What is Pulmonary Valve Stenosis

Pulmonary valve stenosis is a heart problem that some children are born with (congenital heart defect). This defect is a stiffening or narrowing (stenosis) of a one-way valve in the heart.

This is the valve that allows blood to flow from the pumping chamber on the right side of the heart (right ventricle) into the blood vessel that carries blood to the lungs (pulmonary artery). If there is not enough blood flowing through the valve, your child will not get enough oxygen into his or her blood. Your child’s heart will also be forced to work harder.

Pulmonary valve stenosis develops during the first 8 weeks as a child is growing inside the womb (uterus). It sometimes develops with other types of heart abnormalities.

Pulmonary valve stenosis can range from mild to severe. Mild cases may not require treatment. Severe cases may require surgery to open or replace the valve. Signs and symptoms of severe pulmonary valve stenosis may show up soon after birth.

What are the causes?

The cause of this condition is not known. In some cases, genes that are passed down through families (inherited) may contribute to this condition.

What are the signs or symptoms?

Symptoms of this condition depend on how much the valve is slowing down blood flow. Symptoms may include:

• Quick and heavy breathing.
• Bluish color of the skin.
• Trouble feeding.
• Not gaining weight.
• Swelling of the feet, ankles, face, or belly.

Older children may become short of breath when playing and they may have chest pain.

How is this diagnosed?

This condition may be diagnosed based on:

• Your child’s symptoms and a physical exam. During the exam, the health care provider will listen for an abnormal heart sound (heart murmur). This is caused by blood flow through the narrowed valve.
• Tests to confirm the diagnosis. Tests may include:
  • Chest X-ray. This may show an enlarged heart chamber caused by increased work.
  • Electrocardiogram (ECG). This is an electrical tracking of the heart’s rhythm. It may show abnormal heartbeats.
  • Echocardiogram. This test creates an image of the heart using sound waves and a computer. It may show an abnormal valve.
  • Cardiac catheterization. In severe cases, a health care provider may thread a thin, flexible tube (catheter) into the heart through a blood vessel in the groin. This catheter can measure pressure and oxygen levels on both sides of the pulmonary valve.

How is this treated?

Treatment for this condition depends on how severe the condition is.

• Mild cases may not cause symptoms and may not require treatment. Your child’s condition may just need to be managed with frequent checkups.
• Severe cases may require treatment, which may include:
  • Balloon valvuloplasty. This is the most common treatment. In this procedure, a catheter is placed in the heart through the valve. Then, a tiny balloon on the end of the catheter is blown up (inflated) to open the valve.
  • Heart surgery to open, repair, or replace the valve.

Follow these instructions at home:

Follow all instructions from your child’s health care provider.

• Give your child over-the-counter and prescription medicines only as told by his or her health care provider.
• Do not give your child aspirin unless your child’s health care provider or cardiologist tells you to do that.
• Have your child follow activity restrictions if told by your child’s health care provider.. Ask your child’s health care provider what activities are safe for your child. Depending on the severity of the the condition, some children can participate in the usual activities.
• Your child does not have to follow a special diet.
• Make sure your child’s immunizations are up to date.
• Let all health care providers know that your child has pulmonary valve stenosis before any medical procedure.
• Ask your health care provider if your child should take antibiotic medicine before having any medical or dental procedure.
• Keep all follow-up visits as told by your health care provider. This is important.

Get help right away if:

• Your child’s symptoms are getting worse.
• Your child has new symptoms.
• Your child’s skin develops a bluish color.
• Your child has trouble breathing.
• Your child passes out.
• Your child has chest pain.