Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy.
The familial long QT syndrome (LQTS) is now recognized as a genetic channelopathy with a propensity to arrhythmogenic syncope and sudden death. Three genetic mutations have been identified that involve the slow and fast delayed potassium rectifier currents and the sodium current. Distinctive ECG-T wave phenotypes are associated with each of the three genotypes. Current day therapy includes: beta-adrenergic blocking drugs; pacemakers; left cervicothoracic sympathetic ganglionectomy; implanted cardioverter defibrillators; and possibly, drugs that improve mutant ionic channel dysfunction. LQTS has provided unique insight into the complex relationship between ionic channel dysfunction and ventricular tachyarrhythmias.
Moss
A J
AJ
Department of Medicine, University of Rochester School of Medicine and Dentistry, New York, USA.
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable; D015171:Ganglionectomy; D015316:Genetic Therapy; D005838:Genotype; D006801:Humans; D007473:Ion Channels / Q000235:genetics; D008133:Long QT Syndrome / Q000188:drug therapy / Q000235:genetics / Q000601:surgery / Q000628:therapy*; D009154:Mutation / Q000235:genetics; D010138:Pacemaker, Artificial; D010641:Phenotype; D015221:Potassium Channels / Q000235:genetics; D015222:Sodium Channels / Q000235:genetics; D019462:Syncope, Vasovagal / Q000209:etiology; D017180:Tachycardia, Ventricular / Q000209:etiology
D000319:Adrenergic beta-Antagonists; D007473:Ion Channels; D015221:Potassium Channels; D015222:Sodium Channels