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, 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.

The Society of Thoracic Surgeons has rated Norton Children’s Heart Institute’s pediatric heart care among the best in the region. 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:

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.

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 rated higher by the Society of Thoracic Surgeons than the Norton Children’s Heart Institute Pediatric Cardiothoracic Surgery Program.

  • Norton Children’s Hospital is 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 UofL School of Medicine, for advanced heart care.
  • Norton Children’s Heart Institute successfully performs more than 17,500 procedures a year.
  • The Society of Thoracic Surgeons rated Norton Children’s Heart Institute among the best in the region after studying years of our patients’ outcomes and our ability to treat a range of pediatric heart conditions, including the most severe.
  • Norton Children’s Heart Institute has satellite outpatient offices in Ashland, Bowling Green, Campbellsville, Elizabethtown, Frankfort, London, Madisonville, Murray, Owensboro, Paducah and Shepherdsville in Kentucky; as well as Corydon, Jasper, Madison and Scottsburg in Indiana; 28 tele-echocardiography locations in Kentucky and Southern Indiana; and six fetal echocardiography locations across Kentucky.
  • The American Board of Thoracic Surgery has awarded the cardiothoracic surgeons at Norton Children’s Hospital with subspecialty certification in congenital heart surgery.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
  • 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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Heart team helps firefighter ‘feel 100%’ after surgery for Ebstein anomaly

Luke Coward was born with Ebstein anomaly of the tricuspid valve, an extremely rare congenital heart condition that accounts for less than 1% of all congenital heart conditions. From the time he was diagnosed at […]

Read Full Story

Collaborative heart care helps Indiana boy’s Ebstein anomaly

Audrey Sims’ first clue that her twins’ birth would be complicated came at 14 weeks of pregnancy, when a routine ultrasound found that one of her sons, Aiden, had a blocked lymph node, which can […]

Read Full Story

We take a lot of deep breaths

I found out when I was 23 weeks pregnant that Joslyn had Ebstein’s anomaly, a congenital heart defect that I also have. We were under the impression that it wasn’t hereditary — but evidently that’s […]

Read Full Story
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Heart team helps firefighter ‘feel 100%’ after surgery for Ebstein anomaly

Luke Coward was born with Ebstein anomaly of the tricuspid valve, an extremely rare congenital heart condition that accounts for less than 1% of all congenital heart conditions. From the time he was diagnosed at […]

Read Full Story

Collaborative heart care helps Indiana boy’s Ebstein anomaly

Audrey Sims’ first clue that her twins’ birth would be complicated came at 14 weeks of pregnancy, when a routine ultrasound found that one of her sons, Aiden, had a blocked lymph node, which can […]

Read Full Story

We take a lot of deep breaths

I found out when I was 23 weeks pregnant that Joslyn had Ebstein’s anomaly, a congenital heart defect that I also have. We were under the impression that it wasn’t hereditary — but evidently that’s […]

Read Full Story

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