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Management

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The management of a case of congenital LQT syndrome has two objectives:

  • preventing ventricular tachyarrhythmias in the patient:
    • beta-blockade is usually effective
    • surgical adrenergic denervation of the heart may be required
    • these interventions are effective in reducing the incidence of syncope and death

  • diagnosis of LQT syndrome in asymptomatic relatives

The use of an implantable cardioverter defibrillator should be considered.

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