Interrupted aortic arch is a congenital heart defect where the aortic arch, which comes from the left ventricle up towards the head then curves back to the lower body, is underdeveloped..

At the curve of the aortic arch, there are three blood vessels that come off of the aorta:

  • The innominate artery (which delivers blood to the right arm and right neck)
  • The left carotid artery (which delivers blood to the left neck)
  • The left subclavian artery (which delivers blood to the left arm)

In patients with interrupted aortic arch, the aorta comes normally from the left ventricle then stops after one of these blood vessels (usually between the left carotid and left subclavian arteries). The arch starts again and travels the normal path down to the lower body. This gap is called the interruption.

An interrupted arch is usually seen with a ventricular septal defect (VSD), which is a hole in the wall between the two ventricles. The aortic valve, which separates the left ventricle and aorta, is often small.

In most cases, there is no known specific cause for interrupted aortic arch. DiGeorge syndrome is often associated with interrupted aortic arch.

Diagnosing Interrupted Aortic Arch

Interrupted aortic arch is usually diagnosed by:

  • Echocardiogram - an ultrasound of the heart.
  • Fetal echocardiogram -  an ultrasound of the heart before birth
  • Others may not be detected until after birth
  • Failing the newborn pulse oximetry screen. The diagnosis would be confirmed with an echocardiogram

If the diagnosis of interrupted aortic arch is discovered before birth, then your baby’s delivery may be planned.

He or she will go to the neonatal intensive care unit (NICU) after birth. An echocardiogram will be done and the baby’s breathing and oxygen level will be monitored. The baby will be put on a medication called prostaglandins. This medication keeps the PDA open for a while. The same things would happen if the diagnosis is made after birth.

Eventually, the baby will require surgery.

Surgery for Interrupted Aortic Arch

Surgery for interrupted aortic arch involves a few steps that try to fix the heart abnormalities. Surgery is usually done soon after birth, typically within the first week of life. Depending on how big the baby is, surgery may be done in stages.

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The most important part of the surgery is to reconnect the aorta. The surgeon attaches the two ends of the aorta together so that it is no longer interrupted.

The other part of the surgery is closing the VSD. This involves placing a patch on the hole.

In very small babies, it may be too risky to close the hole. For those patients, VSD closure may be done a few months later. The VSD may cause the heart to work harder, pumping extra to keep up with the blood going across the VSD.

The surgeon will place a small band around the pulmonary artery during the first surgery to try to limit the blood flow that goes across the VSD. This will keep the baby from having symptoms of heart failure allowing the baby to grow bigger for the future surgery.

the aortic valve is very small, it may cause the heart to have trouble pumping blood to the body. In some cases, the valve has to be fixed or the surgeon needs to find other ways to route the blood from the left ventricle to the aorta.

Most patients who have interrupted aortic arch will not need repeat surgeries later in life, however there is always the chance of needing a procedure or surgery in the future.

Long-term Prognosis

In general, the long term prognosis for interrupted aortic arch is good. Although patients may need additional surgeries or procedures, they are not typically on a lot of medications. Their activities are typically not limited.

When one family member has a congenital heart defect, there is an increased risk in other family members. A genetic counselor can better determine what the risk would be for future pregnancies.

If the baby has DiGeorge syndrome, parents may be tested to see if they carry the gene abnormality seen in DiGeorge syndrome.

To make an appointment with a Children's Heart Program physician, please call 410-328-4348.

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