Although mutations in the BMPR2 gene account for a considerable portion of patients with familial pulmonary artery hypertension (FPAH), only 20–30% of carriers with mutations in this gene suffer from PH, indicating that other factors contribute to the onset of the disease (Orriols et al., 2017; Zhao et al., 2019). This evidence concerns the gene BMPR2 and heritable pulmonary arterial hypertension.