Understanding your congenital heart condition
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How Ebstein’s anomaly affects your heart
You blood flows around your heart and the rest of your body in one direction, like a one-way traffic system.
Valves inside your heart control the direction of your blood flow. Each valve has very small flaps of skin (called leaflets or cusps) that open and close like doors with every heartbeat. They stop the blood flowing backwards.
Ebstein’s anomaly affects your tricuspid heart valve (a valve on the right side of your heart). The valve is formed in the wrong place and the leaflets do not close properly. This means blood can flow the wrong way in your heart.
If more blood flows backwards into your right atrium (the upper chamber of your heart), it can make your atrium bigger. It can also make your right ventricle (the lower chamber of your heart) smaller and less effective than normal.
Over time, because your heart is working harder than normal, it can lead to heart failure.
- Learn more about how your heart works
Levels of Ebstein’s anomaly
Ebstein’s anomaly can be mild to severe. You’re more likely to have symptoms and need treatment if it’s severe.
You may also have other heart problems if you have Ebstein’s anomaly, such as atrial septal defect (ASD) or patent foramen ovale (PFO).
Symptoms of Ebstein’s anomaly
Some people have no symptoms except for a heart murmur.
This is a swishing noise created by the blood flowing the wrong way in your heart. Doctors can hear a heart murmur when they listen to your heart with a stethoscope.
Other signs and symptoms include:
- blue or grey lips or nails, this may not be as noticeable on darker skin
- feeling very tired (fatigue)
- irregular or extra heart beats, called arrhythmias
- shortness of breath, especially when exercising.
If you have any of these symptoms or your symptoms are getting worse, speak to your doctor. Symptoms can be a sign that your heart is working harder than normal, and you may need treatment.
Diagnosing Ebstein’s anomaly
Most people with Ebstein's anomaly will be diagnosed shortly after they’re born during a routine health check.
If you’re diagnosed as an adult, you may find out when having checks for other conditions or if you’ve been having symptoms.
Tests to diagnose Ebstein’s anomaly:
These tests do not hurt. Speak to a healthcare professional if you have any questions or worries before having them.
Causes of Ebstein’s anomaly
The cause of Ebstein’s anomaly is not known, but it happens when a baby’s heart is developing during pregnancy.
Not knowing what has caused your condition can be frustrating. It can help to find out more about the causes of congenital heart disease.
Treating Ebstein’s anomaly
The treatment for Ebstein’s anomaly depends on:
- how severe it is
- how old you are when you’re diagnosed
- what symptoms you have
- if you have other heart conditions.
If your condition is mild and you have no symptoms, you should have regular appointments to check how your heart is working. It’s important to go to all your appointments even if you feel well.
If you need treatment, you’ll usually have surgery to repair or replace your tricuspid valve. This could include:
- heart valve replacement
- valvuloplasty (a type of heart valve repair)
- annuloplasty (a type of heart valve repair).
If you have ASD or PFO you may have treatment for this at the same time.
Some people with Ebstein’s anomaly will also have abnormal heart rhythms (arrhythmias). Your doctor may recommend you have treatment at the same time, like a pacemaker or an implantable cardioverter defibrillator (ICD).
You may also be given medicine to help your heart work better and improve symptoms.
Your doctor and a specialist team will discuss the possible treatment options with you.
What happens if it’s not repaired?
Some people may not be able to have Ebstein’s anomaly repaired or need other surgeries when they’re adults. This may be because of other health conditions, like pulmonary hypertension, or their own choice. or their own choice.
If you cannot have surgery, or choose not to, your doctor may suggest medicine to help your heart work better and improve symptoms. You’ll also have regular appointments to check how your heart is working.
Your doctor can give you more information on living with Ebstein’s anomaly and how to manage your condition.
Living with Ebstein’s anomaly
It’s normal to feel anxious after being diagnosed. You may feel worried about your condition and how it will affect your life.
Most people live a normal, healthy life after having Ebstein’s anomaly repaired. It's important to go to your follow-up appointments. Your specialist team will let you know how often they need to see you to make sure your heart is healthy.
Read about the emotional impact of living with congenital heart disease.
Questions to ask your doctor
You can talk to your healthcare team about anything. It’s okay to ask questions, even things that feel difficult or uncomfortable to talk about.
You may want to ask about:
• exercising
• diet
• alcohol
• driving
• school or work
• holidays and flying
• sex and contraception
• having children
• support available to you.
It can help to write down questions when you think of them and ask at your next appointment. Having more information can help you to live well with your condition.
Staying active
Most people can exercise normally, and staying active can help you manage your health.
It’s still best to speak to your doctor if you have Ebstein’s anomaly to find out what kind of exercise is best for you.
If you’re having symptoms, like shortness of breath, you may need to change the exercise you do. If your symptoms get worse when you’re exercising, stop the exercise and speak to your doctor.
Family planning
It’s important to talk to your doctor before getting pregnant or getting a partner pregnant, if you have Ebstein’s anomaly. They can discuss options with you, make sure you’re supported and get the right care.
Read more about pregnancy and contraception.
Endocarditis
Endocarditis is a rare infection that causes inflammation of the valves and inner lining of the heart.
People with congenital heart diseases, including Ebstein’s anomaly, have an increased risk of endocarditis. To reduce the risk of developing endocarditis, your doctor or dentist might recommend antibiotics before or after any surgery or procedures.
More information and support
Your healthcare team are here to support you. You can ask them anything. It might feel uncomfortable or embarrassing to ask questions, but most people feel much better once they do.
Here are some ways you can get support.
- Read common questions about congenital heart disease. We talk about things like alcohol, mental health, preparing for surgery, and more.
- If you still have questions, speak to our cardiac nurses on our Heart Helpline by phone, callback, email or online chat (Monday to Friday, 9am to 5pm).
- You can also speak to others with heart conditions by joining a support group or online community.
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Treatments
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Support
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