-signalling inhibitor and ligand trap targeting activins in the transforming growth factor-beta superfamily. Sotatercept may act beyond vasodilatation by counterbalancing pro-proliferative signalling involved in pulmonary vascular remodelling. Known safety concerns for sotatercept include haemoglobin elevation, thrombocytopenia, epistaxis, and telangiectasia.