The detrimental variants in BMPR2 account for nearly 50%–80% of FPAH and 10%–40% of IPAH cases in children (Kerstjens-Frederikse et al., 2013; Levy et al., 2016; Zhu et al., 2018a; Eyries et al., 2019; Zhang et al., 2019; Zhu et al., 2019; Haarman et al., 2020; Abman et al., 2022). Here, BMPR2 is linked to idiopathic pulmonary arterial hypertension.