Peripheral pulmonary stenosis
Learning more about peripheral pulmonary stenosis can help you better understand what's next for your child and family, and what to expect in the days and weeks ahead.
To picture what happens when a child has peripheral pulmonary stenosis, it’s helpful to have a solid understanding of the heart's anatomy. The diagram below depicts the heart’s structures (click to enlarge).
- are responsible for carrying blood from the heart to the lungs
- are the only arteries in the human body (besides the umbilical cord arteries that nourish babies in the womb) carrying blood that has been de-oxygenated—meaning the oxygen has been delivered throughout the body where it is needed, and has been replaced by carbon dioxide
You can think of the pulmonary arteries as a large tree with the following parts:
- the main pulmonary artery, or “trunk,” that reaches from the right ventricle of the heart into the two pulmonary branches
- the pulmonary branches—one on the left and one on the right—each of which branches out many times into the corresponding lung
The term “stenosis” describes an abnormal narrowing within a structure of the body. Peripheral pulmonary stenosis, therefore, describes narrowing in one or more areas within the pulmonary artery branches. This condition should not be confused with pulmonary (valve) stenosis (PVS, PS) or pulmonary vein stenosis, which are different conditions.
Children with peripheral pulmonary stenosis are at risk of getting insufficient oxygen within their blood, placing increased pressure on the heart. The condition can affect each child differently, so it’s important to talk to your clinician about your child’s individual circumstances and the recommended treatment options.
What causes peripheral pulmonary stenosis?
Peripheral pulmonary stenosis is typically a congenital heart defect—something a child is born with, rather than a disease that is acquired later in life. It can occur:
- as a complication of Tetralogy of Fallot, a rare condition in which four different heart defects are all present at birth
- in conjunction with certain genetic syndromes that affect the heart, like Williams Syndrome, Alagille Syndrome and Noonan Syndrome
- if a child is born with only one large vessel that leads out of the heart, causing the oxygen-rich blood coming from the lungs and the de-oxygenated blood going back to the heart to become mixed; this rare condition is called truncus arteriosus
- if a child is born without a pulmonary valve; this condition is called pulmonary atresia
- if the pulmonary valve is narrowed; this is called pulmonary (valve) stenosis (PVS, PS)
- if the opening between the aorta and the pulmonary artery does not close soon enough after birth; this rare condition is called patent ductus arteriosus
- as a complication of certain types of infection, like congenital rubella syndrome, that can weaken the heart
Learn more about how Children’s treats congenital heart defects.
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Signs and symptoms
What are the symptoms of peripheral pulmonary stenosis?
Many children in the mild to moderate stages of peripheral pulmonary stenosis show no visible symptoms. Usually, the only identifiable symptom in these cases is a pronounced heart murmur.
As peripheral pulmonary stenosis progresses over time, children may display one or more of the following symptoms:
- shortness of breath
- heavy, difficult breathing
- rapid, shallow breathing
- rapid or fluttering heartbeat
- swelling in the feet and ankles
- swelling in the face
- swelling in the abdomen
You should seek treatment from a qualified medical professional right away if you notice any of these warning signs in your child.
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Q: Will my child be OK?
A: Peripheral pulmonary stenosis can vary widely in severity and symptoms from child to child. The condition is sometimes progressive, meaning that symptoms can become more pronounced over time.
A child’s long-term health depends greatly on her individual circumstances, especially:
- whether she has other heart problems, such as Tetralogy of Fallot
- the extent of narrowing within the pulmonary branches
- her tolerance for specific medications or procedures
- your family’s preferences for treatment
Children with mild or moderate peripheral pulmonary stenosis may not need any intervention, since they have no noticeable symptoms that disrupt their daily lives. These children can do well for long periods of time with regular monitoring and checkups by their treatment team.
More serious cases of peripheral pulmonary stenosis will need interventional catheterization. However, the good news is that the majority of children who undergo these procedures do very well and can go on to lead healthy adult lives.
Q: Will my child be at greater risk for contracting a bacterial infection?
A: A child with peripheral pulmonary stenosis may need to periodically take antibiotics in order to prevent an infection called bacterial endocarditis. Bacterial endocarditis can cause serious damage to the inner lining of the heart and its valves.
Children with heart conditions may be vulnerable to developing endocarditis while undergoing medical procedures that could release bacteria into the bloodstream. In these cases, a regimen of antibiotics might be prescribed before the child has the procedure. You should always let medical personnel know about your child’s peripheral pulmonary stenosis before making arrangements for a medical procedure, even if the procedure seems minor or unrelated to your child’s cardiac care.
If your child has peripheral pulmonary stenosis, but no other cardiac problems, he probably will not need antibiotics before a routine dental procedure (for example, a teeth-cleaning). Read the American Heart Association’s latest guidelines for dental patients with heart conditions (Adobe Acrobat required).
Q: Does my child have to cut back on physical activities?
A: Whether your child needs to cut back significantly on activities—or cut back at all—depends greatly on whether he has other heart problems, how extensive the narrowing within the pulmonary branches has become and what symptoms (if any) he is experiencing. You should talk to your treating clinician about recommended exercise restrictions or practices for your child.
Q: Is my child at serious risk of having a heart attack?
A: One of the most common misconceptions about peripheral pulmonary stenosis is that children with the condition are automatically at high risk of suffering a heart attack. However, thanks to available diagnostic procedures and imaging technology, peripheral pulmonary stenosis can be detected even when the only noticeable symptom is a heart murmur.
This ability to make an early diagnosis gives clinicians the opportunity to start monitoring kids with peripheral pulmonary stenosis right away, and to easily “stay on top of” the progression of the condition with regular echocardiograms and exams.
Q: What do I need to look out for once my child has been diagnosed with peripheral pulmonary stenosis?
A: Parents of children with peripheral pulmonary stenosis should always be watchful for changes in behavior, appearance, activity level and respiration.
You should seek medical help immediately if your child experiences:
- shortness of breath
- rapid heartbeat
- clammy skin or the feeling of a “cold sweat”
- difficulty “catching a breath”
- heart palpitations
- pale, ashen color in the face
- rapid breathing
- shallow breathing
- swollen feet or legs
- swollen eyelids
- swelling in other parts of the face
- swelling in the abdomen
Q: Can Children’s treat peripheral pulmonary stenosis while a baby is still in the womb?
A: Yes. Children’s Fetal Cardiology Program provides the most advanced, comprehensive care for expectant parents whose babies are diagnosed with heart defects in utero. Among the services we offer are:
- diagnosis, or confirmation of an earlier diagnosis, of heart defects using fetal ultrasound, echocardiography or magnetic resonance imaging
- working with the family to devise customized treatment plans
- fetal interventions such as balloon dilation
Learn more about the Fetal Cardiology Program.
Q: Does Children’s offer genetic testing for peripheral pulmonary stenosis?
A: Yes. Children’s Cardiovascular Genetics Program provides several clinical services to test for heart diseases like peripheral pulmonary stenosis and associated heart problems, such as Tetralogy of Fallot. It also offers genetic counseling. Learn more about the program.
Q: Is there a cure for peripheral pulmonary stenosis?
A: For some (though not all) children with peripheral pulmonary stenosis, the condition is progressive—meaning that the degree of narrowing within the branches can continue to increase over time, and more involved treatments—usually the use of interventional catheterization—will eventually become necessary. The good news is that interventional catheterization techniques have an excellent success rate for restoring heart and lung function and blood flow.
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Questions to ask your doctor
You and your family play an essential role in your child’s treatment for peripheral pulmonary stenosis. It’s important that you share your observations and ideas with your child’s treating physician, and that you have all the information you need to fully understand the treatment team’s explanations and recommendations.
You’ve probably thought of many questions to ask about your child’s peripheral pulmonary stenosis. It’s often very helpful to jot down your thoughts and questions ahead of time and bring them with you, along with a notebook, to your child’s appointment. That way, you’ll have all of your questions in front of you when you meet with your child’s treating clinician and can make notes to take home with you. (If your child is old enough, you can encourage him or her to write down questions, too.)
Some questions to ask your doctor might include:
- How did you arrive at this diagnosis?
- Are there any other conditions my child might have instead?
- Is the peripheral pulmonary stenosis caused by another heart condition?
- Is my child's peripheral pulmonary stenosis linked to a genetic syndrome?
- Does my child require further testing?
- How extensive is the narrowing within the pulmonary branches?
- Is my child’s condition likely to be progressive, with symptoms worsening over time?
- What is the long-term outlook for my child?
- What medications will you prescribe and what are the possible side effects?
- Is interventional catheterization necessary at this time?
- How should I talk to my child about this condition and her long-term health?
- Do I need to restrict my child’s physical activity?
- Does my child need to take antibiotics to protect against a bacterial infection?
- Do I need to make any other changes to my child’s home and school routines?
- What other resources can you point me to for more information?
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