Pulmonary Stenosis (PS)

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What is Pulmonary Stenosis?

Pulmonary stenosis is a very rare condition. It can be diagnosed in utero or shortly after birth. Ultrasound images of the four chambers of the heart in pregnancy may show isolated pulmonary stenosis due to an abnormally thickened or bicuspid valve, or pulmonary stenosis in association with other congenital anomalies, e.g. Fallot’s tetralogy, Noonan’s syndrome.

The heart on the left is normal showing its pulmonary valve wide open accepting blood. The heart on the right has pulmonary stenosis. Note how little blood flow can get through its pulmonary valve

Pulmonary Stenosis is a heart valve disorder where there is a narrowing or blockage of the pulmonary valve. In a healthy heart the pulmonary valve opens to let blood flow from the right ventricle to the lungs. In a patient with pulmonary stenosis the narrowed pulmonary valve means that the right ventricle has to work harder to get the blood past the blockage and to the lung arteries. In time this can cause the heart muscle to become damaged.

Symptoms

Symptoms of pulmonary stenosis vary depending on the severity of the condition.

Usually the only sign of pulmonary stenosis is a heart murmur which may be picked up during a routine medical check (a heart murmur is an unusual sound from the heart, which can be heard through a stethoscope). Other symptoms can include:

  • shortness of breath (dyspnea);
  • chest pain (angina);
  • fainting (syncope), or
  • cyanosis (blueness).

If significant pulmonary stenosis is present then the child may feel tired when playing or doing any physical activity.

In mild cases the patient may not suffer any symptoms at all.

Diagnosis

Diagnosis before birth by an MRI usually means that pulmonary stenosis will be severe and treatment will be needed shortly after birth. However, if Pulmonary Stenosis is not diagnosed before birth, it can be diagnosed by other tests in the form of the following:

Treatment

The pulmonary valve can be treated to improve the narrowing but it can’t be made normal. Treatment will be required when the pressure in the right ventricle is high.

Balloon Valvoplasty

In many cases the narrowing can be widened by balloon valvoplasty. This is where a balloon is placed into an artery in the groin, advanced to the heart, placed across the valve, and inflated in the hope it will relieve the obstruction caused by the narrowed valve. Valvoplasty is a low risk effective treatment of pulmonary stenosis. Out of every 100 children with the condition, 99 will survive. In a small number of cases, the thickened overworked heart muscle doesn’t return to its normal size and the muscle itself can obstruct the normal flow of blood. If this causes significant narrowing inside the heart, the child will need to have surgery to remove some of the muscle.

Open Heart Surgery

For very severe cases of Pulmonary Stenosis surgery will be needed. This is where the heart is stopped and put onto a heart bypass machine. Once the heart has been stopped, the surgeon will open the heart and make a small cut in the valve, to widen the narrowed valve and allow more blood to flow through. The heart is then restarted. If a surgical widening of the valve is not possible then a valve replacement may be done. A homograft valve is usually used to replace the pulmonary valve, which is a valve from another person. This type of valve will need replacing after a few years.

After the operation, your child will have a scar down the middle of the chest, along the breast bone.

After treatment

A good result from a balloon valvoplasty or surgery usually means that further treatment is not required. However, the pulmonary valve will never work normally, and there will be leakage of some degree (this is where some of the blood pumped out from the heart to the lungs flows back into the heart through the valve) This leak is not often very important, but there is a chance that some patients may need further surgery to repair or replace the valve later on in life.

Disclaimer: The facts and opinions shown in this article are as accurate and up to date as possible, but are provided as general “information resources”, which may not be relevant to individual persons. This article is not a substitute for individual assessment and always take advice from a doctor who is familiar with the particular person.

Consult you or your child’s physician regard the specific outlook for you or your child.
Related Links

http://www.americanheart.org/presenter.jhtml?identifier=1321

http://www.childrenshospital.org/az/Site512/mainpageS512P0.html

http://www.yorksandhumberhearts.nhs.uk/templates/Page.aspx?id=421

www.chop.edu/healthinfo/pulmonary-stenosis.html

Researched by Teresa Hawes
Rewritten by Lisa Lathane (twice!!!)

@lopta guessed correctly that we were writing up Pulmonary Stenosis.