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What is peripheral pulmonary stenosis?

Peripheral pulmonary stenosis is a narrowing in one or more of the branches of the pulmonary arteries. These are the arteries that carry blood from the heart to the lungs.

Peripheral pulmonary stenosis is typically a congenital heart defect — a condition a child is born with. Sometimes peripheral pulmonary stenosis is a complication of a heart defect or genetic syndrome.

Peripheral pulmonary stenosis can cause a lack of oxygen the blood. This can put increased pressure on the heart, making the heart work harder. In some cases, peripheral pulmonary stenosis may get worse over time.

Peripheral Pulmonary Stenosis | Symptoms & Causes

What are the symptoms of peripheral pulmonary stenosis?

Many children in the mild to moderate stages of peripheral pulmonary stenosis have no visible symptoms. Usually, the only symptom is a heart murmur.

As peripheral pulmonary stenosis progresses over time, children may have one or more of the following symptoms:

  • fatigue
  • shortness of breath
  • heavy, difficult breathing
  • rapid, shallow breathing
  • rapid or fluttering heartbeat
  • swelling in the feet, ankles, face, or abdomen

What are the causes of peripheral pulmonary stenosis?

Pulmonary stenosis can occur as a complication of other heart conditions, such as tetralogy of Fallot, or in conjunction with certain genetic syndromes that affect the heart, like Williams syndrome, Alagille syndrome, and Noonan syndrome.

Peripheral Pulmonary Stenosis | Diagnosis & Treatments

How is peripheral pulmonary stenosis diagnosed?

In many cases, a clinician may suspect peripheral pulmonary stenosis after hearing a heart murmur during a routine physical examination.

One or more of the following tests may help diagnose peripheral pulmonary stenosis:

What are the treatment options for peripheral pulmonary stenosis?

Peripheral pulmonary stenosis can affect each child differently. Your child’s clinician is the best resource for providing detailed information about your child’s individual situation and treatment options.

Children with mild to moderate peripheral pulmonary stenosis may not require any treatment other than routine monitoring.

Some children with peripheral pulmonary stenosis may need to take medication to:

  • help the heart maintain healthy function and blood flow
  • control blood pressure
  • prevent abnormal heart rhythms, called arrhythmias

A child with peripheral pulmonary stenosis may also need to take antibiotics periodically to prevent an infection in the heart called bacterial endocarditis.

Children with more severe peripheral pulmonary stenosis may need interventional catheterization. This procedure uses a thin tube called a catheter to open narrowed passageways in the pulmonary branches. Some children may need several interventions over time, as they grow and age.

How we care for peripheral pulmonary stenosis

The Boston Children's Hospital Benderson Family Heart Center team has extensive experience treating children, adolescents, and adults with peripheral pulmonary stenosis.

Our specialized training in pediatric cardiology means that we understand the particular challenges, circumstances, and intricacies of working with young people with heart problems.

Peripheral Pulmonary Stenosis | Programs & Services