Pulmonary valve stenosis symptoms & causes in children

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It’s natural for you to be concerned right now about your child’s health—a diagnosis of pulmonary valve stenosis can be upsetting. But you can rest assured that at Boston Children's Hospital, your child is in expert hands.

How does the normal heart work?

When a heart is functioning normally, blood is pumped through its four chambers with the help of four heart valves whose leaflets open and close in a way that allows blood to flow in only one direction.

One of these valves—the pulmonary valve—sends oxygen-poor (blue) blood from the right ventricle to the pulmonary artery. The pulmonary artery sends the oxygen-poor blood into the lungs, where it is enriched with oxygen, goes back to the left side of the heart and pumps through the aorta to the body.

Visit Heart and Blood Vessels for more on how the normal heart works.

What is pulmonary valve stenosis?

The pulmonary valve normally has three leaflets that allow blood to flow forward into the pulmonary artery, but not backward into the right ventricle. Pulmonary stenosis occurs when the pulmonary valve is unable to open completely, making it harder for the leaflets to allow blood to flow forward from the right ventricle to the lungs. In children, 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 don’t open all the way

Pulmonary stenosis may be present in varying degrees, according to how much obstruction to blood flow is present.

  • A child with mild pulmonary stenosis may have few or no symptoms.
  • A moderate or severe degree of obstruction can become worse with time and often requires intervention.
  • A child with severe pulmonary stenosis could be quite ill, with major symptoms noted early in life.

Why is pulmonary stenosis a concern?

Mild pulmonary stenosis usually doesn’t cause any symptoms, and usually doesn’t need treatment. But moderate to severe pulmonary stenosis can cause problems, such as forcing the right ventricle to work harder to move blood through the tight pulmonary valve. The right ventricle will thicken (hypertrophy) in response to the increased load.

Over time, if no intervention is performed on the abnormal valve, the right ventricle can fail to pump efficiently. If the right ventricle fails, pressure can build up in the right atrium (upper chamber), and then in the veins bringing blood back to the right side of the heart. Fluid retention and swelling may occur.

What are the signs and symptoms of pulmonary stenosis?

While mild and even moderate pulmonary stenosis generally doesn’t cause any symptoms, severe pulmonary valve stenosis typically presents in infancy and does.

  • Severe stenosis in newborns causes signs and symptoms of:
    • cyanosis (bluish tinge to skin color)
    • lethargy
    • pale, cool or clammy skin
    • circulatory shock
  • Symptoms in childhood are rare and may include:
    • exercise intolerance
    • heavy and rapid breathing
    • fatigue

How is pulmonary stenosis diagnosed? 

Your child's doctor may have heard a heart murmur during a physical exam and referred your child to a pediatric cardiologist* for a diagnosis. A heart murmur is a noise caused by the turbulence of blood flowing through the obstruction from the right ventricle to the pulmonary artery. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in 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 location within the chest where 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.

Other tests may assist with the diagnosis, including:

  • electrocardiogram (ECG or EKG) which looks at the rhythm of the heart, as well as the orientation and forces of the heart
  • echocardiogram (echo, cardiac ultrasound) which looks at the structures and function of the heart

How is pulmonary stenosis treated? 

Mild pulmonary stenosis generally does not require treatment. Moderate or severe stenosis is usually managed by cardiac catheterization (balloon dilation or valvuloplasty), in which:

  • A small, flexible tube (catheter) with a deflated balloon in the tip is inserted into the narrowed valve.
  • The balloon is inflated to stretch the area open.

Pulmonary valve stenosis can also be repaired by open heart surgery, although this is less common. The vast majority of abnormal pulmonary valves in infants and children are repaired through cardiac catheterization, usually on a non-emergency, scheduled basis.

Some infants who have severe pulmonary valve stenosis will need more emergent procedures and care in Boston Children’s Cardiac Intensive Care Unit (CICU) prior to treatment.

What is the long-term outlook after pulmonary valve repair? 

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 (abnormal heart rhythms), 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 percentage of repaired pulmonary valves will have leakage and calcifications from the repaired valve. Patients who develop symptoms later in life can be candidates for re-intervention or valve replacement.

What causes pulmonary stenosis? 

Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. Most of the time, this heart defect occurs sporadically (by chance), with no clear reason for its development.

Some congenital heart defects may have a genetic link—e.g., a defective gene, a chromosome abnormality or environmental exposure—causing heart problems to occur more often in certain families.

How common is pulmonary valve stenosis?

This heart defect is congenital (present at birth). It is the second most common congenital heart defect, accounting for 5 to 10 percent of all cases. Pulmonary valve stenosis often occurs by itself (in isolation), but it’s also a component of half of all complex congenital heart defects.

How will Boston Children’s research benefit my child?

Boston Children's has pioneered catheterization repair of many types of congenital heart defects, including pulmonary stenosis. Our research has yielded many potential patient benefits, including eliminating the need for open-heart surgery, reduces pain and the chance of infection, and shortens recovery time.

Where can my child find care and support when he grows up?

  • Our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.

    BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.
  • The Adult Congenital Heart Association (ACHA) is a national not-for-profit organization dedicated to improving the quality of life—and extending the life—of adults with congenital heart defects. The organization serves and supports many of the adults with congenital heart defects (thought at this time to be as many as one million), their families and the medical community, as well as conducting research and providing advocacy, outreach and education.

FAQ

Q: What is pulmonary valve stenosis?
A:
The pulmonary valve has three leaflets that normally allow blood to flow forward into the pulmonary artery, but not backward into the right ventricle. Pulmonary stenosis occurs when the pulmonary valve is unable to open completely, making it harder for the leaflets to allow blood to flow forward from the right ventricle to the lungs. In children, 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

Q: What are the symptoms of pulmonary stenosis?
A:
Severe stenosis in newborns causes signs and symptoms of:

  • cyanosis (bluish tinge to skin color)
  • lethargy
  • pale, cool or clammy skin
  • circulatory shock

Symptoms in childhood are rare and may include:

  • exercise intolerance
  • heavy and rapid breathing
  • fatigue

Q: If my child has pulmonary stenosis, will he be OK?
A:
While every child is different, there is good cause for hope. Heart treatments at Boston Children’s have among the highest success rates in the United States among large pediatric cardiac centers.

Q: How is pulmonary valve stenosis usually diagnosed?
A:
Your child’s pediatrician will detect a murmur and refer you to a pediatric cardiologist, who will assess the location of the murmur in the chest, loudness, and quality of the murmur (harsh, blowing, etc.) to diagnose the heart condition. Other tests may be helpful, including:

  • electrocardiogram (ECG or EKG) which looks at the rhythm of the heart, as well as the orientation and forces of the heart
  • echocardiogram (echo, cardiac ultrasound) which looks at the structures of the heart

Q: How does Boston Children’s treat pulmonary stenosis?
A:
Mild pulmonary stenosis generally does not require treatment. Moderate or severe stenosis is usually managed by cardiac catheterization (balloon dilation or valvuloplasty).

Pulmonary valve stenosis can also be repaired by open heart surgery, though this is less common. The vast majority of abnormal pulmonary valves in infants and children are repaired through cardiac catheterization, usually on a non-emergency, scheduled basis.

Q: If my child has a stenotic (closed or defective) pulmonary valve, what should I ask my Boston Children’s doctor?
A:
Some of the questions you may want to ask include:

  • What tests will you perform to further diagnose my child?
  • How severe is his pulmonary valve stenosis?
  • Does my child need a procedure? Will surgery be necessary? How long will he need to recover?
  • Will he need additional procedures during childhood?
  • Will my child be OK if he has congenital heart disease?
  • Will there be restrictions on my child’s activities in the future?
  • Will there be long-term health effects? Can this problem recur?
  • Will there be continuing risks?
  • What can we do at home?

Q: What is the long-term outlook for children who’ve had pulmonary stenosis?
A:
Most children will live healthy lives with normal growth, no restrictions on exercise and activities, and no risks associated with pregnancy later in life.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 (abnormal heart rhythms), 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 percentage of repaired pulmonary valves will have leakage and calcifications from the repaired valve. Patients who develop symptoms later in life can be candidates for re-intervention or valve replacement.

Q: What causes pulmonary valve stenosis?
A:
Most often, this heart defect occurs sporadically (by chance), with no clear reason for its development. It’s important for parents to understand that you’ve done nothing to cause your baby’s pulmonary valve stenosis.

Q: What is Boston Children’s experience treating congenital heart defects?
A:
Boston Children’s cardiologists and cardiac surgeons treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation for large pediatric cardiac centers.

Our experts have pioneered some of medical science’s most advanced heart treatments, now in use around the globe. Boston Children’s is the only major pediatric heart center in the world performing fetal heart interventions in volume for certain congenital defects.

Q: What heart innovation relevant to pulmonary valve stenosis is coming from Boston Children’s?
A:
Boston Children's has pioneered interventional catheterization repair of many types of congenital heart defects, including pulmonary stenosis. In many cases, this eliminates the need for open-heart surgery—resulting in less pain, less chance of infection and shorter recovery time. Research is continuing on the design and creation of heart valves using a patient's own tissue.
 

Causes 

The heart forms during the first eight weeks of fetal development. It’s at this time that defects can develop. Most of the time, these kinds of heart defects occur sporadically (by chance), with no clear reason for their development.

Some congenital heart defects may have a genetic link—e.g., a defective gene, a chromosome abnormality or environmental exposure—causing heart problems to occur more frequently in certain families.

It’s important for parents to understand that you’ve done nothing to cause your baby’s pulmonary valve stenosis.
 

Signs and symptoms

  • Severe stenosis in newborns causes signs and symptoms of:
    • cyanosis (bluish tinge to skin color)
    • lethargy
    • pale, cool or clammy skin
    • circulatory shock
  • Symptoms in childhood are rare and may include:
    • exercise intolerance
    • heavy and rapid breathing
    • fatigue

When to seek medical advice

Call your health care provider immediately if your baby or child:

  • is having difficulty breathing or is breathing rapidly
  • has a bluish color
  • tires too easily
  • is uninterested in eating

Questions to ask your doctor

If your child is diagnosed with pulmonary valve stenosis, you may feel overwhelmed with information. It’s easy to lose track of the questions that occur to you—so lots of parents find it helpful to jot down questions as they arise. This way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

Some of the questions you may want to ask include:

  • What tests will you perform to further diagnose my child?
  • How severe is his pulmonary valve stenosis?
  • Does my child need a procedure? Will surgery be necessary? How long will he need to recover?
  • Will he need additional procedures during childhood?
  • Will my child be OK if he has congenital heart disease?
  • Will there be restrictions on my child’s activities in the future?
  • Will there be long-term health effects? Can this problem recur?
  • Will there be continuing risks?
  • What can we do at home?

Who’s at risk

Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development, so it’s difficult to predict who’s at risk. Familial cases have been reported, but no genetic link has been confirmed. In general, if you have a child with a congenital cardiac defect, the chance of your having another child with a defect is about 2 to 3 percent.

Pulmonary valve stenosis is the second most common congenital heart defect, accounting for 5 to 10 percent of all cases.

Complications

A small percentage of repaired pulmonary valves will have leakage and calcifications from the repaired valve; patients who develop symptoms later in life can be candidates for re-intervention or valve replacement.

Long-term outlook

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

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 (abnormal heart rhythms), 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 percentage of repaired pulmonary valves will have leakage and calcifications from the repaired valve. Patients who develop symptoms later in life can be candidates for re-intervention or valve replacement.

For teens

If you’re a teen with a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you’ll also have to deal with:

  • medical appointments and procedures
  • some delay of your natural wish for independence
  • feeling different
  • assuming a lot of personal responsibility for maintaining your own good health
     

If you feel overwhelmed, depressed or anxious through this important time in your transition to adulthood, speak to your doctor, nurse, parent or counselor—they’re all on your team, and they all want to help.

For adults

If you were treated for congenital heart disease as a child, you’re probably being followed by your cardiologist, since complications from early heart disease can arise in adulthood.

You may need lifelong monitoring and medication, since you’ll continue to be at some risk for arrhythmias (abnormal heart rhythms), blood clots, infections, heart failure or stroke. Going forward, your cardiologist will also advise you on activity levels, pregnancy issues and certain lifestyle choices.

Fortunately, Children’s can help adults with congenital heart defects. Many adults who were patients as babies or children continue to be monitored by the clinicians who have followed them since childhood.

Our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood—whether or not they were originally treated at Children’s.

BACH is an international center for excellence, with physicians and services from Boston Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

Prevention

It’s important to understand that as parents, you’ve done nothing to cause your child’s pulmonary valve stenosis, and there are no precautions you could have taken to prevent it. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

Pulmonary valve stenosis glossary

  • aorta: one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and carries oxygen-rich blood out to the body.
  • cardiac catheterization: a specialized procedure in which a long, flexible tube is inserted into a vein or artery and guided into the heart.
    • It’s often used at Children’s instead of open-heart surgery to treat congenital heart defects.
    • Children’s performs hundreds of cardiac catheterizations each year.
    • Children’s trains more specialists in performing the procedure than any other hospital in the United States.
    • For pulmonary stenosis, catheters are used to open the stenotic valve.
  • cardiac/cardio: pertaining to the heart
  • cardiac surgery: surgical procedure performed on the heart or one of the blood vessels connected to the heart
  • cardiac surgeon: doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.
  • cardiologist: doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants, children and some adults with heart problems.
  • The Center for Families at Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
  • cyanosis (cyanotic): blue color of skin, lips and nailbeds caused by a reduction in the amount of oxygen-rich (red) blood circulating in baby’s bloodstream
  • congenital heart defect: heart defect present at birth. The heart usually starts to form in the first eight weeks of fetal development. It is thought that most congenital heart defects develop during this period.
  • diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools
  • echocardiogram (echo, cardiac ultrasound): a diagnostic tool that evaluates the structure and function of the heart using sound waves that produce a moving picture of your child’s heart and heart valves. The ultrasound can be used to understand flow in the different chambers and to estimate pressures.
  • electrocardiogram (ECG, EKG): a diagnostic tool that evaluates the electrical activity of your child’s heart.
  • heart murmur: a noise caused by the turbulence of blood flow in the heart
  • neonatologist: a doctor who specializes in illnesses affecting newborns, both premature and full-term
  • pediatric cardiologist: a doctor who specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood
  • pulmonary artery: one of the heart’s two great arteries, which normally arises from the right ventricle and carries oxygen-poor blood to the lungs, where it receives oxygen
  • sporadic: occurring by chance, occasionally, not inherited
  • symptoms: the presenting reasons why a child needs medical attention. A symptom’s characteristics—such as onset, quality, triggers and severity—help diagnosticians to determine a disease, or to decide which testing is needed to determine the disease.
     

For a more complete list of cardiovascular terms, visit Boston Children’s Heart Glossary.

For in-depth visual information on several of the conditions, diagnostic tools and procedures described above, visit Boston Children’s Cardiovascular Multimedia Library.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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