Approximately 80% of familial PAH (hPAH) and 20% of idiopathic cases of PAH (iPAH) are associated with mutations in the bone morphogenic type 2 receptor (BMPR2), but a penetrance of 20–30% suggests secondary stimuli, such as inflammation and thrombosis, as important contributors to EC dysfunction and PAH development [18,19,20,21]. Here, BMPR2 is linked to pulmonary arterial hypertension.