• J. Cardiovasc. Electrophysiol. · Jun 1998

    Case Reports

    Management of patients with the hereditary long QT syndrome.

    • A J Moss.
    • Department of Medicine, University of Rochester School of Medicine and Dentistry, New York, USA.
    • J. Cardiovasc. Electrophysiol. 1998 Jun 1;9(6):668-74.

    AbstractThe 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.

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