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Pediatric Ebstein Anomaly Treatment & Management

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

Medical Care

Asymptomatic patients with Ebstein anomaly who have mild tricuspid regurgitation need only outpatient clinic evaluation, which may include periodic ECG, chest radiography, and oxygen saturation measurement. All patients with this diagnosis require lifetime prophylaxis for bacterial endocarditis.

Neonates with severe Ebstein anomaly initially require admission to a neonatal intensive care unit (NICU) for stabilization. If pulmonary blood flow is insufficient or ductal dependent, they also require prostaglandin E1 therapy.

In addition, it is crucial that neonates with a severe form of this disease have an adequate atrial communication. If the patient is born with only a patent foramen ovale (PFO) or a restrictive atrial septal defect (ASD), a balloon atrial septostomy or urgent surgical intervention may be required. Atrial septostomy can be accomplished at the bedside with echocardiographic guidance or in the cardiac catheterization laboratory, under echocardiographic and/or fluoroscopic guidance.

Electrophysiological studies are performed both as a diagnostic tool to determine the cause of an arrhythmia and as a curative procedure using radiofrequency catheter ablation. Catheter ablation for paroxysmal supraventricular tachycardia (SVT) is highly successful in children, with a low complication and recurrence rate; however, the subset of patients with Ebstein anomaly and SVT has been shown to be more challenging to cure, likely because of the derangement in tricuspid valve alignment with the tricuspid annulus and the increased likelihood of multiple accessory pathways.

The reported acute success rate in the Pediatric Radiofrequency Ablation Registry and other sources ranges from 75-90%, and the recurrence rate is reported to be as high as 32%.[5] As expected, success rates, complications, and recurrence rates vary with complex pediatric radiofrequency catheter ablation procedures, depending on operator and institutional experience. Radiofrequency ablation appears to be most successful in patients with a mild degree of tricuspid regurgitation.

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Surgical Care

The surgical care of these patients depends on the severity of the leaflet displacement and on the degree of associated right ventricular outflow tract obstruction.

In neonates with the most severe form of Ebstein anomaly, the functional right ventricle is hypoplastic, and the patient is usually best treated by closing the tricuspid valve and, in effect, creating a tricuspid atresia physiology (Starnes procedure). In addition, these infants require a systemic artery–to–pulmonary artery shunt. When the patient is aged approximately 6 months, a bidirectional Glenn procedure (superior vena cava–to–pulmonary artery anastomosis) and shunt takedown is performed. Fontan completion (inferior vena cava–to–pulmonary artery anastomosis) is usually performed when the patient is aged 2-4 years.

In infants with mild-to-moderate tricuspid regurgitation and severe right ventricular outflow tract obstruction, a systemic artery–to–pulmonary artery shunt is performed in addition to an unrestrictive atrial communication being created. In patients with moderate-to-severe tricuspid regurgitation, the abnormal valve can be replaced with a mechanical or prosthetic valve, a surgical reconstruction, or a combination of the two.[6, 7, 8]

In a study by Brown et al from the Mayo clinic the results of 539 patients who had 604 cardiac operations is described.[9] The mean age of this group was 24 years. The first repair consisted of tricuspid valve repair in 182 patients and tricuspid valve replacement in 337 patients. Late survival was 84.7% at 10 years and 71.2% at 20 years. Preoperative characteristics associated with mortality included increased hematocrit levels, associated mitral valve repair, prior cardiac operation, and moderate-to-severe reduction in right ventricular systolic function.

One study evaluated the outcomes of 32 patients who underwent surgery for Ebstein anomaly. After 16-year follow-up, the results found early and late survival rates of 60% ± 12% versus 85% ± 10 (P = .06), respectively, for patients without pulmonary atresia and early and late survival rates of 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively, for those with pulmonary atresia. The results conclude that biventricular and tricuspid valve repair of Ebstein anomaly is feasible in neonates and young infants with good results, especially those without pulmonary atresia.[10]

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Diet

Special dietary restrictions are not usually required. An infant with severe tricuspid regurgitation may require a high caloric density formula.

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Activity

The activity restrictions of these patients depend on the severity of the leaflet displacement. If the displacement is mild and patients do not have an associated paroxysmal SVT, they should be allowed to determine their own level of activity. For patients with cyanosis, sports participation is usually somewhat restricted. An exercise stress test and other noninvasive assessments might be helpful in making this determination.

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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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