Pulmonary Stenosis

Heart Conditions in Children - Pulmonary Stenosis

What is pulmonary stenosis?

Anatomy of the heart, view of the valves
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Pulmonary stenosis is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy.

The pulmonary valve is found between the right ventricle and the pulmonary artery. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery, but not backward into the right ventricle.

With pulmonary stenosis, problems with the pulmonary valve make it harder for the leaflets to open and permit blood to flow forward from the right ventricle to the lungs. In children, these problems can include:

There are four different types of pulmonary stenosis:

Pulmonary stenosis may be present in varying degrees, classified according to how much obstruction to blood flow is present. A child with severe pulmonary stenosis could be quite ill, with major symptoms noted early in life. A child with mild pulmonary stenosis may have few or no symptoms, or perhaps none until later in adulthood. A moderate or severe degree of obstruction can become worse with time.

Illustration of pulmonary stenosis
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Pulmonary stenosis is a component of half of all complex congenital heart defects.

Pulmonary stenosis is the second most common congenital heart defect, comprising 5 to 10 percent of all cases.

What causes pulmonary stenosis?

Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight 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 clear reason evident 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.

Why is pulmonary stenosis a concern?

Mild pulmonary stenosis may not cause any symptoms. Problems can occur when pulmonary stenosis is moderate to severe, including the following:

What are the symptoms of pulmonary stenosis?

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

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

How is pulmonary 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 the 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 pulmonary stenosis

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

Mild pulmonary stenosis often does not require treatment. Moderate or severe 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 pulmonary valve if the stenosis is severe. Others, who are exhibiting few symptoms, will have the repair scheduled on a less urgent basis.

Repair options include the following:

Postprocedure care for your child

Long-term outlook after pulmonary stenosis repair

Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a period of time following surgery and always for all valve replacements.

Occasionally, repeat interventional cath lab procedures may be necessary during infancy and childhood to stretch the valve open. Replacement of the pulmonary valve is generally recommended later during adolescence or early adulthood to prevent complications such as enlargement of the right ventricle, heart failure, and dysrhythmias (irregular or fast heartbeats).

Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individual's lifespan.

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

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