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Pediatric Heart

Diagnosis & Treatment

Ebstein Anomaly

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How is Ebstein Anomaly Diagnosed?

Diagnosis

If a newborn baby shows signs of a heart problem, or if a pediatrician suspects the presence of a heart condition, the child will be referred to a pediatric cardiologist for a physical examination and other tests if necessary. Some children do not have signs or symptoms of Ebstein anomaly, and the doctor might suspect an issue only after hearing abnormal heart sounds during a routine physical examination.

Tests to diagnose Ebstein anomaly may include:

  • Echocardiogram, an ultrasound of the heart
  • Electrocardiogram (EKG or ECG), is a measurement of the electrical activity of the heart
  • Chest X-ray, which creates an image of the heart, lungs, and blood vessels and can show an enlarged heart
  • MRI (magnetic resonance imaging) uses magnetic fields and radio waves to create detailed images of the heart. It shows the heart chambers and valves, which may indicate potential problems.

How is Ebstein Anomaly Treated?

Treatment

Treatment options for Ebstein anomaly depend on the severity of the defect and related symptoms and include medication, non-surgical treatment, and surgery.

Medication

Medication can help improve symptoms in children with a mild condition, including medicines to help with:

Nonsurgical procedure

Children who have arrhythmias and heartbeat irregularities that cannot be controlled by medication may need a procedure to treat these heart arrhythmias.

  • Radiofrequency catheter ablation is done in children who have fast or abnormal heart rhythms. During the treatment, a small area of heart tissue is damaged intentionally (ablated) to block the abnormal signals that are causing your arrhythmia.

Surgery

Several surgical options for repairing a tricuspid valve and other defects that are associated with Ebstein anomaly are available. These surgical treatments include:

  • Tricuspid valve repair. This procedure is done when there is enough valve tissue to allow repair. The valve is reshaped to allow a normal function.
  • Tricuspid valve replacement. When the tricuspid valve of a child cannot be repaired, the valve is removed and replaced with a mechanical valve or a bioprosthetic valve, made of human or animal tissue.
    • The Cone procedure is the most advanced form of tricuspid valve repair for Ebstein Anomaly. NewYork-Presbyterian has significant expertise here.
  • Bidirectional Glenn procedure. Sometimes when the right ventricle is not functioning, a procedure to direct the blood flow from the upper body veins to the lungs is performed. It replaces the normal way in which the heart pumps the blood to the lungs.
  • Heart transplantation. In severe Ebstein anomaly cases with poor heart function, a heart transplant might be necessary.

FAQs

FAQs

Ebstein anomaly is a rare heart defect that affects the tricuspid valve. About one in 10,000 babies are born with this condition.

Babies might show signs of Ebstein anomaly at birth or in the first year of life. In some cases, Ebstein anomaly is found before birth during a fetal ultrasound. Mild cases of Ebstein anomaly might cause symptoms only later in life.

Get Care

Trust NewYork-Presbyterian for Ebstein Anomaly Treatment

At NewYork-Presbyterian’s Congenital Heart Center, we are dedicated to helping children with congenital heart conditions. We collaborate with experts in other teams at NewYork-Presbyterian to provide treatment for children with complex heart conditions. Our experienced and compassionate doctors and teams, who specialize in pediatric cardiology, pediatric cardiac surgery, and pediatric heart transplant, treat children with congenital (present from birth) heart conditions such as Ebstein anomaly. Learn more about congenital heart defects in children. Contact us to schedule an appointment.