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Pediatric Hypertrophic Cardiomyopathy Medication

  • Author: Christina Y Miyake, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Jan 04, 2016
 

Medication Summary

Beta-blockers and calcium channel blockers are used to treat children with hypertrophic cardiomyopathy (HCM). In individuals with significant tachyarrhythmias, amiodarone and other class III-type antiarrhythmic agents have also been used.

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Beta-Adrenergic Blocking Agents

Class Summary

Beta-blockers may decrease outflow obstruction and increase ventricular compliance. No clear evidence indicates that they decrease sudden death. Approximately one half of patients who use beta-blockers feel improvement in symptoms.

Propranolol (Inderal LA)

 

Propranolol is a nonselective beta-blocker with a long record of use and relative safety. The treatment dose is titrated to produce clinical effect (ie, a reduction in perceived symptoms). Blunting of the maximal heart rate during exercise testing is a good marker for beta-blocker effect. Although propranolol is generally a short-acting agent, long-acting preparations are available. A stable liquid preparation is available and can be used to treat infants.

Atenolol (Tenormin)

 

Atenolol selectively blocks beta1 receptors, with little or no effect on beta2 types. It may be better tolerated than propranolol (it has more favorable pharmacokinetics and frequently has equivalent efficacy).

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Calcium Channel Blockers

Class Summary

Calcium channel blockers are an alternative to beta-blockers. They improve diastolic filling by improving diastolic relaxation and decreasing outflow gradient due to depression of cardiac contractility.

Verapamil (Calan, Isoptin, Verelan)

 

During depolarization, verapamil inhibits calcium ion from entering slow channels or voltage-sensitive areas of the vascular smooth muscle and myocardium. It may have a better effect on exercise performance. Sustained release formulations with daily dosing are available.

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Antiarrhythmics, Miscellaneous

Class Summary

Amiodarone is categorized as a class III antiarrhythmic agent but has antiarrhythmic effects that overlap all 4 Vaughn-Williams antiarrhythmic classes. Its use is generally reserved for potentially life-threatening ventricular arrhythmias.

Amiodarone (Cordarone, Pacerone)

 

Amiodarone is a complex and potent antiarrhythmic agent with multiple effects on cardiac action potential, exceedingly complex pharmacokinetics, and extracardiac pharmacodynamics. Oral efficacy may take weeks. With the exception of disorders of prolonged repolarization (eg, long QT syndrome), amiodarone may be the drug of choice for life-threatening ventricular arrhythmias refractory to beta-blockade and initial therapy with other agents.

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Contributor Information and Disclosures
Author

Christina Y Miyake, MD Assistant Professor of Pediatric Cardiology, Texas Children's Hospital

Christina Y Miyake, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Massachusetts Medical Society, Pediatric and Congenital Electrophysiology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, Heart Rhythm Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Received grant/research funds from Medtronic for consulting.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Christopher Johnsrude, MD, MS Chief, Division of Pediatric Cardiology, University of Louisville School of Medicine; Director, Congenital Heart Center, Kosair Children's Hospital

Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: St Jude Medical Honoraria Speaking and teaching

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

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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Hypertrophic cardiomyopathy. Image courtesy of Michael E. Zevitz, MD
Sarcomeric genes involved in hypertrophic cardiomyopathy (adapted from Priori 1999).
ECG of a 16-year-old with hypertrophic cardiomyopathy (HCM), demonstrating left ventricular hypertrophy pattern and "pseudo-preexcitation."
 
 
 
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