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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 8 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:
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.
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.
Congenital pulmonary stenosis occurs due to improper development of the pulmonary 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 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.
Mild pulmonary stenosis may not cause any symptoms. Problems can occur when pulmonary stenosis is moderate to severe, including the following:
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.
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. However, other tests are needed to help with the diagnosis, and may include the following:
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.
Children who do not require immediate repair in infancy may need to receive antibiotics to prevent an infection of the inner surfaces of the heart known as bacterial endocarditis prior to procedures such as a routine dental check-up and teeth cleaning. Other procedures may also increase the risk of the heart infection occurring. It is important that you inform all medical personnel that your child has pulmonary stenosis so they may determine if the antibiotics are necessary prior to the procedure.
Repair options include the following:
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 which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.
After discharged 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.
Most children who have had a pulmonary stenosis surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal. Infants who had a difficult post-operative course may be less healthy than infants who did not experience complications.
Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis after discharge from the hospital.
Consult your child's physician regarding the specific outlook for your child.
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Online Resources of Cardiovascular Disorders
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