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).

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.

• 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 get worse with exercise or activity.

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