title: The short QT syndrome as a paradigm to understand the role of potassium channels in ventricular fibrillation.
abstract: The recently discovered hereditary channelopathy, the Short QT Syndrome (SQTS), is an important advance in clinical and molecular cardiology that has opened new doors for investigating the manner in which alterations in excitability and action potential morphology may facilitate the occurrence of ventricular fibrillation. In this brief review we address the molecular and genetic features of SQTS in which specific mutations in one of three different potassium channels involved in cardiac repolarization substantially increase the risk of life-threatening tachyarrhythmias. We then summarize new knowledge on the mechanism of wavebreak, which is the hallmark of reentry initiation, and on the role of potassium channels in the ionic mechanisms underlying cardiac excitation and its frequency dependence. The article argues for a detailed understanding of the ionic mechanisms that promote wavebreaks and stable rotors as an essential tool for successful anti-arrhythmic therapy in SQTS and other diseases leading to sudden cardiac death.
authors.affiliation: Institute for Cardiovascular Research and Department of Pharmacology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
authors.forename: M
authors.initials: M
authors.lastname: Cerrone
authors.forename: S
authors.initials: S
authors.lastname: Noujaim
authors.forename: J
authors.initials: J
authors.lastname: Jalife
mesh_terms: D000200:Action Potentials / Q000235:genetics; D000818:Animals; D002478:Cells, Cultured; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004562:Electrocardiography; D006321:Heart / Q000503:physiopathology; D006801:Humans; D008954:Models, Biological; D009154:Mutation; D015221:Potassium Channels / Q000235:genetics*; D024661:Potassium Channels, Inwardly Rectifying / Q000235:genetics; D051381:Rats; D013577:Syndrome; D014693:Ventricular Fibrillation / Q000235:genetics*
chemical_list: C489461:Kir2.1 channel; D015221:Potassium Channels; D024661:Potassium Channels, Inwardly Rectifying