Ebstein anomaly surgical repair

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 have the experience, skill and training to know which medication may alleviate Ebstein anomaly and when more needs to be done.


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


The Society of Thoracic Surgeons has ranked Norton Children’s Heart Institute’s pediatric heart care among the best in the region. With our network of remote diagnostic and treatment services in Kentucky and Southern Indiana, your child can stay close to home for quality care.

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 rhythms (arrhythmias)
  • Swollen arms and legs or an enlarged liver from congestive right heart failure


Your child’s cardiologist may use radiofrequency catheter ablationto 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.

Ebstein anomaly repair complications and after-care

A child who has been treated for Ebstein anomaly will need lifelong care with a cardiologist, as new heart rhythm issues may appear over time. Limiting physical activity may be necessary over time as well, which our pediatric cardiologists will discuss with you after surgery.

Why choose Norton Children’s Heart Institute

No other congenital heart surgery program in Kentucky, Ohio or Southern Indiana is ranked higher by the Society of Thoracic Surgeons than the Norton Children’s Pediatric Cardiothoracic Surgery Program.

  • 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.
  • Our board-certified and fellowship-trained pediatric cardiovascular surgeons are leaders in the field as clinicians and researchers.
  • More than 5,000 children a year visit Norton Children’s Heart Institute, affiliated with the University of Louisville, for advanced heart care.
  • Norton Children’s Heart Institute successfully performs more than 17,500 procedures a year.
  • The Society of Thoracic Surgeons ranked Norton Children’s Heart Institute among the best in the region after studying years of our patients’ outcomes and our ability to handle a range of pediatric heart conditions, including the most severe.
  • Norton Children’s Heart Institute has satellite outpatient centers in Bowling Green, Frankfort, Owensboro and Paducah; 28 tele-echocardiography locations in Kentucky and Southern Indiana; and six fetal echocardiography locations across Kentucky.
  • The American Board of Thoracic Surgery has certified the cardiothoracic surgeons at Norton Children’s Hospital with subspecialty certification in congenital heart surgery.
  • The Jennifer Lawrence Cardiac Intensive Care Unit is under construction to give our patients the most advanced cardiac intensive care unit available.
  • Our multidisciplinary approach to pediatric heart surgery brings together our specialists in cardiothoracic surgery, cardiology, anesthesiology, cardiac critical care and other areas to create a complete care plan tailored for your child.
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Norton Children’s Heart Institute

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