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Overview

Pulmonary valve stenosis is a narrowing of the valve between the lower right heart chamber and the lung arteries. In a narrowed heart valve, the valve flaps may become thick or stiff. This reduces blood flow through the valve.

Usually, pulmonary valve disease is caused by a heart problem that develops before birth. A heart problem present at birth is called a congenital heart defect. In adults, pulmonary valve stenosis may be a complication of another illness.

Pulmonary valve stenosis ranges from mild to severe. Some people with mild pulmonary valve stenosis don't have symptoms. They may need only occasional health checkups. Moderate and severe pulmonary valve stenosis may need a procedure to repair or replace the valve.

Symptoms

Pulmonary valve stenosis symptoms depend on how much blood flow is blocked. Some people with mild pulmonary stenosis do not have symptoms. Those with more-severe pulmonary stenosis may first notice symptoms while exercising.

Pulmonary valve stenosis symptoms may include:

• A whooshing sound called a heart murmur that can be heard with a stethoscope.
• Fatigue.
• Shortness of breath, especially during activity.
• Chest pain.
• Fainting.

Babies with pulmonary valve stenosis may have blue or gray skin due to low oxygen levels.

When to see a doctor

Talk to your health care provider if you or your child has:

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

Prompt diagnosis and treatment of pulmonary valve stenosis can help reduce the risk of complications.

Causes

Pulmonary valve stenosis usually results from a heart problem present at birth. The exact cause is unclear. The pulmonary valve doesn't develop properly as the baby is growing in the womb.

The pulmonary valve is made of three thin pieces of tissue called flaps, also called cusps. The cusps open and close with each heartbeat. They make sure blood moves in the right direction.

In pulmonary valve stenosis, one or more of the cusps may be stiff or thick. Sometimes the cusps may be joined together. That means they are fused. So the valve doesn't open fully. The smaller opening makes it harder for blood to leave the lower right heart chamber. Pressure increases inside the chamber. The increased pressure strains the heart. Eventually the lower right heart chamber wall gets thicker.

Risk factors

Things that may increase the risk of pulmonary valve stenosis include:

• German measles, also called rubella. Having German measles during pregnancy increases the risk of pulmonary valve stenosis in the baby.
• Noonan syndrome. This condition is caused by altered DNA. It can lead to many problems with the heart's structure and function.
• Rheumatic fever. This complication of strep throat can cause permanent damage to the heart and heart valves. It increases the risk of developing pulmonary valve stenosis later in life.
• Carcinoid syndrome. This condition occurs when a rare cancerous tumor releases certain chemicals into the bloodstream. It causes shortness of breath, flushing and other symptoms. Some people with this syndrome develop carcinoid heart disease, which damages heart valves.

Complications

Possible complications of pulmonary stenosis include:

• Infection of the lining of the heart, called infective endocarditis. People with heart valve problems, such as pulmonary stenosis, have an increased risk of developing bacterial infections that affect the inner lining of the heart.
• Irregular heartbeats, called arrhythmias. People with pulmonary stenosis are more likely to have irregular heartbeats. Unless the stenosis is severe, irregular heartbeats due to pulmonary stenosis usually aren't life-threatening.
• Thickening of the heart muscle. In severe pulmonary stenosis, the lower right heart chamber must pump harder to force blood into the pulmonary artery. The strain on the heart causes the muscular wall of the ventricle to thicken. The condition is called right ventricular hypertrophy.
• Heart failure. If the right ventricle can't pump properly, heart failure eventually develops. Symptoms of heart failure include fatigue, shortness of breath, and swelling of the legs and belly area.
• Pregnancy complications. The risks of complications during labor and delivery are higher for those with severe pulmonary valve stenosis than for those without it.

Diagnosis

Pulmonary valve stenosis is often diagnosed in childhood. But it may not be detected until later in life.

A health care provider uses a stethoscope to listen to the heart. A whooshing sound, called a heart murmur, may be heard. The sound is caused by choppy blood flow across the narrowed valve.

Tests to diagnose pulmonary valve stenosis include:

• Electrocardiogram (ECG or EKG). This quick and painless test records the electrical signals in the heart. Sticky patches, called electrodes, are placed on the chest and sometimes the arms and legs. Wires connect the electrodes to a computer, which displays the test results. An ECG can show how the heart is beating and may reveal signs of heart muscle thickening.
• Echocardiogram. An echocardiogram uses sound waves to produce images of the heart. This common test shows how the heart beats and pumps blood. An echocardiogram can show the shape of the pulmonary valve. The test can show how much of the valve is narrowed.

• Cardiac catheterization. A thin tube called a catheter is inserted into the groin and threaded through the blood vessels to the heart. Dye flows through the catheter into the blood vessels to make them show up more clearly on X-rays. This part of the test is called a coronary angiogram.

  During the test, pressures within the heart can be measured to see how forcefully blood pumps through the heart. A provider can determine the severity of pulmonary stenosis by checking the difference in pressure between the right lower heart chamber and the lung artery.

• Other imaging tests. Magnetic resonance imaging (MRI) and computed tomography (CT) scans are sometimes used to confirm the diagnosis of pulmonary valve stenosis.

Treatment

If you have mild pulmonary valve stenosis without symptoms, you may only need occasional health checkups.

If you have moderate or severe pulmonary valve stenosis, you may need a heart procedure or heart surgery. The type of procedure or surgery done depends on your overall health and the appearance of your pulmonary valve.

Surgeries or other procedures

Pulmonary valve stenosis treatment may include:

• Balloon valvuloplasty. The provider inserts a flexible tube with a balloon on the tip into an artery, usually in the groin. X-rays help guide the tube, called a catheter, to the narrowed valve in the heart. The balloon inflates, making the valve opening larger. The balloon is deflated. The catheter and balloon are removed.

  Valvuloplasty may improve blood flow through the heart and reduce pulmonary valve stenosis symptoms. But the valve may narrow again. Some people need valve repair or replacement in the future.

• Pulmonary valve replacement. If balloon valvuloplasty isn't an option, open-heart surgery or a catheter procedure may be done to replace the pulmonary valve. If there are other heart problems, the surgeon may repair those during the same surgery.

  People who have had pulmonary valve replacement need to take antibiotics before certain dental procedures or surgeries to prevent endocarditis.

Self care

If you have valve disease, it's important to take steps to keep your heart healthy. Certain lifestyle changes can decrease your risk of developing other types of heart disease or having a heart attack.

Lifestyle changes to talk about with your health care provider include:

• Quitting smoking.
• Eating a heart-healthy diet that includes fruits and vegetables, low-fat dairy products, whole grains, and lean meat.
• Maintaining a healthy weight.
• Getting regular exercise.

Preparing for your appointment

If you or your child has valve disease, you will likely be referred to a doctor trained in evaluating and treating heart conditions. This type of provider is called a cardiologist.

Here's some information to help you get ready for your appointment.

What you can do

• Write down symptoms you or your child has, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including major stresses or recent illnesses.
• List all medications, vitamins and supplements you or your child takes.
• Write down questions to ask the health care provider.

Preparing a list of questions can help you make the most of your time with your health care provider. For pulmonary valve stenosis, some basic questions include:

• What's the most likely cause of the symptoms?
• Are there other possible causes?
• What tests are needed? Do these tests require any special preparation?
• Is pulmonary valve stenosis temporary or long lasting?
• What treatments are available? Which do you recommend and why?
• What are the risks of a balloon valvuloplasty or open-heart surgery?
• Are there any activity restrictions?
• Are there brochures or other printed material that I can take? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your health care provider is likely to ask many questions, including:

• When did symptoms begin?
• Do the symptoms come and go, or do they always happen?
• Do the symptoms get worse during activity or when lying down?
• Does anything seem to improve symptoms?