title: Congenital long QT syndromes and Brugada syndrome: the arrhythmogenic ion channel disorders.
abstract: Congenital long QT syndromes (LQTS) and Brugada syndrome are hereditary disorders of cardiac ion channels which result in life-threatening cardiac arrhythmias or sudden cardiac death in patients with anatomically normal hearts. The pathogenesis of these dramatic events has been partially elucidated with the identification of the individual ion channels involved and understanding of the effect of some disease-causing mutations on the membrane currents and action potential. The clinical spectrum of congenital LQTS is broader than previously thought and involves certain patients previously diagnosed with idiosyncratic drug-induced proarrhythmia. The initial treatment for congenital LQTS patients involves beta-blockers in most cases. Indications for implantable cardioverter-defibrillator (ICD) or pace-maker (PM) implantation in selected individuals continue to evolve.
authors.affiliation: Department of Cardiology, Institute of Clinical and Experimental Medicine, Vídenská 800, Prague, Czech Republic.
authors.forename: J
authors.initials: J
authors.lastname: Nemec
authors.forename: W K
authors.initials: WK
authors.lastname: Shen
mesh_terms: D000200:Action Potentials; D001145:Arrhythmias, Cardiac / Q000209:etiology*; D002037:Bundle-Branch Block / Q000503:physiopathology / Q000628:therapy*; D002304:Cardiac Pacing, Artificial; D005260:Female; D005838:Genotype; D006801:Humans; D008133:Long QT Syndrome / Q000151:congenital* / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D015221:Potassium Channels / Q000235:genetics* / Q000502:physiology; D015222:Sodium Channels / Q000235:genetics* / Q000502:physiology; D014693:Ventricular Fibrillation / Q000503:physiopathology / Q000628:therapy*
chemical_list: D015221:Potassium Channels; D015222:Sodium Channels