Indeed, the identification of heterozygous loss-of-function mutations in the KCNK3 (potassium two pore domain channel subfamily K member 3) gene that encodes the tandem of P domains in weak inward rectifier K+ channels (TWIK)-related acid-sensitive potassium channel 1 (TASK1) and loss-of-function mutation in the ABCC8 (coding for adenosine triphosphate (ATP)-sensitive potassium channel subunit) as a cause for PAH, has revived interest in the concept of channelopathy [6]. The gene discussed is KCNK3; the disease is channelopathy.