Congenital Pulmonary Valve Stenosis
Information for teenagers and adults with pulmonary valve stenosis
Pulmonary valve stenosis is a congenital heart condition in which the pulmonary valve is narrower than usual.
The pulmonary valve sits between the right ventricle, which is the pumping chamber on the right side of the heart, and the pulmonary artery, which carries blood to the lungs.
Pulmonary stenosis can range from very mild to severe. Some people require treatment during infancy or childhood, while others with mild pulmonary stenosis may not be diagnosed until later in life.
Most people with isolated pulmonary stenosis have an excellent long-term outlook. However, ongoing heart checks may be important, particularly if you have previously had a balloon procedure or surgery on your pulmonary valve.
How does pulmonary stenosis affect the heart?
Normally, the pulmonary valve opens easily each time the right ventricle contracts, allowing blood to flow into the pulmonary artery and towards the lungs.
When the valve is narrowed, the right ventricle has to generate higher pressure to push blood through the valve.
With mild pulmonary stenosis, this may have very little effect on the heart.
If the narrowing is more severe, the muscle of the right ventricle can become thicker because it is working against increased resistance.
Severe pulmonary stenosis can eventually affect the ability of the right side of the heart to function normally.
What causes pulmonary stenosis?
In most people, pulmonary stenosis occurs because the pulmonary valve developed differently before birth.
The valve leaflets may be thickened or partly fused together, preventing the valve from opening normally.
Less commonly, obstruction can occur below the pulmonary valve or in the pulmonary artery above the valve. These are different forms of right ventricular outflow obstruction and may require different treatment.
Pulmonary stenosis can occur on its own or as part of another congenital heart condition or genetic syndrome.
Does pulmonary stenosis get worse over time?
Mild pulmonary stenosis often remains stable, particularly once a person has reached adulthood.
Moderate or severe pulmonary stenosis requires closer monitoring because significant obstruction places additional workload on the right ventricle.
Importantly, people who have previously had their pulmonary valve treated can develop a different problem later in life — pulmonary regurgitation.
This means that follow-up is sometimes required even when the original stenosis has been successfully relieved.
How is pulmonary stenosis treated?
Treatment is generally recommended when pulmonary stenosis is severe or when significant obstruction is affecting the right side of the heart.
Balloon pulmonary valvuloplasty
For most people with typical congenital pulmonary valve stenosis, the preferred treatment is balloon pulmonary valvuloplasty.
This is performed during a cardiac catheter procedure.
A thin tube is passed through a vein, usually from the groin, to the heart. A balloon is positioned across the narrowed pulmonary valve and briefly inflated to separate the fused valve leaflets and improve the opening of the valve.
No surgical incision in the chest is required.
Balloon valvuloplasty is very effective for suitable pulmonary valves and many people require no further treatment for the obstruction.
Surgery
Surgery is required less commonly.
It may be recommended if the pulmonary valve anatomy is not suitable for balloon treatment, if there is obstruction above or below the valve, or if there are other heart abnormalities that need to be treated at the same time.
What happens after balloon valvuloplasty or surgery?
After treatment, the obstruction is usually substantially improved.
However, opening the pulmonary valve can sometimes make it leak. This is called pulmonary regurgitation.
A small amount of pulmonary regurgitation is common after treatment and usually causes no problems.
If the leak is more significant, extra blood returns to the right ventricle with each heartbeat. Over many years, this can cause the right ventricle to enlarge.
This process is usually gradual and may not cause symptoms initially.
For this reason, adults who had significant pulmonary stenosis treated during childhood may still benefit from periodic congenital heart disease follow-up, even if they have felt completely well for many years.
Could I eventually need a pulmonary valve replacement?
Most people with treated isolated pulmonary stenosis will never need a pulmonary valve replacement.
However, replacement may occasionally be considered if severe pulmonary regurgitation develops and causes substantial enlargement or reduced function of the right ventricle, or if significant pulmonary stenosis returns.
A pulmonary valve can be replaced surgically.
For some people with suitable anatomy, a new pulmonary valve can instead be implanted using a catheter procedure. This is known as transcatheter pulmonary valve replacement.
The decision about whether and when a pulmonary valve should be replaced depends on your symptoms, the severity of stenosis or regurgitation, the size and function of the right ventricle and your individual anatomy.
How to stay well
Keep up with your heart checks
How often you need review depends on the severity of your pulmonary stenosis and whether you have previously had treatment.
People with very mild pulmonary stenosis may require only occasional follow-up.
People with moderate or severe stenosis, previous balloon valvuloplasty or surgery, significant pulmonary regurgitation or right ventricular enlargement may require more regular review with a congenital cardiologist.
Stay active
Regular exercise is encouraged for most people with pulmonary stenosis.
People with mild pulmonary stenosis and normal heart function can usually undertake normal physical activity without restriction.
Exercise recommendations may be different if you have:
Severe pulmonary stenosis
Significant pulmonary regurgitation
Enlargement or reduced function of the right ventricle
Abnormal heart rhythms
Symptoms during exercise
If you participate in competitive sport or very high-intensity exercise, discuss this with your cardiologist.
Look after your teeth
Good dental hygiene is important for everyone with congenital heart disease.
Most people with isolated pulmonary stenosis do not require antibiotics before routine dental treatment.
Antibiotic prophylaxis is recommended for certain higher-risk people, including some people with prosthetic heart valves or a previous episode of infective endocarditis.
Your cardiologist or dentist can tell you whether this applies to you.
Don’t smoke or vape
Smoking increases your lifetime risk of cardiovascular and lung disease. Avoid smoking and vaping and seek help to quit if needed.
Pregnancy
Most women with mild or successfully treated pulmonary stenosis and normal right heart function can have a successful pregnancy with relatively low cardiovascular risk.
Pregnancy is generally well tolerated following successful balloon valvuloplasty.
Women with severe pulmonary stenosis, significant pulmonary regurgitation or impaired right ventricular function should have specialist assessment before pregnancy.
If you are considering pregnancy, discussing this with your cardiologist beforehand allows your valve and right ventricular function to be assessed and any treatment to be planned before pregnancy if necessary.
Symptoms to tell your doctor about
Contact your doctor or cardiology team if you develop:
New or increasing shortness of breath
A noticeable reduction in your exercise capacity
Frequent or prolonged palpitations
Dizziness during exercise
Unexplained fainting or near-fainting
New swelling of your ankles or abdomen
A significant change in your usual symptoms
A gradual reduction in exercise capacity can sometimes be an early sign that significant valve disease is affecting the right side of the heart.
Emergency — get help immediately if
Seek urgent medical attention or call 000 if you develop:
Severe or sudden shortness of breath
Fainting during exercise
Fainting associated with chest pain, breathlessness or sustained palpitations
A sustained rapid heartbeat associated with dizziness, chest pain or feeling very unwell
A sudden significant deterioration in your condition
Your regular check-ups
Depending on your condition and previous treatment, follow-up may include:
Clinical review
Your cardiologist will ask about exercise capacity, breathlessness, dizziness and palpitations and examine your heart and circulation.
ECG
An electrocardiogram records the electrical activity and rhythm of your heart.
Echocardiogram
An ultrasound of the heart can measure the severity of pulmonary stenosis, assess pulmonary regurgitation and examine the size and function of the right ventricle.
Cardiac MRI
MRI can provide particularly accurate information about the size and function of the right ventricle and the amount of pulmonary regurgitation. It may be recommended when these need more detailed assessment.
Exercise testing
An exercise test can objectively assess your exercise capacity and may be useful if there is uncertainty about whether valve disease is beginning to affect you.
Cardiac catheterisation
Catheterisation is used when pulmonary stenosis is treated with balloon valvuloplasty and may occasionally be required for other diagnostic or treatment purposes.
Questions for your doctor
How severe is my pulmonary stenosis?
Does it need treatment or can it simply be monitored?
If I have previously had treatment, do I now have pulmonary regurgitation?
Is the size and function of my right ventricle normal?
Could I ever need another balloon procedure or pulmonary valve replacement?
Do I have any restrictions on exercise, work, travel or pregnancy?
How often should I have a heart check-up?
This information is intended as a general guide for teenagers and adults with congenital pulmonary valve stenosis. Individual congenital heart conditions vary, and your cardiologist can provide advice specific to your valve, previous treatment and current health.
