Management of patients with the hereditary long QT syndrome.
The hereditary long QT syndrome is an inherited ion channel disorder with QT prolongation, morphologic changes in the T waves, and a relatively high frequency of syncope, T wave alternans, torsades de pointes-type ventricular tachycardia, and sudden death. Monotherapy with beta blockers is the treatment of first choice. In patients with recurrent syncope despite therapy with beta blockers, pacemakers and/or ganglionectomy may be useful in selected cases, with an implantable cardioverter defibrillator used as a fail-safe approach in high-risk patients.
Moss
A J
AJ
Department of Medicine, University of Rochester School of Medicine and Dentistry, New York, USA.
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D012307:Risk Factors