Pediatric Ebstein Anomaly Treatment & Management

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

Medical Care

Asymptomatic patients with Ebstein anomaly who have mild tricuspid regurgitation need only outpatient clinic evaluation, which may include periodic electrocardiographic (ECG) evaluation, 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, these newborns 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.

Electrophysiologic 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% to 90%, and the recurrence rate is reported to be as high as 32%. [16] 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.

Outpatient monitoring

Patients who are asymptomatic initially may develop increasing cyanosis or congestive heart failure. They require continued outpatient monitoring.

Adult patients are best served by having their follow-up at a center with adult congenital heart disease specialists and technologists. [17]

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Surgical Care

The surgical care of patients with Ebstein anomaly depends on the severity of the leaflet displacement and on the degree of the associated right ventricular (RV) outflow tract obstruction.

In neonates with the most severe form of Ebstein anomaly, the functional RV 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. This surgical repair is akin to single-ventricle physiology.

In infants with mild-to-moderate tricuspid regurgitation and severe RV outflow tract obstruction, a systemic artery–to–pulmonary artery shunt is performed in addition to creation of an unrestrictive atrial communication. In patients with moderate-to-severe tricuspid regurgitation, the abnormal valve can be replaced with a mechanical or prosthetic valve, surgical reconstruction, or a combination of the two. [18, 19, 20]  This type of surgical approach will result in two-ventricle physiology.

In some infants and children, a one and a half ventricle repair strategy may be used, in which a bidirectional Glenn operation is performed in addition to closing the atrial septal defect/patent foramen ovale (ASD/PFO) and repair of the tricuspid valve. Bidirectional Glenn operation decreases the volume load, allowing the hypoplastic RV handle venous return from inferior vena cava only. This strategy is suitable for certain select patients. [21]

Brown et al described their results from 539 patients (mean age, 24 y) who had 604 cardiac operations. [22]  The first repair in 182 patients consisted of tricuspid valve repair, and 337 patients underwent tricuspid valve replacement. Late survival was 84.7% at 10 years and 71.2% at 20 years. Preoperative characteristics associated with mortality included increased hematocrit levels, an associated mitral valve repair, prior cardiac operation, and moderate-to-severe reduction in RV systolic function. [22]

Another study evaluated the outcomes of 32 patients who underwent surgery for Ebstein anomaly. [23] After 16-years' follow-up, the results for early and late survival rates for patients without pulmonary atresia were 60% ± 12% versus 85% ± 10 (P = .06), respectively, and those for early and late survival rates in patients with pulmonary atresia were 60% ± 12% (9/15) versus 94.1% (16/17) (P< .05), respectively. The investigators concluded that biventricular and tricuspid valve repair of Ebstein anomaly is feasible in neonates and young infants with good results, especially those without pulmonary atresia. [23]

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Diet and Activity

Diet

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

Activity

The activity restrictions of patients with Ebstein anomaly depend on the severity of the leaflet displacement. If the displacement is mild and patients do not have an associated paroxysmal supraventricular tachycardia, 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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