Pulmonary valve stenosis

Pulmonary valve stenosis is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy. This condition is different from pulmonary vein stenosis.

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 valve 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 infants, 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 valve stenosis may be present in varying degrees, classified according to how much obstruction to blood flow is present. A child with severe pulmonary valve stenosis could be quite ill, with major symptoms noted early in life. A child with mild pulmonary valve 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 valve stenosis is a component of half of all complex congenital heart defects. It is the second most common congenital heart defect, comprising 5 to 10 percent of all cases.

What causes pulmonary valve stenosis?

Congenital pulmonary valve 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, though most of the time this heart defect occurs sporadically (by chance), 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.

Why is pulmonary valve stenosis a concern?

Mild pulmonary valve stenosis may not cause any symptoms. Problems can occur when pulmonary valve stenosis is moderate to severe, including the following:

• The right ventricle has to work harder to try to move blood through the tight pulmonary valve. Eventually, the right ventricle is no longer able to handle the extra workload, and it fails to pump forward efficiently. Pressure builds up in the right atrium, and then in the veins bringing blood back to the right side of the heart. Fluid retention and swelling may occur.
• There is a higher than average chance of developing an infection in the lining of the heart known as bacterial endocarditis.

What are the symptoms of pulmonary valve stenosis?

The following are the most common symptoms of pulmonary valve 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

The symptoms of pulmonary valve stenosis may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

How is pulmonary valve stenosis diagnosed?

Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction from the right ventricle to the pulmonary artery. Symptoms your child exhibits will also help with the diagnosis.

A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. However, other tests are needed to help with the diagnosis, and may include the following:

• Chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
• Electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.
• Echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
• Cardiac catheterization - a cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.

Treatment for pulmonary valve stenosis

Specific treatment for pulmonary valve stenosis will be determined by your child's physician based on:

• Your child's age, overall health, and medical history
• Extent of the condition
• Your child's tolerance for specific medications, procedures, or therapies
• Expectations for the course of the condition
• Your opinion or preference

Mild pulmonary valve stenosis often does not require treatment. Moderate or severe pulmonary valve stenosis is treated with repair of the obstructed valve. Several options are currently available.

Some infants will be very sick, require care in the cardiac intensive care unit (CICU) prior to the procedure, and could possibly even need emergency repair of the pulmonary valve if the stenosis is severe. Others, who are exhibiting few symptoms, will have the repair scheduled on a less urgent basis.

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. Other procedures may also increase the risk of the heart infection occurring. It is important that you inform all medical personnel that your child has aortic stenosis so they may determine if the antibiotics are necessary prior to the procedure.

Pulmonary valve 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. 

Postoperative care for your child

After pulmonary valve surgery, your child will go to the cardiac intensive care unit (CICU). While your child is in the ICU, special equipment will be used to help him/her recover from surgery, and may include the following:

• Ventilator - a machine that helps your child breathe while he/she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he/she is too sleepy to breathe effectively on his/her own. Many children remain on the ventilator for a while after surgery so they can rest.
• Intravenous (IV) catheters - small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation.
• Arterial line - a specialized IV placed in the wrist, or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.
• Nasogastric (NG) tube - a small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
• Urinary catheter - a small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys to remove excess fluid from the body.
• Chest tube - a drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
• Heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values. 

Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.

Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.

After being discharged from the ICU, your child will recuperate in another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.

Long-term outlook after pulmonary valve stenosis surgical repair

Most children who have had a pulmonary valve stenosis surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal. Infants who had a difficult post-operative course may be less healthy than infants who did not experience complications.

Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis after discharge from the hospital.

Consult your child's physician regarding the specific outlook for your child.