Pulmonary valve disease is a type of heart valve disease. It affects the valve between the heart's lower right chamber and the artery that delivers blood to the lungs. That artery is called the pulmonary artery. The valve is called the pulmonary valve.

A diseased or damaged pulmonary valve doesn't work the way it should. It changes how blood flows from the heart to the lungs.

Types of pulmonary valve disease include:

• Pulmonary valve stenosis. The valve is narrowed. This reduces the blood flow from the heart to the pulmonary artery and lungs.
• Pulmonary valve regurgitation. The flaps of the pulmonary valve don't close tightly. Blood moves backward into the right lower heart chamber, called the right ventricle.
• Pulmonary atresia. This condition is present at birth. That means it is a congenital heart defect. The pulmonary valve isn't formed. Instead, a solid sheet of tissue blocks blood flow from the right side of the heart. Blood can't go to the lungs to pick up oxygen.

Symptoms of pulmonary valve disease depend on the specific valve condition and how much blood flow is changed or blocked. Some babies are born with pulmonary valve disease. Symptoms may be noticed soon after birth. But sometimes symptoms aren't seen until later in life.

In general, pulmonary valve disease symptoms may include:

• Chest pain.
• Shortness of breath at rest or when active or lying down.
• Fatigue.
• Fainting.

Babies born with pulmonary valve disease may have blue or gray skin due to low oxygen levels. Depending on skin color, these changes may be harder or easier to see.

When to see a doctor

Make an appointment for a health checkup if you or your child has:

• Shortness of breath.
• Chest pain.
• Fainting.

Call 911 or your local emergency number if chest pain is sudden or can't be explained.

Quick diagnosis and treatment of pulmonary valve disease can help reduce the risk of complications.

Usually, pulmonary valve disease is caused by a heart condition that develops before birth, called a congenital heart defect. But pulmonary valve disease may happen later in life as a complication of another illness.

To understand how pulmonary valve disease happens, it might help to know how the valve works.

The pulmonary valve is like a one-way door from the lower right heart chamber, called the right ventricle, to the lungs. Blood usually flows from that chamber through the pulmonary valve and into the lungs. Blood gets oxygen in the lungs to take to the body.

When the pulmonary valve doesn't work as it should, the heart must work harder to pump blood to the lungs. The extra work can cause the right ventricle to become thick.

Things that can raise the risk of pulmonary valve disease are:

• A heart condition that you're born with, also called a congenital heart defect. Some congenital heart defects affect the shape of the pulmonary valve and how it works.
• Carcinoid syndrome. This condition happens when a rare cancerous tumor sends chemicals into the bloodstream. It can cause carcinoid heart disease, which commonly damages the pulmonary and tricuspid valves.
• Chest injury. An injury to the chest, such as from a car accident, may cause damage that leads to pulmonary valve regurgitation.
• German measles, also called rubella. Having German measles during pregnancy increases the risk of pulmonary valve stenosis in the baby.
• Infection of the lining of the heart and heart valves, also called endocarditis. This condition can damage the pulmonary valve. IV drug misuse makes a person more likely to get endocarditis.
• Noonan syndrome. This condition is caused by changes in genes. Pulmonary valve stenosis is a common heart complication seen in people with Noonan syndrome.

Some types of pulmonary valve stenosis are diagnosed at birth or soon after. But sometimes, pulmonary valve disease isn't noticed until later in life.

To diagnose pulmonary valve disease, a healthcare professional uses a stethoscope to listen to the heart. A whooshing sound, called a heart murmur, may be heard.

Tests

Tests are done to check the heart and confirm a diagnosis of pulmonary valve disease. Tests may include:

• Electrocardiogram (ECG or EKG). This quick and painless test shows how the heart is beating. Sticky patches with sensors on them, called electrodes, attach to the chest and sometimes the arms and legs. Wires connect the electrodes to a computer, which prints or displays the results.
• Echocardiogram. Sound waves create images of the heart. An echocardiogram shows blood flow through the heart and heart valves. It can show the shape of the pulmonary valve.
• Chest X-ray. A chest X-ray shows the heart and lungs. It can tell if the heart is larger than usual or if there is fluid around the lungs. Some types of heart valve disease can cause fluid buildup.
• Cardiac catheterization. This test isn't often used to diagnose heart valve disease. But it may be done to tell how severe pulmonary valve disease is. A long, thin flexible tube called a catheter is inserted in a blood vessel, usually in the groin or wrist. It's guided to the heart. Dye flows through the catheter into the arteries in the heart. The dye helps the arteries show up more clearly on X-ray images and video.
• Other imaging tests. Magnetic resonance imaging (MRI) and computerized tomography (CT) scans are sometimes used to confirm pulmonary valve disease, such as stenosis.

Heart valve disease stages

After testing confirms a diagnosis of heart valve disease, your healthcare team may tell you the stage of disease. Staging helps determine the most appropriate treatment.

The stage of heart valve disease depends on many things, including symptoms, disease severity, the structure of the valve or valves, and blood flow through the heart and lungs.

Heart valve disease is staged into four basic groups:

• Stage A: At risk. Risk factors for heart valve disease are present.
• Stage B: Progressive. Valve disease is mild or moderate. There are no heart valve symptoms.
• Stage C: Asymptomatic severe. There are no heart valve symptoms but the valve disease is severe.
• Stage D: Symptomatic severe. Heart valve disease is severe and is causing symptoms.

Pulmonary valve disease treatment depends on:

• The symptoms.
• The stage of the disease.
• If the disease is getting worse.

Treatment may include:

• Regular health checkups.
• Surgery to repair or replace the valve.

If symptoms are mild, the only treatment may be regular health checkups and imaging tests to see how the heart is working.

Surgeries or other procedures

Surgery or another treatment may be needed to fix or replace the pulmonary valve.

The decision to repair or replace a damaged pulmonary valve depends on many things, including:

• The stage of pulmonary valve disease.
• Symptoms.
• Age and overall health.
• Whether or not the condition is getting worse.
• Whether surgery is needed to correct another valve or heart condition.

Pulmonary valve surgery may be done at the same time as other heart valve surgeries. In general, surgeons recommend pulmonary valve repair when possible, as it saves the heart valve and improves heart function.

Types of surgery or procedures used to treat pulmonary valve disease include:

• Balloon valvuloplasty. This treatment is often used to treat infants and children with a narrowed pulmonary valve. During balloon valvuloplasty, a surgeon places a thin, hollow tube called a catheter into a blood vessel, usually in the groin, and guides it to the heart. A balloon on the tip of the catheter is inflated, which makes the valve opening wider. The balloon is deflated and removed.
• Pulmonary valve replacement surgery. If the pulmonary valve can't be fixed, a surgeon might replace the valve with a mechanical valve or one made from cow, pig or human heart tissue. A tissue valve also is called a biological tissue valve.
• Transcatheter pulmonary valve replacement. Sometimes, the pulmonary valve can be replaced with a minimally invasive procedure, which uses thin, flexible tubes and smaller surgical cuts. A transcatheter procedure also may be used to place a new pulmonary valve in a previously replaced valve that's now failing. This is called a valve-in-valve procedure.