Coarctation of the Aorta

Overview, Causes, & Risk Factors

The aorta is the main artery of the body and carries blood away from the heart. Coarctation is an abnormal narrowing in the aorta that is present at birth, causing congenital heart disease.

What is going on in the body?

It is not known why coarctation of the aorta occurs. The narrowing in the aorta decreases the amount of blood that can flow through it. The worse the narrowing, the worse the symptoms. Severe cases may lead to early symptoms in infancy. Milder cases may not be discovered until adulthood.

What are the causes and risks of the condition?

The cause of coarctation of the aorta is unknown. Those who are at higher risk for this condition include:

males, who are more commonly affected than females

those with Turner syndrome, a condition present at birth that affects only females and is caused by an abnormal number of chromosomes

those with other heart defects present at birth, often called congenital heart disease

Symptoms & Signs

What are the signs and symptoms of the condition?

Symptoms of coarctation of the aorta may be mild or severe. In severe cases, symptoms usually begin shortly after birth. The main symptoms are due to congestive heart failure, as the heart cannot pump blood through the narrowed passage. Congestive heart failure in children can cause:

a rapid heartbeat

swelling in the abdomen

swelling in the legs

trouble breathing or fast breathing

If symptoms do not occur until later in life, they may include:

cold legs

headaches

high blood pressure in the arms with low blood pressure in the legs

leg cramps

nosebleeds

symptoms of congestive heart failure, including swelling of the legs and shortness of breath

an upper body that is stronger and better developed than the lower body

Most of these symptoms tend to get worse with exercise. They are mostly due to the fact that blood can flow more easily to the upper body, which is above the narrowed portion of the aorta. The lower body, which is below the narrowed section of aorta, does not get as good a blood supply, so it does not grow as well.

Diagnosis & Tests

How is the condition diagnosed?

The diagnosis of coarctation of the aorta may be suspected from the medical history and physical exam. A heart tracing, called an EKG, and a chest X-ray are commonly done and may be abnormal. Imaging tests of the heart, such as a test that uses sound waves called echocardiography, can confirm the diagnosis. A chest MRI and chest CT scan can also show the defect.

An X-ray procedure called cardiac catheterization may be done in some cases. In this test, a thin tube is inserted through the skin and into an artery, usually in the groin. The tube is then advanced along the artery and up into the heart. A contrast agent can then be squirted through the tube and into the blood. X-ray pictures of the contrast agent can be taken as it flows through the heart and aorta.

Prevention & Expectations

What can be done to prevent the condition?

There are no known ways to prevent coarctation of the aorta.

What are the long-term effects of the condition?

In severe cases, newborns with coarctation of the aorta can die without immediate treatment. Long-term effects include:

bleeding into the brain

congestive heart failure

high blood pressure

infections of the heart

rupture or tearing of the aorta, which is called aortic dissection

Adults rarely survive past the age of 40 without treatment because of these long-term effects. Timely treatment can avoid most of these effects.

What are the risks to others?

Coarctation of the aorta is not catching and poses no risks to others.

Treatment & Monitoring

What are the treatments for the condition?

Severely affected infants with coarctation of the aorta may need surgery shortly after birth. Before surgery, these infants may need aggressive treatment in the intensive care unit. This may include powerful medicines and an artificial breathing machine called a ventilator.

For those with no symptoms at birth, surgery is often advised later in childhood or in early adulthood when symptoms or heart damage begin. The goal of surgery is to remove or bypass the narrowed part of the aorta.

What are the side effects of the treatments?

Surgery carries a risk of bleeding, infections, allergic reactions to anesthesia, and even death.

What happens after treatment for the condition?

After recovering from surgery, most people do well. Long-term follow-up care is needed to watch for or treat high blood pressure, which is fairly common after surgery. Narrowing in the aorta can recur, especially in children less than 5 years old. In the absence of high blood pressure, most people can resume a normal life after recovery.

How is the condition monitored?

Long-term monitoring of blood pressure is needed. Other monitoring may be needed if heart damage or other heart defects are present before surgery. Any new or worsening symptoms should be reported to the doctor.