Pediatric Ebstein Anomaly Medication

Updated: Aug 14, 2018
  • Author: Duraisamy Balaguru, MBBS, MRCP, FACC, FAAP, FSCAI; Chief Editor: Stuart Berger, MD  more...
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Medication

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 supraventricular tachycardia 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 the 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 the Medscape Drugs and Diseases topic 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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