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What is coarctation of the aorta?

Coarctation of the aorta is a narrowing of the aorta, the main artery that delivers oxygen-rich (red) blood to the body. A coarctation is located just past the aortic arch, which has branches providing blood to the head and arms. When this defect is present, blood flow is restricted and the left ventricle of the heart must pump harder to push blood through the narrowed opening.

In severe cases, this blockage can cause the left ventricle to weaken and make your child very sick. In older children, this blockage can cause the left ventricle to thicken and also cause high blood pressure in the brain. If the muscle becomes too thick and is no longer able to function efficiently and handle its workload, it can eventually fail. If uncorrected, or corrected late, coarctation can result in lifelong high blood pressure.

Coarctation of the aorta occurs in about 6 to 8 percent of all children with congenital heart disease, and twice as frequently in boys. It also occurs in about 10 percent of girls who have Turner syndrome, a chromosomal abnormality.

Seventy-five percent of children with coarctation of the aorta also have a bicuspid aortic valve, in which the aortic valve has two leaflets, instead of the usual three. Other commonly associated defects can include:

How we care for coarctation of the aorta

Boston Children’s Hospital has vast experience treating infants and children who have coarctation of the aorta. Our team in the Benderson Family Heart Center treats some of the most complex pediatric heart conditions in the world with overall success rates approaching 98 percent — among the highest in the nation for large pediatric cardiac centers.

The majority of coarctations are treated surgically although we have developed increasing experience with catheter-based interventions as well. Treatment is individualized to the child’s specific anatomy, age, size and other associated cardiac conditions.

Our areas of innovation for coarctation of the aorta

Innovations at Boston Children’s in the treatment of coarctation include:

  • achieving repairs without the heart/lung machine
  • all-natural tissue repairs
  • advanced brain-protection strategies
  • minimally-invasive and catheter-based solutions
  • “hybrid” collaborations between interventional cardiology and cardiac surgery

Coarctation of the Aorta | Symptoms & Causes

What are the symptoms of coarctation of the aorta?

The more severe the narrowing of the aorta, the more signs and symptoms a child will have, and the earlier the problem will be noticed. In infants, the onset of symptoms in a previously “fine” baby can be sudden and severe. On the other end of the spectrum, mild narrowing may not cause any symptoms at all.

Common signs include:

  • heavy or rapid breathing
  • pale skin
  • poor feeding
  • sweating
  • irritability

The most common physical finding for coarctation of the aorta in an infant is that the baby’s femoral (leg) pulses are weak and difficult for the doctor to feel.

In some cases, coarctation of the aorta may not be detected until school age, adolescence or even adulthood — often during a routine blood pressure test. As the child grows, signs and symptoms can appear, such as:

  • high blood pressure
  • nosebleeds
  • heart murmur
  • headaches (from high blood pressure)
  • cramps in the lower sections of the body (from low blood pressure) 

What causes coarctation of the aorta?

Coarctation of the aorta may be due to improper development of the aorta in the first eight weeks of fetal growth. Congenital heart defects, like coarctation of the aorta, usually occur by chance, with no clear reason for their development.

This condition also may be associated with other heart defects such as Shone’s syndrome, bicuspid aortic valve and ventricular septal defect.

Coarctation of the Aorta | Diagnosis & Treatments

How is coarctation of the aorta diagnosed?

The first line of diagnosis for coarctation of the aorta is a clinical exam, beginning with obtaining the child’s vital signs. The pediatric cardiologist obtains a four-limb blood pressure — measuring the blood pressure in both arms and both legs.

Coarctation of the aorta is suspected when the doctor notes a lower blood pressure in the legs. The child’s leg or foot pulses will be weak and therefore difficult for the doctor to feel. Other tests that help with the diagnosis — or with planning for treatment — may include:

Can coarctation of the aorta be diagnosed prenatally?

Coarctation of the aorta can sometimes be detected prenatally by fetal echocardiogram. But the aorta isn’t usually obstructed in the fetus because of the open ductus arteriosus (the fetal connection that ensures blood flow between the aorta and the pulmonary artery). The obstruction can become evident after birth once the ductus arteriosus closes.

How is coarctation of the aorta treated?

Coarctation of the aorta is treated by removing or patching the narrow part of the vessel. Newborns who are very sick and require care in the cardiac intensive care unit (CICU) may need urgent repair of the coarctation. 

For most infants, surgery is the standard treatment. The surgeon cuts out the narrowed segment and sews the two healthy ends of the aorta back together, establishing normal blood flow through the vessel. 

If the child also has a large ventricular septal defect, our surgeons usually repair the defect at the same time as the coarctation. If there's a bicuspid aortic valve, it will need to be followed by your cardiologist to make sure that no blockage (stenosis) or leakage (regurgitation) develop later.

Some patients have narrowing of the aortic arch as well. In this case, our surgeons will repair the arch and coarctation at the same time using the heart/lung machine. Depending on the anatomy, this repair can be achieved with all natural tissue or a patch.

In a small percentage of patients, the narrowing can return. In that small number of cases, a catheterization with balloon dilation is very effective. Rarely, a stent is required.

What is the long-term outlook for children with coarctation of the aorta?

The long-term outlook for children with coarctation of the aorta is excellent. Nearly all children who've had surgery for coarctation of the aorta will live normal, healthy lives.

Coarctation of the Aorta | Programs & Services