Not only are deleterious mutations in BMPR2 associated with both heritable (~80%) and idiopathic (~20%) PAH [6,7,8], but decreased BMPR2 expression and signaling has also been demonstrated in other subtypes of PAH and non-PAH pulmonary hypertension (PH) in the absence of mutations [9,10]. Here, BMPR2 is linked to pulmonary arterial hypertension.