Pulmonary valve stenosis is a condition in which a deformity on or near your pulmonary valve narrows the pulmonary valve opening and slows the blood flow. The pulmonary valve is located between the lower right heart chamber (right ventricle) and the pulmonary arteries. Adults occasionally have pulmonary valve stenosis as a complication of another illness, but mostly, pulmonary valve stenosis develops before birth as a congenital heart defect.
Pulmonary valve stenosis ranges from mild and without symptoms to severe. Mild pulmonary stenosis doesn't usually worsen over time, but moderate and severe cases may worsen and require surgery. Fortunately, treatment is generally highly successful, and most people with pulmonary valve stenosis can expect to lead normal lives.
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:
Talk to your doctor if you or your child has:
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 fetal 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.
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.
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.
Sometimes other medical conditions or having an artificial valve can cause the condition.
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:
Pulmonary stenosis may be associated with the following:
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 checkup 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 checkups.
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.
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.
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:
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.
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:
Don't hesitate to ask other questions.
Your doctor is likely to ask you a number of questions, including:
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