To better understand the mechanism by which ActRIIA-Fc exerts vascular anti-remodeling effects, in the present study we used RNA-seq and pathway analyses to determine the impact of ActRIIA-Fc therapy on the pulmonary gene signature in severe experimental PAH and to determine the degree to which this model of severe angio-obliterative PAH resembles the gene signature in PAH patients. This evidence concerns the gene ACVR2A and pulmonary arterial hypertension.