Pulmonary stenosis is a birth defect of the heart (congenital). It can happen when the pulmonary valve doesn’t develop as it should during the first 8 weeks of pregnancy. The pulmonary valve connects the right ventricle to the pulmonary artery. It normally has 3 flaps (leaflets) that work like a one-way door. This means they allow blood to flow from the right ventricle to the pulmonary artery, but not backward from the pulmonary artery to the right ventricle.
With pulmonary stenosis, it is harder for the flaps to open and the blood to flow as it should. The flaps may be stuck together. Or the flaps may be thick and not able to open all the way. In some cases, the valve may be narrowed. Sometimes the stenosis may not be related to a problem with the valve leaflets but with the area directly below and above the valve.
Pulmonary stenosis varies according to how much of the blood flow is blocked. A child with severe pulmonary stenosis is likely to be quite ill and have obvious symptoms. A child with mild pulmonary stenosis may have few or no symptoms. He or she may get symptoms later in life. The blockage and symptoms can get worse over time. It is uncommon for this condition to be identified during pregnancy.
Pulmonary stenosis often occurs as part of other complex congenital heart defects.
Pulmonary stenosis occurs when the pulmonary valve doesn’t develop as it should or the area below or above the valve doesn't develop fully during the first 8 weeks of pregnancy. Why this happens isn't known.
Some congenital heart defects are passed down through families (genetic defects).
Some children with pulmonary stenosis do not have symptoms. The more severe the stenosis, the more likely the child is to have symptoms. The most common symptoms may include:
The symptoms of pulmonary stenosis can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.
The healthcare provider will ask about your child’s symptoms and health history. He or she will give your child a physical exam. The provider will listen to your child's heart and lungs with a stethoscope. The provider may hear an abnormal heart sound (heart murmur). He or she may also find other signs or symptoms. The provider may refer your child to a pediatric cardiologist. This is a doctor with special training to treat heart problems in children.
The cardiologist will also examine your child. He or she will carefully listen for a heart murmur. The details about the heart murmur will help with the diagnosis. Your child may have tests, such as:
Mild pulmonary stenosis often does not need treatment. Moderate or severe stenosis needs repair.
Some infants will be very sick and need care in the intensive care unit (ICU) before the defect can be fixed. Some infants may need an emergency repair if the stenosis is very severe. Prostaglandins, a medicine that keeps the ductus arterious open, will be given so that enough blood flows in the lungs. Once the child is stabilized, a procedure will be done to repair the valve. Less severe stenosis will have the repair scheduled.
Repair choices include:
The outlook for children with pulmonary stenosis is usually excellent. For a period of time, your child's cardiologist may recommend your child take antibiotics to prevent infection of the heart lining and valves (bacterial endocarditis). Your child may need to take them before medical and dental procedures.
Sometimes your child may need to have procedures done again during infancy and childhood to stretch the valve open. Your child may need a pulmonary valve replacement as a teen or young adult to prevent complications.
Your child will need regular follow-up care at a congenital cardiac care center throughout life.
Check with your child's cardiologist about your child's outlook.
Call your child’s healthcare provider if he or she has symptoms, especially:
Tips to help you get the most from a visit to your child’s healthcare provider:
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