Pulmonic valve stenosis


Pulmonic valve stenosis

Description, Causes and Risk Factors:

Abbreviation: PVS.

Pulmonic 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.

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 pulmonic 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. Pulmonic valve stenosis is a rare disorder. In some cases, pulmonic valve stenosis runs in families.

Other risk factors:

    Carcinoid syndrome.

  • Rheumatic fever.

  • Noonan syndrome.

In most cases it is not a serious problem and needs no treatment, but if the narrowing is very severe the heart cannot pump normally and this may require treatment.

Preventive Measures:

    Eating heart-healthy diet.

  • Maintaining healthy weight.

  • Exercising.

  • Quit smoking if you smoke.

  • Consulting your doctor on regular interval.

Symptoms:

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 thrive in infants with severe blockage.

  • Shortness of breath [SOB].

  • Sudden death.

Diagnosis:

The health care provider may hear a heart murmur when listening to your heart using a stethoscope. Murmurs are blowing, whooshing, or rasping sounds heard during a heartbeat.

Tests used to diagnose pulmonic valve stenosis may include:

    ECG.

  • Echocardiogram.

  • MRI of the heart.

  • Chest x-ray.

  • Cardiac catheterization.

The health care provider will grade the severity of the valve stenosis to plan treatment.

Treatment:

Balloon Valvuloplasty: If the valve is severely narrowed treatment may be needed, usually as keyhole treatment rather than open heart surgery. Most cases can be treated by stretching the narrow valve open with a balloon (called "balloon Valvuloplasty"). This is done under general anaesthetic and involves passing a long tube with a collapsed balloon on the end of it (a balloon catheter) into the vein at the top of the leg and feeding it into the heart and across the narrow valve. Using X-ray pictures, the balloon is positioned in the narrow valve and is blown up, stretching the valve open. This is usually a very effective form of treatment and is very low risk - the chance of death during the procedure is less than 1 in 100. Usually only one night's stay in hospital is necessary. Sometimes if the narrowing is only partly relieved by the balloon it is worth repeating the procedure at a later date.

Because the thickened heart muscle (due to the narrow valve) itself can cause some narrowing, it is not always possible to tell straight after the procedure exactly how successful it has been - sometimes we have to wait for a few weeks for the thickened heart muscle to return to normal. In a small proportion of patients the thickened heart muscle does not return to normal and if this causes important narrowing inside the heart surgery is needed to remove some of the muscle.

Surgical treatment: Sometimes the pulmonary valve cannot be stretched open using a balloon and open heart surgery will be needed. This involves opening the chest (usually in the middle at the front) and the function of the heart and lungs being taken over by a machine so the surgeon can open the heart and cut the narrow valve open. There is a very small risk of death (less than 1 in 100) and a very small risk of major complications such as brain damage (less than 1 in 100). Other complications such as fluid collecting around the heart or lungs can occur after the operation but these are very rarely serious. After operation you would need to stay in hospital for about 5-7 days, although a longer stay might be necessary if there are any complications.

If you have not needed any treatment by the time you become an adult you are unlikely to need treatment, but in rare cases the valve can become narrower in later adult life. It is therefore important to attend a specialist Cardiology Clinic, to monitor your heart from time to time. Patients who have a good result from balloon stretching of the valve or surgery usually do not need any further treatment. However, after any kind of treatment for pulmonary stenosis, the pulmonary valve never works completely normally, and will leak to some extent (some of the blood pumped out of the heart to the lungs flows back into the heart through the valve). If you have a leak on the valve there is a chance that you may need surgery to replace or repair the valve in later life.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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