Pulmonary valve stenosis (PVS)

Pulmonary valve stenosis explained

Your heart has four valves that help blood flow through the heart. Your pulmonary valve is a valve on the right side of your heart.

Your pulmonary valve opens to let blood flow to your lungs through the pulmonary artery and closes to stop blood flowing backwards into your heart.

If you have pulmonary valve stenosis, your pulmonary valve and the area around it can be narrow. This means blood does not flow properly through your heart. And your heart has to work harder to pump blood to your lungs.

Over time, the muscle around the lower chamber on the right side of your heart (your right ventricle) can become thicker to try and help pump blood better.

Pulmonary valve stenosis is sometimes called pulmonary stenosis.

Types of pulmonary valve stenosis

• Valvular pulmonary stenosis is when your pulmonary valve is narrow. This is the most common type of pulmonary valve stenosis.
• Subvalvular pulmonary stenosis is when there is a narrowing before your pulmonary valve. This can happen because your heart muscle is thicker.
• Supravalvular pulmonary stenosis is when there is a narrowing in your pulmonary artery. You may be more likely to have this type of pulmonary valve stenosis if you have a genetic condition like Williams Syndrome.

Speak to your doctor about what type of pulmonary stenosis you have and what this means for you.

Levels of pulmonary valve stenosis

There are different levels of pulmonary valve stenosis. No matter what level you have, your pulmonary valve will not work as well as it should.

• Mild – enough blood is pumped to the lungs. 
• Moderate or severe – the lungs do not get enough blood, and your heart has to work harder.

If you’re diagnosed with moderate or severe stenosis, you’ll usually need treatment. If your stenosis is mild, you’ll normally have regular appointments to check how your heart works.

If your heart works harder over a long time, it can cause problems like your heart muscle can become thicker. It can also cause other conditions, such as:

• heart failure
• irregular heart rhythm
• Eisenmenger syndrome.

Symptoms of pulmonary valve stenosis

Most people with pulmonary valve stenosis have no symptoms except for a heart murmur.

This is a swishing noise created by the blood flowing the wrong way in your heart. Doctors can hear a heart murmur when they listen to your heart with a stethoscope.

If your condition is severe, you may feel more tired than usual (fatigue). If this happens speak to your doctor.

Diagnosing pulmonary valve stenosis

Most people will be diagnosed shortly after they’re born during a routine health check. Some adults may be diagnosed during a health check or if you’ve been having symptoms.

Tests to diagnose pulmonary valve stenosis include:

• electrocardiogram (ECG)
• echocardiogram (echo)
• chest x-ray
• CT scan
• MRI
• stress test
• blood tests.

Your doctor may also ask you to have a certain type of heart catheterisation to check how your heart works in more detail.

These tests do not hurt. Speak to a healthcare professional if you have any questions or you’re worried about tests you’re having.

Causes of pulmonary valve stenosis

We do not know why pulmonary valve stenosis happens. Not knowing what has caused your condition can be frustrating. It can help to find out more about the causes of congenital heart disease.

Treatment for pulmonary valve stenosis

Pulmonary valve stenosis is usually treated with surgery when you’re a baby.

This could be either:

• balloon valvoplasty (also called balloon dilation)
• open heart surgery.

As you get older you may need more treatment. You may need more surgery to repair your pulmonary valve. You may also need other treatments if your condition has caused other problems with your heart.

Balloon valvoplasty (also called balloon dilation)

This is a type of keyhole surgery to make the opening of the pulmonary artery wider. This means blood can be pumped to the lungs at a lower pressure. It stops the heart from having to work so hard.

Keyhole surgery means surgery using smaller cuts and specialist equipment to fix the problem. Your recovery time can be quicker than open heart surgery.

A thin tube is put into a vein at the top of your leg (your groin). It’s guided to your heart and into your pulmonary artery. A balloon at the end of the tube is inflated to stretch your pulmonary artery.

You’ll usually stay in hospital for 1 or 2 days.

Open heart surgery

Open heart surgery is where the surgeon will work directly on your heart to fix your pulmonary heart valve.

The surgeon can make the area around your pulmonary valve bigger, or they may replace your pulmonary valve.

You’ll usually stay in hospital for 5 to 7 days.

• Read about heart valve repair
• Read about heart valve replacement surgery

Living with pulmonary valve stenosis

It’s normal to feel anxious after being diagnosed. You may feel worried about your condition and how it will affect your life.

Most people who have treatment or have mild pulmonary valve stenosis can live well with their condition.

It’s important to go to your appointments, even if you feel well. Your doctors will check how your heart is working and can help answer any questions you have.

Staying active

The exercise you can do will depend on:

• what level of pulmonary valve stenosis you have
• if you’ve had treatment
• if you have any other conditions
• how well your heart is working.

Generally, people with moderate stenosis should avoid high-intensity exercise and sports like climbing or gymnastics. If you have severe stenosis, you should only do low-intensity exercise.

Your healthcare team can give you more information about what kind of exercise is best for you.

Family planning

If you’re pregnant or planning to have a family, it's important to speak to your doctor. They can discuss options with you, make sure you’re supported and get the right care.

Endocarditis

Endocarditis is a rare infection that causes inflammation of the valves and inner lining of the heart.

People with pulmonary valve stenosis have an increased risk of endocarditis.

To reduce the risk of developing endocarditis, your doctor or dentist might recommend antibiotics before or after any surgery or procedures.

More information and support

Here are some ways you can get support.

• Read common questions about congenital heart disease. We talk about things like alcohol, mental health, preparing for surgery, and more.
• If you have questions, speak to our cardiac nurses on our Heart Helpline by phone, callback, email or online chat (Monday to Friday, 9am to 5pm).
• You can also speak to others with heart conditions by joining a support group or online community.