Bringing a newborn baby home is a joyous occasion for a family. But at the same time, it can also bring a little fear to parents. "Is the baby doing well?", "Is the baby breathing well?", "Why is he crying so much?" You may have had questions like these. While most of the time these are normal things, sometimes there are rare situations that require us to be a little more careful. Today we are going to talk about a very rare heart condition that can occur in newborn babies, but which requires immediate treatment. That is Interrupted Aortic Arch, or IAA for short.
Simply put, what is Interrupted Aortic Arch (IAA)?
To understand this, let's first take a look at how our heart works. Imagine that your heart is a four-chambered main water pump.
- The two upper chambers (Atria): Deoxygenated "blue" blood from the body enters one chamber, and oxygenated "red" blood from the lungs enters the other.
- The two lower chambers (ventricles): These are the main pumping chambers. On one side, they send oxygen-poor blood to the lungs to pick it up. On the other side, they pump oxygen-rich red blood to be distributed throughout the body.
The main blood vessel that carries oxygenated blood throughout the body is called the Aorta . It is usually shaped like a cane, or an arch.
Now, in the case of Interrupted Aortic Arch (IAA) , the arch of the aorta is incomplete. It's like the main pipe that carries water from the water tank to the entire house has broken into two parts . Because of this, no matter how hard the heart pumps, it cannot send oxygen-rich blood to the lower parts of the body. This is a very serious, life-threatening condition. Because if parts of the body don't get blood, those organs can fail and pose a great danger to the baby.
This condition is very rare. It affects about two out of every 100,000 babies born in Sri Lanka. So while it's important to be aware of this, don't panic unnecessarily.
How do you know if your baby has this condition? What are the symptoms?
A baby with IAA usually shows symptoms within the first few days after birth. This is because before birth, the baby's heart is receiving oxygen from the mother's body, which is not a problem, but after birth, the baby's heart has to work on its own. That's when this problem arises.
You should be very careful about the following symptoms.
| Symptom | Simply explained |
|---|---|
| Difficulty in breastfeeding and excessive fatigue | Even when a baby sucks a little milk, he or she seems to be struggling, sweating, and working very hard. Because even that small task requires a lot of effort from the heart. |
| Rapid breathing | Because the body is not getting enough oxygen, the baby starts breathing rapidly to compensate. The chest may appear to be throbbing. |
| Rapid heartbeat | Because the heart has to work harder to pump blood throughout the body, the heart starts to beat faster. |
| Skin turning gray or blue | Especially since oxygen-rich blood does not flow properly to the lower parts of the baby's body, such as the legs and feet, the skin in those areas may turn gray or melancholy. |
| Unusual drowsiness and weakness | If your baby is constantly sleepy and lethargic, it may be because their body is not getting the energy it needs. |
If you notice any of these symptoms, see a doctor immediately without delay. If necessary, take your baby to the nearest hospital's Emergency Treatment Unit (ETU).
Other heart defects that may occur with this condition
Babies with IAA may have several other heart defects at the same time.
- Ventricular Septal Defect (VSD): This is a hole in the wall between the two lower pumping chambers (ventricles) of the heart. Surprisingly, having a VSD can sometimes be a temporary help in cases of IAA, as it allows some oxygenated blood to pass through the hole to the body.
- Atrial Septal Defect (ASD): This is a hole in the wall between the two upper chambers of the heart.
- Truncus Arteriosus: This is where the two main blood vessels coming out of the heart (the aorta and the pulmonary artery) join together to form one.
Why does this happen to babies? What is the reason?
As parents, the first question that comes to your mind when you hear this is, "Is this our fault?" Of course not.
Interrupted Aortic Arch (IAA) is a condition caused by a genetic defect . Simply put, our chromosomes are the 'book' that contains all the instructions for how our bodies are built. In this condition, a very small piece of a chromosome that contains the instructions for how the heart and blood vessels are built is missing. This causes the aorta to not form properly.
Often, babies with IAA also have another genetic condition called DiGeorge syndrome . This is also caused by a missing part of the same chromosome. Children with DiGeorge syndrome may have frequent infections and certain facial changes (such as cleft lip).
How do doctors find this out exactly?
When you take your baby to the hospital, if the doctors examine the baby and suspect a heart condition like this, the main test to confirm it is an echocardiogram .
An echocardiogram is like watching a video of a baby's heart. It's painless. It uses ultrasound waves to clearly see the heart's chambers, valves, and blood vessels. In the case of IAA, it can clearly show the blockage in the aorta.
Sometimes, a heart defect like this can be detected during an anomaly scan (ultrasound scan) performed while the baby is still in the womb.
What are the treatments for this? Can the baby be cured?
Yes, absolutely. Although this is a serious condition, it can be completely cured with surgery . The treatment process can be divided into two main parts.
1. What to do immediately before surgery
After diagnosis, the baby is immediately admitted to a neonatal intensive care unit (NICU). The primary goal is to maintain blood flow to the body until the baby is prepared for surgery.
Doctors use a clever trick to do this. Before every baby is born, there is a temporary connection (ductus arteriosus) between the aorta and the artery that carries blood to the lungs. This closes on its own within a few hours or days after birth.
But in the case of IAA, doctors don't let this temporary connection close. It's like a 'bypass' when the main road is blocked. To keep this bypass open, a drug called Prostaglandin E1 is given to the baby in saline. This allows the baby's lower body to get some blood until the surgery.
2. Life-saving surgery
Once the baby's condition is stabilized, the surgery is performed by pediatric cardiac surgeons.
- The main thing: The two parts of the aorta that have been severed and separated are reconnected, creating a complete arch.
- Additional things: At the same time, if the baby has a VSD (a hole in the heart), it will also be closed during this surgery. The bypass (PDA) that was opened with medication earlier will also be closed, because now that the main path has been completed, there is no need for it.
This is a very complex surgery, but skilled doctors in Sri Lanka successfully perform such surgeries.
What will the baby's future be like after the surgery?
This is the most important thing. After a successful surgery, most children can live a normal, healthy life . According to statistics, even 10 years after surgery, more than 81% of people are still alive.
But the most important thing to remember is that even after surgery, you need to be under the follow-up of a cardiologist for the rest of your life . You need to go to clinics at the scheduled time and have tests like an echocardiogram to see if your heart is functioning properly. Sometimes, when you get older, you may need some treatment again. Therefore, it is essential to follow your doctor's instructions.
It's normal to feel like your whole world has come crashing down when you find out your baby has this condition. But remember, medical science is very advanced today. If the disease is diagnosed early and treated properly, your baby has a good chance of being as happy as any other child.
Take-Home Message
- Interrupted Aortic Arch (IAA) is when the main blood vessel that carries blood from the baby's heart to the body is incomplete.
- This is a very rare, but serious condition that requires immediate medical attention .
- Be very careful about symptoms like difficulty in breastfeeding, rapid breathing, and gray skin.
- This is caused by a genetic defect, not the fault of the parents.
- The treatment is surgical, and after successful surgery, most children can live a normal life .
- It is essential to remain under the supervision of a cardiologist for the rest of your life, even after surgery.
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