Pulmonary Stenosis

Pulmonary Stenosis is a narrowing of the valve that lets the blood flow from the lower-right chamber (the right ventricle) into the lungs. When this valve narrows, the right ventricle has to work harder and it becomes enlarged.

The Pulmonary Valve is found between the right ventricle and the pulmonary artery. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery, but not backward into the right ventricle.

With Pulmonary Stenosis, problems with the pulmonary valve make it harder for the leaflets to open and permit blood to flow forward from the right ventricle to the lungs. In children, these problems can include:

* A valve that only has one or two leaflets instead of three.

* A valve that has leaflets that are partially fused together.

* A valve that has thick leaflets that do not open all the way.

Pulmonary Stenosis may be present in varying degrees, classified according to how much obstruction of blood flow is present. A child with severe pulmonary stenosis could be quite ill, with major symptoms noted early in life. A child with mild pulmonary stenosis may have few or no symptoms, or perhaps none until later in adulthood. A moderate or severe degree of obstruction can become worse with time.

Pulmonary Stenosis is the second most common congenital heart defect, comprising 5 to 10 percent of all cases.

CAUSES OF PULMONARY STENOSIS

Pulmonary Stenosis occurs due to improper development of the pulmonary valve in the first 8 weeks of fetal growth. It can be caused by a number of factors, with no clear reason evident for its development.

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families.

SYMPTOMS OF PULMONARY STENOSIS

The following are the most common symptoms of Pulmonary Stenosis. However, each child may experience symptoms differently. Symptoms may include:

* Heavy or rapid breathing

* Shortness of breath

* Fatigue

* Rapid heart rate

* Swelling in the feet, ankles, face, eyelids, and/or abdomen

* Fewer wet diapers or trips to the bathroom.

DIAGNOSIS

The doctor will be confirming to Pulmonary Stenosis by the following:

* Abnormal chest sounds, such as a heart murmur or click

* Noticeable chest heave or vibration when the doctor's hand is held over the heart

To confirm the diagnosis, tests may include:

* CHEST X-RAY – a test that uses radiation to take pictures of structures inside the chest.

* ELECTROCARDIOGRAM(ECG, EKG) – a test that records the heart's activity by measuring electrical currents through the heart muscle; it may show signs of heart strain or enlargement.

* ECHOCARDIOGRAM – a test that uses high-frequency sound waves (ultrasound) to examine the size, shape, and motion of the heart.

* CARDIAC CATHETERIZATION – an x-ray of the heart's circulation that is done after injection of a contrast dye.

TREATMENT FOR PULMONARY STENOSIS

Mild Pulmonary Stenosis often does not require treatment. Moderate or Severe Stenosis is treated with repair of the obstructed valve.

Children who do not require immediate repair in infancy may need to receive antibiotics to prevent an infection of the inner surfaces of the heart known as Bacterial Endocarditis prior to procedures such as a routine dental check-up and teeth cleaning.

Repair options include the following:

* BALLOON DILATION or VALVULOPLASTY - in a cardiac catheterization procedure, a small, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. The tube has a deflated balloon in the tip. When the tube is placed in the narrowed valve, the balloon is inflated to stretch the area open.

* VALVOTOMY - surgical release of adhesions that are preventing the valve leaflets from opening properly.