Ebstein anomaly or Ebstein malformation is a rare congenital (present at birth) heart defect that affects the tricuspid valve. The flaps of the valve can be abnormal in size, shape or position, causing the valve to be displaced downward. The result is a bad valve that can cause issues with the right atrium and right ventricle. An Ebstein anomaly surgical repair depends on how severe the tricuspid valve defect is and whether the child has other congenital heart conditions.

Medication may be all that’s needed to control a mild Ebstein anomaly. Children whose anomaly is severe may need surgery to repair the valve.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, have the experience, skills and training to know which medication may alleviate Ebstein anomaly and when more needs to be done.

Norton Children’s Heart Institute is the leading provider of pediatric heart care in Louisville and Southern Indiana.

Norton Children’s Heart Institute has a network of remote diagnostic and treatment services in Kentucky and Southern Indiana..

What Happens in an Ebstein Anomaly Repair?

Children with a mild anomaly may be able to use medication alone to control symptoms, including:

• Abnormal heart rate
• Abnormal heart rhythm (arrhythmia)
• Swollen arms and legs or an enlarged liver from congestive right heart failure

Your child’s cardiologist may use radiofrequency catheter ablation to treat your child’s heart rhythm and heartbeat issues that medicines cannot control.

Surgical options to repair the tricuspid valve and other defects associated with Ebstein anomaly include:

• Surgical valve repair: Pediatric cardiothoracic surgeons surgically reconfigure the shape of the tricuspid valve leaflets so the valve functions better. This is the preferred procedure because it uses the body’s own tissue and the tissue will grow with the child. In addition, if possible, the existing valve may be shifted to the best position between the atrium and ventricle.
• Tricuspid valve replacement: A pediatric cardiothoracic surgeon will replace the existing valve with a mechanical valve or a valve made of human or animal tissue. This is done only if your child’s existing valve cannot be repaired.
• Maze procedure: If your child has heart rhythm issues (dysrhythmia), the pediatric cardiothoracic surgeon may perform a maze procedure during the valve surgery. During this procedure, the surgeon can interrupt the pathway of a dysrhythmia arising in the right atrium, resulting in a healthier heart rhythm.

Why Choose Norton Children’s Heart Institute

• Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
• The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
• The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
• More than 17,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
• Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
• The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.