A to Z: Interrupted Aortic Arch

May also be called: IAA; Aortic Arch Interruption

Interrupted aortic (ay-OR-tik) arch is a rare congenital (present at birth) problem. It happens when a baby's aorta doesn't develop correctly, leaving a gap between two parts of the aorta that are normally connected.

More to Know

The aorta, the body's largest blood vessel, carries oxygen-rich blood from the heart to the rest of the body. It starts from the heart's left ventricle, goes up, and then does a U-turn around the top of the heart before heading down to the rest of the body. The curved part above the heart is the aortic arch.

When an aortic arch is interrupted it means that at some point along the curve, the part going up (the ascending aorta) ends and is not attached to the part going down (the descending aorta). This means that the heart can't send blood through the aorta to the lower body.

Interrupted aortic arch (IAA) is a life-threatening condition. When a baby is born with IAA, the only way for the lower body to get blood is through a small blood vessel called the ductus arteriosus. This duct allows most of the blood to bypass the lungs in a developing fetus. It is present when a child is born but usually closes on its own after a day or two. Once the duct closes, a child with IAA needs surgery to fix the interruption and connect the ascending aorta to the descending aorta. Babies with IAA are given medicine to keep the ductus arteriosus open until surgery can be done.

Babies with IAA usually seem healthy at birth but become very ill within the first days of life. Symptoms include rapid breathing, rapid heartbeat, poor feeding, and a gray skin tone in areas that aren't getting any blood.

Keep in Mind

Doctors can diagnose IAA with an imaging scan while a fetus is still in the womb or after birth with an echocardiogram. After surgery, children treated for IAA usually will take antibiotics for the rest of their lives to prevent heart infections, but their long-term outlook is excellent.

All A to Z dictionary entries are regularly reviewed by KidsHealth medical experts.