When Heather had her yearly physical, her doctor noticed that her blood pressure was high and ordered tests to determine what was causing it. It turned out Heather had a heart condition known as coarctation of the aorta.

Heather was surprised, and understandably a little worried. But she was relieved to hear that the problem could be treated and that she could still live a normal life.

What Is Coarctation of the Aorta?

The aorta (pronounced: ay-or-tuh) is the major artery that carries blood away from the heart to the body. When someone has coarctation of the aorta, that person's aorta is narrowed at some point.

Here's how a healthy heart and aorta work: Blood that needs oxygen comes from all over the body and enters the right side of the heart, which pumps it to the lungs. The lungs fill the blood with oxygen, and this oxygen-rich blood returns from the lungs to the left side of the heart. The left side of the heart finishes by pumping the blood out through the aorta. From the aorta, the blood travels through arteries that reach all of the body's organs and tissues, bringing them oxygen. Then the blood returns to the heart through veins and restarts the cycle.

When part of the aorta is narrowed, called a coarctation (pronounced: ko-ark-tay-shun), the defect can affect the body's blood circulation because the left side of the heart has to work harder to pump blood through the narrowed aorta. Sometimes the narrowing is minor and might not even cause symptoms. In other cases the aorta may be more constricted, placing a strain on the heart's left ventricle (the chamber that pumps blood to the aorta and out to the body).

A coarctation can occur anywhere in the aorta, but most often is found just beyond the point where the arteries that carry the blood to the upper body and head branch off from the aorta.

What Causes Coarctation of the Aorta?

Coarctation of the aorta, COA for short, is a congenital defect, meaning that someone is born with it. Doctors don't know for sure why certain people are born with COA.

Guys are almost twice as likely to have COA than girls. In many people, the defect shows up with other birth defects or conditions, such as a ventricular septal defect (a hole in the wall between the heart's left and right ventricles). COA is also fairly common in girls born with Turner syndrome, a genetic disorder in which one of a girl's two X chromosomes is incomplete or missing.

Most people with COA are diagnosed when they are babies or young kids. But some, like Heather, may not be diagnosed until they are teens or even adults. When people aren't diagnosed until their teens, it's usually because the narrowing in the aorta is not severe enough to cause serious symptoms while they're very young. But even people who don't have major symptoms usually need treatment because COA can eventually cause problems. The heart defect won't just go away on its own.

Signs and Symptoms

Often the first sign that someone has COA is an abnormal blood pressure test. During a physical exam, the doctor may find that a person with a coarctation has higher blood pressure in the arms than in the legs. The doctor also might hear a heart murmur or notice that the pulses felt in the groin area or on the feet are weak or even absent.

Teens and adults who have COA often do not have any symptoms or have only mild signs that are discovered during a regular visit to the doctor. Someone with symptoms may notice some or all of these:

• cold legs and feet
• shortness of breath, especially when exercising
• chest pain
  

Diagnosis and Treatment

If a teen has the signs or symptoms of COA, the doctor may refer him or her to a pediatric cardiologist — a doctor who specializes in diagnosing and treating heart disease in kids and teens. The cardiologist will listen to the heart, feel the pulses, measure blood pressure, and probably order an echocardiogram(a test that uses sound waves to create a picture of the heart and its circulation). A cardiologist may also use other tests that produce images of the heart, such as a chest X-ray, a magnetic resonance imaging (MRI) test, or a computerized tomography (CT) scan to look for a narrowing of the aorta.

Doctors often recommend that COA be treated quickly, especially in teens and adults. The longer a COA is left untreated, the more likely it is that the person will have high blood pressure even after the COA is fixed. If it's left untreated indefinitely, the defect can be fatal in many people by the age of 40.

Coarctation of the aorta can be repaired in several ways. Surgery can very effectively repair a narrowing of the aorta, usually by removing the narrow section and reconnecting the two good ends of the aorta.

In some cases, doctors might do a procedure known as balloon dilation (also called balloon angioplasty). For this procedure, a cardiologist inserts a tiny catheter (plastic tube) into a blood vessel in the leg and uses a very thin wire to thread it up to the aorta. This specialized catheter has a deflated balloon inside. When the catheter is in the aorta, the doctor inflates the balloon, expanding the narrow spot. After the area has been widened, the balloon is removed. The cardiologist may also implant a device called a stent, which is a tube made of metal meshwork, to keep the area open after the procedure.

Taking Care of Yourself

After the defect is fixed, most symptoms of COA disappear quickly because the blockage that caused them is gone. Some people will still have high blood pressure for a while and may have to take medicine to control it.

People who have surgery often feel completely better after a couple of weeks, and those who have had the balloon treatment feel better even sooner, often within a couple of days. But doctors recommend that all patients avoid certain physical activities — especially lifting heavy objects or playing certain sports that could cause an impact to the chest — for several weeks or months to give their bodies enough time to heal. Someone whose blood pressure remains high may have to continue to limit certain activities as long as he or she has high blood pressure.

People who've had a COA corrected will still need to be monitored over time. In some, the narrowing of the aorta can return after surgery or balloon dilation treatment. Regular visits to a cardiologist — often every year or two after the person has fully recovered — let the doctor keep an eye on a person's blood pressure and look for signs that COA could be returning.

Patients also need to monitor themselves and call the doctor if they have shortness of breath, chest pain, or fainting. But overall, teens like Heather who have coarctation of the aorta can expect to continue leading a normal life after treatment.

Reviewed by: Steven B. Ritz, MD
Date reviewed: August 2010