The Coronary Artery Anomalies Program (CAAP) at Texas Children’s Hospital is a multidisciplinary team dedicated to the evaluation and management of patients affected by coronary artery anomalies, especially anomalous aortic origin of a coronary artery (AAOCA) and myocardial bridge. The program was created in December 2012 and cares for hundreds of patients and families affected by this condition. The program’s goals include prospective data collection, enrolling patients in a registry to advance our understanding of coronary anomalies and help us provide a holistic approach to patients and families seeking our guidance.

What is anomalous aortic origin of a coronary artery (AAOCA)?

The coronary arteries are vessels that arise from the aorta (the biggest vessel in the body) and provide blood supply and oxygen to the heart. There are usually two main arteries: the left and right coronary arteries. The vessels arise from one of three outpouchings of the aorta called sinuses of Valsalva.

 Anomalous aortic origin of a coronary artery (AAOCA) is a congenital cardiac abnormality (present at birth) in which the origin or course of a coronary artery that arises from the aorta is atypical.

AAOCA is the second leading cause of sudden cardiac death (SCD) in young athletes in the United States.

People with AAOCA may be completely asymptomatic, as in up to 50% of cases or more, and the first manifestation of the disorder can be sudden cardiac arrest (SCA) or sudden cardiac death (SCD). Others may develop symptoms leading to this diagnosis upon medical evaluation.

Types of AAOCA

The figure to the right shows the usual types of AAOCA we evaluate and manage in our program, among other types of anomalies, such as myocardial bridge.

The left hand panel shows AAOCA that have an interarterial course. The interarterial course is a condition in which the coronary artery travels between the two great arteries – the aorta (the artery that takes blood to the body) and the pulmonary artery (the artery that takes blood to the lungs to receive oxygen).

The graphic shows anomalous coronary arteries that have an intramural course, in addition to an interarterial course. The intramural course is a condition in which the first part of the coronary artery has grown within the aortic wall and may increase risk for ischemia, which is decreased blood flow to the heart muscle.  

A particular type of AAOCA is the one that includes an intraseptal course, which means that the anomalous coronary travels inside the heart muscle for a specific length before surfacing the heart. Patients may be asymptomatic (without symptoms) but some may experience symptoms.

During your child’s clinic visit, the cardiology provider will go over (in great detail) the type of AAOCA your child has.

Image above: CT scan of the left coronary
artery with an intraseptal course