Coarctation of the aorta

Definition

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

The signs and symptoms of coarctation of the aorta depend on its seriousness. 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.

Risk factors

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.
• Ventricular septal defect. In this condition, there's a hole in the wall that separates the two lower chambers (ventricles) of the heart. Oxygen-poor blood from the right ventricle mixes with oxygen-rich blood from the left ventricle, which pumps blood out to the body.
• 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.
• cause:
  

Doctors aren't certain what causes aortic coarctation in most cases. 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. Severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) may narrow your aorta, leading to aortic coarctation. In rare cases, trauma may lead to coarctation of the aorta.

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.

How is coarctation of the aorta diagnosed?

Your child's physician may have heard a heart murmur during a physical exam and referred your child to a pediatric cardiologist for testing. A heart murmur is simply a noise caused by the turbulence of blood flowing through a narrow region. Symptoms your child exhibits also will help with the diagnosis.

• A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood.
• The cardiologist will perform a physical exam, listen to your child's heart and lungs, and make other observations that help in the diagnosis.

The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur will give the cardiologist an initial idea of which heart problem your child may have. Other tests are needed to help with the diagnosis, and may include:

• chest x-ray
• echocardiogram (echo)
• electrocardiogram (ECG or EKG)
• magnetic resonance imaging (MRI)

Treatment & Care

Coarctation of the aorta is treated with repair of the narrowed vessel. This may be done through a cardiac catheterization procedure or through surgery in an operating room.

Very sick infants who require care in our intensive care unit (ICU), may need emergency repair of the coarctation. Others with few symptoms may have the repair scheduled on a less urgent basis.

• Cardiac catheterization
  • During the procedure, your child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart.
  • Once the catheter is in the heart, the cardiologist will pass an inflated balloon through the narrowed section of the aorta to stretch the area open.
  • A small device, called a stent, may also be placed in the narrowed area after the balloon dilation to keep the aorta open.
• Surgery
  • The surgical repair is done through an incision on the left side of the chest below your child’s armpit.
  • The ribs are spread, the lung moved aside, and the aorta exposed near the heart.
  • The aorta is clamped on either side of the narrowing and the narrow segment is cut out.
  • The two ends of aorta are then sewn together, clamps are removed and flow through the aorta is reestablished.