What is Ebstein's Anomaly
A rare birth defect that affects the tricuspid valve in the heart.
Ebstein’s Anomaly is a rare birth defect that affects the tricuspid valve in the heart. The severity of the condition varies, but there is treatment available for moderate to severe cases.
In this guide, we’ll discuss what exactly Ebstein’s Anomaly is, what the symptoms are and how your child can go on to have a healthy, normal life.
Key Points:
- Ebstein’s Anomaly is a heart condition that affects the tricuspid valve.
- It can be mild, moderate or severe and your child may not show any signs or symptoms at all.
Medicine or surgery can be used to treat the defect and children who are treated will most likely live long, healthy lives.
Ebstein Anomaly is a rare congenital heart defect that affects the function of the tricuspid valve, which is one of the four valves in the heart.
Its name comes from Wilhelm Ebstein, who first described the condition in 1866.
Learn more about Epsteins Anomaly
Ebstein’s Anomaly and children
Ebstein’s Anomaly can range from mild, with little to no symptoms, to severe; but it really depends on how much blood is able to get to the lungs.
Most children who have surgery for Ebstein’s Anomaly will go on to live normal, healthy lives. Your child may need periodic monitoring as they grow to make sure their heart is healthy and functioning correctly, as well as to ensure they don’t need any additional surgery.
What are the symptoms of Ebstein’s Anomaly?
Some children may not show any symptoms at all, with the exception of a heart murmur, which is a swishing noise caused by the blood flowing the wrong way in the heart.
Other signs and symptoms of Ebstein’s Anomaly can include:
- Blue or grey lips and nails (this sometimes less noticeable on darker skin)
- Fatigue and becoming tired easily
- Irregular heartbeats (arrhythmia)
- Shortness in breath, especially while exercising
- Slow weight gain
Symptoms of Ebstein’s Anomaly can be similar to other conditions, which is why it is important you speak to a healthcare professional to get the right diagnosis.
If the condition is mild, your child may not display symptoms until they are slightly older.

How Ebstein’s Anomaly affects your heart
In a normal heart, the tricuspid valve controls blood flowing from the top chamber and bottom chamber, i.e. Right Atrium and Right Ventricle. The valve has 3 cups, or leaflets, that open and close to allow blood to flow through while also preventing a backflow.
However, with Ebstein’s Anomaly, the leaflets are malformed and are positioned too low in the right ventricle. Sometimes the leaflets are shaped differently, with some being larger than the other.

Ebstein’s Anomaly can lead to a number of different problems, including:
- Tricuspid Valve Regurgitation – The leaflets are formed incorrectly and don’t close properly, allowing blood to flow backwards into the atrium.
- Right Atrium Enlargement – Blood flowing back into the atrium can cause it to become larger.
- Right Ventricle Shrinkage – The right ventricle can become constrained and less effective.
- Heart Failure – Over time, if left untreated, the heart working harder can result in heart failure.
- Atrial Septal Defect (ASD) – A hole in the atrial septum that separates the two upper chambers of the heart.
- Arrhythmia – Irregular heartbeat.
How is Ebstein’s Anomaly diagnosed?
Most children born with Ebstein’s Anomaly will be diagnosed shortly after birth during a routine health check, typically when a heart murmur is detected.
A heart murmur may be the sign that your child has a heart condition and they will likely be referred to a paediatric cardiologist to confirm the diagnosis through other tests, such as:
- Echocardiogram – An ultrasound of the heart
- Electrocardiogram (ECG) – This test measures the electrical activity of the heart
- Exercise Stress Test – This is a type of ECG used while you exercise to measure how the heart functions when it works hard.
- Chest X-Rays – Imaging of the chest
- MRI Scans – Imaging of the body
How is Ebstein’s Anomaly treated?
The treatment of Ebstein’s Anomaly depends on:
- The severity of the condition
- How old your child is when the condition was diagnosed
- What symptoms your child has
- If your child has other heart conditions
If your child’s condition is mild and they are not showing any symptoms, they may only need regular visits to their cardiologist to check how well their heart is working. If symptoms are showing but are mild, your child may need to take medication to help improve the heart’s function.
If your child needs treatment, they will usually have surgery to repair the tricuspid valve. This is the preferred treatment because it will use your child’s own tissue and will result in their heart healing more effectively. If repairing the valve isn’t an option, the valve can be replaced with a mechanical valve.
If your child has another heart condition, as well as Ebstein’s Anomaly, it is likely that this will also be treated during surgery. For example, if your child has ASD, the surgeon will close the hole during surgery.
What is the life expectancy of a child with Ebstein’s Anomaly?
Without the right treatment, children born with Ebstein’s Anomaly will have a shortened life expectancy of 15 to 30 years old.
However, children who receive the right treatment for Ebstein’s Anomaly will have an increased lifespan, with many going to live long, healthy and active lives. Your child’s symptoms are likely to disappear after corrective surgery, helping them to grow and develop like other children.
Living with Ebstein’s Anomaly
It is normal to feel anxious about your child after they have been diagnosed, and you may worry how it will affect their life.
Most children who have been treated will go on to live normal, healthy lives. It is essential your child goes to all of their follow up appointments as this allows doctors to see any changes in the heart and monitor your child’s development.
If you have any questions or concerns, your doctor will be more than happy to discuss them with you and explain how you may need to accommodate your child’s needs.
You may want to ask about:
- Exercise
- Diet
- School
- Holidays and flying
- Additional support available