EDN1 and pulmonary arterial hypertension: As a consequence, the endothelium switches from a quiescent to an overactive state, where it starts to secrete vasoconstrictive factors, like endothelin-1 (ET-1) [13] and thromboxane [14], and proliferative factors, like vascular endothelial growth factor (VEGF), fibroblast growth factor 2 (FGF2) [15], CXCL12 [16], and reduce the secretion of vasodilators, like nitric oxide (NO) and prostacyclin, which indicates that EC dysfunction might play a central role in the pathogenesis of PAH.