Inherited Cardiac Arrhythmia Syndrome: Role of Potassium Channels

Potassium channels are key players in the control of cardiac action potential, and their dysfunction can lead to atrial and ventricular arrhythmias. Mutations in genes encoding voltage-gated potassium channels, leading to their loss of function, cause long QT syndrome and Andersen-Tawil syndrome. Mutations in potassium-channel genes, leading to their gain of function, cause short QT syndrome and familial atrial fibrillation. Recently, the potassium-channel KATP has been implicated in early repolarization syndrome and in Brugada syndrome