Anomalous Coronary Arteries

An anomalous coronary artery (ACA) is a coronary artery with a congenital abnormality. It may eventually cause chest pain, heart failure, or cardiac death.

The main function of the coronary arteries is to supply blood to the heart muscle. The left main coronary artery supplies blood to the left ventricle and left atrium. The right coronary artery, which divides into the right posterior descending and acute marginal arteries, supplies blood to the right ventricle, right atrium, and sinoatrial node. Additional arteries branch off the two main coronary arteries to supply the heart muscle with blood. They include the circumflex artery (Cx) and the left anterior descending artery (LAD).

Since coronary arteries deliver blood to the heart muscle, any coronary artery disorder or disease can reduce the flow of oxygen and nutrients to the heart, leading to a heart attack or death.

What happens once you have anomalous coronary arteries?

The symptoms of ACA vary depending on the type of anomalous artery. Some types have no symptoms and may be found later in life. Other ACA types may cause symptoms related to decreased blood flow to the heart tissue, such as chest pain.

Symptoms may begin in infancy. More often, they may not appear until adulthood. An older child or adult may complain of chest pain or dizziness and fainting during exertion.

Heart failure, with symptoms of shortness of breath after moving and bloating, may be the hallmark symptom if chest pain has been vague or ignored.

Both chest pain and heart failure symptoms serve as early warning signs in adults that the heart muscle is no longer receiving enough blood supply.

What causes anomalous coronary arteries?

The vast majority of congenital heart defects have no known cause. A baby's heart is completely formed by eight weeks into the pregnancy. Congenital heart defects develop during this crucial first eight weeks.

ACA is serious because there may be no indication that the condition is present until a severe event such as heart attack, or even sudden death, occurs.

Individuals with ACA involved in strenuous activity or athletics may be at risk for sudden death and might need to modify their exercise routines. Between 4 and 15 percent of young people who experience sudden cardiac death have a coronary artery anomaly. ACA is the second most common cause of sudden death in young athletes.

What types of tests are used to diagnosis anomalous coronary arteries?

ACA might be detected during tests for other conditions:

• CT angiography: Coronary CTA is the primary imaging tool to evaluate high-risk anatomic features in patients with either a suspected or incidental finding of ACA.


• Stress test: For non-invasive testing, physical exercise with a maximal heart rate is preferred to mimic real-life conditions.


• Cardiac catheterization: Cardiac catheterization can be performed in people with ACA if the anatomy cannot be defined with non-invasive imaging and in adults with a high probability for coexistent coronary artery disease (CAD).

What types of treatments and procedures are used to treat anomalous coronary arteries?

Specific treatment for anomalous coronary artery will be determined by your doctor based on:

• Your preference.


• The extent of the disease.


• Expectations for the course of the defect.


• Your age, overall health, and medical history.


• Which type of ACA is present and its effect on you.


• Your tolerance for specific medications, procedures, or therapies.

Treatment for ACA may include:

• Oxygen therapy.


• Activity limitations.


• Surgery techniques such as angioplasty.


• Medications that strengthen the heart, lower blood pressure, and/or remove extra fluid from the body (diuretics or "water pills").

What can I do to support my health when I have anomalous coronary arteries?

Although many people diagnosed with ACA have no symptoms, a thorough evaluation again in adolescence or early adulthood is recommended. In some cases, periodic stress tests may be recommended to assess for changes in coronary artery status.

Following surgical repair of ACA, there may be some increased risk of developing early coronary artery disease. Outcomes following surgical repair may be improved by following guidelines for a healthy diet and exercise.

Why choose Presbyterian for anomalous coronary arteries treatment?

Presbyterian’s Pediatric and Congenital Cardiology team has many different options to help you manage your or your child’s heart condition. The team performs various diagnostic tests and procedures to help form an accurate diagnosis and create individualized treatment plans. Depending on the type of heart condition your child has and its underlying cause, the team can recommend a wide variety of treatment options. Our pediatric cardiologists, pediatric interventional cardiologists, and pediatric cardiovascular surgeons work closely together for cases in which cardiac repair or surgery is the best treatment option.