Pediatric coronary artery disease

What is pediatric coronary artery disease?

Coronary artery disease affects blood flow to the heart muscle. When there are problems with the coronary arteries, the heart muscle doesn’t get enough oxygen. Without treatment, some coronary artery conditions can lead to heart failure.

What are the different types of pediatric coronary artery diseases?

There are different types of coronary artery conditions. The causes, symptoms and treatments vary depending on the type.

Coronary artery conditions may include:

• Congenital heart disease - Present at birth

• Acquired heart disease - Develops later in life

• Congenital anomalous coronary artery diseases - Occurs when the coronary arteries connect to the wrong places. An anomaly is something that is different than expected. Congenital coronary artery anomalies affect about 2% of people who have congenital heart disease. Children with these rare conditions receive advanced care at our dedicated Congenital Coronary Anomaly Program.

Congenital coronary artery diseases include:

• Anomalous left coronary artery from the pulmonary artery (ALCAPA) - The left coronary artery connects to the pulmonary artery instead of to the aorta. This means oxygenated blood doesn’t get to some parts of the heart muscle, which increases the risk of heart failure. Symptoms appear within the first few months of life. Without treatment, an infant may have a heart attack.

• Anomalous aortic origin of a coronary artery (AAOCA) - This condition occurs when both the left and right coronary arteries attach to the same side (either the left or right side) of the aorta. In a healthy heart, the left coronary artery attaches to the left side, and the right artery attaches to the right side. This condition may not cause symptoms, or a child may have chest pain and feel dizzy after exercise. It is the second leading cause of sudden cardiac arrests (unexpected stopping of the heart) in young people.

• Acquired coronary artery diseases - Acquired coronary artery diseases are not present at birth. A child develops a problem later in life, usually after having some type of infection.

Acquired conditions that affect the coronary arteries include:

• Kawasaki disease - This condition inflames and weakens the coronary arteries. It increases the risk of blood clots, aneurysms (weak spots in an artery wall) and heart attack. Children often develop Kawasaki disease after having another illness, but there’s no known specific infection that causes the disease.

• Multisystem inflammatory syndrome in children (MIS-C) - This condition affects a small number of children (typically between the ages of 5 and 13) who get COVID-19. For unknown reasons, the virus causes inflammation in the heart, lungs, kidneys or intestines.

What are the signs and symptoms of pediatric coronary artery disease?

Symptoms vary depending on your child’s age and their condition. Children with anomalous aortic origin of a coronary artery may not have symptoms. Older children may have chest pain, dizziness or fainting during physical activity.

Infants may have:

• Difficulty gaining weight

• Pale or gray skin or lips

• Rapid breathing

How are pediatric coronary artery diseases diagnosed?

At Children’s Health, your child sees heart doctors who are experts at detecting rare coronary artery conditions. We perform a thorough physical examination and use advanced cardiac imaging to accurately diagnose conditions that others may overlook or misdiagnose.

These tests produce images of the heart and blood vessels to help us make a diagnosis:

• CT scan

• Echocardiogram (Echo)

• Electrocardiogram (ECG)

• MRI

• Exercise stress test - We may also use an exercise stress test in older children to check the function of the coronary arteries. This test shows how the heart responds to physical exertion.

What are the causes of pediatric coronary artery diseases?

Congenital coronary artery disease develops in the womb. For unknown reasons, an unborn baby’s heart or arteries don’t form as they should. Heart defects aren’t caused by anything a woman does or doesn’t do during pregnancy.

Infections from viruses or bacteria may cause acquired coronary artery conditions. Researchers are still trying to determine why some children develop Kawasaki disease or MIS-C while others don’t.

How are pediatric coronary artery diseases treated?

Treatments depend on the type of coronary artery disease which may include:

• Heart surgery - Surgery to reposition the coronary arteries and improve blood flow and heart function. Surgery can prevent heart failure in children with congenital coronary artery conditions.

• IV immunoglobulin (IVIG) - Infusions of antibodies (proteins), to help children with Kawasaki disease fight infections. A child may also get aspirin and steroids.

• Medications and fluids - Helps children recover from MIS-C.