Coarctation of the Aorta

What is coarctation of the aorta?

Coarctation of the aorta is a common congenital (present at birth) heart defect where the aorta is pinched in or narrowed, which restricts blood flow. The defect can affect a single spot or a larger section of the aorta.

Who is affected by coarctation of the aorta?

Coarctation of the aorta affects about 1 in 10,000 people and accounts for 5 to 10 percent of all congenital heart defects.

What are the long-term effects of coarctation of the aorta?

Coarctation of the aorta causes high blood pressure. Without treatment, the condition can lead to stroke, coronary artery disease at a young age, brain aneurysm, ruptured aorta, kidney failure and liver failure.

More than half of people with coarctation of the aorta also have a congenital heart valve defect (bicuspid aortic valve). The valve condition usually gets worse with time, and surgery may be needed to repair or replace the valve. People who have valve disease are also at risk for an aortic aneurysm —the walls of the aorta get weak, which causes it to bulge out and possibly rupture.

What are the symptoms of coarctation of the aorta?

Symptoms depend on how narrow the aorta is. Severe cases are often diagnosed at birth or within the first months of life. In mild cases, symptoms might not start until adulthood. Symptoms in an adult may include not being able to exercise, headache, shortness of breath, chest pain, nose bleeds, cold feet or leg pain after exercise.

How is coarctation of the aorta diagnosed?

Aortic coarctation usually happens between the area where the blood vessels branch off the aorta to the upper body and the area where the blood vessels branch to the lower body. Because of this, aortic coarctation causes high blood pressure in the arms and upper body and low blood pressure in the lower body and legs. This blood pressure difference is one of the most classic signs of the condition. Others include differences between the pulse at the groin and the neck and a distinctive harsh heart murmur that can be heard when a stethoscope is on the back.

If you have coarctation of the aorta, your cardiologist may order tests to find out how severe it is.

Tests include:

Electrocardiogram (ECG).
Chest X-ray.
Echocardiography.
Chest computed tomography (CT or CAT) scan.
Magnetic resonance imaging (MRI) of the chest.
Cardiac catheterization.

What treatments are available?

In the past, surgery was the only treatment available for patients with aortic coarctation. Surgery is still considered the gold standard, but treatment options for children and adults with this condition now also include balloon angioplasty and stenting. The choice of treatment is based on your overall health, the size and severity of the coarctation and its exact location.

If you have coarctation of the aorta, you should be under the care of an experienced congenital heart specialist at a major medical center. A cardiac surgeon experienced in the procedures used to treat patients with this condition can best determine the timing and type of treatment that is best for you.

Surgery

When the coarctation is fairly small, the surgeon can remove the narrowed section of aorta and rejoin the two ends. This is called an end-to-end anastamosis and is often the best surgical treatment option.

Other types of surgery include various types of bypass surgeries in which a graft is stitched onto the aorta to move blood around the area of the defect. A bypass can be done at the same time as valve surgery, if needed.

Angioplasty

Angioplasty is another option for treating patients with coarctation of the aorta. The procedure is similar to angioplasty for patients with coronary artery disease. A long, thin tube called a catheter is placed into a blood vessel through a small incision in the groin. The catheter is guided into the aorta. A tiny balloon on the end of the catheter is inflated once the catheter reaches the coarctation. This opens up the narrow area of the aorta. Once the area is widened, the balloon catheter is removed.

A small metal mesh tube called a stent may be placed at the site of the coarctation to keep the aorta open. Stenting can lower your risk of an aneurysm and ruptured aorta. Your doctor will talk to you about the best treatment for you.

Recurring coarctation

There is a small risk after treatment that you can have aortic coarctation again. The risk for adults is between 5 and 10 percent after surgery. If you have angioplasty or stenting, the risk is between 11 and 15 percent for adults. Your doctor will talk to you about the benefits of the procedure as well as the risk of complications. If coarctation does happen again after any treatment, angioplasty with stenting is the preferred treatment.

Follow-up care

Ongoing care from a congenital heart disease specialist is very important after treatment for aortic coarctation. This includes regular visits to check your blood pressure and a yearly exam. You should also have Doppler ultrasound and MRI tests every two to five years.

If your blood pressure is normal after treatment, you probably won’t have any activity restrictions. But, if your blood pressure stays high, you will most likely need to avoid some strenuous activities and sports.