Anomalous Coronary Artery

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with anomalous coronary artery, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

What Is It?

Anomalous coronary artery is a group of abnormalities in which the coronary arteries have abnormal connections to the blood vessels leaving the heart (“congenital” means that a child was born with the condition). The coronary arteries are the blood vessels that deliver blood to the heart muscle itself. They usually arise from the aorta (the main blood vessel carrying blood from the heart to the body) just above the aortic valve, which separates the pumping chamber of the heart from the aorta. There are usually two openings (called coronary “ostia”) in the aorta — one at the back where the left main coronary artery leaves to feed primarily the left side of the heart, and the one in the front where the right coronary artery leaves to feed the right side of the heart.

Categories of Anomalous Coronary Artery

There are three main categories of anomalous coronary artery:

Anomalous Aortic Origins of the Coronary Arteries

The last category is called “anomalous aortic origins of the coronary arteries.” In this case, the coronary arteries arise from the aorta as they should, but in an abnormal location. Most commonly, there are still two openings in the aorta (“coronary ostia”), but they arise next to each other (either both in back from the area of the aorta called the “left coronary sinus,” or both in front from the area called the “right coronary sinus”).

There are a couple of reasons why this may be a problem:

  • The abnormal coronary artery — instead of running where it’s supposed to on the heart — must travel between the aorta and the pulmonary artery. This is called an “inter-arterial” (meaning within the arteries) course. In this location, the coronary artery may be pinched between the two arteries (particularly when people exercise).
  • In other cases — instead of the coronary artery leaving the aorta — it runs within the wall of the aorta for a few millimeters or centimeters. This is called an “intra-mural” (meaning within the wall) course. Again, the coronary artery can be pinched when the aorta is at a higher pressure, also usually during exercise.

The effects of abnormal aortic origin of the coronary arteries (also called “AAOCA”) aren’t clear. Although some children with each of these abnormal connections have been found to have sudden death (probably due to pinching of the coronary artery and decreased blood flow to the heart), others live to old age without any problems. That’s why our job at the Nemours Cardiac Center is to diagnose and treat children with anomalous coronary artery as soon as possible — so that they can be as healthy as possible.

Anomalous Coronary Artery Off of the Pulmonary Artery

An anomalous coronary artery off the pulmonary artery happens when one of the coronary arteries arises from the pulmonary artery, rather than arising from the aorta as it should. This is usually an abnormal connection of the left coronary artery called an “anomalous left coronary artery off of the pulmonary artery” (or “ALCAPA,” for short).

The problem with this abnormal connection is that the blood pressure in the pulmonary artery is much lower than the blood pressure in the aorta, so there’s less force pushing blood into the coronary artery and from there into the heart muscle. In many cases, blood will actually flow backward out of the muscle and into the pulmonary artery. Also, while the aorta carries oxygen-rich blood, the pulmonary artery carries oxygen-poor blood. This means that in children with ALCAPA, the heart muscle is being fed by lower pressure blood with less oxygen — so not enough oxygen is delivered to portions of the heart and, eventually, there’s damage to the heart muscle.

Coronary Arteriovenous Fistulas

In children with coronary arteriovenous (or “AV”) fistula, there’s an abnormal connection between one of the coronary arteries and either a coronary vein (a vein that drains blood from the muscle of the heart) or one of the chambers of the heart. This can cause the oxygen-rich blood to drain back into the heart without first going to the muscle, and can prevent the heart muscle from getting enough oxygen.

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at also offer these helpful resources to help both you and your child understand how the heart works:

How & When Is It Diagnosed?

The different types of anomalous coronary artery are diagnosed differently. But all of them are difficult to diagnose because they may not cause symptoms early on — and when they do, they may be difficult to see on imaging studies like an echocardiogram.

Anomalous Aortic Origin of the Coronary Arteries

These abnormalities are difficult to diagnose because they rarely cause symptoms. They’re often diagnosed when an echo or other test is done for unrelated reasons, although some children may have episodes of fainting or chest pain that may or may not be related to the abnormal coronary artery. Often, an echo suggests that there may be an abnormal connection and then we use further testing to clearly define the origin the location of the artery.

Anomalous Coronary Artery Off the Pulmonary Artery

This type of abnormal connection almost always causes symptoms. Because of the poor delivery of oxygen to the heart muscle, children with anomalous left coronary artery off of the pulmonary artery (“ALCAPA”) usually have early symptoms, within the first months to years after birth, of heart failure including enlargement of the heart, poor growth, rapid breathing and rapid heart rate. We often perform an echo to detect the abnormal connection, but children usually need additional tests such as a cardiac catheterization, a CT scan or an MRI.

Coronary AV Fistulae

Most commonly, coronary AV fistulae cause either a murmur or occasionally symptoms of heart failure. We can usually make the diagnosis with an echocardiogram (“echo”) — a completely safe and painless test that uses ultrasound (sound waves) to build a series of picture of the heart. Further testing isn’t usually necessary, although sometimes we may perform a cardiac catheterization, which involves inserting a thin plastic, flexible tube (called a “catheter”) into an artery and vein that lead to the heart. Cardiac catheterization is done under sedation with local anesthesia or under general anesthesia, depending on your child’s age and condition.

Learn More About Diagnostic Tests »

How & When Is It Treated?

The different types of anomalous coronary artery are also treated differently.

Anomalous Aortic Origin of the Coronary Arteries

As noted above, these abnormal connections are usually asymptomatic (they don’t cause any symptoms) and the decision to proceed with repair may be complicated. Currently the exact risks for each child with this diagnosis isn’t clear. We usually decide whether or not to repair the abnormality based on the exact anatomy of the child’s heart and whether or not the child is having symptoms.

Because anomalous coronary artery has only been commonly discovered within the past decade, cardiologists and heart surgeons all over the world are still unclear of what treatments will affect children over time. In order to address the fact that no one really knows either the long-term results of doing surgery or the results of not doing surgery, the Nemours Cardiac Center is part of a consortium run by the Congenital Heart Surgeons’ Society that’s following the outcomes of these children (whether or not they have surgery) so that we can learn the best path of treatment.

Anomalous Coronary Artery Off the Pulmonary Artery

When an anomalous coronary artery off of the pulmonary artery is diagnosed, surgery is required to fix it. Because this defect can be hard to diagnose, children often come in extremely ill and may need mechanical support (either extracorporeal membrane oxygenation or a ventricular assist device) to try to stabilize them before surgery and support them after surgery. (Extracorporeal membrane oxygenation, or “ECMO,” is a life-saving technique that simulates the natural function of the heart and lungs, allowing the child to rest and the organs to recuperate. During ECMO treatment, the heart continues to beat but a mechanical pump does most of the work. A ventricular assist device, or “VAD,” is a mechanical pump used to support blood flow and heart function in patients with weakened hearts.)

Our pediatric heart surgeons may use different types of repair to correct an anomalous coronary artery off of the pulmonary artery. In some cases, we may move (or “translocate”) the coronary artery from the pulmonary artery to the aorta. In other cases, we may construct a tunnel to deliver oxygen-rich blood from the aorta to the anomalous coronary artery.

Coronary AV Fistulae

These abnormalities typically don’t close on their own — and, over time, they may cause heart failure, which will make repair necessary. Depending on the exact anatomy of the fistula, our Nemours Cardiac Center experts usually repair these defects either with open-heart surgery or by using a catheter. The catheter technique involves closure of the fistula with a synthetic device that plugs the hole. Our pediatric cardiac catheterization physician inserts the device through a heart catheter, which is passed through a vein in the leg that leads up to the heart. If open-heart surgery is necessary, our pediatric heart surgeon will sew on a patch to close the fistula. The patch material may be a portion of the patient’s own pericardium (the sac around the heart) or a synthetic material.

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How Will It Affect My Child Long Term?

The different types of anomalous coronary artery affect children in different ways. However, after repair for coronary AV fistulae, anomalous coronary artery off the pulmonary artery, or anomalous aortic origin of the coronary arteries, most children usually don’t need any further surgical procedures over the course of their lifetime. As we collect data as part of the Congenital Heart Surgeons’ Society consortium, we should begin to better understand the long-term outcomes in these children. 

If your child has anomalous coronary artery, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

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Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


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Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »