title: [Short QT syndrome as an inherited condition].
abstract: Inherited ion-channel disorders can lead to life-threatening cardiac arrhythmias. A recent, rare entity has been discovered and termed short QT syndrome due to its electrocardiac features in conjunction with atrial and ventricular tachyarrhythmias as well as syncope and sudden cardiac death. The basis of the new syndrome is genetic and this review covers the genes responsible for the condition as well as the pathophysiology and diagnostic challenges involved in the syndrome. Furthermore, treatment for this new arrhythmic syndrome is reviewed.
authors.affiliation: Sektion for Forskning og Udvikling, Klinisk Biokemisk og Immunologisk Afdeling, Statens Serum Institut, 85/141, Artillerivej 5, 2300 København S, Denmark. dvm@ssi.dk
authors.forename: Daniel Vega
authors.initials: DV
authors.lastname: Møller
authors.forename: Paula L
authors.initials: PL
authors.lastname: Hedley
authors.forename: Morten
authors.initials: M
authors.lastname: Olesen
authors.forename: Jørgen
authors.initials: J
authors.lastname: Kanters
authors.forename: Jesper Hastrup
authors.initials: JH
authors.lastname: Svendsen
authors.forename: Michael
authors.initials: M
authors.lastname: Christiansen
mesh_terms: D000200:Action Potentials / Q000235:genetics / Q000502:physiology; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology / Q000628:therapy; D020746:Calcium Channels, L-Type / Q000235:genetics; D000072237:ERG1 Potassium Channel; D004562:Electrocardiography; D051638:Ether-A-Go-Go Potassium Channels / Q000235:genetics; D006801:Humans; D051657:KCNQ1 Potassium Channel / Q000235:genetics; D015221:Potassium Channels / Q000235:genetics; D024661:Potassium Channels, Inwardly Rectifying / Q000235:genetics; D013575:Syncope; D013577:Syndrome
chemical_list: C079146:CACNA1C protein, human; C107597:CACNB2 protein, human; D020746:Calcium Channels, L-Type; D000072237:ERG1 Potassium Channel; D051638:Ether-A-Go-Go Potassium Channels; C489457:KCNJ2 protein, human; D051657:KCNQ1 Potassium Channel; C496909:KCNQ1 protein, human; D015221:Potassium Channels; D024661:Potassium Channels, Inwardly Rectifying