Coarctation of the aorta is a narrowing of the aorta. The aorta is the main blood vessel carrying oxygen-rich blood from the left ventricle of the heart to the rest of the body.
The coarctation occurs most often in a short piece of the aorta just beyond where the arteries to the head and arms take off. This is the area of the aorta where another blood vessel, called the ductus arteriosus, typically inserts.
The ductus arteriosus is a blood vessel that is normally present in a fetus. It has special tissue in its wall that causes it to close after birth, in the first hours or days of life. Coarctation may be caused by having extra ductal tissue.
In some babies with coarctation, the aortic arch prior to area of most significant narrowing may also be small (hypoplastic). Coarctation may also occur with other cardiac defects, typically involving the left side of the heart. The defects usually seen with coarctation are bicuspid aortic valve and ventricular septal defect. Coarctation may also be seen as a part of more complex, single ventricle heart defects. Coarctation of the aorta is common in some patients with genetic disorders, such as Turner's syndrome.
When a coarctation exists, the left ventricle must work harder to create a higher pressure than normal to force blood through the narrow part of the aorta to the lower part of the body.
If the narrowing is severe early in life, the ventricle may not be strong enough to do this extra work. This can lead to congestive heart failure or not enough blood flow to the organs of the body.
Coarctation of the aorta is typically present from birth or shortly after birth. The age at which coarctation is found depends on the severity of the narrowing.
In about 25% of cases of isolated coarctation (without other associated cardiac defects), the narrowing is severe enough to cause symptoms in the first days of life. When the ductus arteriosus closes, the left ventricle must suddenly pump against much higher resistance to blood flow at the area of the coarctation. This can lead to heart failure and shock. Because these newborns are well until the ductus arteriosus closes, symptoms appear quickly. They are often severe.
In patients who do not develop heart failure as newborns, coarctation may not be found until the child is several years old. In these older patients, coarctation is often first thought of because of a heart murmur or high blood pressure.
Coarctation is also considered when the doctor is unable to feel pulses in a child’s legs. High blood pressure in the arms (but not the legs) is often present. A heart murmur is usually present. It may be loudest in the back where the aorta is located.
The diagnosis of coarctation is confirmed with echocardiography. This can look at the anatomy of the aorta. It can also check for other cardiac anomalies that may be present and give important information about the function of the heart. Sometimes other tests, such as a cardiac MRI or CT scan, may be used to look at the aorta and coarctation in more detail prior to intervention.
Coarctation may also be diagnosed on fetal echocardiograms. It is one of the cardiac defects that may be found on screening ultrasounds. Early diagnosis allows for prompt intervention at the time of birth. However, while the ductus arteriosus is open in utero it can be unclear in some patients if there is a risk of a coarctation forming. Some infants may need to be observed closely after birth as the ductus arteriosus closes to detect if there is coarctation.
Planning to deliver an infant at a hospital capable of newborn resuscitation is important in improving the chances for a good outcome.
Learn more about our Fetal Heart Program.
Last Updated 04/2024
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