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Coarctation of the aorta

Coarctation (ko-ahrk-TAY-shun) of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrow part of your aorta.

Coarctation of the aorta is generally present at birth (congenital). Coarctation of the aorta may range from mild to severe, and may not be detected until adulthood, depending on how narrowed the aorta is.

Coarctation of the aorta often occurs along with other heart defects. While treatment for coarctation of the aorta is usually successful, it's a condition that requires careful follow-up through infancy and throughout adulthood.

Symptoms Causes Risk factors Complications Prevention

Coarctation of the aorta symptoms depend on the seriousness of the condition. Children with serious aortic narrowing tend to show signs and symptoms earlier in life, while mild cases may not be diagnosed until adulthood.

Babies with severe coarctation of the aorta usually begin having signs and symptoms shortly after birth. These include:

  • Pale skin
  • Irritability
  • Heavy sweating
  • Difficulty breathing

Left untreated, aortic coarctation in babies may lead to heart failure and death.

Older children and adults with the condition often don't have symptoms, because they tend to have less severe narrowing of the aorta. If signs or symptoms appear, the most common sign is high blood pressure (hypertension) measured in the arm. Signs and symptoms may include:

  • High blood pressure
  • Shortness of breath, especially during exercise
  • Headache
  • Muscle weakness
  • Leg cramps or cold feet
  • Nosebleeds

When to see a doctor

Seek medical help if you or your child has the following signs or symptoms:

  • Severe chest pain
  • Fainting
  • Sudden shortness of breath
  • Unexplained high blood pressure

While experiencing these signs or symptoms doesn't necessarily mean that you have a serious problem, it's best to get checked out quickly. Early detection and treatment may help save your life.

Doctors aren't certain what causes aortic coarctation. For unknown reasons, mild to severe narrowing develops in part of the aorta. Although aortic coarctation can occur anywhere along the aorta, the coarctation is most often located near a blood vessel called the ductus arteriosus. The condition generally begins before birth.

Rarely, coarctation of the aorta may develop later in life. Traumatic injury may lead to coarctation of the aorta. Rarely, severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) may narrow your aorta, leading to aortic coarctation.

Coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and before the blood vessels that lead to your lower body. This often means you'll have high blood pressure in your arms, but low blood pressure in your legs and ankles.

Coarctation of the aorta often occurs along with other congenital heart defects, although doctors don't know what causes multiple heart defects to form together. If you or your child has any of the following heart conditions, you or your child is more likely to have aortic coarctation:

  • Bicuspid aortic valve. The aortic valve separates the lower left chamber (left ventricle) of the heart from the aorta. A bicuspid aortic valve has two leaflets instead of the usual three.
  • Patent ductus arteriosus. While a baby is still in the womb, the ductus arteriosus is a blood vessel connecting the left pulmonary artery to the aorta, allowing blood to bypass the lungs. Shortly after birth, the ductus arteriosus usually closes. If it remains open, it's called a patent ductus arteriosus.
  • Aortic valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta. This means your heart has to pump harder to get adequate blood flow to your body. Over time, this can thicken the muscle of your heart and lead to heart failure.
  • Mitral valve stenosis. This is a narrowing of the valve that lets blood flow through the left side of your heart. This means blood may back up into your lungs, causing shortness of breath or lung congestion. Like aortic valve stenosis, this condition can also lead to heart failure.

Coarctation of the aorta is also more common in people who have certain genetic conditions, such as Turner syndrome. Women and girls with this syndrome have 45 chromosomes, with one missing or incomplete X chromosome, instead of 46. About 10 percent of women and girls with Turner syndrome have aortic coarctation.

Untreated coarctation of the aorta frequently leads to complications. Several of the complications are a result of long-standing high blood pressure caused by the aortic coarctation.

Complications of coarctation of the aorta include:

  • High blood pressure
  • Stroke
  • Rupture of the aorta
  • Premature coronary artery disease — narrowing of the blood vessels that supply the heart
  • Weakened or bulging artery in the brain (cerebral aneurysm)

In addition, if the coarctation of the aorta is severe, your heart may not be able to pump adequate blood to the organs of your body, resulting in the failure of organs such as your kidneys or liver.

Coarctation of the aorta can't be prevented, because it's usually present at birth. However, if you or your child has a condition that increases the risk of aortic coarctation, such as Turner syndrome, bicuspid aortic valve or another heart defect, or a family history of congenital heart disease, early detection can help. Discuss the risk of aortic coarctation with your doctor.

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