Referenced in: none

Automatically associated channels: Kv7.1 , Slo1

Title: Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy.

Authors: A J Moss

Journal, date & volume: , 1997 Aug , 20, 2058-60

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/9272508

Abstract
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.