title: Prevention of ventricular arrhythmias in the congenital long QT syndrome.
abstract: Life-long therapy is necessary for patients with symptomatic long QT syndrome to prevent arrhythmic death. The merits and limitations of the different therapeutic modalities are discussed. beta-blockers remain the mainstay of therapy, but this medication may not be sufficient for cardiac arrest survivors and for those with the LQT3 genotype. "Genotype-specific" therapy, like potassium-channel openers for patients with inadequate potassium outflow (LQT1 and LQT2 genotypes) or sodium-channel blockers for patients with excessive sodium inflow (LQT3), significantly shortens the QT interval, but the effects of these drugs on arrhythmia prevention is less well established. Cardiac pacemakers may be especially beneficial for patients with LQT2 or LQT3 and for those with pause-dependent torsade de pointes. More important is to recognize that device programming for preventing tachyarrhythmias in patients with long QT differs from the standard pacemaker programming. Finally, implantable defibrillators with dual-chamber pacing capability are indicated for patients at high risk for arrhythmic death, including all cardiac arrest survivors.
authors.affiliation: Department of Cardiology, Sourasky-Tel Aviv Medical Center, Sackler-School of Medicine, Tel Aviv University, Weizman 6, Tel Aviv 64239, Israel. viskin_s@netvision.net.il
authors.forename: S
authors.initials: S
authors.lastname: Viskin
authors.forename: R
authors.initials: R
authors.lastname: Fish
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000008:administration & dosage*; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use*; D002304:Cardiac Pacing, Artificial / Q000379:methods*; D018849:Controlled Clinical Trials as Topic; D017147:Defibrillators, Implantable; D004554:Electric Countershock / Q000295:instrumentation; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000150:complications* / Q000151:congenital* / Q000175:diagnosis / Q000401:mortality; D008297:Male; D011379:Prognosis; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000517:prevention & control*
chemical_list: D000319:Adrenergic beta-Antagonists; D000889:Anti-Arrhythmia Agents