Pulmonary Valve Stenosis | Diagnosis and Treatment

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How is pulmonary valve stenosis diagnosed?

The most common signs of pulmonary valve stenosis in a newborn are blueness (cyanosis, lower oxygen levels) and the presence of a heart murmur. The degree of cyanosis is measured with a pulse oximeter device that is now found in all newborn nurseries. If your newborn has any symptoms that suggest a heart problem such as pulmonary valve stenosis, your pediatrician will refer you to a pediatric cardiologist.

The cardiologist will do a complete exam and listen for a heart murmur. The specific location and sound of the murmur will give the cardiologist an initial idea of what kind of heart problem your baby may have.

Your child’s doctor may also order one or more of the following tests to diagnose:

What are the treatment options for pulmonary valve stenosis?

Children with mild pulmonary valve stenosis don’t usually need treatment. However, they do require follow up over time, since in some patients, the degree of obstruction can progress.

Those who have moderate to severe stenosis are most often treated by cardiac catheterization (balloon dilation or valvuloplasty). During this procedure the doctor inserts a small, flexible tube (catheter) with a deflated balloon on the tip. Once the balloon is positioned across the narrowed valve, the balloon is inflated to stretch the valve open. This procedure is performed under sedation or anesthesia to ensure the patient does not feel any discomfort. Most patients are discharged either the same day of the procedure or the following day, after a brief period of observation.

In some cases, pulmonary valve stenosis may be repaired by open-heart surgery, although this is less common. Some infants with severe pulmonary valve stenosis may need emergency care prior to treatment.

What is the long-term outlook for pulmonary valve stenosis?

Most children will live healthy lives with normal growth, no restrictions on exercise and activities, and no risks associated with pregnancy later in life.

Even so, most people who’ve had congenital-heart-disease repair will have an ongoing relationship with their cardiologist, since they’ll always be at some risk for arrhythmias, infections and other problems. Your child’s cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood.

A small number of children may have leakage and calcifications (calcium deposits) from the repaired valve later in life. In some cases, they may need further treatment or valve replacement. Valve replacement may be done using open-heart surgery or using a technique in which a valve is inserted through a catheter and then expanded in the location of the pulmonary valve. Both of these procedures are carried out at Boston Children's Hospital.

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- Sandra L. Fenwick, President and CEO

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