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Coarctation of the Aorta

Coarctation of the aorta is a common heart defect. About 5%-8% of people with heart defects have coarctation.

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What is coarctation?
People with coarctation are born with an aorta that is too narrow. The aorta is the longest blood vessel in the body, and branches out into the right arm, head, and left arm. In coarctation the aorta can be narrow in different places and in different ways. Some people have a short, narrow section. Others have long areas of their aorta that are too narrow. If the aorta is very narrow, blood cannot pass. This kind of coarctation is usually found at birth. If the narrowing is not as bad, it may not be detected until later in life.

How common is coarctation?
Coarctation is a common heart defect. About 5%–8% of people with heart defects have coarctation. 

How does coarctation develop?
Right now there are several ideas about what causes the aorta to become too narrow. Coarctation often occurs near the ductus arteriosus, an artery in the heart that is open before birth and normally closes in the newborn. It is possible that when the ductus closes it pinches the aorta. This may cause coarctation.

It is also possible that coarctation develops because the blood flow in the growing aorta is not normal because of problems in the heart itself. Another idea is that people with coarctation have an in-born problem in the cells in the walls of their aorta.

What causes coarctation?
We do not know enough about why coarctation happens. In some cases there is a family history and/or genetic factor, such as Turner’s Syndrome. Scientists suspect that as we continue to study coarctation new genetic factors may emerge. Coarctation is not known to be caused by environmental factors during pregnancy.

If I have coarctation, do my relatives have a higher risk for heart problems?
If you have coarctation, your parents, sisters, and brothers are more likely to have certain kinds of heart defects. These can include bicuspid aortic valve, abnormal mitral valve, and coarctation. Your family members may want to get tested for heart defects. This usually involves an echocardiogram, which is an ultrasound picture of the heart.

What other defects are common in coarctation?
50%-85% of coarctation patients also have a bicuspid aortic valve. This means the aortic valve has two flaps instead of three. Some people with coarctation are also born with a hole in the lower wall of the heart (ventricular septal defect). A smaller number have problems with other heart valves such as the mitral valve. People with coarctation are also at risk from swelling in their blood vessels (aneurysms). The causes and risks of aneurysms are discussed below.

How is coarctation diagnosed?
Severe coarctation is usually diagnosed at birth. These babies may have heart failure or go into shock. They are often very ill. Surgery is needed right away.

If coarctation is not found in birth, then later in life, a heart murmur or high blood pressure may lead to a diagnosis. One common clue is the blood pressure in the arms and legs is not the same. This can mean the aorta is narrow. The diagnosis can be confirmed by using an echocardiogram, CAT scan, MRI, or heart catheterization.

What is the treatment for newly-diagnosed coarctation?
There are now different ways to widen the aorta. In most centers the narrow section is removed surgically. The two ends of the aorta are then sewn together.

Another less-common option is to open up the aorta with a catheter. A catheter is a long, thin tube that the doctor guides into the heart. An attached “balloon” can be used to widen the aorta. Sometimes the doctor will also place a mesh tube (stent) in the aorta to help keep it open.

All repaired coarctation patients are at risk of having their aortas re-narrow. This risk seems to be higher in patients who have been treated via catheter.

If you are an adult who needs coarctation repair or re-repair, it is very important that you seek treatment from specialists in adult congenital heart disease (ACHD). You and your ACHD doctors can discuss risks and benefits of surgery and other treatments.

What are common long-term complications in repaired coarctation?
All repaired coarctation patients are at risk of developing the following problems:

  • Re-narrowing of the Aorta: In some patients the aorta re-narrows again. This can occur in those who had surgery as well as catheter treatment. Some patients will need to have their aorta re-opened.
  • High Blood Pressure: Many people with coarctation continue to have high blood pressure. Some people may have normal pressure at rest but get high blood pressure when they exercise. Some patients will need to take medicine to keep their blood pressure low.
  • Swelling in the Aorta (Aneurysms): Some patients develop swollen areas in their aorta (aneurysms). If these grow too quickly and/or get too big they can burst. This can cause death. When diagnosed early, aneurysms can be treated to prevent this from occurring. It is important that coarctation patients have regular testing to check the size of their aorta.
  • Brain Aneurysm/Stroke: Some people with coarctation also have something wrong in the brain’s blood vessels, and they can swell and burst. Coarctation patients should know how to identify signs of stroke and should report any worrisome symptoms to their doctor promptly. New methods exist to diagnose and treat these problems before they cause damage. Good control of high blood pressure may also help prevent this problem.
  • Valve Problems: As discussed above, many coarctation patients have a bicuspid valve. This valve can begin leaking over time. It can also become too narrow. It is important that this valve’s function continue to be checked regularly.
  • Coronary Artery Disease:Some patients with repaired coarctation develop coronary artery disease earlier than expected. This may be due to high blood pressure. It is important that coarctation patients get regular screenings for acquired heart disease. As in all CHD patients, heart-healthy habits are advised.
  • Infection: Some people with coarctation are at risk of developing an an infection of the lining of the heart and valves (endocarditis). They may also be prone to infection in the aorta (endarteritis). This includes patients with a history of heart infection and patients who have recently received a stent or graft. It is important that patients with coarctation protect themselves from infection as directed by their heart doctor.

Can repaired coarctation patients exercise normally?
Most patients with repaired coarctation can exercise normally. Some repaired coarctation patients should avoid heavy weight lifting. Your ACHD doctor can tell you more about what exercise is right for you.

Can women with coarctation have children?
Coarctation should be repaired before you become pregnant. Women with unrepaired coarctation are at highest risk for problems during pregnancy.

Most women with repaired coarctation can have successful pregnancies. It is very important that all women with coarctation see an ACHD specialist before getting pregnant. The ACHD specialist should order a detailed image of your entire aorta. It is important to make sure that you do not have any new narrowing or areas of swelling. Pregnancy is a bigger risk if your aorta is still narrow or an aneurysm is forming. You may need additional treatment to make pregnancy safer for you.

The most common complication in pregnancy in women with coarctation is high blood pressure during pregnancy. All pregnant women with coarctation should have their blood pressure checked regularly. If you have coarctation, your risk of having a child with a heart defect is higher than in the general population. You should talk about this with your ACHD doctor. 

What kind of cardiology care is recommended for adults with repaired coarctation?
We used to think that coarctation was a simple defect, but we now realize that it is much more than a narrowed aorta. People with coarctation may have an in-born abnormality in the cells throughout all their arteries. This would explain why so many patients experience new narrowing and other problems after coarctation is repaired.

It is important that coronary artery disease be found and treated. This means you may need treatment for high cholesterol. It also means that controlling your weight is especially important. If you smoke, try to get the help you need to stop.

Coarctation is a lifelong disorder. But the good news is that most people with coarctation do well. There are now many treatments available. According to the Guidelines for the Management of the Adult with Congenital Heart Disease, if you have coartctation, you should be seen yearly by an ACHD doctor. By getting the ACHD care you need and taking good care of your heart health, you can help your “funny plumbing” continue to thrive.