Pulmonic Valve Stenosis

The heart is made up of two pairs of chambers. The upper chambers (atria) are smaller and less muscular than the lower chambers (ventricles).

In a heart that is working normally, blood filled with oxygen from the lungs flows into the left atrium. From there, the blood flows into the left ventricle, which pushes it into the arteries of the body. When the oxygen in the blood has been used up, the blood is pumped into the right atrium. From there it is pumped into the right ventricle, which pumps the blood into the lungs to receive more oxygen.

In pulmonic valve stenosis there is a narrowing in the opening of the valve between the pulmonary artery and the right ventricle. This condition causes about 10% of all congenital heart disease.

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Symptoms

In newborns this narrow opening only allows a small amount of blood to flow from the right ventricle. This condition is usually an emergency that requires an immediate diagnosis.

In older children the bluish, purplish skin color that shows a lack of oxygen may not be present, but poor skin color may be present on the hands and feet. A heart murmur with a noticeable click may also be present.

Diagnosing this condition in older children is usually based on:

  • Symptoms
  • Presence of a heart murmur with a noticeable click
  • Electrocardiography
  • X-rays
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Treatments

In newborns immediate surgery is needed to create a shunt to restore adequate pulmonary blood flow. Until this is done, drugs may be used. Replacement of the pulmonary valve may be necessary, and additional surgery may be needed to improve the flow of blood from the right ventricle as the child grows older. Balloon valvulopasty may be used for infants with a normal or near-normal right ventricle.

In older children the preferred approach is balloon valvuloplasty with catheterization. During this procedure flexible tubes (catheters) are put into blood vessels, usually through the groin area. They are long enough to reach inside the heart and the major blood vessels. As the catheters are moving inside the blood vessels and heart, they are monitored by fluoroscopy, which uses radiation energy to create a picture on a TV monitor for surgeons to watch during the procedure.

While the catheters are inside the heart and blood vessels:

  • The pressures inside the lower right chamber of the heart (right ventricle) and the pulmonary artery are measured
  • The ring of tissue that supports the pulmonary valve is measured
  • A balloon is inserted so that it is centered across the pulmonary valve, and it is inflated two to three times to widen the valve
  • After the balloon has been used to widen the valve, the measurements of the pressures inside of the right ventricle and the pulmonary artery and of the diameter of the valve are repeated to make sure that there has been an improvement in how the valve is working

This procedure allows doctors to evaluate the wall between the two upper chambers of the heart and accurately measure the pressure in the pulmonic valve. Replacement of the valve may be needed. If the condition is not severe, treatment may be delayed until just before preschool.

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