Is your baby suffering from a rare heart condition? Let's learn about Ebstein's Anomaly in simple terms.

Does your little one feel tired all the time? Does he/she seem to have trouble breathing? Does his/her skin sometimes look blue? While these can often be normal, sometimes they could be due to a congenital heart problem. Today we are going to talk about one such rare but important heart condition. It is called Ebstein's Anomaly.

What exactly is Ebstein's Anomaly?

Simply put, this is a congenital heart condition. That is, a defect in the heart that develops while the baby is still in the womb. Our heart has four chambers, two upper chambers and two lower chambers. Between these chambers are valves that act like doors. These control the flow of blood in only one direction.

Ebstein's Anomaly mainly affects the tricuspid valve, which is located between the upper chamber (right atrium) and the lower chamber (right ventricle) on the right side of the heart. Two main things happen in this condition:

1. The valve is positioned incorrectly: This tricuspid valve is positioned much lower than it should normally be, into the right lower chamber.

2. The valve leaflets are not formed properly: The valve leaflets are abnormally shaped. Sometimes they may even stick to the heart wall.

Think of what happens when a door doesn't close properly. In the same way, because this valve doesn't close properly, the blood that needs to be pumped from the lower chamber leaks back into the upper chamber.

This causes the upper chamber on the right side of the heart to become larger. Also, the lower chamber, which has a valve that is positioned lower, has less room to function properly. Over time, this enlarged right side of the heart can weaken and even lead to heart failure.

Often, children with `Ebstein's Anomaly` have other heart conditions. The most common is a hole in the wall between the two upper chambers of the heart. This is called `(Atrial Septal Defect - ASD )`.

What are the symptoms of this condition?

The range of symptoms is very wide. Sometimes, if the disease is very severe, symptoms may appear within a few days of birth. However, if the disease is not so severe, there may be no symptoms at all until adolescence.

Why is this happening? What is the reason?

In fact, it is often difficult to find a specific cause for this. But doctors believe that genetic changes may play a role. There is also a belief that exposure to certain medications such as lithium or benzodiazepines during pregnancy may also pose a small risk. But these are still in the research stage.

How does the doctor find this? (Testing)

If your child has these symptoms, the doctor will first examine the child carefully. Then, they may order several tests to determine exactly how the heart is functioning.

• Echocardiogram: This is the most important test. Similar to an ultrasound scan of a baby in the womb, it takes a detailed picture of the heart. It can show where the valves are, their shape, whether blood is leaking backward, how much, and how large the heart chambers are.
• Electrocardiogram (EKG): This measures the electrical activity of the heart. This helps detect irregular heartbeats (arrhythmias).
• Cardiac MRI: This can produce clearer, three-dimensional images of the heart and valves. This is very important in situations such as before surgery.
• Exercise Stress Test: For older children, an EKG is done while they walk on a treadmill. This helps to see how the heart responds to exertion.
• Holter Monitor: This is a small device that is worn on the body for 24 to 48 hours. It records the heart rate throughout the day and is used to detect any irregular heartbeats that occur during the day.

What are the treatments for this?

Treatment depends on the severity of the disease and the child's symptoms.

Treatment with medication

If the symptoms are not severe, the doctor may decide to monitor the child's condition. If there are signs of heart failure, they may give medications to help the heart work better or to remove excess fluid from the body. If the heartbeat is irregular, there are also medications to control it.

Surgery

If symptoms are severe or the heart gradually weakens, surgery may be necessary.

• Valve Repair: Surgeons' first choice is always to try to repair and fix the child's own valve. Using the child's own tissue, the valve flaps are adjusted so that they work properly.
• Valve Replacement: If the valve is damaged beyond repair, an artificial valve is implanted in its place. These can be made of metal (Mechanical valve) or made of animal tissue (Biological valve). If a metal valve is inserted, you will need to take blood thinners for the rest of your life.
• Other procedures: During surgery, the hole in the heart (ASD) can be closed and the part of the heart that is causing the irregular heartbeat (catheter ablation) can be treated.

What are the special things to be careful about?

A child with this condition requires lifelong monitoring by a cardiologist . Even after surgery, it is essential to visit clinics for checkups at the scheduled times.

If your child has this condition, keep an eye out for the following symptoms:

• If the skin turns blue more often.
• If the child's activity, running, and playing decreases.
• If there is swelling in the body.
• If you feel your heart beating faster.

If you notice any changes like this, inform your doctor immediately .

Also, in some cases, such as before teething, the doctor may advise children to take antibiotics to prevent heart infections. Always follow the doctor's instructions exactly.