Loss of function variants in channelopathy genes potassium two pore domain channel (KCNK3) [26] and ATP-binding cassette subfamily member 8 (ABCC8) [27], as well as membrane reservoir gene caveolin-1 (CAV1) [28–30], are causative for PAH. Here, KCNK3 is linked to pulmonary arterial hypertension.