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J Cardiovasc Electrophysiol. 1998 Jun;9(6):668-74.

Management of patients with the hereditary long QT syndrome.

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  • Department of Medicine, University of Rochester School of Medicine and Dentistry, New York, USA.


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.

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