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Pulmonary Valve Stenosis

What is pulmonary valve stenosis?

Your pulmonary valve rests between your right ventricle and your pulmonary artery. The pulmonary artery sends oxygen-poor blood to your lungs. The valve acts as a doorway that lets blood into and out of your heart. Pulmonary valve stenosis is when your pulmonary valve does not open properly or wide enough. 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 symptoms of pulmonary valve stenosis?

Pulmonary valve stenosis affects the body’s ability to get oxygenated blood. Many children do not show symptoms until adulthood. Examples of pulmonary valve stenosis symptoms include:

  • heart murmur
  • prominent and enlarged jugular vein
  • bluish tint to the skin
  • chest pain
  • fainting
  • heart palpitations
  • unexplained fatigue
  • failure to thrive
  • difficulty breathing

Pulmonary valve stenosis can cause sudden death in severe instances. This is why diagnosis and treatment is vital to your health. Symptoms may in some instances not appear until the stenosis becomes severe.

What causes pulmonary valve stenosis?

Doctors don’t know the exact cause of pulmonary valve stenosis. The valve in the fetus may fail to develop properly during pregnancy. The disease may also have a genetic component.

The condition may accompany other congenital heart defects. If you are diagnosed with pulmonary valve stenosis, your doctor will often recommend that you undergo additional tests to ensure your heart is healthy.

Adults can also experience the condition as a complication of an illness that affects the heart. Conditions that can have pulmonary valve stenosis as a complication include rheumatic fever and carcinoid tumors in the digestive system.

What are the potential complications of pulmonary valve stenosis?

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

Any structural defect in the heart can increase the risk of an infection inside the heart.

The extra effort your heart must exert can lead to heart failure and death if pulmonary valve stenosis is left untreated. Make a doctor’s appointment if you suspect you or your child may be experiencing pulmonary valve stenosis.

How is pulmonary valve stenosis diagnosed?

Pulmonary valve stenosis can cause a heart murmur. A heart murmur sounds like an extra click, blowing, whooshing, or rasping sound when a doctor listens to your heart. The murmur can be an initial indicator of pulmonary valve stenosis. It may be a sign that further testing is required.

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 doctor see where blood may not be flowing freely or if there is narrowing of the pulmonary valve.

How is pulmonary valve stenosis treated?

Your doctor will use imaging scans and other examinations to determine the best course of treatment for pulmonary valve stenosis. They might not recommend intervention if the stenosis is mild and not causing symptoms.

Seek medical treatment if you experience chest pain, shortness of breath, or fainting as a result of your pulmonary valve stenosis. These symptoms can indicate your condition is advancing.

There is a chance mild stenosis will improve with time. Though some patients will improve, others will continue to experience the same symptoms. And others will experience worsening symptoms that likely require interventions.

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.

Surgery may be required to replace the pulmonary valve in severe cases.

How can I improve my long-term outlook?

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.

Be sure to visit your doctor and cardiologist on a regular basis. They can help you keep close tabs on your heart health and reduce the likelihood of you developing any other forms of heart disease.

Content licensed from:

Written by: Rachel Nall
Medically reviewed on: Jun 20, 2016: Deborah Weatherspoon, Ph.D, MSN, RN, CRNA

This feature is for informational purposes only and should not be used to replace the care and information received from your health care provider. Please consult a health care professional with any health concerns you may have.
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