Pulmonary Valve Stenosis

Written by Rachel Nall
Medically Reviewed by George Krucik, MD

What Is Pulmonary Valve Stenosis?

Your pulmonary valve rests between your right ventricle and your pulmonary artery, which sends oxygen-poor blood to your lungs. The valve acts as a doorway that lets blood into and out of your heart. If your pulmonary valve does not open properly or wide enough, this is known as pulmonary valve stenosis. This very rare disorder is typically present at birth. Pulmonary valve stenosis does not always require medical treatment.  However, some patients may need medications or surgeries to correct the condition.

What Are the Condition’s Symptoms?

Pulmonary valve stenosis affects the body’s ability to get oxygenated blood. Examples of pulmonary valve stenosis symptoms include:

  • protruding abdomen
  • bluish tint to the skin
  • chest pain
  • fainting
  • heart palpitations
  • unexplained fatigue
  • failure to thrive
  • difficulty breathing

In severe instances, pulmonary valve stenosis can cause sudden death. This is why diagnosis and treatment is vital to your health. In some instances, symptoms do not result until the stenosis becomes severe.

What Causes the Condition?

Physicians do not know the exact cause of pulmonary valve stenosis. The valve in the fetus may fail to develop properly during pregnancy. The disease also may have a genetic component.

The condition also may accompany additional congenital heart defects. Your physician will often recommend performing additional tests to ensure your heart is healthy.

Adults also can experience the condition due to a complication from an illness that affects the heart. These include rheumatic fever or carcinoid tumors in the digestive system.

What Are the Condition’s Effects?

Untreated pulmonary valve stenosis can lead to a number of harmful and deadly side effects. One example is right ventricular hypertrophy or heart enlargement, which can weaken and permanently damage the heart. A lack of blood to your tissues can cause cyanosis, which causes a blue tint to your skin and affects your breathing.

The extra effort your heart must put forth to work can lead to heart failure and death if left untreated. If you suspect you or your child may be experiencing pulmonary valve stenosis, make an appointment to see your physician.

How Is the Condition Diagnosed?

Pulmonary valve stenosis can cause a heart murmur, which sounds like an extra click, blowing, whooshing, or rasping sound when a medical provider listens to your heart. The murmur can be an initial indicator of pulmonary valve stenosis that can highlight the need for further testing.

A physician may order certain imaging tests to visualize the heart’s anatomy. Examples include:

  • chest X-ray
  • electrocardiogram (ECG)
  • echocardiogram
  • magnetic resonance imaging scan (MRI)

These imaging tests can help your physician see where blood may not be flowing as freely or if there is narrowing of the pulmonary valve.

How Is the Condition Treated?

Your physician will use imaging scans and other examinations to determine the best course of treatment for pulmonary valve stenosis. If the stenosis is mild and not causing symptoms, a physician may not recommend interventions to treat pulmonary valve stenosis.

If you have untreated pulmonary valve stenosis, seek medical treatment if you experience chest pain, shortness of breath, or fainting. These symptoms can indicate your condition is advancing.

According to PubMed Health, even if you are experiencing symptoms associated with pulmonary valve stenosis, there is a chance mild stenosis will improve with time. An estimated one-third of patients will improve while one-third will stay the same. The remaining third will experience worsening symptoms that will likely require interventions. (National Institutes of Health)

A physician may prescribe medications that make it easier for blood to flow through the heart’s chambers. Examples of medications prescribed may include:

  • prostaglandins to improve blood flow
  • blood thinners to reduce clotting
  • water pills to reduce excess fluid in the blood stream
  • pills that prevent irregular heart rhythms

A surgical procedure known as a valvuloplasty can stretch the pulmonary valve’s walls, enlarging it to improve blood flow. This treatment option involves inserting a catheter that has a balloon on the end that can inflate and stretch the heart’s walls.

In severe instances, surgery to replace the pulmonary valve may be required.

Maintaining a heart-healthy lifestyle also can be beneficial when you have pulmonary valve stenosis. This means refraining from smoking, exercising regularly, and eating a healthy diet full of fruits, whole grains, and vegetables.

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