Sotatercept, a novel fusion protein targeting the transforming growth factor-β (TGF-β) superfamily, has shown efficacy in pulmonary arterial hypertension and although PVOD is pathophysiologically distinct from IPAH, shared features of vascular remodeling raise the possibility that sotatercept or related agents may have therapeutic value in PVOD. The gene discussed is TGFB1; the disease is idiopathic pulmonary arterial hypertension.