What is Anomalous Coronary Artery Disease?
An anomalous aortic origin of a coronary artery (AAOCA) is a condition in which one of the two coronary arteries comes from an uncommon place on the aorta (the large blood vessel that supplies blood to the body). Most people are born with two coronary arteries, a right and a left. The coronary arteries are the main blood vessels that supply oxygenated blood to the heart muscle. This allows it to pump (squeeze) the right way. The coronary arteries are the blood vessels that often get clogged with plaque / fat in later life. This can lead to a heart attack.
Children can be born with either an AAORCA or an AAOLCA. AAORCA is a condition in which the right coronary artery comes off the left side of the aorta. AAOLCA is a condition in which the left coronary artery comes off the right side of the aorta. It is believed that these conditions happen in less than 1% of people.
Anomalous Coronary Artery Disease Symptoms
Not all people have the same AAOCA symptoms. Some people have symptoms like chest pain, shortness of breath, fainting, feeling dizzy or a feeling that their heart is not beating right during physical activity. For some patients, the first symptom is aborted sudden cardiac arrest (heart attack) during physical activity. People who have any of these symptoms need to see a cardiologist (heart doctor) right away. AAOCA does not cause a heart murmur.
Anomalous Coronary Artery Disease Diagnosis
Most people are told they have AAOCA when the abnormal location of the coronary artery is found by chance when they are having imaging of their heart done for some other reason (like an unrelated heart murmur, testing before starting chemotherapy, or an abnormal EKG).
Testing that may be used to diagnose AAOCA:
Echocardiogram (Echo)- is an ultrasound of the heart. This test allows heart doctors to see the structures, coronary arteries, and function (squeeze) of the heart. An echo is a non-invasive test that can be done in clinic to help find out if a patient has AAOCA. An cho often takes less than 45 minutes to do. An AAOCA diagnosis can often be made from this test.
Electrocardiogram (EKG)- is a test that looks at the electrical activity of the heart. Electrodes are attached to the patient’s chest with stickers. The EKG looks for heart rhythm problems. The test often takes less than 10 minutes. EKG changes are not always seen in patients who have AAOCA.
Exercise Test- also known as a stress test or graded exercise test (GXT). An exercise test is a useful tool for getting information about a child's heart function and fitness. It looks for decreased oxygenated blood flow (ischemia) through the coronary arteries using an EKG to track changes with increasing activity. This test can also be given with an echo and / or an IV treatment of a cardio-specific radioisotope medication (sestamibi). Ultimately, an exercise test can help to find out how the blood flows through the coronary arteries, possibly revealing AAOCA.
Cardiac MRI or CT scan- Cardiac MRIs and CTs are done on MRI machines that have special software and cardiac equipment. Both can help show the coronary artery structure in a better way. This can help confirm the diagnosis of AAOCA. There is no radiation used with an MRI. An MRI can also be done with IV medicine that helps provide the best images possible. This can help show if the blood flow through the coronary arteries is good or decreased.
Anomalous Coronary Artery Disease Treatment
Since AAOCA is an abnormal coronary artery structure, it cannot be treated with medicine. Surgery is most often needed for successful treatment. Treatment is determined based on the type of coronary anomaly and whether the stress testing showed a risk. Surgery is usually recommended for patients with AAORCA if they have symptoms such as passing out, abnormal heart rhythms, chest pain with activity or ischemia noted on stress testing. Most children older than 10 years of age with AAOLCA are referred for surgery even if they do not have symptoms. This is due to the increased risk of sudden cardiac death. The most common non-surgical strategy includes placing a limit on physical activity. The doctor will decide what is best based on the risk assessment.
Anomalous Coronary Artery Disease Risk and Follow Up
Recent data shows that risk of heart attack related to AAOCA is thought to be one in 1,000,000, although this may be under reported. The exact reason for the ischemia (lack of oxygenated blood to the heart muscle leading to a heart attack) is not known. A cardiac risk assessment is done to figure out how to manage this.
All patients with AAOCA will be followed by a heart doctor over their lifetime. How often follow-up visits are needed will be based on which treatment options are chosen. It is normal for patients who do not have a surgical repair to have a yearly follow up. This follow up includes testing such as an echo, EKG and exercise test every one to three years. Patients who have surgery for repair of AAOCA are watched more closely. Their follow up is based on assessment and review of testing after their surgery.
