Interrupted aortic arch occurs when 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 from the left ventricle then stops after one of the three blood vessels (usually between the left carotid and left subclavian arteries). The arch starts again after the blood vessel and travels the normal path down to the lower body. This gap for the aorta to get past the blood vessel is called the interruption.

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

Interrupted Aortic Arch – Causes and Diagnosis

In most cases, there is no known specific cause for interrupted aortic arch. DiGeorge syndrome is often associated with this condition. Our team of pediatric cardiologists also works closely with our clinical geneticists to explore all the possibilities if there is concern for a genetic syndrome.

An interrupted aortic arch can be diagnosed in utero or after birth, usually by:

  • Echocardiogram – an ultrasound of the heart
  • Fetal echocardiogram –  an ultrasound of the heart before birth
  • Failing the newborn pulse oximetry screen. The diagnosis would be confirmed with an echocardiogram

If this condition is discovered during pregnancy, your baby will go to the neonatal intensive care unit (NICU) after being born. An echocardiogram will be done and the baby’s breathing and oxygen level will be monitored. The baby will be put on a medication that keeps the PDA open for a while until surgery.

Care Providers

The University of Maryland Children's Heart Program team that treats ASD includes a pediatric cardiac surgeon, pediatric cardiologists, pediatric and neonatal intensive care doctors and nurses, pediatric cardiac anesthesiologists, cardiac operating room staff, cardiac nurses and many others with expertise in providing this specialized cardiac care. 

Treating Interrupted Aortic Arch

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

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 by 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 happen a few months later.  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, allowing the baby to grow bigger for future surgery.

Since the aortic valve is very small at this stage of the baby's life, it may be difficult for the heart to pump 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.

There is the chance of needing a procedure or surgery in the future.

Contact Us

  • To learn more about Maternal Fetal Heart medicine, please call 410-328-3865.
  • To make an appointment with a Children's Heart Program physician, please call 410-328-4348.