Coarcation of the Aorta

What is coarctation of the aorta?

Coarctation of the aorta is a narrowing of the aorta. The aorta is the largest blood vessel that carries blood from the heart to the rest of the body. Coarctation of the aorta makes the heart work harder to pump blood to the body. The extra work causes the heart muscle to weaken. It can also cause the heart muscle to become bulky (hypertrophy) and stiff. In some extreme cases, coarctation of the aorta can lead to congestive heart failure. This is a condition in which the heart is unable to pump the normal amount of blood to the body.

Coarctation of the aorta can lead to higher blood pressure in the arteries in the area before the narrowing. These arteries include blood vessels supplying the head and arms. The pressure is lower in the arteries in the area after the obstruction. These arteries include blood vessels that send blood to the abdomen, kidneys, spinal cord, and legs. Different blood pressures in the upper and lower body can lead to complications, such as stroke, kidney failure, and intestinal damage.

Are there any known causes?

Coarctation of the aorta is a congenital heart defect. This means that a child is born with this structural heart defect. The defect forms as the heart is developing. We don't yet know why this heart defect forms. Coarctation of the aorta occurs more often in families with a history of left-sided heart problems, such as aortic stenosis, bicuspid aortic valve, coarctation, or hypoplastic left heart syndrome. It also occurs in children with certain genetic abnormalities such as Turner Syndrome. In some patients, coarctation of the aorta can also be diagnosed with other congenital heart defects, such as bicuspid aortic valve, ventricular septal defect, atrial septal defect or patent ductus arteriosus.

What are common symptoms? How does this problem present?

Children with mild to moderate coarctation of the aorta may appear to be in normal health and have no symptoms at first. Older children with long standing obstruction can have symptoms that include:

  • Shortness of breath
  • Decreased stamina
  • Leg pain
  • Abdominal pain
  • Headaches

Children with “critical” coarctation of the aorta present at birth. They generally have severe symptoms that include:

  • Heavy breathing and grunting
  • Trouble feeding (in infants)
  • Circulatory collapse (child becomes gray, cold, and the heart may stop beating)

When do these symptoms require care from someone who specializes in children's heart disease?

Some forms of coarctation of the aorta can be detected by a fetal echocardiogram or an ultrasound of a baby's heart before birth. This test uses sound waves to form a picture of the baby's heart. This test can be done when the mother is in her second or third trimester.

If coarctation of the aorta is not detected before birth, signs of a heart problem may be found during a physical exam after birth. Possible signs include high blood pressure or poor pulses in the legs.

If a heart problem is suspected, the child can be referred to a pediatric cardiologist, who diagnoses and treats heart problems in children.

What kind of testing and diagnostic tools are used to determine diagnosis or disease stage?

To confirm a diagnosis of coarctation of the aorta, several tests may be done. These include:

  • 4 extremity blood pressure measurements to see if the blood pressure is lower in the legs than in the arms
  • Chest x-ray: X-rays are used to take still pictures of the heart and lungs
  • Electrocardiogram (ECG): An ECG is used to measure the electrical activity of the heart
  • Echocardiogram: An ultrasound which create a movie of the heart and looks for structural defects and other problems
  • Cardiac MRI or CT: Studies that use magnets or x-rays to create a 3-dimensional image of the heart defect

What are possible treatment options?

Coarctation of the aorta can be treated with heart surgery or cardiac catheterization. There are many techniques for surgical repair. The exact technique will depend on the degree of the narrowing, location, and associated problems.

Cardiac catheterization is generally done on older children. The pediatric cardiologist will evaluate your child's heart and discuss the best treatment options with you.

What happens after treatment? Will my child require care throughout childhood/adulthood?

After repair of coarctation of the aorta, most children can be active and participate in sports and physical activities. Regular follow-up visits with the pediatric cardiologist will be needed. The frequency of these visits may decrease over time. Medications may be prescribed to treat problems such as high blood pressure.

The risk of coarctation of the aorta occurring again is low, but possible. Adults with coarctation treated in childhood require periodic cardiac MRI or CT to be sure the repaired area has not become dilated or narrow. Further treatment may be needed when your child is older.

To make an appointment with the Children's Heart Program, please call 410-328-4FIT (4348) or 1-800-373-4111 (referring physicians only).

This page was last updated: June 21, 2013

         
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