Pulmonary Valve Stenosis

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KEY POINTS

• Pulmonary valve stenosis (PVS) is a narrowing in the opening of the pulmonary valve in the heart. The narrowing reduces the amount of blood that is pushed out of the heart to the lungs with each heartbeat, which can damage the heart muscle over time.
• PVS may be mild and not need treatment. If pulmonary valve stenosis is serious, surgery to repair or replace the valve will be needed.
• Follow your child’s healthcare provider's instructions. Ask your child’s healthcare provider what symptoms or problems you should watch for and what to do if your child has them.

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What is pulmonary valve stenosis?

Pulmonary valve stenosis (PVS) is a narrowing in the opening of the pulmonary valve in the heart. The valve may be misshapen or too thick and does not open completely. The pulmonary valve is between the right lower heart chamber and the artery that carries blood to the lungs.

The pulmonary valve is narrow, which reduces the amount of blood that is pushed out of the heart to the lungs with each heartbeat. The right side of the heart must pump harder to move enough blood through the narrow valve. This can damage the heart muscle over time.

What is the cause?

Most of the time, the cause of PVS is not known. It is a type of congenital heart disease, which means that the heart did not develop normally while the baby was in the womb.

Some conditions that increase the risk of being born with PVS and other heart problems include:

• Other family members have congenital heart disease.
• The mother has an infection during pregnancy such as rubella (German measles).
• The baby has a problem with genes, which are in every cell of the body. Genes contain the information that tells the body how to develop and work. Changes in the genes can be passed from parents to children

Some medicines or drugs taken by the mother during pregnancy or even a month or 2 before pregnancy may increase the risk that a baby will be born with a heart problem.

What are the signs and symptoms?

Mild PVS may not be noticed until the adult years, and there may be no symptoms.

Sometimes, PVS causes symptoms soon after birth. Signs and symptoms of serious PVS in a baby or child may include:

• Being short of breath or breathing fast
• Being weak or tired
• Not feeding well, or choking, gagging, or vomiting
• Having trouble gaining weight
• Irritability
• Sweating, especially when feeding
• Turning blue around the lips or fingers

How is it diagnosed?

The healthcare provider will ask about your child's symptoms and medical history and examine your child. Your healthcare provider may find a problem before birth by using ultrasound. Ultrasound uses sound waves to see how a baby is growing during pregnancy.

Tests may include:

• Echocardiogram, which uses sound waves (ultrasound) to see how well the heart is pumping
• ECG (also called an EKG or electrocardiogram), which measures and records the heartbeat
• Chest X-ray
• Heart catheterization (coronary angiogram), which uses a small tube called a catheter inserted into a blood vessel, contrast dye, and X-rays to look at the blood vessels and heart
• MRI, which uses a strong magnetic field and radio waves to show detailed pictures of the heart

How is it treated?

Pulmonary valve stenosis may be mild and not need treatment. If pulmonary valve stenosis is more serious, it will need repair. The repair may be done during these procedures:

• Heart catheterization (coronary angiogram) uses a small tube called a catheter inserted into a blood vessel. The healthcare provider will use tools put through the catheter to repair the valve.
• In open heart surgery, your healthcare provider repairs the valve.

How can I take care of my child?

Follow your child’s healthcare provider's instructions. Ask your child’s healthcare provider:

• How and when you will get your child’s test results
• How long it will take for your child to recover
• If there are activities your child should avoid and when your child can return to normal activities
• How to take care of your child at home
• If your child should take antibiotic medicine to prevent infection before having dental work or procedures that involve the rectum, bladder, or vagina
• What symptoms or problems you should watch for and what to do if your child has them

Make sure you know when your child should come back for a checkup. A child who has had a valve repaired may need more surgery as an older child or as an adult. Keep all appointments for provider visits or tests.