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Pediatric Long QT Syndrome Medication

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

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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Contributor Information and Disclosures
Author

Sreekanth S Raghavan, MBBS, , FACC Consulting Pediatric Cardiologist, Head and Director of Pediatric Cardiac Services, Manipal Heart Institute, India

Sreekanth S Raghavan, MBBS, , FACC is a member of the following medical societies: American College of Cardiology, American Society of Echocardiography, Pediatric Cardiac Society of India

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Acknowledgements

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References
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Marked prolongation of QT interval in a 15-year-old male adolescent with long QT syndrome. Abnormal morphology of repolarization can be observed in almost every lead (ie, peaked T waves, bowing ST segment). Bradycardia is a common feature in patients with long QT syndrome. R-R = 1 s; QT interval = 0.56 s; QT interval corrected for heart rate (QTc) = 0.56 s.
Genetically confirmed long QT syndrome with borderline values of QT corrected for heart rate (QTc) duration in a 12-year-old girl. Note the abnormal morphology of the T wave (notches) in leads V2-V4. R-R = 0.68 s; QT interval = 0.36 s; QTc = 0.44 s.
ECG of a 13-year-old female who had a syncopal event while running to a school bus. She awoke after a few seconds, and her subsequent clinical course was uneventful.
Table 1. Genetic Basis of Long QT Syndrome, Including Jervell and Lang-Nielsen (JLN) Syndrome
Type of Long QT Syndrome Chromosomal Locus Mutated Gene Ion Current Affected
LQT1 11p15.5 KVLQT1or KCNQ1 (heterozygotes) Potassium (IKs)
LQT2 7q35-36 HERG, KCNH2 Potassium (IKr)
LQT3 3p21-24 SCN5A Sodium (INa)
LQT4 4q25-27 ANK2, ANKB Sodium, potassium and calcium
LQT5 21q22.1-22.2 KCNE1 (heterozygotes) Potassium (IKs)
LQT6 21q22.1-22.2 MiRP1, KNCE2 Potassium (IKr)
LQT7 (Andersen syndrome) 17q23 KCNJ2 Potassium (IK1)
LQT8 (Timothy syndrome) 12q13.3 CACNA1C Calcium (ICa-Lalpha)
JLN1 11p15.5 KVLQT1or KCNQ1 (homozygotes) Potassium (IKs)
JLN2 21q22.1-22.2 KCNE1 (homozygotes) Potassium (IKs)
Table 2. Genetic Basis of Long QT Syndrome
Group Prolonged QTc (s) Borderline QTc (s) Reference Range (s)
Children and adolescents (< 15 y) >0.46 0.44-0.46 < 0.44
Men >0.45 0.43-0.45 < 0.43
Women >0.46 0.45-0.46 < 0.45
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