Valvular pulmonary stenosis

Alternative names
Pulmonary valve stenosis; Heart valve pulmonary stenosis

Definition
Pulmonary valve stenosis is a condition, usually present at birth (congenital), in which outflow of blood from the right ventricle (lower chamber) of the heart is obstructed at the level of the pulmonic valve (the valve which separates the heart from the pulmonary artery).

Causes, incidence, and risk factors

Pulmonary valve stenosis is most often caused by a malformation during fetal development. The cause is unknown. A narrowing may occur in the pulmonary valve or below the pulmonary valve at the pulmonary artery.

The defect may occur alone, but is relatively common in connection with other heart defects. The condition can be mild or severe. It occurs in approximately 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

     
  • shortness of breath  
  • fatigue  
  • bluish coloration to the skin (cyanosis)  
  • chest pain  
  • fainting  
  • poor weight gain or failure to thrive in infants  
  • sudden death

Note: There may be no symptoms until the disorder is severe. Symptoms, when present, may be aggravated by exercise or activity.

Signs and tests

A heart murmur may be heard by stethoscope.

Tests used in the diagnosis of pulmonary stenosis may include:

     
  • chest X-ray  
  • ECG  
  • echocardiogram  
  • cardiac catheterization

Treatment

In some cases, treatment may not be required.

Percutaneous balloon pulmonary dilation (valvuloplasty) has recently been found quite successful as treatment for the form of Pulmonary valve stenosis that occurs without the presence of other heart defects.

Surgical repair of the defect (heart valve surgery) is usually performed when the child has reached preschool age. Oxygen may be required prior to surgery if symptoms are severe.

Medications used before surgery may include prostaglandins (PGE) to maintain pulmonary blood flow, water pills to remove the excess fluid, anti-arrhythmics to improve the heart function, and blood thinners to prevent clots.

Expectations (prognosis)

The outcome may be poor without surgical repair. The outcome is good with successful surgery.

Complications

     
  • heart failure  
  • right ventricular hypertrophy (enlargement)

Calling your health care provider

Call your health care provider if symptoms associated with Pulmonary valve stenosis occur.

Call your health care provider if swelling (of the ankles or any area), difficulty breathing, or other new symptoms develop in a person with treated or untreated Pulmonary valve stenosis.

Johns Hopkins patient information

Last revised: December 8, 2012
by Brenda A. Kuper, M.D.

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