Mutations in the bone morphogenetic protein receptor 2 (BMPR2), a member of the transforming growth factor-β (TGFβ) superfamily, are the most common cause for hereditary PAH and account for ~75% of cases and have also been identified in ~25% of patients with idiopathic PAH [5,6,7]. This evidence concerns the gene BMPR2 and pulmonary arterial hypertension.