As presented in Table 3, and similar to BMPR2 carriers with PAH as described by Evans et al. [182], KCNK3 mutations carriers patients are younger than idiopathic patients at diagnosis (mean age 29 ±11.22 vs. 42 ± 17.8 years) and have a higher mean pulmonary artery pressure (76 ± 17.95 vs. 56.4 ± 15.3 mmHg) [14]. Here, BMPR2 is linked to pulmonary arterial hypertension.