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Heart, section through the middle
Heart, section through the middle


Heart, front view
Heart, front view


Heart valves
Heart valves


Definition:

Pulmonary valve stenosis is a condition in which the flow of blood from the heart (right ventricle, or lower chamber) is blocked at the valve that separates the heart from the pulmonary artery (pulmonic valve). This narrowing is usually present at birth (congenital).



Alternative Names:

Valvular pulmonary stenosis; Heart valve pulmonary stenosis



Causes, incidence, and risk factors:

Pulmonary valve stenosis is most often caused by a problem that occurs when the unborn baby (fetus) is developing. The cause is unknown, but genetics may play a role.

Narrowing that occurs in the pulmonary valve is called pulmonary valve stenosis. Narrowing that occurs below the pulmonary valve is called subvalvar pulmonary stenosis. Another form of the condition, supravalvar pulmonary stenosis, is when narrowing occurs above the main pulmonary valve.

The defect may occur alone. However, it can also occur with other heart defects. The condition can be mild or severe. It occurs rarely, in only about 10% of patients with congenital heart disease.

Pulmonary stenosis can also occur later in life as a result of conditions that cause damage or scarring of the heart valves. These include rheumatic fever , endocarditis , and other disorders.



Symptoms:

Note: Patients with mild-to-moderate blockage may not have any symptoms. There may be no symptoms until the disorder is severe. Symptoms, when present, may get worse with exercise or activity.



Signs and tests:

The health care provider may hear a heart murmur by stethoscope. Tests used in the diagnosis of pulmonary stenosis may include:



Treatment:

Sometimes, treatment may not be required.

Percutaneous balloon pulmonary dilation (valvuloplasty) using a catheter can be successful for pulmonary valve stenosis that occurs without other heart defects.

Surgery may be performed to repair the defect.

Medications used before surgery may include:

  • Anti-arrhythmics to improve the heart function
  • Blood thinners to prevent clots
  • Prostaglandins
  • Water pills to remove the excess fluid


Support Groups:



Expectations (prognosis):

As a general rule with mild stenosis, one-third of patients get better, one-third stay the same, and one-third get worse. The outcome is good with successful surgery or cardiac catheterization. Other congenital heart defects may also be a factor.



Complications:
  • Cyanosis
  • Death
  • Heart failure
  • Leaking of blood back into the right ventricle (pulmonary regurgitation) after repair
  • Right ventricular hypertrophy (enlargement)


Calling your health care provider:

Call your health care provider if you have symptoms of pulmonary valve stenosis.

Call your health care provider if you have treated or untreated pulmonary valve stenosis and you develop swelling (of the ankles or any area), difficulty breathing, or other new symptoms.



Prevention:



References:

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.




Review Date: 5/15/2008
Reviewed By: Robert A. Cowles, MD, Assistant Professor of Surgery, Columbia University College of Physicians and Surgeons, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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