Pulmonary Stenosis is a narrowing of the pulmonary artery or the pulmonary valve causing an obstruction (blockage) of blood flow out of the right ventricle to the lungs. 

The most common type of pulmonary stenosis is a narrowing of the pulmonary valve

The normal pulmonary valve consists of three (3) thin and flexible valve leaflets.  When the right ventricle contracts (constricts) these valve leaflets spread apart easily allowing the blood to flow out of the heart and to the lungs.

If the pulmonary valve or pulmonary artery is small, or if the pulmonary valve leaflets are not formed normally, blood flow out of the heart may be more difficult or blocked. 

A Pediatric Cardiologist—a specialist in heart problems of babies and children—sees babies with pulmonary stenosis.

Murmur—This is the sound made as blood in the heart flows through the obstructed pulmonary artery or valve.  It is heard when the doctor, nurse practitioner and/or the nurse listens to your baby’s chest.

Cyanosis—This means a bluish coloring to the baby’s lips and skin.  The cyanosis is not usually seen immediately after birth.  It usually develops or becomes more noticeable in the first few days of life.

Infants/children with mild Pulmonary Stenosis usually do not have cyanosis.

Rapid/fast breathing (Tachypnea).

Work harder to breathe—have retractions.

Rapid/fast heart rate (Tachycardia).

Tire easily—even with everyday baby care, like feeding, handling, changing the diaper.

 If the neonatologist notices any of the symptoms listed above and thinks the baby has PS an echocardiogram may be ordered. 

An echocardiogram is a painless ultrasound of the heart.  It uses high frequency sound waves to measure blood flow and is done at your baby’s bedside. 

The pediatric cardiologist will see your baby and will decide if your baby’s heart defect needs:

	Regular checkups
	Special tests and procedures
	Surgery.

Special tests and procedures

Echocardiography (ECHO):  An ECHO is an ultrasound of the heart.  It is a painless procedure that uses high-frequency sound waves to create a picture of the inside of your baby’s heart.  It also measures the blood flow through his heart.  The test will not hurt your baby.  An ECHO is important in eliminating other problems that may be associated with PS, such as an ASD or a VSD.

Cardiac catheterization:  A cardiac catheterization, often called a “cardiac cath,” can evaluate the size of the pulmonary artery, give us information about the blood flow through the heart and lungs, as well as information about the movement of the pulmonary valve.  This procedure is done in the Cardiac Cath Department and the baby is asleep for this procedure.  Often we may be able to fix the pulmonary valve during the cardiac cath procedure.

If your baby does need a “cardiac cath” and/or surgery, he may be transferred to either Duke University Medical Center or The University Hospital at Chapel Hill for the surgery.

	If the decision is to transfer your baby to another hospital you will be asked to sign a consent giving Cape Fear Valley Medical Center permission to transfer your baby.
	If your baby is transferred to another hospital, the doctors there will also talk with you about your baby’s cardiac cath procedure or surgery.
	Sometimes the cardiac cath procedure or surgery can be delayed until your baby is older.

The procedure is done in the Operating Room and the baby is asleep.  You will be asked to sign a consent (give permission) for the procedure.

Once your baby’s condition is stable and the doctors are happy with the baby’s progress, the baby will be returned to Cape Fear Valley Medical Center’s Neonatal Intensive Care Unit.

 Both the pediatric cardiologist and our neonatologist will talk with you about your baby and what they recommend. 

 Please feel free to ask questions at any time. 

An important part of the baby getting better is the parents’ love and concern.

We encourage you to talk and sing to your baby, gently touch or rub them and hold them close if tolerated by the baby.  Talk with your baby’s nurse about this. 

Your love and attention will go a long way in helping your little one on the road to recovery. 

Once your baby returns to Cape Fear Valley Medical Center, keep up with your baby’s progress each time you visit or call.