What is coarctation of the aorta in children?

Coarctation of the aorta (COA) is a heart defect that is present at birth (congenital). It means the aorta is narrower than it should be. The aorta is the large artery that carries oxygen-rich blood from the left ventricle to the body. This narrowing means that less oxygen-rich blood is sent to the body.

The amount of narrowing can vary. A child with greater narrowing of the aorta will have more symptoms. The symptoms will also be seen at an early age. In some cases, coarctation is seen in infancy. In others, it may not be seen until school-age or teen years. COA may be diagnosed in babies, school-age children, or teens. It is more often seen in males. There is an increased chance of having the condition if another family member has it. It also occurs more often in certain genetic syndromes such as Turner Syndrome. Coarctation of the aorta is often linked to other cardiac defects. These include a bicuspid aortic valve, aortic stenosis, or an underdeveloped left ventricle.

What causes COA in a child?

Most of the time, COA has no known cause. Some heart defects present at birth have a genetic link. This means they happen more often in certain families. There is an increased risk in families with a history of left-sided heart obstructive lesions.

What are the symptoms of COA in a child?

These are the most common symptoms of coarctation of the aorta:

• Grouchiness

• Pale skin

• Sweating

• Heavy or fast breathing

• Enlarged liver (hepatomegaly)

• Poor feeding or eating

• Poor weight gain

• Cold feet or legs

• Weak pulses in the feet, or no pulses in the feet

• Blood pressure in the arms that is much higher than the blood pressure in the legs

• Chest pain

• Pain in lower legs with walking (claudication)

Mild narrowing may not cause symptoms at all. The healthcare provider may find high blood pressure. Or he or she may hear an abnormal sound (heart murmur) when listening to your child's heart with a stethoscope. Adults with the condition are at increased risk of intracranial aneurysms. This is a weakening and ballooning of blood vessels in the brain. This is especially the case if they have high blood pressure. The symptoms of COA can seem like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is COA diagnosed in a child?

The healthcare provider will ask about your child’s symptoms and health history. He or she will give your child a physical exam. The provider may refer you to a pediatric cardiologist. This is a doctor with special training to treat heart problems in children. The cardiologist will examine your child and listen to his or her heart and lungs. Your child may also have tests such as:

• Echocardiogram. This uses sound waves to make a moving picture of the heart and heart valves. Most aortic coarctations are diagnosed by echocardiography.

• Chest X-ray. A chest X-ray may show an enlarged heart or other changes caused by COA.

• Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It can find abnormal rhythms (arrhythmias) and heart muscle damage.

• Cardiac catheterization.  A thin, flexible tube (catheter) is moved through the blood vessels into the heart. Dye is used to get a clear picture of the heart and aorta. A catheterization may also be used to treat the narrowing.

• CT scan. This can make detailed pictures of the aorta and show the area of narrowing.

• MRI. This makes detailed pictures of the heart and aorta. It can clearly show where the narrowing is and how large it is.

How is COA 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.

COA is treated by fixing the narrowed vessel. It may be fixed by:

• Cardiac catheterization.  During the procedure, your child is given medicine to help him or her relax (sedation). The healthcare provider puts a catheter through a blood vessel to the narrowed part of the aorta. A balloon at the tip of the catheter is inflated to stretch the area open. The provider may put a small metal mesh tube (stent) in the narrowed area to keep the aorta open. Your child will usually need to stay overnight in the hospital.

• Surgery. Your child will be given sleep medicine (general anesthesia). The surgeon will make a cut (incision) into your child's chest. The location of the incision will depend on the extent of narrowing of the aorta. It will also depend on if there are other defects that need to be fixed during the same surgery. The surgeon will cut out the narrowed area or make it larger and connect the 2 ends of the aorta together. In some cases, the surgeon will have to use other nearby tissue or patch material to make the aorta longer.

What are possible complications of COA in a child?

If untreated, COA causes several problems:

• The left ventricle, which pumps blood through the aorta and to the body, becomes weak and fails

• Blood pressure is too high in the upper body and too low in the lower body

• Kidneys don't work as they should

• Coronary artery disease

• Infection of the heart and blood vessel wall

• The aorta or other arteries can tear, causing uncontrolled bleeding or stroke

• Death

How can I help my child live with COA?

All children with COA will need to have the defect fixed. Some babies will be very sick and need care in the intensive care unit before the procedure. In some cases, a child may need emergency repair. Children who have few symptoms will have scheduled repairs. Most children who have had a COA repair will live healthy lives. Activity levels, appetite, and growth should return to normal in time.

After the repair, your child's cardiologist may advise your child to take antibiotics before surgeries or procedures, such as dental work. This is done to prevent infection.

As the child grows, the aorta may again become narrow. If this happens, another cardiac catheterization or surgery may be done.

Controlling blood pressure is very important. Your child's blood pressure may be higher after aortic coarctation repair. He or she may need to take medicines to help lower blood pressure.

Your child will need lifelong regular follow-up care at a center offering pediatric or adult congenital heart care.

When should I call my child's healthcare provider?

Call your child's healthcare provider right away if you notice changes in your child's normal symptoms. For example, your child might have more trouble breathing or is less able to eat.

Key points about COA in children

• COA is a congenital heart defect. It means the aorta is narrower than it should be.

• Symptoms can vary from mild to more severe. They may include trouble breathing, poor weight gain, poor feeding, and pale skin.

• If not treated, it can lead to coronary artery disease, kidneys not working well, high blood pressure in the upper body and low blood pressure in the lower body, and even death.

• COA can be fixed by cardiac catheterization or surgery.

• After surgery, most children live healthy lives. But regular follow-up care is needed.

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.