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Pediatric Ebstein Anomaly Medication

  • Author: Raymond T Fedderly, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 04, 2016
 

Medication Summary

In patients with Ebstein anomaly who are asymptomatic, the only drug therapy normally required is prophylaxis for bacterial endocarditis. In patients with congestive heart failure, digoxin and diuretics may be required. Patients with cyanosis are at an increased risk for a paradoxical embolus and may require therapy with warfarin (Coumadin) or aspirin. Patients who have paroxysmal SVT may require a beta-blocking or calcium channel–blocking agent or, possibly, a Vaughn-Williams class I or III antiarrhythmic agent.

The choice of antiarrhythmic agent somewhat depends symptom severity, tachycardia mechanism, potential medical contraindications, patient and physician preferences, and alternate therapeutic choices, such as catheter ablation. Antibiotics for endocarditis prophylaxis are required before performing procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

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Inotropic agents

Class Summary

These agents increase cardiac output.

Digoxin (Lanoxin)

 

Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

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Diuretics

Class Summary

These agents decrease pulmonary or systemic edema.

Furosemide (Lasix)

 

Increases excretion of water by interfering with chloride-binding cotransport system, which inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.

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Prostaglandins

Class Summary

Neonates with severe Ebstein anomaly and insufficient or ductal dependent pulmonary blood flow require prostaglandin E1 therapy.

Alprostadil (Prostaglandin E1, Prostin VR Pediatric injection)

 

Temporary maintenance of patency of ductus arteriosus in neonates with ductal-dependent congenital heart disease until surgery can be performed. The underlying conditions may present as cyanotic or acyanotic heart disease. Provides vasodilation by direct effects on vasculature and ductus arteriosus smooth muscle.

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Contributor Information and Disclosures
Author

Raymond T Fedderly, MD Associate Professor, Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin

Raymond T Fedderly, MD is a member of the following medical societies: American College of Cardiology, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic 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, Society for Cardiovascular Angiography and Interventions

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.

Additional Contributors

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.

References
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Frontal chest radiograph in an infant with severe Ebstein anomaly shows a large heart that leaves little space for the lung. Although the appearance is relatively nonspecific, the large heart should suggest Ebstein anomaly in the differential diagnosis.
 
 
 
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