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Approach Considerations All patients with long QT syndrome (LQTS) :should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium level. Potassium and magnesium deficiency should be corrected. Although treating asymptomatic patients is somewhat controversial, a safe approach is to treat all patients with congenital LQTS because sudden cardiac death can be the first manifestation of LQTS. Beta-blockers are drugs of choice for patients with LQTS. The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. They may also reduce the QT interval in some patients. Consultations A cardiologist and a cardiac electrophysiologist are typically consulted when patients with LQTS are evaluated. In families of patients with genotypically confirmed LQTS, genetic counseling of patients and family members should be considered. Inpatient care Patients with LQTS are frequently hospitalized in a monitored unit after they have a cardiac event (eg, syncope, cardiac arrest) to enable immediate rescue if cardiac arrhythmias recur. Follow-up Asymptomatic individuals with LQTS usually do not require hospitalization. However, carefully evaluate them and provide follow-up care in an ambulatory setting. A cardiologist or a cardiac electrophysiologist should examine patients with LQTS on a regular basis. Guidelines The following is a summary of guidelines for the management of patients with LQTS, as suggested by the American College of Cardiology, the American Heart Association, and the European Society of Cardiology, in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society[9, 10] : •No participation in competitive sports for patients with the diagnosis established by means of genetic testing only •Beta-blockers should be given to patients who have QTc-interval prolongation (>460 ms in women and >440 ms in men) and are recommended (class IIa) for patients with a normal QTc interval •An implantable cardioverter-defibrillator (ICD) should be used in survivors of cardiac arrest and is recommended (class IIa) for patients with syncope while receiving beta-blockers; ICD therapy can be considered (class IIb) for primary prevention in patients with characteristics that suggest high risk (including LQT2, LQT3, and QTc interval >500 ms)
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