Double Aortic Arch

What is a double aortic arch?

A double aortic arch is a rare heart defect that affects infants and children. Normally your baby’s aorta (the large artery that supplies blood to their entire body) develops as one large vessel that leaves their heart. The aorta extends from your baby’s heart down into their belly. The curved part at the top, above their heart, is called the aortic arch.

In double aortic arch, there are two aortic arches, as a result of extra blood vessels attached to your baby’s aortic arch. These vessels form a second aortic arch connected to the main aortic arch. The arches form a circle that wraps around your baby’s trachea (windpipe) and esophagus (food pipe). If the circle is too tight, it can cause severe symptoms like trouble breathing, noisy breathing or swallowing difficulties.

The aortic arch forms early in pregnancy, between weeks two and seven. During this time, many complex changes happen as your heart and blood vessels develop. Some portions of the developing heart form for a short time and then go away because they’re no longer needed. The double aortic arch forms this way. It’s made of blood vessels that should’ve gone away during the fifth week of pregnancy. But for unknown reasons, those vessels stay and form the double aortic arch.

The double aortic arch belongs to a group of congenital heart defects called “vascular rings.”

Babies with double aortic arch have extra blood vessels that compress their trachea and esophagus, which can affect their breathing and feeding.

Vascular rings

Vascular rings are abnormal aortic arch formations that can compress your child’s trachea (airway) or esophagus (food pipe), or both. Vascular rings are rare congenital heart defects. About 1 in 100 babies are born with some type of congenital heart disease. About 1 in 10,000 babies are born with a vascular ring.

Types of vascular rings include:

• Double aortic arch. Two aortic arches (left and right) surround the trachea and esophagus.
• Right aortic arch with aberrant left subclavian artery and left ligamentum. There’s only one aortic arch, but it descends to the right of the trachea rather than the left (where it should usually be). In this condition, the left subclavian artery (which supplies blood to your baby’s arm) arises from the aorta and runs behind the trachea and esophagus. Along with the left ligamentum arteriosus (a remnant of a fetal blood vessel), it completes the vascular ring.
• Left aortic arch with aberrant right subclavian artery. Here, the right subclavian artery travels behind the trachea and esophagus and can compress these structures.
• Innominate artery compression. The innominate (brachiocephalic) artery branches off the aorta. In this condition, it arises to the left of the trachea and then travels to the right, in front of the trachea. This puts pressure on the trachea.
• Pulmonary artery sling. The left pulmonary artery extends from the right pulmonary artery and travels between the trachea and esophagus.

Types of double aortic arches

There are three types of double aortic arches. They’re defined by which arch is bigger and where the bigger arch is located (to the right or to the left of the trachea).

• Dominant right arch with a small left arch (80% of all cases).
• Dominant left arch with a small right arch (about 10%).
• Balanced arches with both arches being similar in size (about 10%).

Which type of vascular ring is most common?

Researchers used to think that the double aortic arch was the most common type of vascular ring. However, new research has shown that the right aortic arch is actually the most common type.

Babies with a right-sided aortic arch are more likely than babies with a double aortic arch to have other forms of congenital heart disease. But they’re less likely to be diagnosed right after birth. That’s because a right-sided aortic arch may not cause early symptoms. So, babies may not have surgery to treat the problem until six to 18 months after birth.

The double aortic arch is the type of vascular ring most likely to cause symptoms. So, your baby’s healthcare provider will diagnose the issue sooner. And your baby may need surgery sooner after birth compared with babies who have other types of vascular rings.

What are the symptoms of a double aortic arch?

Symptoms of a double aortic arch usually relate to the compression of your baby’s trachea, esophagus or both. This compression can affect your baby’s breathing, eating or heart function.

Symptoms usually appear early in infancy (especially in severe cases). But sometimes they can occur by age three or even in the teenage years.

Some children are diagnosed with asthma because the symptoms are similar. When asthma treatments don’t help, further tests can lead to the diagnosis.

Respiratory symptoms

Respiratory symptoms affect 91% of babies and children with a double aortic arch. These include:

• Noisy breathing with stridor (a high-pitched sound) or wheezing.
• A cough that sounds like a “seal-bark” similar to the cough caused by croup.
• Repeated lower respiratory tract infections.

Gastrointestinal symptoms

Gastrointestinal symptoms affect 40% of babies and children with a double aortic arch. These include:

• Choking while being fed.
• Trouble swallowing (dysphagia). This usually occurs later on when a baby begins solid food.
• Trouble gaining weight.
• Regurgitation/reflux (food comes back up from the esophagus).

Heart symptoms

Heart symptoms affect 30% of babies and children with a double aortic arch. These include:

• Chest pain.
• Heart murmur.
• Blue skin (cyanosis).

What causes a double aortic arch?

The exact cause of a double aortic arch isn’t known. It’s more common in babies conceived by in-vitro fertilization, but we don’t know why.

A double aortic arch is linked with other medical conditions. Up to 12% of babies with a double aortic arch have another congenital heart defect. These include ventricular septal defect (VSD) and tetralogy of Fallot. Up to 20% of babies with a double aortic arch can have a genetic disorder such as chromosome 22q11 deletion and trisomy 21.

How is a double aortic arch diagnosed?

A double aortic arch is diagnosed accidentally or else when symptoms begin. Sometimes it’s diagnosed during imaging tests done for other reasons, either during pregnancy or soon after birth. The number of cases diagnosed through prenatal imaging has been rising since 2004. This is helpful because treatment can be planned for soon after the baby’s birth.

A baby can also be diagnosed after birth when symptoms begin. Your baby’s healthcare provider will talk with you about the symptoms and then run some tests.

Tests to diagnose a double aortic arch

Tests to diagnose a double aortic arch include:

• Chest X-ray. This is often done first.
• CT scan.
• Echocardiogram (echo). This test is most helpful for finding other heart defects.
• MRI.

Bronchoscopy may be used to check your baby’s airway. This may happen before a double aortic arch diagnosis, in response to respiratory symptoms. Or, it may happen later on, before surgery, to see details that will help the surgeon.

Genetic testing can be useful for diagnosing genetic disorders.

How do you fix a double aortic arch?

Surgery is needed to fix a double aortic arch. Babies without symptoms should have surgery at 6 to 9 months of age. Babies with symptoms may need surgery sooner than that, especially if the symptoms are severe.

The double aortic arch was first identified in 1737, and the first surgery to fix it was in 1947. We’ve come a long way since that time! Babies with this defect can live a long and healthy life thanks to surgical repair.

Double aortic arch repair

Double aortic arch repair is safe and has excellent outcomes. Your baby doesn’t need to be on a cardiopulmonary bypass (heart-lung machine).

The surgery involves a lateral thoracotomy. This means the surgeon makes a cut on one side of your baby’s chest between two ribs. It’s usually done on the left side since that’s where most babies have the “extra” smaller arch. Imaging tests before the procedure will allow the surgeon to see your baby’s anatomy and plan accordingly.

The surgeon uses a ligating and dividing technique. This means the smaller arch is tied off to stop blood from flowing through it. The surgeon then divides (cuts) it and sews stitches. These steps are repeated until the arch is fully divided and no longer pressing on the trachea and esophagus.

Your baby will recover in the hospital for up to a week and then come home.

Repair of Kommerell diverticulum

A small number of babies with a double aortic arch also have something called a Kommerell diverticulum (KD). It’s more common in babies with a right-sided aortic arch, but it can rarely happen with a double aortic arch, too.

A KD is an aneurysm that forms in the descending aorta near the aortic arch. This aneurysm can take up space and press on a baby’s trachea and esophagus. It may not cause problems right away. But surgeons prefer to remove it while fixing the double aortic arch to prevent issues down the road.

Repair of other heart defects

If your baby has other heart defects that require surgery, the double aortic arch can be fixed at the same time. This may require a median sternotomy, which is a vertical incision in the middle of the chest. Your baby’s surgeon will discuss the best approach with you, based on the problems that need treatment.

Complications of surgery

The risk of complications from double aortic arch repair is low. Rarely, a baby may have trouble with feeding or have persistent breathing problems. Any lingering breathing problems usually go away within a year after the surgery.

Complications from surgeries that repair other heart defects along with a double aortic arch vary. Ask your baby’s surgeon to discuss all risks and complications with you.

How can a double aortic arch be prevented?

A double aortic arch can’t be prevented. It forms early in pregnancy and is present at birth.

What is the outlook for people with a double aortic arch?

Babies and children who have double aortic arch repair have an excellent outlook. They enjoy a normal life and activities with no limitations.

After surgery, your baby may have persistent noisy breathing for up to one year. That’s because a double aortic arch prevents your baby’s trachea from developing properly. So, it takes some more time for the trachea to get stronger.

Rarely, babies and children have respiratory symptoms more than one year after surgery.

Make sure to take your baby for regular check-ups and tell their provider about any lingering symptoms. Long-term follow-up care is important for all children who have heart surgeries.

When should I call my baby’s doctor?

Call your baby’s doctor if you notice any new or worsening symptoms. Babies and children go through so many changes that it can be hard to tell what’s normal and what’s not. But if your baby has the telltale “seal-bark” cough or noisy breathing, tell their doctor right away. It may just be a normal childhood illness. But in rare cases, it could be a sign of a heart problem that’s affecting your baby’s ability to breathe.

When to call 911

A double aortic arch can put life-threatening pressure on a baby’s windpipe. Call 911 if your baby’s skin looks blue (cyanosis) or if your baby is visibly struggling to breathe.

What questions should I ask my baby’s doctor?

If your baby has been diagnosed with a double aortic arch, you’ll have many questions. It’s important to learn more about your baby’s condition and when surgery will be needed. You also need to know if your baby has other health issues. You may want to ask:

• When will my baby need surgery?
• Where will the surgery take place, and who will provide it?
• What symptoms should I look out for at home?
• How can I make my baby comfortable at home?
• Does my baby have other heart defects?
• Should my baby or others in our family have genetic testing?
• What’s the outlook for my baby?

What is aortic arch syndrome?

Aortic arch syndrome is a very rare form of vasculitis. It’s more commonly known as Takayasu's arteritis.

Vasculitis is an inflammation of your blood vessel walls. It can happen in any of your blood vessels, including the tiniest ones called capillaries. Takayasu’s arteritis is inflammation that affects medium-sized and large blood vessels. It commonly occurs in the aortic arch and its major branches. So, it’s been called aortic arch syndrome.

Takayasu’s arteritis (aortic arch syndrome) develops in teenagers and young adults. It’s a different problem than the congenital double aortic arch, and it needs different treatment.

A note from Cleveland Clinic

Usually, the birth of a baby brings joy and celebration. When your baby is diagnosed with a heart problem during pregnancy or soon after birth, the mood can shift to worry and uncertainty. If you’ve just found out your baby has a double aortic arch, you’re probably wondering what comes next.

Your healthcare team will help you take things one step at a time. You’ve already taken the first step by learning more about your baby’s condition. And the good news is that surgery for a double aortic arch is safe and highly successful. If your baby has other heart defects or a genetic syndrome, treatment may be a bit more complex. But advances in neonatal care can help your baby get the proper care, recover and enjoy a happy childhood.