Interrupted aortic arch repair

Also known as: IAA surgery.

What is interrupted aortic arch repair?

Interrupted aortic arch repair is a surgical procedure to fix interrupted aortic arch (IAA). IAA is a heart defect in which the aorta is incomplete. The aorta is the main artery that carries blood with oxygen out of the heart to the body. In a child with IAA, there is a disconnection between the top part of the aortic arch and the lower, descending aorta. Arteries that deliver blood to the head, arms and other parts of the upper body branch off at the top of the arch. Arteries that deliver blood to the abdomen, legs, and other parts of the lower body branch off from the lower, descending aorta.

A newborn can survive with a disconnection in the aorta as long as a blood vessel called the ductus arteriosus remains open. The ductus arteriosus is an alternate route for oxygenated blood to reach the lower body. This vessel exists in the fetus, but closes within hours or days of birth. After it closes, an infant with interruption of the aortic arch will quickly become very sick without medical intervention. This condition is life-threatening.
 

What happens during the procedure?

Surgery will occur as soon as possible, once your baby is stabilized. The cardiac surgeons will make the aorta into a continuous vessel by attaching the top part of the aortic arch and the lower part of the aorta together. As the child grows, heart tissue will grow over the patch and the stitches.

Surgery varies depending on the child’s heart anatomy; more than one surgery may be required. The Heart Program team will explain the procedures to you in detail.
 

Is any special preparation needed?

Before the surgery, the baby is given medication called prostaglandin E1 to keep the patent ductus arteriosus open. This helps keep the baby alive until the procedure can be performed.
 

What are the risk factors?

There are significant risks related to the surgery, but the outlook is generally good. Without the procedure, there is no chance of survival.
 
Interrupted aortic arch repair at Nicklaus Children’s Hospital: IAA repairs are performed by Nicklaus Children’s Hospital’s team of top-notch pediatric heart surgeons using the newest techniques.


Reviewed by: Bhavi Patel, DO

This page was last updated on: 7/4/2018 11:07:02 AM

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Jun Sasaki, MD of Nicklaus Children's Hospital is a pediatric cardiologist with The Heart Program.