Coarctation of the Aorta

What is coarctation of the aorta?

Coarctation of the aorta 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 (red) 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 teenage years. Coarctation of the aorta may be diagnosed in infants, school-age children, or teens.

About half of children with coarctation of the aorta also have an abnormal mitral valve. This is the valve between the left upper and lower chambers of the heart.

What causes coarctation of the aorta?

Most of the time, coarctation of the aorta has no known cause. Some heart defects present at birth have a genetic link. This means they happen more often in certain families.

What are the symptoms of coarctation of the aorta?

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

• Irritability
• Pale skin
• Sweating
• Heavy or fast breathing
• 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

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.  Some older children may complain of headaches or cramps in their lower body.

The symptoms of coarctation of the aorta can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is coarctation of the aorta diagnosed?

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 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:

• Chest X-ray. A chest X-ray may show an enlarged heart or other changes caused by coarctation of the aorta.
• Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It can find abnormal rhythms (arrhythmias) and heart muscle damage.
• Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. Most aortic coarctations are diagnosed by echocardiography.
• Cardiac catheterization (cath). A heart cath uses a thin, flexible tube (catheter) that is moved through the blood vessels into the heart. Dye is used to get a clear picture of the heart and aorta. Catheterization may also be used to correct the narrowing.
• MRI. MRI makes detailed pictures of the heart and aorta. It can clearly show where the narrowing is and how large it is.

How is coarctation of the aorta treated?

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

Coarctation of the aorta is treated by fixing the narrowed vessel. It may be fixed by:

• Cardiac cath. 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 surgeon will either cut out the narrowed area or make it larger. The surgeon may use nearby tissue or a patch for this.

What are the complications of coarctation of the aorta?

If untreated, coarctation of the aorta causes several problems:

• The left ventricle, which pumps blood through the aorta and to the body, becomes weak.
• Blood pressure is too high in the upper body and too low in the lower body.
• Kidneys don't work as they should.
• The aorta or other arteries can tear. This can cause uncontrolled bleeding or stroke.

Living with coarctation of the aorta

All children with coarctation of the aorta will need to have the defect fixed. Some infants will be very sick and need care in the intensive care unit (NICU) 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 coarctation of the aorta repair will live healthy lives. Activity levels, appetite, and growth should return to normal in time.

After the repair, your child's cardiologist may recommend your child 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 balloon procedure 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 regular follow-up care at a center offering pediatric or adult congenital cardiac care throughout life.

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 coarctation of the aorta

• Coarctation of the aorta 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.
• Coarctation of the aorta can be fixed by cardiac catheterization or surgery.
• After surgery, most children live healthy lives.

Next steps

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

• Before your visit, write down questions you want answered.
• At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
• 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.

Patients can be seen by Texas Children's experts in Heart Center.

To learn more about our outcomes, see our Heart Center Outcomes section.