Journal of cardiovascular electrophysiology
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J. Cardiovasc. Electrophysiol. · Jun 1998
Case ReportsManagement 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.