Pulmonary Valve Disease

What is pulmonary valve disease?

Pulmonary valve disease is any issue that keeps this specific heart valve from working the way it should. Your pulmonary valve is the gatekeeper that lets your blood move from your heart’s right ventricle (lower chamber) to your lungs via your pulmonary artery. This allows your blood to get fresh oxygen to distribute to your body.

A problem with your pulmonary valve makes it harder for your blood to travel to your lungs, which delays getting oxygen to your tissues, cells and organs.

Types of pulmonary valve disease

Types of pulmonary valve disease are:

• Pulmonary valve regurgitation (leaking).
• Pulmonary valve stenosis (narrowing).
• Pulmonary atresia (missing or poorly formed valve at birth).

How common is pulmonary valve disease?

Pulmonary valve regurgitation is very common. Some people are born with different valve tissue, and that physiological difference can make them more likely to have pulmonary valve regurgitation. However, many cases are incredibly mild and this doesn’t mean it’s a worrisome condition that needs to be treated.

Pulmonary valve stenosis happens in 7% of people with congenital (present at birth) heart disease.

About 1 in 7,000 babies has pulmonary atresia at birth in the U.S.

What are the symptoms of pulmonary valve disease?

Pulmonary valve disease symptoms may include:

• Chest pain.
• Fatigue.
• Lightheadedness.
• Shortness of breath.
• Inability to tolerate exercise.

People with mild stenosis or regurgitation don’t have symptoms.

Babies with pulmonary atresia have difficulty breathing or eating, are very sleepy and have a blue tone to their skin (cyanosis).

What causes pulmonary valve disease?

Causes of pulmonary valve disease vary by condition.

• Pulmonary stenosis causes: Many cases of pulmonary stenosis have an unknown cause, but some may be due to several genetic disorders or a birthing parent having rubella during pregnancy.
• Pulmonary regurgitation causes: These causes may include pulmonary hypertension, endocarditis, rheumatic fever or certain heart issues present at birth or their treatments. It’s very common in people with repaired Tetralogy of Fallot.
• Pulmonary atresia causes: Many cases of atresia have unknown causes, but atresia causes include changes in a fetus’ genes or exposure to something harmful in the environment or in something ingested during pregnancy.

What are the complications of pulmonary valve disease?

Pulmonary valve disease can strain, damage and/or enlarge your heart’s right ventricle because it has to use more effort to move blood forward to your lungs. In rare cases, this can lead to heart failure.

Children with pulmonary atresia may develop liver disease or abnormal heart rhythms.

How is pulmonary valve disease diagnosed?

While listening to your heart during a physical exam, a provider can hear a heart murmur. That’s a sign of pulmonary regurgitation or pulmonary stenosis.

What tests will be done to diagnose this condition?

A provider can use these tests to diagnose pulmonary valve disease:

• Fetal echocardiogram (for atresia).
• Echocardiogram (for atresia, stenosis or regurgitation).
• Pulse oximetry (for atresia).
• Cardiac catheterization (for atresia or stenosis).
• Electrocardiogram or EKG (for atresia or regurgitation).
• Heart MRI or magnetic resonance imaging (for stenosis or regurgitation).
• Chest X-ray (for regurgitation).

How is pulmonary valve disease treated?

Pulmonary valve disease treatments range from medicine to surgery, depending on the condition you have.

Specific medicines and procedures

Pulmonary valve disease treatments include:

• Balloon valvuloplasty to widen your pulmonary valve.
• Medicine or a stent (for atresia) to keep a baby’s ductus arteriosus open instead of allowing it to close soon after birth.
• Diuretics to keep you from retaining fluid because of heart failure from a leaky valve.
• Medicines to treat pulmonary hypertension or endocarditis (for regurgitation).
• Transcatheter procedure or open-heart surgery to repair a leaky valve or put in a new one.

Complications/side effects of the treatment

Some babies may have trouble eating after surgery and may need to get food through a tube in their nose.

After having a valve replacement, your child will need to take antibiotics before having dental work. This medicine lowers their risk of getting a heart infection called endocarditis.

After a pulmonary valve repair, the valve may leak. A provider may need to widen it again or replace it.

Any surgery can cause an infection or bleeding.

How long does it take to recover from this treatment?

Your child may be in the hospital for one or two weeks after surgery for pulmonary atresia. It can take weeks or months to recover after surgery for pulmonary valve regurgitation. After a valvuloplasty for pulmonary stenosis, avoid doing anything strenuous for about a week.

How can I lower my risk?

You can’t lower your risk of having a child with pulmonary valve disease from unknown causes. But if you plan to become pregnant, you should be sure you’re up to date with the rubella vaccine.

You may be able to prevent pulmonary valve regurgitation by preventing its causes: pulmonary hypertension, endocarditis and rheumatic fever.

What can I expect if I have pulmonary valve disease?

Your experience with pulmonary valve disease may be different from that of another person. Some people have mild symptoms and don’t need surgery. Other people may need to have more than one pulmonary valve surgery in their lifetime.

Outlook for pulmonary valve disease

Children who undergo valvuloplasty for pulmonary valve stenosis (widening a narrow valve) have an excellent prognosis (outlook). They usually don’t need anything more except checkups with a pediatric cardiologist. Some people need a provider to widen their pulmonary valve again 15 years or more in the future.

Pulmonary atresia is fatal without treatment, but most children live into adulthood after surgery.

The outlook is good for people who have mild or moderate pulmonary valve regurgitation. For those with more severe disease, the outlook depends on how quickly they get treatment.

How do I take care of myself?

Keep your heart healthy by managing things you can, like:

• Managing your blood pressure and cholesterol.
• Avoiding tobacco products.
• Exercising regularly.
• Eating fruits, vegetables and other foods that have little or no saturated fat.

When should I see my healthcare provider?

Keep your follow-up appointments with your cardiologist (heart specialist) so they can make sure your valve continues to work right. They may also want to repeat some tests they used to diagnose you. These may include:

• Echocardiogram (Echo).
• Computed tomography (CT).
• Heart MRI.
• Electrocardiogram (EKG).

When should I go to the ER?

You should go to the emergency room if you have:

1. Fainting episodes.
2. Shortness of breath.
3. Heart palpitations.
4. Signs of infection or bleeding after surgery, like a fever or bloody stool (poop).

What questions should I ask my healthcare provider?

You may want to ask your provider:

• What’s the best treatment for me (or my child)?
• Do you know the cause of my (or my child’s) pulmonary valve disease?
• How often do I (or does my child) need follow-up appointments with you?
• Is there a support group for parents of children with heart issues?

A note from Cleveland Clinic

Learning that your child (or your own heart) has a pulmonary valve disease is unsettling. But providers who specialize in heart care deal with valve issues all the time. They can draw on their experience to decide on the best treatment possible for you or your child. Don’t be afraid to ask questions about anything that isn’t clear. Be sure to go to all your follow-up appointments with your provider and take any medicines they prescribe for you.