Pulmonary valve stenosis is a heart defect that is present at birth (congenital). It happens when the fetal heart doesn’t develop normally during the first two months of pregnancy.

The pulmonary valve is within the heart's right ventricle. It is the opening to the pulmonary artery, which takes blood to the lungs. The pulmonary valve has three flaps that function like a one-way door. It allows blood to flow forward into the pulmonary artery, but not backward into the right ventricle.

Pulmonary valve stenosis is a narrowing of the pulmonary valve. Because of the narrowing of the valve, the right ventricle needs to work harder to get blood past the blockage and into the lungs. In children, pulmonary stenosis can include a valve that:

• Has only one or two flaps instead of three
• Has flaps that are partially connected
• Has thick flaps that do not open all the way

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

• Your child's age, overall health and medical history
• Extent of the condition
• Your child's tolerance for specific medications, procedures or therapies
• Expectations for the course of the condition
• Your opinion or preference

Mild pulmonary valve stenosis often does not require treatment. Moderate or severe pulmonary valve stenosis is treated with repair of the valve. Several options are currently available.

Some infants will be very sick and require care in the cardiac intensive care unit (CICU) before the procedure. Some 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.

Pulmonary valve repair options include the following:

• Balloon dilation or valvuloplasty: This is a cardiac catheterization procedure. A small, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. The tube has a deflated balloon in the tip. When the tube is placed in the narrowed valve, the balloon is inflated to stretch the area open.
• Valvotomy: Surgical release of connections that are preventing the valve flap from opening properly. 

For some families, it can be helpful to come prepared with a plan before your appointment with a cardiologist. Here are things to consider documenting and having ready for your appointment:

• Symptoms your child is experiencing: Sharing when these symptoms appeared and how frequently they occur is helpful to the medical team. Don’t be afraid to share symptoms that seem unrelated — congenital heart disease can affect other parts of the body.
• Medications: This should also include any supplements or vitamins your child takes.
• Related history: This includes family medical history and recent changes in your family’s life.
• Your questions: This will help ensure you don’t forget to ask all your questions. It is okay to ask the same question several times if you are unclear. Our team is here to help you understand the big picture and make sure you walk away with your questions answered.

Your child will need to be monitored by a cardiologist in case new problems develop. Once they reach adulthood, they should seek out a program that specializes in adult congenital heart disease. The Herma Heart Institute’s Adult Congenital Heart Disease Program is the largest in the state.