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 ch annelopathy with a propensity to arrhythmogenic syncope and sudden dea th. 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 thr ee genotypes. Current day therapy includes: beta-adrenergic blocking d rugs; pacemakers; left cervicothoracic sympathetic ganglionectomy; imp lanted cardioverter defibrillators; and possibly, drugs that improve m utant ionic channel dysfunction. LQTS has provided unique insight into the complex relationship between ionic channel dysfunction and ventri cular tachyarrhythmias.