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). The program was created in December 2012 and cares for hundreds of patients and families affected by this condition. The program also aims at prospective data collection enrolling patients in a Registry with the mission of advancing our understanding in coronary anomalies and providing a hollistic 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 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 cardiac abnormality present at birth (congenital) 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, in up to 50% of cases or more, and the first manifestation of the disorder can be sudden cardiac arrest (SCA). Others may develop symptoms leading to this diagnosis upon medical evaluation.

 


Types of AAOCA

The table below shows the usual types of AAOCA we see and manage in our program, among others. The column to the right shows anomalous coronaries that have an intramural (in-tra-mur-al) course, in addition to an interarterial (in-ter-are-teer- ee-al) course (shown in the column to the left). The intramural course is when 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. The column to the left shows the anomalous coronary arteries that do not have an intramural course but do have an interarterial course which is a course between the two great arteries – the aorta and the pulmonary artery (the artery that takes blood to the lungs to receive oxygen).  The cardiology provider will go over the type of AAOCA the child has during the clinic visit in great details.

A particular type of AAOCA is the one that includes an intraseptal course, which means that the anomalous coronary had a course inside the heart muscle for a specific length. Patients may be asymptomatic but some may experience symptoms. Treatment options, if indicated, may be challenging depending on the results of studies and patients’ symptoms.