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What is Ebstein’s Anomaly?

Ebstein’s Anomaly, a rare congenital heart defect, occurs when the tricuspid valve of the heart does not form correctly. Normally, the tricuspid valve has three flaps that allow blood to flow from the right atrium of the heart into the right ventricle. In Ebstein’s Anomaly, one or two flaps are malformed, which causes blood to leak backwards into the right atrium.

This heart defect can cause oxygen-poor blood to be pumped out to the body. Ebstein’s Anomaly can also cause the fetus’ heart to be enlarged, which may impact lung development.

Some children with Ebstein’s Anomaly have Wolff-Parkinson-White syndrome, a syndrome that leads to heart rhythm disorders. Common symptoms after the baby is born with Ebstein’s include bluish skin, failure to thrive, breathing problems, and a fast heartbeat.

How is Ebstein’s Anomaly diagnosed?

During pregnancy, Ebstein’s Anomaly is diagnosed through fetal echocardiogram, if the tricuspid valve looks abnormal or if the heart is dilated and the tricuspid valve is leaking. The impact of Ebstein’s Anomaly can range from mild to severe. Because of this, an experienced fetal heart team should help you decide on the best possible course of treatment.

How is Ebstein’s Anomaly in a fetus monitored during pregnancy?

Because severe cases of Ebstein’s Anomaly carry an increased risk of fetal demise compared to other congenital heart defects, close monitoring through fetal echocardiograms and ultrasound is important. Regular monitoring provides the Fetal Heart Program team with a picture of the fetus’ overall well being, and can alert them to any changes that would necessitate an early delivery, or an EXIT (Ex Utero Intrapartum Treatment) procedure to ease the transition from fetal to neonatal life.

How does Ebstein’s Anomaly impact delivery?

Most babies with Ebstein’s Anomaly can be delivered vaginally, unless there are obstetric indications for another mode of delivery. It is best for these babies to be delivered at a medical center where there is immediate access to a team of pediatric cardiologists and neonatologists, along with an established NICU (Neonatal Intensive Care Unit).

After delivery, the baby may need PGE, a special medicine that maintains patency of the ductus arteriosus after birth, which allows for blood flow from the aorta to the pulmonary arteries.

The Fetal Heart Program at the St. Louis Fetal Care Institute has the experience, facilities and technology to handle the most medically challenging deliveries. In the most severe cases the team can perform cesarean sections and EXIT procedures at SSM Cardinal Glennon Children’s Medical Center. The EXIT procedure allows the fetal and cardiothoracic surgeons to perform life-saving procedures on the baby before cutting the umbilical cord. If additional intervention is needed, the baby is next door to the pediatric operating room and the pediatric hybrid cardiac catheterization suite.

What are the treatments and surgery for Ebstein’s Anomaly?

For mild cases, medication may be all your child needs to control his or her symptoms. For more severe cases, if your child’s health is in danger, surgery is necessary.

There are a few possible surgeries, depending on the nature of the heart defect, and the age of the child at the time of surgery:

Surgical valve repair: If the tricuspid valve is salvageable, the surgeon repairs the structure of the tricuspid valve.

Starnes procedure: If the tricuspid valve is not salvageable, the surgeon sews over the tricuspid valve to completely block off flow to the right ventricle, and then adds a pulmonary shunt to provide flow to the lungs. After this procedure, your child will need additional surgeries later in life to separate oxygen poor and oxygen rich blood.

Tricuspid valve replacement: If the tricuspid is too malformed to be repaired, surgeons can replace the existing valve with either a mechanical one or a bioprosthetic valve.

Radiofrequency ablation for arrhythmias: Older children who have arrhythmias and heartbeat irregularities that medication can’t effectively control may need this minimally invasive procedure. During the procedure, the cardiologist places the tip of a catheter into the heart, through a vein. Radio waves travel through this to eliminate the tissue area that is causing the irregular heart beats.

What happens after surgery?

Many children who have Ebstein’s Anomaly will require monitoring and many need medication throughout their life. However, many develop normally following surgery. Those with more severe cases may need additional surgeries throughout their lifetime.

What is the long-term prognosis for children with Ebstein’s Anomaly?

The long-term prognosis after surgery for Ebstein’s Anomaly is good, in many cases. After surgery, most children will be able to lead relatively normal, healthy, and full lives. The prognosis is more guarded for babies with poor lung development due to significant cardiomegaly.