Pulmonary valve stenosis is a heart valve disorder that involves the pulmonary valve.
This valve separates the right ventricle (one of the chambers in the heart) and the pulmonary artery. The pulmonary artery carries oxygen-poor blood to the lungs.
Stenosis, or narrowing, occurs when the valve cannot open wide enough. As a result, less blood flows to the lungs.
Valvular pulmonary stenosis; Heart valve pulmonary stenosis; Pulmonary stenosis; Stenosis – pulmonary valve; Balloon valvuloplasty – pulmonary
Causes, incidence, and risk factors
Narrowing of 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 heart defects that are present at birth (congenital). The condition can be mild or severe.
Pulmonary valve stenosis is a rare disorder.
In some cases, pulmonary valve stenosis runs in families.
Many cases of pulmonary valve stenosis are mild and do not cause symptoms. These infants are usually found to have a murmur on a routine heart examination.
When the valve narrowing (stenosis) is moderate to severe, the symptoms include:
Abdominal distention Bluish color to the skin(cyanosis) in some patients Chest pain Fainting Fatigue
- Poor weight gain or
failure to thrivein infants with severe blockage Shortness of breath
- Sudden death
Symptoms may get worse with exercise or activity.
Signs and tests
The health care provider may hear a
Tests used to diagnose pulmonary stenosis may include:
- Chest x-ray
ECG Echocardiogram MRI of the heart
The health care provider will grade the severity of the valve stenosis to plan treatment.
Sometimes, treatment may not be needed if the disorder is mild.
When there are also other heart defects, medications may be used to:
- Help blood flow through the heart (prostaglandins)
- Help the heart beat stronger
- Prevent clots (blood thinners)
- Remove excess fluid (water pills)
- Treat abnormal heartbeats and rhythms
Percutaneous balloon pulmonary dilation (valvuloplasty) may be performed when no other heart defects are present.
- This procedure is done through an artery in the groin.
- The doctor sends a flexible tube (catheter) with a balloon attached to the end up to the heart. Special x-rays are used to help guide the catheter.
- The balloon stretches the opening of the valve.
Some patients may need heart surgery to repair or replace the pulmonary valve. The new valve can be made from different materials. If the valve cannot be repaired or replaced, other procedures may be needed.
Patients with mild disease rarely get worse. However, those with moderate to severe disease will get worse. The outcome is good with successful surgery or balloon dilation. Other congenital heart defects may be a factor in the outlook.
Most often, the new valves can last for decades. Others wear out and will need to be replaced.
- Abnormal heartbeats (
Heart failureand enlargement of the right side of the heart
- Leaking of blood back into the right ventricle (pulmonary regurgitation) after repair
Calling your health care provider
Call your health care provider if:
- You have symptoms of pulmonary valve stenosis
- You have been treated or have untreated pulmonary valve stenosis and have developed swelling (of the ankles, legs, or abdomen), difficulty breathing, or other new symptoms
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