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 narrowed part of your aorta.
Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and might not be detected until adulthood, depending on how much the aorta is narrowed.
Coarctation of the aorta often occurs along with other heart defects. While treatment is usually successful, the condition requires careful lifelong follow-up.
Coarctation of the aorta symptoms depend on the severity of the condition. Most people don't have symptoms. Children with serious aortic narrowing may show signs and symptoms earlier in life, but mild cases with no symptoms might not be diagnosed until adulthood. People may also have signs or symptoms of other heart defects that they have along with coarctation of the aorta.
Babies with severe coarctation of the aorta may begin having signs and symptoms shortly after birth. These include:
- Pale skin
- Irritability
- Heavy sweating
- Difficulty breathing
- Difficulty feeding
Left untreated, aortic coarctation in babies might lead to heart failure or death.
Older children and adults with coarctation of the aorta often don't have symptoms because their narrowing may be less severe. If you have signs or symptoms that appear after infancy, you most commonly will have high blood pressure (hypertension) measured in your arms. However, your blood pressure is likely to be lower in your legs. Signs and symptoms might include:
- High blood pressure
- Headache
- Muscle weakness
- Leg cramps or cold feet
- Nosebleeds
- Chest pain
Doctors aren't certain what causes coarctation of the aorta (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 (congenital). Congenital heart defects are the most common of all birth defects.
Rarely, coarctation of the aorta develops later in life. Traumatic injury might lead to coarctation of the aorta. Rarely, severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu's arteritis) can narrow the 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 can often lead to high blood pressure in your arms but low blood pressure in your legs and ankles.
With coarctation of the aorta, the lower left heart chamber (left ventricle) of your heart works harder to pump blood through the narrowed aorta, and blood pressure increases in the left ventricle. This may cause the wall of the left ventricle to thicken (hypertrophy).
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. The condition is more common in males than in females. You or your child may be more likely to have aortic coarctation if certain heart conditions exist, including:
- 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 flaps (cusps) instead of the usual three. Many people with coarctation of the aorta have a bicuspid aortic valve.
- Patent ductus arteriosus. Before birth, 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.
- Holes in the wall between the left and right sides of the heart. You may have a hole in the wall (septum) between the upper chambers of the heart (atrial septal defect) or the lower chambers of the heart (ventricular septal defect) when you're born.
This causes oxygen-rich blood from the left side of the heart to mix with oxygen-poor blood in the right side of the heart.
- Aortic valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta (aortic valve). This means your heart has to pump harder to get adequate blood flow to your body.
Over time, this can cause your heart muscle to thicken and lead to symptoms such as chest pain, fainting spells and breathlessness, or heart failure.
- Aortic valve regurgitation. This occurs when the aortic valve doesn't close tightly, causing blood to leak backward into the left ventricle.
- Mitral valve stenosis. This is a narrowing of the valve (mitral valve) between the upper left heart chamber (left atrium) and the left ventricle that lets blood flow through the left side of your heart.
In this condition, 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.
- Mitral valve regurgitation. This occurs when the mitral valve doesn't close tightly, causing blood to leak backward into the left atrium.
Coarctation of the aorta is also more common in people who have certain genetic conditions, such as Turner syndrome. Women and girls with Turner 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.
Talk to you doctor if you or your child has of any of these risk factors or a family history of congenital heart disease.
Untreated coarctation of the aorta frequently leads to complications. Some complications may be a result of long-standing high blood pressure caused by the aortic coarctation. Complications are also possible after treatment for coarctation of the aorta.
Complications of coarctation of the aorta may include:
- Narrowing of the aortic valve (aortic stenosis)
- High blood pressure
- Stroke
- Enlargement in a section of the wall of the aorta (aneurysm)
- Aortic rupture or tear (dissection)
- Premature coronary artery disease — narrowing of the blood vessels that supply the heart
- Heart failure
- A weakened or bulging artery in the brain (brain aneurysm) or bleeding in the brain (hemorrhage)
In addition, if the coarctation of the aorta is severe, your heart might not be able to pump enough blood to your other organs. This can cause damage to your heart and also can result in kidney failure or other organ failure.
If your coarctation of the aorta was treated when you were young, you have a risk of the aorta re-narrowing (re-coarctation) over time. You also have a higher risk of developing high blood pressure. You will need lifelong follow-up for coarctation of the aorta, and you may require additional treatments.