According to a recent article, a team at the Heart Institute is working on creating a guide to help cardiologists decide what treatment is best for patients diagnosed with anomalous aortic origin of a coronary artery.

Anomalous Aortic Origin of a Coronary Artery (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.

Symptoms:

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

Unknowns About the Condition

AAOCA may cause sudden cardiac arrest, especially in kids and adolescents. Due to this, a team at the Heart Institute has decided to create a customized algorithm that will guide cardiologists in how they image and treat their patients. The leaders of the team are John David Cleveland, MD, a cardiothoracic surgeon at Children’s Hospital Los Angeles, and Grace Kung, MD.

Dr. Kung says that every patient is unique and therefore should be treated differently depending on their needs. However, there should be a set approach that is a basis for care for patients and their families.

Creating the Guide

Depending on the location and type of the artery anomaly, it will be considered high-risk or low-risk. In a healthy person, the right and left coronaries come from the aorta in different locations. Therefore, when one of the arteries connects to the aorta from a contrary location, it increases the patient’s risk.

Dr. Cleveland explained that an anomalous coronary artery can be crowded from the structures around it or the artery itself can be an abnormal shape which reduces the blood supply to the heart, thus increasing the risk of the patients having sudden death due to loss of heart function.

In particular, when the left and right coronary come from the same place on the aorta, surgery will be needed. Additionally, patients that experience symptoms from a right coronary coming off of the left artery location, surgery will also be recommended in most cases. The surgery involves surgeons opening up the wall between the coronary and aorta to help with blood flow.

The gray area comes with patients who do not experience any symptoms but have the right and left coronaries arising from the same area. In most cases, this is considered low-risk but not always. This leads doctors to question whether the patient should undergo surgery to see if it would be helpful in the long run, or if they should just wait and see what happens with the patient over time.

Surgery comes with its own risks, such as aortic insufficiency and other potential outcomes with the most severe being death.

How It Works

To start off, patients should have a CT scan. This will allow doctors to see and understand the anatomy and any risk factors. This is recommended only for patients who are 9 years or older and is not recommended for anyone younger unless they are symptomatic. Following their CT scans, patients will have a cardiac MRI.

The team stresses that this algorithm is just a guide for doctors, and that they should still customize their treatment to best fit what their patient needs.