Alternate Names: Valvular pulmonary stenosis; Heart valve pulmonary stenosis

Causes and Risks:

Pulmonary stenosis is most often caused by a malformation during fetal development for unknown reasons. A narrowing may occur in the pulmonary valve or below the pulmonary valve at the pulmonary artery. The defect may occur alone, or with other heart defects. The condition can be mild or severe. It affects about 1 out of 8000 infants. Pulmonary stenosis can occur later in life as a result of conditions that cause damage or scarring of the heart valves. This includes rheumatic fever, endocarditis, and other disorders.

Prevention: Unknown

Symptoms:

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

Note: There may be no symptoms until the disorder is mild-to-moderately 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
• Doppler ultrasound

Treatment:

In some cases, treatment may not be required.

Surgical repair of the defect (heart valve surgery) is usually performed in preschool-aged children. Oxygen may be required prior to surgery if symptoms are severe. Medications used before surgery may include diuretics to remove the excess fluid, anti-arrhythmics to improve the heart function, and anticoagulants to prevent clots.

Prognosis:

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

Complications:

• heart failure
• right ventricular hypertrophy (enlargement)

Call your health care provider if symptoms of pulmonary stenosis are present.

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