Coarctation of the aorta (CoA)

What is a coarctation of the aorta (CoA)?

Coarctation of the aorta (CoA) is a congenital heart disease, which means a child is born with it. A child with coarctation of the aorta has a narrowing in the body’s main blood vessel (the aorta). This narrowing restricts the flow of blood with oxygen to the lower body.

Some children develop symptoms in infancy, while others learn they have the condition later in childhood or the teen years. When the aorta is too narrow, the heart has to work harder to circulate blood.

Without surgical treatment, a child may experience:

• Heart failure - Enlarged heart and poor muscle pumping capacity

• High blood pressure - Hypertension in the upper body

• Low blood pressure - Hypotension in the lower body

Risk factors

Coarctation of the aorta (CoA) is one of the more common congenital heart diseases. Approximately 2,200 babies are born with this heart defect every year. The condition affects 4 out of every 10,000 newborns.

What are the signs and symptoms of a coarctation of the aorta (CoA)?

Coarctation of the aorta doesn’t always cause noticeable symptoms, especially early in life.

If an infant has a severely narrowed aorta, these symptoms may occur:

• Breathing problems

• Difficulty eating and poor weight gain

• Excessive sweating

• Extreme fussiness or irritability

• Pale or gray-looking skin

High blood pressure is often the main symptom of coarctation of the aorta in an older child or teenager. A child with hypertension may complain of headaches or get frequent nose bleeds.

Other signs of coarctation of the aorta in a child or teen include:

• Chest pain

• Cold feet or legs

• Leg cramps during exercise

• Poor growth

• Shortness of breath

How is a coarctation of the aorta (CoA) diagnosed?

Because of our doctors’ deep expertise diagnosing this condition in children, we often make a diagnosis based on a physical examination to check blood pressure, heart murmurs (unusual heart sounds) and other symptoms.

We use advanced cardiac imaging to confirm the coarctation of aorta diagnosis and determine the severity of the aortic narrowing.

Your child may get one or more of these tests:

• Chest X-ray

• CT scan

• Echocardiogram

• MRI

What causes a coarctation of the aorta (CoA)?

Like most congenital heart defects, researchers are still trying to figure out why some children develop this condition. Heart defects are not brought on by anything a woman does – or doesn’t do – during pregnancy.

• Turner syndrome - Children with a rare chromosomal disorder called Turner syndrome are more likely to have coarctation of the aorta. Turner syndrome only affects girls. If your child has Turner syndrome, our heart specialists work with the Turner syndrome specialists at Children’s Health to meet all your child’s healthcare needs.

How is a coarctation of the aorta (CoA) treated?

A child with coarctation of the aorta needs treatment to open up the narrowed section of the aorta. Treatment depends on how much of the aorta is affected and the severity of the narrowing.

Heart surgery

Nearly all infants and young children need heart surgery to correct this condition. Your child’s doctor will determine the best surgical approach based on your child’s unique heart condition and structure.

Most children with coarctation need surgery. The incision is most commonly made under the left arm. The narrowed part of the aorta is removed, and the two ends are sewn together to rebuild the aorta.

In some patients who have a relatively long area of narrowing, the incision is made on the front of the chest and a heart-lung machine is used to reconstruct the aorta (open heart surgery). Many patients who have other heart problems such as a ventricular septal defect need open heart surgery to repair these problems.

Catheterization

Aortic coarctation in older children and teenagers can sometimes be treated during a procedure in the cardiac catheterization lab. Our doctors pass a small tube (catheter) with a balloon on the end from a blood vessel in the upper leg up to the region of the coarctation. Once in place, the balloon is inflated and expands the narrowed aorta. We may also place a stent (small mesh tube) to keep the aorta open.