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Pulmonary Valve Stenosis (PVS) Pulmonary Valve Stenosis (PVS)
Pulmonary valve stenosis or PVS is the second most commonly diagnosed congenital heart defect in the US. The pulmonary valve is the valve... Pulmonary Valve Stenosis (PVS)

by Kimberly Allen RN

Pulmonary valve stenosis or PVS is the second most commonly diagnosed congenital heart defect in the US.  The pulmonary valve is the valve that opens to allow the deoxygenated blood to leave the heart and go to the lungs to be reoxygenated.  PVS almost always develops while the fetus is in utero however, occasionally adults can develop it as a complication of some other illness.  Children born with PVS have a defect on or near the pulmonary valve that decreases the blood flow to their lungs.
PVS develops because the valve doesn’t develop properly during fetal growth and development.  Many children with PVS also have other congenital heart abnormalities though the exact cause is unknown.  Many children born with PVS do not have any symptoms however, those born with sever PVS can have symptoms as newborns.  Newborns with severe PVS will usually have a bluish tint to their skin , they’ll be lethargic and may go into circulatory shock.  Usually if the PVS is severe enough to cause symptoms it is discovered shortly after birth however it may not be noticed until later in childhood.  Symptoms that your child may demonstrate in childhood include the inability to tolerate physical activity, increased fatigue and rapid, heavy breathing.  Some complain of chest pain and feeling like their heart is beating “funny”, which is usually palpitations and some children faint easily.
Mild cases of PVS usually doesn’t cause complications, however those with severe PVS can potentially develop numerous complications including arrhythmias and infections.  Most children born with PVS will have some degree of arrhythmia, also known as an irregular heart beat.  Usually these arrhythmias are mild and pose no problems unless the PVS is severe.  Children born with problems with the heart structure are much more prone to bacterial infections called endocarditis.  Endocarditis is an infection of the lining of the heart.  Other potential problems include pumping issues which in turn can lead to heart failure.  When there is severe PVS the heart has to pump harder to push the blood out into the blood vessel known as the pulmonary artery.  This increases the pressure in the right ventricle causing the wall to thicken which then increases ventricular chamber.  As this process  progresses the heart weakens and stiffens.  Once this happens heart failure begins.  As the heart failure develops there will be swelling in the legs and abdomen, increased fatigue and shortness of breath.
Treatment for PVS depends on the severity of the stenosis.  If the PVS is mild and there are no symptoms no treatment may be necessary.  However, your Dr will want to monitor your child for any changes.  In more severe cases of PVS surgery may be needed to repair the stenosisl  There are two options available, balloon valvuloplasty using a cardiac catheter or open heart surgery.  Both options are not viable in every situation.  In order to decide which method of treatment is best for your child the Dr will need to know the severity and location of the stenosis.  If the stenosis.  If the stenosis is  above or below the pulmonary valve balloon valvuloplasty is not an option that is available and open heart surgery is the only viable option for repairing and/or replacing the valve.  The potential for complications is greater and the recovery time is much longer with open heart surgery than with cardiac catheterization, however, cardiac catheterization is not always the best option.
Research and become knowledgeable about your child’s illness so that when you sit with your childs Dr to discuss treatment you can ask questions and make an informed decision.

Kimberly Allen is a registered nurse with an AND in nursing. She has worked in ACF, LCF and psychiatric facilities, although she spent most of her career as a home health expert. She is now a regular contributor to, dispensing advice and knowledge about medical issues and questions. You can reach her with any comments or questions at