Of note, repeated administration of ONO-1301 attenuated pulmonary vascular remodeling and improved survival in a monocrotaline-induced pulmonary hypertension rat model [5], in association with upregulation of endogenous vascular endothelial growth factor (VEGF), hepatocyte growth factor (HGF), and stromal cell-derived factor (SDF)-1 [6]. Here, VEGFA is linked to pulmonary arterial hypertension.