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Pulmonary Valve Stenosis

Last updated Jan. 25, 2019

Approved by: Krish Tangella MD, MBA, FCAP

Pulmonary Valve Stenosis is a congenital heart valve disorder affecting the pulmonary valve. A stenotic or narrow pulmonary valve hinders the flow of blood from the heart to the lungs.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Heart Valve Pulmonary Stenosis
  • Rheumatic Pulmonary Valve Obstruction
  • Valvular Pulmonary Stenosis

What is Pulmonary Valve Stenosis? (Definition/Background Information)

  • The heart is divided into 4 chambers namely the right atrium, right ventricle, left atrium, and the left ventricle
  • The pulmonary valve allows oxygen-poor blood to flow from the right ventricle to the pulmonary artery and then to the lungs. The blood is then oxygenated in the lungs and it returns to the left side of the heart, to be supplied to the whole body
  • Pulmonary Valve Stenosis is a congenital heart valve disorder affecting the pulmonary valve. A stenotic or narrow pulmonary valve hinders the flow of blood from the heart to the lungs
  • This condition can range in intensity from mild to severe. If an individual has mild stenosis of the valve, the symptoms may go unnoticed for several years. Patients with moderate to severe obstruction may have bluish discoloration of the skin, breathing difficulties, chest pain, fatigue, and heart murmur
  • When treatment is necessary, the condition is treated either with balloon valvuloplasty or an open heart surgery. Mild cases of Pulmonary Valve Stenosis have an excellent prognosis
  • A moderate or severe form of the condition will usually require surgery to dilate the stenotic valve. In patients with valve replacement the prognosis is generally better

Who gets Pulmonary Valve Stenosis? (Age and Sex Distribution)

  • Pulmonary Valve Stenosis typically affects the newborn babies (neonates). It is considered to be a congenital defect, which may be associated with an abnormally developed heart
  • Individuals with mild obstruction of the pulmonary valve may present signs and symptoms later in childhood
  • There is no gender, racial, or ethnic group predilection observed; the condition is observed worldwide

What are the Risk Factors for Pulmonary Valve Stenosis? (Predisposing Factors)

Pulmonary Valve Stenosis occurs due to factors that arise prior to birth; currently, the risk factors have not been clearly identified. Even though rare, the probability of getting the condition can increase later in one’s life, in the presence of the following factors:

  • Carcinoid syndrome: A syndrome associated with carcinoid tumour of the small intestine, colon, appendix, and lungs
  • Rheumatic fever: It results from a bacterial infection that affects the heart valves
  • Noonan syndrome: A genetic disorder that affects normal growth and development

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pulmonary Valve Stenosis? (Etiology)

Pulmonary Valve Stenosis is a narrowing of the pulmonary valve which occurs at birth. The exact cause of the condition is unknown.

  • However, it seems to be associated with genetic factors, as it takes place during the fetal development stage
  • This defect can be present on its own or can be seen with other heart conditions
  • Pulmonary Valve Stenosis may be observed in other members of the family as well

What are the Signs and Symptoms of Pulmonary Valve Stenosis?

Pulmonary Valve Stenosis generally presents with mild to no symptoms. If the patient has mild narrowing of the pulmonary valve, the symptoms may go unnoticed for several years; although upon examination, the physician will be able to detect an abnormal heart sound.

Individuals with moderate to severe Pulmonary Valve Stenosis may present with the following signs and symptoms:

  • Bluish skin color, especially under the nail beds
  • Difficulty breathing, regularly feeling tired
  • Chest pain
  • Fainting
  • Presence of heart murmur: It is an abnormal heart sound that can be heard through a stethoscope and is caused by a turbulent flow of blood

How is Pulmonary Valve Stenosis Diagnosed?

The diagnosis of Pulmonary Valve Stenosis may involve the following exams and procedures:

  • Complete evaluation of medical history and a thorough physical exam. During the physical exam, the physician will try to listen to abnormal heart sounds using a stethoscope
  • Echocardiogram: It is the primary diagnostic test that is performed to identify the stenotic valve
  • Other tests, such as a chest MRI scan or a chest x-ray, can also help diagnose the condition

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pulmonary Valve Stenosis?

The complications associated with Pulmonary Valve Stenosis include:

  • Abnormal heart rhythm
  • Infection of the pulmonary valve
  • Enlargement of the right side of the heart
  • Heart failure
  • Sudden death may occur in some severe cases

How is Pulmonary Valve Stenosis Treated?

A mild form of Pulmonary Valve Stenosis does not require any treatment; only regular health check-ups by a cardiologist are recommended. Moderate to severe forms of Pulmonary Valve Stenosis do require treatment. They are generally treated by any of the following methods:

  • Balloon valvuloplasty: In this procedure, a catheter with un-inflated balloon is passed through the leg vein towards the pulmonary valve. The balloon is then inflated to dilate the narrowed valve, which increases the blood flow. The balloon is then removed
  • Open heart surgery: During this procedure, the pulmonary valve is either repaired or replaced with an artificial valve

How can Pulmonary Valve Stenosis be Prevented?

Currently, there are no effective preventative measures available for Pulmonary Valve Stenosis.

What is the Prognosis of Pulmonary Valve Stenosis? (Outcomes/Resolutions)

  • Pulmonary Valve Stenosis has an excellent prognosis when the condition is mild
  • In cases of moderate or severe forms of the condition, surgery is usually required to dilate the stenotic valve. Individuals, who have undergone a valve replacement, show a better prognosis

Additional and Relevant Useful Information for Pulmonary Valve Stenosis:

The following DoveMed website link is a useful resource for additional information:


What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: July 30, 2015
Last updated: Jan. 25, 2019