Deleterious genetic variants in the KCNK3, KCNJ8, ABCC8 and ABCC9 genes (which encode the TASK1 and KIR6.1 channels and the auxiliary subunits SUR1 and SUR2, respectively) and possibly in KCNA5 (encodes for KV1.5 channels) have been associated to PAH, representing paradigmatic examples of channelopathies8. This evidence concerns the gene KCNK3 and pulmonary arterial hypertension.