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Anomalous Coronary Artery

What is an anomalous coronary artery?

An anomalous coronary artery (ACA) is a heart defect. This is something your baby is born with (congenital). In ACA, the blood vessels that supply blood to your child’s heart muscle aren’t normal. In some cases, the arteries don't arise from the correct place, or there are other problems with size, connections or other issues.

ACAs usually aren’t diagnosed until a person is a teen or adult. This is because the condition doesn’t cause many symptoms. It may also be hard to tell that an ACA is causing the symptoms.

What causes an ACA?

Most congenital heart problems have no known cause. An ACA may happen with other congenital heart problems. For instance, it may occur with transposition of the great arteries and tetralogy of Fallot.

What are the symptoms of an ACA?

The symptoms of an ACA vary depending on the defect. Your child may not have any symptoms. In fact, people often don’t know they have this issue until they have a heart test done later in life for other reasons. Other people may have chest pain when exercising or resting.  

Depending on the type of ACA, symptoms may start in babies. A baby with an ACA may have chest pain from a decreased blood supply to the heart muscle (angina). Your baby may also have the following symptoms:

  • Irritability or fussiness
  • Sweating
  • Pale skin
  • Poor feeding

Your baby may also have symptoms of heart failure. These can include:

  • Trouble breathing
  • Swelling of the legs, ankles, feet, or other areas

An older child may complain of chest pain, dizziness, and fainting during exercise. Older children may have symptoms of heart failure. These can include shortness of breath with exercise and swelling of the legs, ankles, and feet.

People with an ACA may not have symptoms until adulthood. These can include chest pain and symptoms of heart failure. Symptoms may start because of a decreased blood supply to the heart muscle. There may have been enough blood supplied to the heart muscle when they were younger. People may also have sudden cardiac death before the condition is diagnosed.

The symptoms of an ACA may look like other health issues or heart problems. Make sure your child sees his or her healthcare provider for a diagnosis.

How is an ACA diagnosed?

Your child's healthcare provider will give your child an exam. He or she will listen to your child’s heart and lungs, and look for other symptoms.

Your child's healthcare provider will refer your child to a pediatric cardiologist. This is a doctor with special training to diagnose and treat heart problems in babies and children. Your child's doctor may recommend other tests such as:

Chest X-ray

A chest X-ray shows your child’s heart and lungs. The X-ray may show changes in the lungs because of extra blood flow.

Electrocardiogram (ECG)

An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress.

Echocardiogram (echo)

An echo uses sound waves to make a moving picture of the heart and heart valves.

Cardiac catheterization (cardiac or heart cath)

A cardiac catheterization gives detailed information about the structures inside the heart. In this test, a small, thin, flexible tube (catheter) is put into a blood vessel in your child’s groin. Then your child’s healthcare provider guides it to your child’s heart. Your child will get an injection of contrast dye. This is used to see the heart more clearly. Your child’s healthcare provider will give him or her medicine to help relax and prevent pain (sedation). Your child’s blood pressure and oxygen levels will be checked during the test.

Cardiac computed tomography angiography (CCTA)

CCTA shows detailed pictures of the blood vessels.

Cardiac magnetic resonance angiography (CMRA)

This type of MRI shows blood flow through the arteries of the heart.

Coronary angiography

This test uses dye and special X-rays to see the arteries of the heart.

Nuclear imaging

These are scans that find abnormal blood flow to the heart. It can find how much the heart is damaged. It can also measure heart function.

How is an ACA treated?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is and what type of ACA your child has.

Treatment for an ACA may include:

  • Medical treatment. Your child may take medicines to help the heart pump better pump and to control blood pressure. You child may also need oxygen therapy.
  • Surgical treatment. Your child may need surgery to fix the defect.

Your child may also need to limit his or her activities.

What are the complications of an ACA?

The coronary arteries deliver blood to the heart muscle. Any problems with these arteries may lead to a heart attack or death.

Many people with an ACA don’t know they have it until a severe event happens. These can include chest pain, a heart attack, or sudden death.

Children with an ACA who are active or athletic may be at risk for sudden death. They may need to change their exercise routines. ACA is the second most common cause of sudden death in young athletes.

ACA may also increase the risk for early development of fatty buildup inside the arteries of the heart (coronary artery disease). This increases the risk for a heart attack.

Living with an anomalous coronary artery

Work with your child’s healthcare provider to create a care plan. Your child should follow it closely. Even if your child doesn’t have symptoms, he or she will need heart checkups. Your child may also need exercise stress tests to check for changes in the coronary arteries.

If your child has surgery for an ACA, he or she may still have a higher risk for early heart disease. Ask your child's healthcare provider about your child's diet and physical activity. This can help reduce your child’s risk for heart disease.

Ask your child's healthcare provider about your child’s outlook.

When should I call my child’s healthcare provider?

Call your child’s healthcare provider if your child has any new or worse symptoms. If your child has chest pain, get medical help right away. This is an emergency.

Key points about an anomalous coronary artery

  • In an ACA, the blood vessels that supply blood to the heart muscle aren’t normal.
  • ACAs are present at birth. But they are usually not diagnosed until the late teen years or adulthood. This is because they don't often cause symptoms.
  • This condition may lead to a heart attack or sudden death.
  • If your child has chest pain, get medical help right away. This is an emergency.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

  • Know the reason for the visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
  • Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
  • Ask if your child’s condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if your child does not take the medicine or have the test or procedure.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.
Online Medical Reviewer: Ayden, Scott, MD
Online Medical Reviewer: Bass, Pat F., III, MD, MPH
Date Last Reviewed: 7/1/2016
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