Robert Wood Johnson University Hospital
 

Aortic Stenosis

Heart Conditions in Children - Aortic Stenosis

What is aortic stenosis?

Illustration of the anatomy of the heart, normal
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Aortic stenosis is a heart defect that may be congenital (present at birth) or acquired (develop later in life). If the problem is congenital, then something occurred during the first 8 weeks of pregnancy to affect the development of the aortic valve.

The aortic valve is found between the left ventricle and the aorta. It has three leaflets that function like a one-way door, allowing blood to flow forward into the aorta, but not backward into the left ventricle. Aortic stenosis is the inability of the aortic valve to open completely.

With aortic stenosis, problems with the aortic valve make it harder for the leaflets to open and permit blood to flow forward from the left ventricle to the aorta. In children, these problems can include a valve that:

Illustration of aortic stenosis
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Aortic stenosis may be present in varying degrees, classified according to how much obstruction to blood flow is present. A child with severe aortic stenosis will be quite ill, with major symptoms noted early in life. A child with mild aortic stenosis may have few symptoms, or perhaps none until later in adulthood. The degree of obstruction can become worse with time.

Congenital aortic stenosis occurs in 4 to 6 percent of all children with congenital heart disease. Relatively few children are symptomatic in infancy, but the incidence of problems increases sharply in adulthood.

Congenital aortic stenosis occurs three times more often in boys than in girls.

What causes aortic stenosis?

Congenital aortic stenosis occurs due to improper development of the aortic valve in the first 8 weeks of fetal growth. It can be caused by a number of factors, though, most of the time, this heart defect occurs sporadically (by chance), with no apparent reason for its development.

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families.

Acquired aortic stenosis may occur after a strep infection that progresses to rheumatic fever.

Why is aortic stenosis a concern?

Mild aortic stenosis may not cause any symptoms. Several problems may occur, however, when aortic stenosis is moderate to severe, including the following:

What are the symptoms of aortic stenosis?

The following are the most common symptoms of aortic stenosis. However, each child may experience symptoms differently. Symptoms may include:

The symptoms of aortic stenosis may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

How is aortic stenosis diagnosed?

Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction from the right ventricle to the pulmonary artery. Symptoms your child exhibits will also 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 examination, listening 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 (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Some tests that may be recommended include the following:

Treatment for aortic stenosis:

Specific treatment for aortic stenosis will be determined by your child's physician based on:

Aortic stenosis is treated with repair of the obstructed valve. Several options are currently available.

Some infants will be very sick, require care in the intensive care unit (ICU) prior to the procedure, and could possibly even need emergency repair of the aortic stenosis. Others, who are exhibiting few symptoms, will have the repair scheduled on a less urgent basis.

Activity may be limited in children who have moderate aortic stenosis prior to repair. For instance, competitive sports that require endurance may be restricted.

Repair options include the following:

Postoperative care for your child:

After surgery, your child will go to the intensive care unit (ICU). While your child is in the ICU, special equipment will be used to help him/her recover from surgery, and may include the following:

Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.

Your child will be kept as comfortable as possible with several different medications; some of which relieve pain and some of which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.

After discharge from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.

Long-term outlook after aortic stenosis surgical repair:

Most children who have had an aortic stenosis surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal.

As the child grows, a valve that was ballooned may once again become narrowed. If this happens, a second balloon procedure or operation may be necessary to repair aortic stenosis. Sometimes the aortic tissue itself may be abnormal, which might lead to complications in the teen or adult years. Regular follow-up care at a specialized cardiac center should continue throughout life.

Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis after discharge from the hospital.

Individuals who had a mechanical valve replacement may need to take anticoagulants (blood thinners) to prevent blood clots from forming on the artificial valve surfaces. Regular monitoring of the blood's clotting status is very important in maintaining the most appropriate dose of anticoagulants.

Initial valve replacement is often performed using a tissue valve to avoid the need for anticoagulation, especially for females of childbearing age. Anticoagulation during pregnancy is very difficult to manage, and requires special treatment.

Repeat valve replacement is not uncommon during the lifespan. In addition, blood pressure should be closely monitored and managed.

Consult your child's physician regarding the specific outlook for your child.

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