Ectopia Cordis: What you need to know about the rare condition in which a baby's heart is located outside the body

When talking to the doctor during pregnancy, sometimes we come across words we have never heard of, or rare medical conditions. It is normal to feel a lot of fear and anxiety at such times. Today we are going to talk about one such very rare and serious birth defect. We call this condition Ectopia Cordis. Don't be scared when you hear this. Let's understand everything about this simply, one by one.

What exactly is Ectopia Cordis?

Simply put, Ectopia Cordis is when a baby is born with all or part of their heart located outside their body. This is a very rare condition. Just think, this condition occurs in only eight out of a million babies born worldwide.

Because this is a very serious condition, many babies born with Ectopia Cordis die in the womb or within three days of birth. However, there is a hopeful side to this story. Skilled medical teams of doctors, nurses, physiotherapists and other specialists have been able to keep some babies born with this condition alive for weeks, and sometimes even years.

What is the reason for this?

To be honest, experts still haven't found a single definitive reason for this, but they do have a few ideas.

• Developmental problems: One theory is that as the baby grows in the womb , physical changes occur that interfere with the baby's normal growth. For example, fibrous bands form in the fluid-filled sac (amniotic sac) that surrounds the baby, which can restrict the baby's growth.
• Chest development problem: Another theory is that the baby's chest is not developing properly. This prevents the heart from moving to its proper place inside the chest. This can lead to Ectopia Cordis and other heart defects.
• Association with other disorders: Ectopia cordis is often seen in conjunction with other birth defects that occur in the midline of the body. For example, it is associated with defects in the sternum, pericardium, and abdominal wall. It can also be associated with chromosomal conditions such as Trisomy 18 and Turner syndrome.
• Genetic causes : One recent theory is that some fetuses lack a specific gene called ``(BMP2)''. This gene is essential for the proper development of the heart and the front of the chest. So they believe that its deficiency could be a cause.

What are the symptoms that can be identified?

The main and most obvious symptom of Ectopia Cordis is that the heart is located outside the body. However, these babies may also have other midline defects along the midline of the body (the line from the head down).

How do doctors diagnose this?

In most cases, if your baby has Ectopia Cordis, it can be detected during a routine ultrasound scan during pregnancy. It can be detected as early as the first trimester of pregnancy. You may need to have another round of ultrasound scans to confirm the diagnosis. The results of these tests are very important for you to decide what to do next.

If you decide to continue your pregnancy, your doctor will monitor your baby's development very closely. This may include MRI ( magnetic resonance imaging).)` and `(Echocardiography)` tests will be used. Echocardiography is a special test that uses sound waves to take detailed pictures of the heart.

Doctors also classify this condition according to where the heart is located.

What are the treatments for this?

When talking about treatment options , the first thing to think about is the safety of the baby. Therefore, the only way to deliver the baby is by cesarean section (C-section). That is the best way to get the baby out without damaging the heart outside.

After birth, your baby may need extra help breathing. To do this, doctors will insert a flexible plastic tube into your baby's windpipe to ensure airflow. They may also inject a special fluid through the tube that coats the lungs to help them absorb oxygen better.

The main goal of treating Ectopia Cordis is to surgically close the opening in the chest, reinsert the heart into the chest, and repair any other heart defects. These surgeries are often not done all at once. They are done in several stages.

The first and biggest challenge is to fit the heart into a smaller-than-normal chest. After the doctor does that, he repositions the breastbone (sternum). In cases like this, a heart transplant is usually not an option.

What is "Comfort Care"?

In some cases, surgery is too risky and the chances of success are very low. In such cases, your doctor will talk to you about an option called "Comfort Care."

The aim is to minimize the pain and suffering the baby experiences and allow them to live the rest of their lives in comfort and peace. This is a special form of care that aims to provide comfort to the baby and family during this difficult time, rather than treating and curing them.