BMPR2 and idiopathic pulmonary arterial hypertension: In 2000, the International primary pulmonary hypertension Consortium and Deng et al. identified mutations in the BMPR2 gene as a cause of familial PH.16,17 By then, further investigations confirmed that mutations in BMPR2 are responsible for familial PH, with ~26% of IPAH cases also linked to BMPR2 mutations.18