Interrupted aortic arch (IAA) is a heart defect someone is born with (congenital). This occurs when there is abnormal development of the heart and aorta during pregnancy. 

The aorta is like the heart’s main highway, bringing blood out to the body. It takes oxygen-rich blood to the body through 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. This creates a roadblock that stops blood from getting to the lower part of a baby’s body.

This isn’t a problem during pregnancy. Before birth, a temporary blood vessel known as the ductus arteriosus, that connects the lung artery to the lower part of the aorta, carries blood to the lower half of a baby’s body. The ductus arteriosus usually closes in the first hours or days after birth. The aorta then takes over. But if the aortic arch is missing a section, the heart can’t get blood to the lower half of the body.

There are three types of IAA (Type A, Type B and Type C) depending on where in the aorta the defect is.

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 for, 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 lower chambers of the heart (ventricles). Some might have other heart defects as well.

Specific treatment for interrupted aortic arch will be determined by your child’s physician 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 may 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. Your child may be given other medications as well.

Surgical Repair 
Babies with IAA will require surgery in the first few weeks of life, to close the gap in the aorta. Doctors will also close a ventricular septal defect with a patch, if needed.

Cardiac Catheterization
Sometimes the repaired portion of the aortic arch narrows over time. If this happens, it might require a cardiac catheterization procedure. During that procedure, 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.

For some families, it can be helpful to come prepared with a plan before your appointment with a cardiologist. Here are things to consider documenting and having ready for your appointment:

Symptoms your child is experiencing: Sharing when these symptoms appeared and how frequently they occur is helpful to the medical team. Don’t be afraid to share symptoms that seem unrelated — congenital heart disease can affect other parts of the body.

Medications: This should also include any supplements or vitamins your child takes.

Related history: This includes family medical history and recent changes in your family’s life.

Your questions: This will help ensure you don’t forget to ask all your questions. It is okay to ask the same question several times if you are unclear. Our team is here to help you understand the big picture and make sure you walk away with your questions answered.

The prognosis for IAA is generally good with early care and treatment. 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. Once your child reaches adulthood, they should seek out a program that specializes in adult congenital heart disease. The Herma Heart Institute’s Adult Congenital Heart Disease program is the largest in the state.

Consult your child's physician regarding the specific outlook for your child.