Pulmonary Stenosis
What is Pulmonary Stenosis
Pulmonary stenosis is a congenital heart defect that occurs when the pulmonary valve does not develop properly during pregnancy.
The pulmonary valve is the valve between the right ventricle and the pulmonary artery. In a heart that has developed normally, the pulmonary valve has three leaflets that allow blood to flow from the right ventricle to the pulmonary artery, but not the other way.
With pulmonary stenosis, the pulmonary valve is too small, narrow, or stiff, impacting the way blood is able to flow from the right ventricle to the pulmonary artery.
Types of pulmonary stenosis
There are four types of pulmonary stenosis, defined by what is causing the obstruction of blood flow.
A person has valval pulmonary stenosis if they have:
- A valve that has partially fused leaflets
- A valve that has thickened leaflets that do not open all the way
A person has supra valvar pulmonary stenosis if:
- They have a valve that is too small (hypoplastic)
- The main pulmonary artery just above the pulmonary valve is narrowed
A person has sub valvar pulmonary stenosis if:
- The muscle under the valve area is thickened, narrowing the outflow tract from the right ventricle
A person has branch/peripheral pulmonary artery stenosis if:
- The right or left pulmonary artery is narrowed, or both may be narrowed
What are the symptoms of pulmonary stenosis?
Some children with pulmonary stenosis do not have symptoms. The more severe the issue, the more likely the child is to have symptoms. The most common symptoms may include:
- Hard or fast breathing
- Bluish color around the lips or fingers
- Shortness of breath
- Feeling tired, especially with activity or exercise
- Accelerated heart rate
- Swelling of the legs, ankles, feet, face, or abdomen
- Fainting
- Chest pain
What treatment options are there?
In instances of mild pulmonary stenosis, no treatment may be needed. If the condition is moderate or severe, the valve will need to be repaired or replaced.
Options for repair or replacement include:
Balloon dilation or valvuloplasty: A catheter with a balloon on the tip is put into the heart. When the catheter reaches the narrowed valve or area, the balloon is inflated to stretch it open. Children who have had balloon dilation may need to take antibiotics to prevent heart infection after being discharged from the hospital.
Valvotomy: A valvotomy removes scar tissue from the pulmonary valve leaflets, allowing the valve to open as it should.
Valvectomy: A valvectomy is surgery to remove the valve. Sometimes a patch is used to help the blood flow from the right ventricle into the pulmonary artery.
Patch enlargement: Patches are used to enlarge narrowed areas. They may be added to the right ventricle or the pulmonary artery.
Pulmonary valve replacement: Sometimes, the pulmonary valve will need to be replaced. A tissue valve (pig or human) may be used. Children who have had valve replacement will need to take antibiotics before medical and dental procedures in the future.
