Ebstein Anomaly
Ebstein anomaly is a rare congenital heart defect – meaning a baby is born with it – that can range from mild to severe. The condition affects the tricuspid valve located between the upper and lower chambers of the right side of the heart. The pediatric heart team at University Hospitals Rainbow Babies & Children’s offers expert treatment options for Ebstein anomaly, including advanced surgical repair in severe cases.
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What is Ebstein Anomaly?
The tricuspid valve controls blood flow from the right atrium (the upper chamber that receives blood from the body) to the right ventricle (the lower chamber that pumps blood to the lungs). In Ebstein anomaly, two of the leaflets, or flaps, of the tricuspid valve are in the wrong place. This prevents the valve from working well, causing blood to leak back into the right atrium. This can enlarge the atrium and cause issues such as heart rhythm problems and heart failure. Ebstein anomaly can also occur with other heart defects, such as pulmonary valve stenosis or atresia, atrial septal defect or ventricular septal defect.
What Are the Symptoms of Ebstein Anomaly?
Ebstein anomaly can range from mild to severe. In milder cases, symptoms may not appear until the child is older. Signs of Ebstein anomaly may include:
- Skin and nails turn blue (cyanosis)
- Shortness of breath
- Growth problems
- Easily tired
- Coughing
- Rapid heartbeat
- Swelling (edema) in the legs
- Heart murmur (abnormal heartbeat)
What Causes Ebstein Anomaly?
Experts are unclear on what causes a baby to develop Ebstein anomaly in the womb. Most are caused by a chance error during the fetal development of the heart, without any apparent reason. Overall, Ebstein anomaly is very rare, occurring in about one in 10,000 babies.
Diagnosing Ebstein Anomaly
Your child’s care provider might suspect a heart condition if your newborn or infant has a bluish tint to their skin, or if they detect a heart murmur during an examination with a stethoscope. In some cases, Ebstein anomaly may also be diagnosed before birth through a fetal ultrasound. Your child may be referred to a pediatric cardiologist, who will provide a more thorough examination and order a series of tests to confirm a diagnosis.
Tests may include:
Echocardiogram (echo)
Uses sound waves to show blood flow patterns in the heart.
Cardiac magnetic resonance imaging (MRI)
Assesses the function and structure of the heart
Chest X-ray
An imaging test to look at the heart and observe its size and structure
Electrocardiogram (ECG)
An imaging test to look at the heart and observe its size and structure
Pulse oximetry
Measures blood oxygen levels through a finger monitor
Treatment for Ebstein Anomaly
Treatment options for Ebstein anomaly will vary depending on the severity of your child’s condition. Mild cases may not need treatment at all; instead, your child will be monitored closely by their doctor in case the condition worsens. Severe cases in which a child develops symptoms of congestive heart failure or arrhythmia (abnormal heart rhythm) may require surgery.
In these cases, surgical repair or replacement of the tricuspid valve may be recommended. This may be done soon after birth if your newborn is experiencing severe symptoms, but in most cases surgery can be delayed for several months or even years. Non-surgical treatments can include giving the child oxygen to treat low levels of oxygen in the blood and medications to manage an irregular heartbeat or treat heart failure.
Procedures to treat Ebstein Anomaly are performed in our state-of-the-art hybrid catheterization and surgical suites. Our facility offers cutting-edge technology, including the most advanced imaging available worldwide and 3-D capabilities. This helps our surgeons better understand and visualize each patient’s unique anatomy while making complex repairs, leading to improved patient safety and outcomes.
Follow-up Care
Most children who undergo surgery for Ebstein anomaly recover very well. They will need to be monitored by a congenital heart expert throughout childhood and into adulthood. Though children and adults with a mild form may not experience any symptoms, people with Ebstein anomaly are at an increased risk of developing heart rhythm problems. If this happens it may require limiting physical activity, medications or further surgical procedures to manage the condition.