In the last two decades, more than 20 genes have been identified to be associated with a genetic predisposition to PAH, including two genes that encode for potassium channel proteins: KCNK3 (Potassium Two Pore Domain Channel Subfamily K Member 3) and ABCC8 (ATP-binding cassette subfamily C member 8) (3–5). Here, ABCC8 is linked to pulmonary arterial hypertension.