Does your little one sometimes feel like he or she is having trouble breathing? Does he or she choke while feeding or nursing? Or does he or she make strange noises while breathing? While these may be common childhood illnesses, there could be a more serious, but treatable, condition behind this. One such rare but important condition to be aware of is called "Double Aortic Arch." Let's talk about it in detail today.
Simply put, the main blood vessel that carries blood from your baby's heart to the rest of his body is the aorta . It is usually a single, large vessel. The curved part of this vessel above the heart is called the aortic arch .
However, in a baby with a "Double Aortic Arch", this "aortic arch" is formed in two. That is, an additional blood vessel is connected to this main "aortic arch", forming a second arch. These two arches come together, forming a ring around the baby's windpipe (trachea) and food pipe (esophagus) . Imagine, what happens if this ring gets blocked? That's when the baby starts to have severe symptoms such as difficulty breathing, making noises while breathing, and difficulty swallowing food.
This "aortic arch" is formed in the very early stages of pregnancy, between two and seven weeks. During this period, the heart and blood vessels develop in a very complex way. Some parts are formed, and after a while they are no longer needed and disappear. The "double aortic arch" is formed because of something like this. Around the fifth week of pregnancy, a blood vessel that should have disappeared, for some unknown reason, does not disappear and this "double aortic arch" is formed. This is a congenital heart defect , which means that the baby is born with it.
This condition, called "double aortic arch," belongs to a group of congenital heart defects called "vascular rings." These cause the baby's windpipe and esophagus to become compressed.
What are these "vascular rings"?
Okay, "Vascular Rings" are, as I mentioned before, conditions where the "aortic arch" develops abnormally, which can cause the baby's windpipe (trachea), food pipe (esophagus), or both to become compressed. These are very rare congenital heart defects . Think about it, about one in 100 babies is born with some kind of congenital heart defect. But a "vascular ring" only affects one in 10,000 babies. It's that rare.
What are the main types of "Vascular Rings"?
There are several main types of these "vascular rings". Let's see what they are:
- Double Aortic Arch: In this, two "aortic arches" (left and right) surround the trachea and esophagus. This is the main topic we are talking about.
- Right aortic arch with aberrant left subclavian artery and left ligamentum: There is one aortic arch here, but it is on the right side of the trachea (normally on the left). In this case, the left subclavian artery, which supplies blood to the baby's arms, begins at the aorta and passes behind the trachea and esophagus. This vascular ring is completed by joining with the left ligamentum arteriosus, a remnant of a blood vessel that was present during fetal life and then disappears.
- Left aortic arch with aberrant right subclavian artery: Here, the right subclavian artery passes behind the trachea and esophagus, compressing them.
- Innominate artery compression: The innominate (brachiocephalic) artery is a branch of the aorta. In this case, it starts on the left side of the trachea, then passes in front of the trachea to the right. This puts pressure on the trachea.
- Pulmonary artery sling: The left pulmonary artery starts from the right pulmonary artery and passes between the trachea and esophagus.
What happens in all of these is that the airway or esophagus is affected in some way.
Are there types of "Double Aortic Arch"?
Yes, there are three main types of "double aortic arch." These are distinguished by which arch is larger, and whether that larger arch is on the right or left side of the trachea.
- Dominant right arch with a small left arch: This is the most common type, occurring in about 80 percent of cases.
- Dominant left arch with a small right arch: This is seen in about 10 percent of cases.
- Balanced arches: This is also seen in about 10 percent of cases.
What type of "vascular ring" is most commonly seen?
Doctors used to think that the "double aortic arch" was the most common type of "vascular ring." But new research has found that the "right aortic arch" is actually more common.
But there's something else. Babies with a "right aortic arch" are more likely to have other congenital heart defects than babies with a "double aortic arch." However, they are less likely to be diagnosed at birth. This is because a "right aortic arch" may not cause symptoms in the early stages. So, surgery for this problem may be necessary between 6 and 18 months of age.
A condition called ``Double Aortic Arch'' is the most common type of vascular ring. Therefore, your baby's doctor can diagnose the problem early. Compared to babies with other types of vascular rings, babies with ``Double Aortic Arch'' may need surgery shortly after birth.
What are the symptoms of a "Double Aortic Arch"?
Symptoms of a "double aortic arch" usually occur when the baby's windpipe, esophagus, or both are compressed. This compression can affect the baby's breathing, feeding, or heart function.
Most often, these symptoms appear in early childhood (especially if the condition is severe), but sometimes they can appear as early as three years of age, or even in adolescence.
Some children may be misdiagnosed as having asthma because the symptoms are similar. The condition is only diagnosed when asthma treatment fails and further testing is done.
Respiratory symptoms
About 91 percent of babies and children with "double aortic arch" develop these breathing-related symptoms. They are:
- A strange sound when breathing (a high-pitched sound called `stridor` or a wheezing sound called `wheezing`) .
- A cough that sounds like a "seal barking." This is similar to the cough that occurs during a condition called "croup."
- Frequent lower respiratory tract infections .
Gastrointestinal symptoms
About 40 percent of babies and children with "double aortic arch" experience these digestive symptoms. They include:
- Choking while breastfeeding or feeding.
- Difficulty swallowing (dysphagia) . This often becomes apparent after the baby starts eating solid foods.
- Difficulty gaining weight .
- Food coming back up into the throat (`Regurgitation/reflux`) .
Heart symptoms
About 30 percent of babies and children with "double aortic arch" develop heart-related symptoms. These include:
- Chest pain .
- An abnormal heart sound (`Heart murmur`) .
- Blue skin (cyanosis) .
What causes "Double Aortic Arch"?
The exact cause of "double otic arch" has not yet been discovered . Although this condition is commonly seen in babies conceived through "in-vitro fertilization" (IVF) , the exact cause is not known.
Double aortic arch can also be associated with other medical conditions. About 12 percent of babies with double aortic arch have another congenital heart defect, such as ventricular septal defect (VSD) and tetralogy of Fallot . In addition, about 20 percent of babies with double aortic arch have genetic conditions such as chromosome 22q11 deletion and trisomy 21 (Down syndrome) .
How to recognize "Double Aortic Arch"?
Double aortic arch is diagnosed either by chance or when symptoms start. Sometimes, it is discovered incidentally during pregnancy, after birth, or during imaging tests done for another reason. Since 2004, the number of people who are diagnosed with this condition through scans during pregnancy has increased. This is very useful, as treatment can be planned as soon as the baby is born.
Or, it can be diagnosed when symptoms appear after the baby is born. Your baby's doctor will talk to you about the symptoms and then do some tests.
Tests to diagnose "Double Aortic Arch"
The main tests used to diagnose this condition are:
- Chest X-ray: This is often the first test done.
- CT scan.
- Echocardiogram (Echo) test: This is most helpful in finding out if there are other heart defects.
- MRI (MRI) test.
Sometimes a bronchoscopy may be done to examine the baby's windpipe. This may be done in response to breathing difficulties before a double aortic arch is diagnosed. Or, it may be done before surgery to help the surgeon determine what is causing the problem.
Genetic testing helps identify genetic conditions.
How to cure "Double Aortic Arch"?
Surgery is required to correct the condition of "double aortic arch." Even for babies who are asymptomatic, surgery is best performed between 6 and 9 months of age. Babies who are symptomatic, especially if the symptoms are severe, may need surgery even earlier.
Just think, the condition "double aortic arch" was first identified in 1737. The first surgery for it was performed in 1947. How far we have come since then! Babies with this birth defect have been able to live long, healthy lives thanks to surgical treatment.
"Double Aortic Arch" surgery
The "Double Aortic Arch" surgery is very safe, and the results are very successful . Your baby will not need to be connected to a "cardiopulmonary bypass" (heart-lung machine) .
The surgery is done using a method called a "lateral thoracotomy." This means that the surgeon makes an incision on one side of the baby's chest, between two ribs. This is often done on the left side, because that's where most babies have that "extra" little rib cage. Imaging tests done before the surgery allow the surgeon to get a good look at the inside of the baby's body and plan the surgery accordingly.
The surgeon uses a "ligating and dividing" technique . This means that the small arch is tied to stop the bleeding through it. Then it is cut and sutured. This process is repeated until the arch is completely separated and the pressure on the windpipe and esophagus is eliminated.
Your baby will be in the hospital for about a week, then recover and come home.
Treatment of "Kommerell diverticulum" (`Kommerell diverticulum - KD`)
Rarely, some babies with a "double aortic arch" may also have something called a "Kommerell diverticulum (KD)." This is more common in babies with a "right aortic arch," but is also very rare with a "double aortic arch."
This `KD` is an `aneurysm`, a blood vessel that develops in the descending `aorta` near the `aortic arch`. This bulge can take up space and press on the baby's windpipe and food pipe. It may not cause any immediate problems. However, surgeons like to remove this as well when creating the `double aortic arch` to prevent problems later.
Treatment of other heart defects
If your baby has other heart defects that require surgery, they may be treated at the same time as the double aortic arch. This may require a procedure called a ``median sternotomy'' , which involves making an incision down the middle of the chest. Your baby's surgeon will discuss the best option for you, depending on the issues that need to be treated.
What are the possible complications of surgery?
The risk of complications from double aortic arch surgery is very low . Rarely, the baby may have difficulty feeding or persistent breathing problems. If there are any persistent breathing problems, they usually go away within a year of surgery.
The complications of surgeries to correct double aortic arch and other heart defects can vary. Discuss all of these risks and complications with your baby's surgeon.
Can "Double Aortic Arch" be prevented?
No, a condition called "double aortic arch" cannot be prevented . It is something that develops early in pregnancy and is present at birth.
What is the future of people with "Double Aortic Arch"?
Babies and children who have undergone "Double Aortic Arch" surgery have a very bright future . They can live and engage in normal activities without any restrictions.
After the surgery, your baby may continue to have noisy breathing for up to a year. This is because the baby's windpipe is not fully developed due to the "double aortic arch." So, it will take some time for the windpipe to become stronger.
Very rarely, babies and children may still have breathing symptoms more than a year after surgery.
The most important thing is to take your baby for regular checkups and tell your doctor if you have any ongoing symptoms. Long-term follow-up care is very important for every child who has had heart surgery.
When should I call my baby's doctor?
If you notice any new symptoms, or if existing symptoms seem to be getting worse, talk to your baby's doctor right away. Babies and young children change so much that it can sometimes be hard to tell what's normal and what's abnormal. But if your baby has that "seal-like" cough or a wheezing sound , tell your doctor right away. It could be a normal childhood illness. But rarely, it could be a sign of a heart problem that's affecting your baby's ability to breathe.
When should you call 911 (emergency number)?
A "double aortic arch" can put life-threatening pressure on your baby's windpipe. If your baby's skin appears blue (cyanosis) , or if your baby is having severe difficulty breathing , call 911 immediately.
What questions should I ask my baby's doctor?
If your baby is diagnosed with "double aortic arch," it's normal for you to have a lot of questions. It's important to know your baby's condition, including when surgery is needed, and whether your baby has any other health problems. You may want to ask questions like:
- When should my baby have surgery?
- Where is the surgery performed? Who is performing it?
- What symptoms should I watch out for at home?
- What can I do to make it easier for my baby to stay at home?
- Does my baby have other heart defects?
- Should I have genetic testing for my baby or the rest of my family?
- What will my baby's future be like?
What is "Aortic Arch Syndrome"? Is it different from "Double Aortic Arch"?
Yes, don't confuse the two. "Aortic Arch Syndrome" is a very rare type of "Vasculitis" . It is also known as "Takayasu's arteritis" .
"Vasculitis" is an inflammation of the walls of your blood vessels. It can occur in any blood vessel, even the smallest blood vessels, the capillaries. "Takayasu's arteritis" is an inflammation of medium-sized and large blood vessels. It most commonly occurs in the "aortic arch" and its major branches. That's why it's also called "aortic arch syndrome."
Takayasu's arteritis (also known as "aortic arch syndrome") occurs in young adults and young adults . This is a completely different problem than the congenital "double aortic arch" we discussed earlier, and the treatment required is different.
The birth of a baby is usually a time of joy and celebration. However, when you find out that your baby has a heart condition, either during pregnancy or after birth, that joy is replaced by anxiety and uncertainty. If you've just found out that your baby has a "double aortic arch," you're probably wondering what's next.
Don't worry. Your medical team will help you through all of this step by step. You've already taken the first step, which is to learn more about your baby's condition. The good news is that double aortic arch surgery is very safe and successful. If your baby has other heart defects or genetic conditions, treatment can be a little more complicated. However, with advances in newborn care, you can help your baby get the proper care they need, recover, and have a happy childhood.
So, what are the most important things to remember from what we've talked about? (Take-Home Message)
Okay, let's summarize some of the things you need to keep in mind from what we've talked about with regard to this "Double Aortic Arch":
- "Double aortic arch" is a congenital heart defect . The baby's aorta is divided into two arches, causing compression of the trachea and esophagus.
- The main symptoms are difficulty breathing, wheezing, difficulty swallowing, and frequent respiratory infections .
- This condition is diagnosed through tests such as `CT scan`, `MRI`, `Echocardiogram` . Sometimes it can even be diagnosed during pregnancy.
- The treatment is surgery . This surgery is very safe and successful.
- After surgery, the baby can live a normal, healthy life .
- If your baby has symptoms such as difficulty breathing or choking, seek medical advice immediately .
- Don't worry . With the advancement of modern medicine, this condition can be successfully treated. Your medical team will provide you with the support you need.
` Double Aortic Arch, Congenital Heart Disease, Vascular Rings, Infant Asphyxia, Infant Health, Heart Surgery
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