Beta blockers remain the first-line therapy for hereditary long QT syndrome, with device therapy or ganglionectomy reserved for refractory or high-risk patients.
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Supports beta-blocker first-line use in long QT syndrome; leaves open optimal device or surgical thresholds in refractory cases.
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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Arthur J. Moss (1998) studied this question.
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