Interrupted Aortic Arch Repair or Advancement

An interrupted aortic arch is a very rare congenital heart condition. It occurs when the curved arch-shaped section of the aorta (an important blood vessel) doesn't completely form. This part of the blood vessel is vital because it helps send oxygenated blood to the lower part of the body.

An infant with an interrupted aortic arch needs heart surgery soon after birth to close this gap and improve blood flow. At The Children's Health℠ Heart Center, our heart surgeons are exceptionally skilled at performing this complex corrective heart surgery.

What is aortic arch repair or advancement?

During aortic arch surgery, our heart surgeons rebuild the missing part of the aorta. Surgery usually takes place within an infant’s first week of life.

The aorta is the body’s largest blood vessel. In a healthy heart, the aorta carries blood upward through the ascending aorta. It then curves into an arch, which helps send blood downward through the descending aorta.

There are two ways to correct an interrupted aortic arch. Our surgeons have a lot of experience and high success rates with these methods:

Aortic arch advancement

Our doctors suture together the disconnected lower and upper ends of the aorta to make an arch.

Aortic arch repair

If the gap is too big to safely bring the ends together, our surgeons place a stretchable tube between the ends to form the arch.

What are the benefits of aortic arch repair or advancement?

When the aortic arch is missing or too small, it’s difficult for oxygenated blood to reach the legs or organs like the kidneys, liver and intestines. Surgery to rebuild the arch helps restore healthy blood flow to the body, preventing heart failure or another type of organ failure.

What are the risks of aortic arch repair or advancement?

Over time, the heart valves or blood vessels may become narrow, causing a condition called stenosis. Our doctors perform cardiac catheterization procedures to treat these problems through a catheter (thin tube) inserted into a blood vessel.

The tube used for aortic arch repair can’t grow as a child’s heart gets bigger. In the past, a child needed additional heart surgeries to replace the tube. At Children’s Health, we often use a stretchable tube that we can enlarge using cardiac catheterization.

What can I expect with aortic arch repair or advancement?

Doctors at our Fetal Heart Program have the expertise to detect an interrupted aortic arch heart defect during pregnancy. We partner with your maternity hospital to quickly transport your newborn to The Heart Center for treatment. Surgery for an interrupted aortic arch typically needs to happen within the first week of life.

What can I expect before aortic arch repair or advancement?

Each child’s surgery will be a little different depending on their unique heart structure and the types of heart defects. We dedicate time before the surgery to help you understand your baby’s heart condition and treatment plan. We make sure you know what to expect on the day of surgery and during your child’s recovery.

In the few days before surgery, we give your baby IV medicine to keep a blood vessel open between the aorta and the pulmonary artery. All newborns are born with this opening called the ductus arteriosus. It should close soon after birth. The use of medicine to prevent this closure (patent ductus arteriosus) ensures that the organs receive enough oxygenated blood to function until surgery takes place.

What can I expect during aortic arch repair or advancement?

An aortic arch surgery can take up to six hours depending on the number and complexity of the heart defects.

The majority of babies with interrupted aortic arches also have holes in the heart, such as ventricular septal defects or atrial septal defects. These openings allow oxygen-rich and oxygen-poor blood to mix inside the heart. If not corrected, a child may experience heart failure.

Your baby may also have truncus arteriosus, a heart defect that causes the aorta and pulmonary artery to fuse together. Our surgeons will close any holes or separate the arteries (if needed) during one surgery.

What can I expect after Interrupted Aortic Arch Repair or Advancement?

Babies who undergo heart surgeries at Children’s Health recover in the region’s most comprehensive pediatric cardiac intensive care unit (CICU). Your child receives dedicated care from skilled pediatric heart specialists.

As your baby recovers in our hospital, you meet with our cardiac nurses to learn how to care for your baby at home. We help you feel confident caring for a baby recovering from a heart procedure. You should be able to take your baby home within one week.

What questions should I ask my provider about Interrupted Aortic Arch Repair or Advancement?

  • How many aortic arch repair surgeries have you performed?
  • Are there other treatment options for my child’s heart condition?
  • Will my child need additional heart procedures? If so, when?
  • How soon will I be able to see my child after surgery?

Interrupted Aortic Arch Repair or Advancement Doctors and Providers

Frequently Asked Questions

  • How common is interrupted aortic arch?

    Interrupted aortic arch is an extremely rare heart defect. It accounts for about 1% of all congenital heart defects.

  • How does interrupted aortic arch and its surgical treatment affect my child’s development?

    There may be some discomfort at the surgical site immediately after the procedure, but we will advise you on the best medication and treatment to help keep your child comfortable.