doctor listening to pediatric patient's heart
doctor listening to pediatric patient's heart
doctor listening to pediatric patient's heart

Coarctation of the Aorta (COA) in Children

What is coarctation of the aorta in children?

Coarctation of the aorta (COA) is a heart defect that is present at birth (congenital). It means the aorta is narrower than it should be. The aorta is the large artery that carries oxygen-rich (red) blood from the left ventricle to the body. This narrowing means that less oxygen-rich blood is sent to the body.

The amount of narrowing can vary. A child with greater narrowing of the aorta will have more symptoms, and the symptoms will be seen at an early age. In some cases, coarctation is seen in infancy. In others, it may not be seen until school-age or teenage years. COA may be diagnosed in infants, school-age children or teens. It is more commonly seen in males. There is an increased chance of having the condition if another family member has it. It also occurs more often in certain genetic syndromes, such as Turner syndrome.

Coarctation of the aorta is often linked to other cardiac defects, such as a bicuspid aortic valve, aortic stenosis, or hypoplastic left heart syndrome.

What causes COA in a child?

Most of the time, COA has no known cause. Some heart defects present at birth have a genetic link. This means they happen more often in certain families. There is an increased risk in families with a history of left-sided heart obstructive lesions.

What are the symptoms of COA in a child?

These are the most common symptoms of coarctation of the aorta:

  • Irritability
  • Pale skin
  • Sweating
  • Heavy or fast breathing
  • Enlarged liver (hepatomegaly)
  • Poor feeding or eating
  • Poor weight gain
  • Cold feet or legs
  • Weak pulses in the feet, or no pulses in the feet
  • Blood pressure in the arms that is much higher than the blood pressure in the legs
  • Chest pain
  • Pain in lower legs with walking (claudication)

Mild narrowing may not cause symptoms at all. A physician or other health care provider may find high blood pressure, or he or she may hear an abnormal sound (heart murmur) when listening to your child’s heart with a stethoscope. Adults with the condition are at increased risk of intracranial aneurysms. This is a weakening and ballooning of blood vessels in the brain. This is especially the case if they have high blood pressure.

The symptoms of COA can be like other health conditions. Make sure your child sees a doctor for a diagnosis.

Interrupted aortic arch 

The most severe and life-threatening form of coarctation of the aorta is called interrupted aortic arch. With interrupted aortic arch, rather than a narrowed vessel, the aorta is incomplete or interrupted at a certain point so that no blood flows to the lower part of the body. The top of the aorta delivers blood to the upper body, but the lower body needs to receive blood from another source (usually a patent ductus arteriosus).

Interrupted aortic arch can be associated with DiGeorge syndrome, as well as an atrial septal defect, ventricular septal defect or truncus arteriosus.

How is COA diagnosed in a child?

Coarctation of the aorta may sometimes be diagnosed before birth during a prenatal ultrasound, although this is very difficult. However, most cases are often not definitively diagnosed until after birth.

All babies are born with a patent ductus arteriosus (PDA), an opening between the pulmonary artery and the aorta that allows blood to flow directly from the heart to the lungs before the baby is born. In babies with coarctation of the aorta, the PDA helps to supply blood to the lower parts of the body while the baby is in utero.

A baby’s PDA should close within the first few days after birth. At that point, if coarctation of the aorta is suspected, further testing will be recommended.

Milder cases may not be detected until the child is older. A doctor or other health care provider will ask about your child’s symptoms and health history. He or she will perform a physical exam, and may refer your child to a pediatric cardiologist, a doctor with special training to treat heart problems in children. The cardiologist will examine your child and listen to his or her heart and lungs. Your child may also have tests such as:

  • Chest X-ray. A chest X-ray may show an enlarged heart or other changes caused by COA.
  • Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It can find abnormal rhythms (arrhythmias) and heart muscle damage.
  • Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. Most aortic coarctations are diagnosed by echocardiography.
  • Cardiac catheterization (cath). A heart cath uses a thin, flexible tube (catheter) that is moved through the blood vessels into the heart. Dye is used to get a clear picture of the heart and aorta. Catheterization may also be used to correct the narrowing.
  • CT scan. CT can make detailed pictures of the aorta and show the area of narrowing.
  • MRI. MRI makes detailed pictures of the heart and aorta. It can clearly show where the narrowing is and how large it is.

How is COA treated in a child?

Treatment will depend on your child’s symptoms, age and general health. It will also depend on how severe the condition is.

COA is treated by fixing the narrowed vessel. It may be fixed by:

Cardiac catherization. During the procedure, your child is given medicine to help him or her relax (sedation). A doctor puts a catheter through a blood vessel to the narrowed part of the aorta. A balloon at the tip of the catheter is inflated to stretch the area open. The doctor may put a small metal mesh tube (stent) in the narrowed area to keep the aorta open. Your child will usually need to stay overnight in the hospital.

Surgery. Your child will be given sleep medicine (general anesthesia). The surgeon will make a cut (incision) into your child’s chest. The location of the incision will depend on the extent of narrowing of the aorta, as well as if there are additional defects that need to be fixed during the same surgery. The surgeon will cut out the narrowed area or make it larger, and connect the two ends of the aorta together. In some instances, the surgeon will have to use additional nearby tissue or patch material to make the aorta longer.

What are the complications of COA in a child?

If untreated, COA causes several problems:

  • The left ventricle, which pumps blood through the aorta and to the body, becomes weak and fails
  • Blood pressure is too high in the upper body and too low in the lower body
  • Kidneys don't work as they should
  • Disease of the coronary arteries
  • Infection of the heart and blood vessel wall
  • The aorta or other arteries can tear, causing uncontrolled bleeding or stroke
  • Death

How can I help my child live with COA?

Most children with COA will need to have the defect fixed. Mild defects may only require monitoring. Some infants will be very sick and need care in the intensive care unit before the procedure. In some cases, a child may need emergency repair. Children who have few symptoms will have scheduled repairs. Most children who have had a COA repair will live healthy lives. Activity levels, appetite and growth should return to normal in time.

After the repair, your child’s cardiologist may recommend your child take antibiotics before surgeries or procedures, such as dental work. This is done to prevent infection.

As the child grows, the aorta may again become narrow. If this happens, another balloon procedure, stent or surgery may be done.

Controlling blood pressure is very important. Your child’s blood pressure may be higher after aortic coarctation repair. He or she may need to take medicines to help lower blood pressure.

Your child will need regular follow-up care throughout their life with a cardiologist who has expertise in congenital heart defects.

When should I call my child's doctor?

Call your child’s pediatrician right away if you notice changes in your child’s symptoms. For example, your child might have more trouble breathing or is eating less.

Pediatric Heart Care at Johns Hopkins

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