Overexpression of both tissue ET-1 and ET receptors is shown in various pathologies including essential hypertension, pulmonary arterial hypertension, chronic kidney disease, and diabetes mellitus, which has led to the development of multiple selective ETAR and mixed ETAR and ETBR antagonists over the years [5, 10, 11•]. Here, EDN1 is linked to pulmonary arterial hypertension.