Pulmonary stenosis is most often unknown and due to a malformation during fetal development, in which the flow 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). It may occur later in life as a result of conditions that cause damage or scarring of the heart valves (as with rheumatic fever or endocarditis).
Symptoms:
Symptoms include shortness of breath, fatigue, bluish coloration to the skin, chest pain, poor weight gain or failure to thrive in infants, or sudden death. There may be no symptoms until the disorder is severe. Symptoms, when present, may get worse with activity.
Risk Factors:
Narrowing in the pulmonary valve is usually present at birth (congenital). It is 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 valve itself is called pulmonary valve stenosis. There may also be narrowing just before or just after the valve.
The defect may occur alone or with other congenital heart defects. The condition can be mild or severe.
Pulmonary valve stenosis is a rare disorder.
How it is Diagnosed:
A murmur may be heard with the stethoscpore. Tests used in the diagnosis may include an electrocardiogram (ECG), a chest XRay, and/or an echocardiogram.
Treatment Options:
In some cases, treatment is not needed. Percutaneous balloon pulmonary dilation (valvuloplasty) has been used for treatment of pulmonary stenosis that occurs without the presence of other heart defects. Heart valve surgery is usually performed in children when they reach preschool age. Oxygen may be needed if symptoms are severe. Medications used before surgery may include prostaglandins (PGE) to maintain pulmonary blood flow, water pills to remove excess fluid, anti-arrhtyhmics to improve the heart pump function, and blood thinners to prevent clots.