Pediatric Long QT Syndrome Medication

Updated: Jun 26, 2014
  • Author: Sreekanth S Raghavan, MBBS, , FACC; Chief Editor: Stuart Berger, MD  more...
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Medication

Medication Summary

Hobbs et al found that in patients who had suffered syncope in the previous 2 years, beta-blocker treatment was associated with a 64% risk reduction for aborted cardiac arrest and sudden cardiac death during adolescence. [4] However, there seems to be variation in the efficacy in preventing cardiac events among the different classes of beta-blockers, and metoprolol seems to have the greatest risk of recurrent cardiac events. [21]

The data favor treating asymptomatic patients younger than 40 years at the time of diagnosis with beta-adrenergic blockers. Sodium channel blockers are promising agents under investigation.

Risk of cardiac events increases during pregnancy and the postpartum period. Because of this increased risk, pregnant women with long QT syndrome should be treated with beta-blockers. [22] Physicians should be aware that high doses of beta blockade in the second and third trimesters may cause neonatal bradycardia at birth. Propranolol and nadolol are the preferred beta-blockers during pregnancy.

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Beta-Adrenergic Blocking Agents

Class Summary

These agents currently represent the first-choice therapy in patients with symptomatic long QT syndrome unless specific contraindications are present. Patients with long QT syndrome who are unable to take beta-blockers may require an ICD as first-line therapy.

Propranolol (Inderal, InnoPran XL)

Propranolol reduces the effect of sympathetic stimulation on the heart. It decreases conduction through the atrioventricular (AV) node and has negative chronotropic and inotropic effects. Consult a cardiologist because dosing practice varies and is individualized in patients with long QT syndrome. Patients with asthma should use cardioselective beta-blockers. Patients with long QT syndrome who are unable to take beta-blockers may require an ICD as first-line therapy.

Nadolol (Corgard)

Nadolol is frequently prescribed because of its long-term effect. It reduces the effect of sympathetic stimulation on the heart. Nadolol decreases conduction through the AV node and has negative chronotropic and inotropic effects. Consult a cardiologist because dosing practice varies and is individualized in patients with long QT syndrome. Patients with asthma should use cardioselective beta-blockers. Patients with long QT syndrome who are unable to take beta-blockers may require an ICD as first-line therapy.

Metoprolol (Lopressor, Toprol XL)

Metoprolol is a selective beta1-adrenergic receptor blocker that decreases the automaticity of contractions. During IV administration, carefully monitor blood pressure, heart rate, and ECG. Consult a cardiologist because dosing varies and is individualized in patients with long QT syndrome. Patients with long QT syndrome who cannot take beta-blockers may require an ICD as first-line therapy.

Atenolol (Tenormin)

Atenolol selectively blocks beta1-receptors, with little or no effect on beta2 types. Consult a cardiologist because dosing varies and is individualized in patients with long QT syndrome. Patients with long QT syndrome who cannot take beta-blockers may require an ICD as first-line therapy.

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