Among rare inherited variants, variants in BMPR2 are causal in ~6.5–7% of both pediatric- and adult-onset PAH; most of the cases are FPAH or idiopathic PAH (IPAH), rarely PAH associated with other diseases (APAH) [11,22], and no occurrences in PPHN have been reported to date [23]. Here, BMPR2 is linked to heritable pulmonary arterial hypertension.