Reduced endothelial bone morphogenetic protein type 2 receptor (BMPR2) signalling has been implicated in the pathobiology of pulmonary arterial hypertension (PAH). However, precisely how BMP signalling is involved in endothelial dysfunction and whether it can be exploited therapeutically remains uncertain. Here, Long et al. show that the BMPR2 ligand BMP9 prevents apoptosis, decreases angiogenesis and promotes monolayer integrity in human pulmonary arterial endothelial cells. Moreover, BMP9 effectively prevented and reversed disease in three different rodent models of PAH.