What is pulmonary valve stenosis?

Pulmonary valve stenosis is a heart condition affecting the pulmonary valve, the valve that moves the blood from the heart to the lungs, that develops before birth. The condition occurs when the pulmonary valve does not fully open.

Patients with mild pulmonary valve stenosis may never experience symptoms or need treatment, while those with moderate to severe pulmonary stenosis may need surgery as the disease progresses. Most people who are treated for pulmonary valve stenosis have successful outcomes.

If left untreated, pulmonary valve stenosis can lead to infectious endocarditis, heart failure, arrhythmia and problems with the heart pumping.

Aortic Valve Stenosis (Aortic Stenosis) Mitral Valve Stenosis (Mitral Stenosis) Tricuspid Valve Stenosis (Tricuspid Stenosis)

Causes of pulmonary valve stenosis

Most cases of pulmonary valve stenosis are from congenital heart defects, when the pulmonary valve does not form correctly in the womb. Babies who are born with pulmonary valve stenosis typically are born with other abnormalities.

Other causes include:

  • Carcinoid syndrome — results from release of carcinoid tumors in the digestive system.
  • Rheumatic fever — develops from untreated strep throat that can damage the heart valves.

Risk factors for pulmonary valve stenosis

There are few known risk factors of pulmonary valve stenosis if it develops as a congenital heart defect.

If the pulmonary stenosis develops later in life, the following conditions can increase your risk of developing the conditions:

  • Carcinoid syndrome
  • Rheumatic fever
  • Noonan’s syndrome

Symptoms of pulmonary valve stenosis

Some patients with pulmonary valve stenosis do not experience symptoms. Others with more severe stenosis first experience symptoms while exercising.

Symptoms of pulmonary valve stenosis include:

  • Heart murmur
  • Shortness of breath
  • Cyanosis
  • Dyspnea
  • Dizziness
  • Upper thorax pain
  • Developmental disorders

Diagnosis of pulmonary valve stenosis

Pulmonary valve stenosis is frequently diagnosed in childhood. Doctors may detect a specific heart murmur during a checkup and may order diagnostic tests to confirm the diagnosis.

Diagnostic tests include:

  • Echocardiogram (echo) — uses sound waves to produce images of the pulmonary valve, the test can determine the severity of the stenosis.
  • Electrocardiogram — measures the electrical impulses in the heart to determine if the right ventricular wall has thickened.
  • Cardiac catheterization — measures the blood pressure in the chambers of the heat and blood vessels.

Treatment for pulmonary valve stenosis

Treatment for pulmonary valve stenosis can depend on the severity of your case as well as the disease progression. Based on the blood pressure in the right ventricle and pulmonary artery, pulmonary valve stenosis is classified as mild, moderate or severe.

Mild pulmonary stenosis may only require close monitoring. As the disease progresses, your doctor will determine a procedure that will best relieve your symptoms.

Pulmonary stenosis surgical treatments may include:

  • Balloon valvotomy (balloon valvuloplasty) — during this procedure, a balloon is inserted into the pulmonary valve and inflated to widen the pulmonary valve.
  • Pulmonary valve repair or replacement — in severe cases a valve repair or replacement may be necessary. When possible, your surgeon will repair your valve using your own heart tissue, but may be replaced with a mechanical or tissue valve from a pig, cow or human heart.

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