Anomalous Aortic Origin of the Coronary Artery (AAOCA)

What is AAOCA?

Anomalous aortic origin of the coronary artery (AAOCA) is a rare congenital heart condition in which one (in rare cases, both) of the coronary arteries arises from the wrong aortic sinus.

In a normal heart, the right coronary artery arises from the front of the aorta in the right coronary sinus and the left coronary artery arises from behind the left coronary sinus. When one of the arteries is connected to the aorta from the wrong side, it gets routed through the heart or around the heart in a way that can inhibit blood flow to the heart or cause a dangerous heart rhythm. This risk increases during exercise when the arteries fill with more blood flow.

There are several types of AAOCA, depending on the specific location and path of the coronary arteries. The type of AAOCA your child has determines their risk for a serious heart problem.

AAOCA is rare. It occurs in about 1 to 5 of every 1,000 people.

What are the symptoms of AAOCA?

Children may begin to have symptoms at around age 10, when they begin competitive sports or more vigorous exercise. In some cases, the first sign of AAOCA is sudden cardiac arrest.

Symptoms can include:

  • chest pain during exercise
  • dizziness
  • fainting during exercise
  • rapid heartbeat

What are the causes of AAOCA?

AAOCA is a congenital condition that a baby is born with. Experts don’t know what causes it to occur.

How is AAOCA diagnosed?

This condition is usually diagnosed with an echocardiogram. Your doctor may confirm the diagnosis with a cardiac MRI or computed tomography angiogram (CTA). Additional testing may include an exercise stress test with stress echo.

What are the treatment options for AAOCA?

Your doctor will discuss your treatment options based on your child’s specific diagnosis.

Surgery may involve unroofing (opening up the wall between the coronary and the aorta) of the anomalous coronary artery or taking a button of coronary and repositioning it back into its normal sinus. Sometimes, a combination of these surgeries is used.

Children with AAOCA will need lifelong follow-up care with a cardiologist to make sure the blood flow through the coronary arteries is normal.

How we care for AAOCA

At Boston Children’s Hospital, we have operated on more than 100 children and young adults with AAOCA, with excellent results. In addition, Boston Children’s submits data to the Congenital Heart Surgeon’s Society’s AAOCA registry to help develop protocols for imaging and interpretation of images.