Anomalous Aortic Origin of a Coronary Artery

Anomalous aortic origin of a coronary artery (AAOCA) is a congenital heart defect in which one of the coronary arteries originates in an unusual location or follows an unusual course or pathway.

About Anomalous Aortic Origin of a Coronary Artery

Coronary arteries are the blood vessels that supply the muscle of the heart with oxygen-rich blood. These arteries originate from the aorta (the major blood vessel that takes blood from the heart to the rest of the body) at two separate sinuses (openings) that are located on the right and left side of the aorta. From the sinuses, the coronary arteries travel along the surface of the heart and form different branches of the coronary artery system.

One of the coronary arteries is supposed to originate from the right of the aorta while the other originates from the left before traveling along the surface of the heart. Anomalous aortic origin of a coronary artery (AAOCA) is congenital heart defect in which the coronary arteries do not originate from their normal locations or they follow an unusual pathway. When one of the coronary arteries is connected to the aorta from the wrong side, it gets routed through or around the heart in a way that can inhibit blood flow to the heart or cause a heart rhythm that can be dangerous, particularly during exercise.

There are several types of AAOCA, depending on the specific location and path of the coronary arteries. The type of AAOCA that your child has determines their risk for a serious heart problem. AAOCA is the second most common cause of sudden cardiac death.

Symptoms of Anomalous Aortic Origin of a Coronary Artery

Symptoms related to AAOCA are uncommon in children under the age of 10.

Symptoms of AAOCA may include:

• Chest pain, particularly during exercise
• Irregular heartbeats or palpitations
• Passing out, particularly during exercise

Diagnosing Anomalous Aortic Origin of a Coronary Artery

Your child’s doctor may refer you to a pediatric cardiologist if your child is experiencing concerning symptoms, such as chest pain or fainting during exercise or irregular heartbeats or palpitations. A diagnosis can also occur following an episode of aborted sudden cardiac death.

Tests performed when diagnosing AAOCA may include:

• Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make the heart’s structure more visible on an X-ray.
• Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies. A cardiac MRI or CT scan can help determine exactly where the coronary arteries arise.
• Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs.
• Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood.
• Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.

Treating Anomalous Aortic Origin of a Coronary Artery

Treatment options vary based on the location and course of the anomalous coronary artery, symptoms experienced, and test results.

Treatment options for AAOCA may include:

• Observation: Some forms of anomalous coronaries can be monitored and do not require intervention.
• Surgery: If surgery is required, an individualized treatment plan will be created for your child and the type of surgery will be dependent upon the details specific to their condition.

Care Team Approach

The Texas Center for Pediatric and Congenital Heart Disease, a clinical partnership between Dell Children’s Medical Center and UT Health Austin, takes a multidisciplinary approach to your child’s care. This means your child and your family will benefit from the expertise of multiple specialists across a variety of disciplines. Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way. We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your child’s care with referring physicians and other partners in the community to ensure that we are providing comprehensive, whole-person care.

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