Abnormalities in BMPR2 are the most commonly identified mutations in children with PAH, in approximately 50%–80% of FPAH and 10%–40% of IPAH, with a pattern of autosomal dominant inheritance (Zhu et al., 2018a; Rosenzweig et al., 2019; Zhu et al., 2019; Ivy and Frank, 2021). Here, BMPR2 is linked to idiopathic pulmonary arterial hypertension.