Interrupted Aortic Arch (IAA)

The aorta is like the heart’s main highway, helping oxygen-rich blood get to the organs and tissues of the body via arteries that branch off like highway exits. But in this rare condition, the portion of the aorta that arches over the top of the heart is missing a section, creating a roadblock that prevents blood from reaching the lower part of a baby’s body. Interrupted aortic arch (IAA) is a very rare condition, affecting 2 out of every 100,000 babies and approximately 1.5% of people with congenital heart defects.

What is an interrupted aortic arch (IAA)?

In this condition, the aortic arch does not form completely. It usually isn’t a problem during pregnancy because before and during birth, a temporary blood vessel known as the ductus arteriosus carries blood to the lower half of a baby’s body. The ductus arteriosus usually closes in the first hours or days after birth, at which point the aorta takes over. But if the aortic arch is incomplete, the heart can’t get blood to the lower half of the body. A medicine called prostaglandin can temporarily reopen the ductus arteriosus until surgery can be planned.

Types of Interrupted Aortic Arch: 

  • Type A: The gap occurs just beyond the left subclavian artery
  • Type B: The most common, where the gap occurs between where the left carotid artery and the left subclavian artery branch off the aortic arch
  • Type C: The rarest form, the gap occurs just between the innominate artery and the left carotid artery 

Most children with IAA also have a ventricular septal defect (VSD), which is a hole between the two ventricles (pumping chambers), and some might have other heart defects, such as truncus arteriosus.

Experts in treating interrupted aortic arch (IAA)

Get a second opinion

Interrupted aortic arch is a complex condition that should be treated at a center that specializes in congenital heart defects. The Herma Heart Institute is one of the nation’s top pediatric heart programs and consistently delivers some of the best congenital heart surgery outcomes for even the most complex types of heart disease. If your child has already been diagnosed at another center, we invite you to get a second opinion for interrupted aortic arch (IAA) at the Herma Heart Institute.

The Herma Heart Institute is also home to our Fetal Heart Program and Adult Congenital Heart Disease Program, allowing for seamless, high quality care for IAA across your child’s lifespan.

What causes interrupted aortic arch (IAA)?

Interrupted aortic arch develops during the early stages of pregnancy. It is sometimes related to a genetic condition, such as DiGeorge syndrome, but other times there is no known cause.

Why is interrupted aortic arch (IAA) a concern?

An interrupted aortic arch can starve the lower half of the body of the oxygen-rich blood flow it needs to survive, damaging the liver, kidneys and intestines. Without proper treatment, this condition can quickly become life-threatening.

What are the symptoms of interrupted aortic arch (IAA)?

Most babies with IAA show symptoms on the first day of birth. As the ductus arteriosus closes, a baby with this condition might show the following symptoms:

  • Weak, unresponsive manner
  • Gray, cool legs
  • Weak or absent pulse in the legs and possibly left arm
  • Rapid breathing
  • Poor feeding

Always consult your child’s doctor if you have concerns or notice anything new.

How is interrupted aortic arch (IAA) diagnosed?

This condition is sometimes detected on prenatal ultrasound and can be diagnosed via fetal echocardiogram. A blood test will likely be recommended to test for a genetic condition like DiGeorge syndrome. Physical examination after your baby’s birth will also reveal important information, and tests to confirm the IAA diagnosis after birth might include:

  • Chest x-ray - Uses electromagnetic energy beams to produce images of internal tissues, bones and organs onto film
  • Electrocardiogram (ECG or EKG) - Records the electrical activity of the heart, shows abnormal rhythms and detects heart muscle stress
  • Echocardiogram (echo) - Evaluates the heart’s structure and function by using sound waves recorded on an electronic sensor to produce a moving picture of the heart and heart valves 
  • Cardiac catheterization - A small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the heart to get very detailed information about the structures inside the heart
  • Cardiac MRI - Uses a magnet, radio waves and a computer to create very detailed, 3D still and moving images of major blood vessels and the heart as it beats

Treatment for interrupted aortic arch (IAA)

Children’s IAA specialists will recommend the best treatment for your child based on:

  • Your child’s age, overall health and medical history
  • Extent of the disease
  • Your child’s tolerance for specific medications, procedures or therapies
  • Expectations for the course of the disease
  • Your opinion or preference

Treatment could include:

Medical management – After your baby’s IAA is diagnosed, your baby will be given a medication called prostaglandin to keep the ductus arteriosus open until the aortic arch can be repaired. Other medications your child might need include:

  • Digoxin - To strengthen the heart muscle, enabling it to pump more efficiently
  • Diuretics - To help the kidneys remove excess fluid from the body, which is sometimes needed when the heart isn’t working as well as it could
  • Antibiotics - To prevent a heart infection called bacterial endocarditis after routine dental work or other procedures, which can be a risk for children with certain heart defects

Surgical repair - Patients with IAA will require surgery shortly after birth to close the gap in the aorta and to patch a VSD if needed. A surgical repair is performed under general anesthesia. Sometimes the repaired portion of the aortic arch narrows over time, which might require a cardiac catheterization procedure. During that procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass a special balloon device to stretch the narrowed portion of the aorta. Your cardiologist will advise you on the best options for your child.

Postoperative care for your child

After surgery, your child will spend time in the cardiac intensive care unit (CICU). Major heart surgery on newborn babies usually requires many days to weeks in the CICU. Frequently the chest is left open and covered with a small patch. This allows for the swelling from surgery (a normal process) to improve over time. After a few days the surgical team will close your child’s chest at the bedside. During this time your baby will be on a ventilator to help with breathing, as well as sedative medicines and medicines to help the heart beat strongly. Over time the breathing tube will be removed, and your baby will be able to wake up and start feedings.

While your child is in the CICU, Children’s might use the following equipment:

  • Ventilator - This machine helps your child breathe. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while they are under anesthesia.
  • Intravenous (IV) catheters - These small, plastic tubes inserted into blood vessels provide IV fluids and important medications that help your child recover from the operation.
  • Heart lines - Small catheters are placed in the heart through the chest wall to measure pressure and provide certain medicines. These are removed at the bedside when they are no longer needed.
  • Arterial line - A specialized IV placed in the wrist, or other area of the body where a pulse can be felt, measures blood pressure continuously during surgery and while your child is in the ICU.
  • Nasogastric (NG) tube - A small, flexible tube keeps the stomach drained of acid and gas bubbles that could build up during surgery.
  • Urinary catheter - This small, flexible tube drains urine out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. Some swelling and puffiness is common after surgery, and your child might be given diuretics to help the kidneys remove excess fluids.
  • Chest tube - A drainage tube might be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding can occur for several hours or even a few days after surgery.
  • Heart monitor - This machine constantly displays a picture of your child’s heart rhythm and monitors heart rate, arterial blood pressure and other values.

Your child might need other supportive equipment while in the CICU or afterward. They will be kept as comfortable as possible with medications that relieve pain and anxiety, and the staff might also ask for your input on how best to soothe and comfort your child.

After your child is discharged from the CICU, they will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home and will receive instructions on medications, activity limitations and follow-up appointments before your child is sent home.

Care for your child at home following interrupted aortic arch (IAA) surgical repair

Most children feel fairly comfortable when they go home, and your physician might recommend pain medications, such as acetaminophen or ibuprofen, to keep them comfortable. The majority of issues for newborn care, such as feeding and care of the wound, are settled before discharge, but careful attention to feeding and your baby’s overall health is important.

Long-term outlook after interrupted aortic arch (IAA) surgical repair

The prognosis for IAA is generally good with prompt surgical repair and preventative cardiology care. Children might have some exercise limitations but can otherwise live healthy and productive lives. Your child will always need to be monitored by a cardiologist in case new problems develop, and once they reach adulthood, they should seek out a program that specializes in adult congenital heart disease like Children’s ACHD program.