Pulmonary Valve Stenosis

Pulmonary valve stenosis signs and symptoms vary, depending on the extent of the obstruction. People with mild pulmonary stenosis usually don't have symptoms. Those with more significant stenosis often may first notice symptoms while exercising.

Pulmonary valve stenosis signs and symptoms may include:

• Heart murmur — an abnormal whooshing sound heard using a stethoscope, caused by turbulent blood flow
• Fatigue
• Shortness of breath, especially during exertion
• Chest pain
• Loss of consciousness (fainting)

When to see a doctor

Talk to your doctor if you or your child has:

• Shortness of breath
• Chest pain
• Fainting

If you have pulmonary stenosis or another heart problem, prompt evaluation and treatment can help reduce your risk of complications.

Pulmonary valve stenosis usually occurs when the pulmonary valve doesn't grow properly during foetal development. Babies who have the condition may have other congenital heart abnormalities, as well. It's not known what causes the valve to develop abnormally.

Normal pulmonary valve anatomy

The pulmonary valve is made up of three thin pieces of tissue called cusps that are arranged in a circle. With each heartbeat, the valve opens in the direction of blood flow — into the pulmonary artery and continuing to the lungs — then closes to prevent blood from flowing backward into the heart's right ventricle.

What happens in pulmonary valve stenosis

One or more of the cusps may be defective or too thick, or the cusps may not separate from each other properly. If this happens, the valve doesn't open correctly, restricting blood flow.

Other contributing conditions

Sometimes other medical conditions or having an artificial valve can cause the condition.

• Carcinoid syndrome. This syndrome — a combination of signs and symptoms, including flushing of the skin and diarrhoea — results from the release of a chemical, serotonin, from growths called carcinoid tumours in the digestive system.
• Rheumatic fever. This complication of an infection caused by streptococcus bacteria, such as strep throat, may injure the heart valves.

Risk factors

Because pulmonary valve stenosis usually develops before birth, there aren't many known risk factors. However, certain conditions and procedures can increase your risk of developing pulmonary valve stenosis later in life, including:

• Carcinoid syndrome
• Rheumatic fever
• Noonan syndrome
• Pulmonary valve replacement

Pulmonary stenosis may be associated with the following:

• Infection. People with heart valve problems, such as pulmonary stenosis, have a higher risk of developing bacterial infections in the inner lining of the heart (infective endocarditis) than people without heart valve problems.
• Heart-pumping problems. In severe pulmonary stenosis, the heart's right ventricle must pump harder to force blood into the pulmonary artery. Pumping of the right ventricle against increased pressure causes the muscular wall of the ventricle to thicken (right ventricular hypertrophy). Eventually, the heart becomes stiff and may weaken.
• Heart failure. If the right ventricle is unable to pump efficiently, heart failure develops. This results in swelling of the legs and abdomen and can cause fatigue and shortness of breath.
• Irregular heartbeat (arrhythmia). People with pulmonary stenosis are more likely to have an irregular heartbeat. Unless the stenosis is severe, irregular heartbeats associated with pulmonary stenosis usually aren't life-threatening.

Pulmonary valve stenosis is often diagnosed in childhood, but sometimes it isn't detected until later in life. If your doctor hears a heart murmur during a routine check-up and suspects pulmonary stenosis, he or she may then use a variety of tests to confirm the diagnosis.

• Echocardiogram. Sound waves bounce off your heart and produce moving images that can be viewed on a video screen. This test is useful for checking the structure of the pulmonary valve, the location and severity of the narrowing (stenosis), and right ventricle size and function.

Doctors may also perform a 3-D echocardiogram.

• Electrocardiogram. During this procedure, patches with wires (electrodes) are placed on your chest, wrists and ankles. The electrodes measure electrical activity in your heart, which is recorded on paper. This test helps determine if the muscular wall of your right ventricle is thickened (right ventricular hypertrophy).
• Other imaging tests. MRI and CT scans are sometimes used to confirm the diagnosis of pulmonary valve stenosis.
• Cardiac catheterization. During this procedure, your doctor inserts a thin, flexible tube (catheter) into an artery or vein in your groin and threads it up to your heart or blood vessels. Dye injected through the catheter makes your blood vessels visible on X-rays. Doctors also use cardiac catheterization to measure the blood pressure in the heart chambers and blood vessels.

Doctors generally use this test only if they suspect that you or your child will need a balloon valvuloplasty, a procedure that can be done at the same time as cardiac catheterization.

Pulmonary valve stenosis is classified as mild, moderate or severe, depending on a measurement of the blood pressure difference between the right ventricle and pulmonary artery. Mild pulmonary stenosis that isn't causing symptoms doesn't usually require treatment, just routine check-ups.

Depending on the degree of obstruction, more-serious cases may need either a balloon valvuloplasty or open-heart surgery.

• Balloon valvuloplasty. Using the small tube that was threaded through a vein in your leg to your heart for a cardiac catheterization, your doctor places an uninflated balloon through the opening of the narrowed pulmonary valve. He or she then inflates the balloon, widening the narrowed valve to increase blood flow, and then removes the balloon.

The most common side effect of a balloon valvuloplasty is blood leakage back through the pulmonary valve (valve regurgitation). As with most procedures, there is a risk of bleeding, infection or blood clots.

• Open-heart surgery. When a balloon valvuloplasty isn't an option, you may require open-heart surgery. During surgery, your doctor either repairs the pulmonary artery or valve or replaces the valve with an artificial valve. Repairs to other congenital heart defects can be made during the surgery, as well. There's a slight risk of bleeding, infection or blood clots associated with the surgery.

While there's little you can do to prevent pulmonary valve stenosis, you can take steps to ensure you won't develop complications of your condition.

Your doctor will also likely recommend regular follow-up appointments to evaluate your condition.

Preventive antibiotics

In most cases, you won't need to take antibiotics to prevent an infection of the inner lining of the heart (infective endocarditis). Your doctor will recommend antibiotics if you've had endocarditis before or if you've had a pulmonary valve replacement.

Heart-healthy lifestyle

Adopting a heart-healthy lifestyle decreases your risk of developing other types of heart disease, such as a heart attack. Lifestyle changes to talk to your doctor about include:

• Quitting smoking
• Eating a heart-healthy diet, such as a variety of fruits and vegetables, low-fat dairy products, whole grains, and lean meat
• Maintaining a healthy weight
• Regular physical activity

Pregnancy

Pregnancy generally isn't a problem for women who have mild and moderate pulmonary valve stenosis. If you have severe pulmonary valve stenosis, the risks of complications during labor and delivery are higher than those for women without the condition. If necessary, it's possible to undergo a balloon valvuloplasty during pregnancy.

You're likely to start by seeing your family doctor, a general practitioner or your child's doctor. You'll probably then be referred to a doctor trained in evaluating and treating heart conditions (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 doctor.

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

• What's the most likely cause of my or my child's 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, and which do you recommend?
• What are the risks of a balloon valvuloplasty or open-heart surgery?
• I have other health conditions. How can I best manage them together?
• Do I need to restrict my or my child's activity?
• Are there brochures or other printed material that I can take? What websites do you recommend?