Anomalous Coronary Artery in Children

What is an ACA in a child?

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 or connections

The vast majority of 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. A specific type of ACA may occur in young babies. This type of ACA is called anomalous left coronary artery from the pulmonary artery (ALCAPA).

What causes an ACA in a child?

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 (TGA) and tetralogy of Fallot (TOF).

What are the symptoms of an ACA in a child?

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:

• Irritability or fussiness
• Sweating
• Pale skin
• Trouble 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 their healthcare provider for a diagnosis.

How is an ACA diagnosed in a child?

Your child's healthcare provider will give your child an exam. They 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 healthcare provider with special training to diagnose and treat heart problems in babies and children. Your child's healthcare provider may advise 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 or the size of the heart.

Electrocardiogram (ECG)

An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and findings, including heart muscle stress (dilation or enlargement).

Echocardiogram (echo)

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

Cardiac catheterization (cardiac or heart cath)

A cardiac catheterization gives detailed information about the structures inside the heart as well as blood vessels connected to 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 them 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 in a child?

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:

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

Your child may also need to limit their activities.

What are possible complications of an ACA in a child?

The coronary arteries send 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. That can include chest pain, a heart attack, syncope (passing out), 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 fatty buildup inside the arteries of the heart (coronary artery disease). This increases the risk for a heart attack.

How can I help my child live with an ACA?

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, they 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, they may still have a higher risk for early heart disease. Ask your child's healthcare provider about 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 ACA in children

• In an ACA, the blood vessels that supply blood to the heart muscle aren’t normal or are connected abnormally.
• 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 and on weekends and holidays. This is important if your child becomes ill and you have questions or need advice.