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Pediatric Pulmonary Stenosis

What is Pediatric Pulmonary Stenosis?

Pediatric pulmonary stenosis is a heart condition that occurs when the pulmonary valve of the heart is narrowed or obstructed, reducing the ability of the heart to pump oxygen-rich blood from the lungs back into the heart. In a healthy heart, the pulmonary valve has three tiny flaps of skin (leaflets) to regulate blood flow like a one-way street. The valve controls the blood flow from the lungs through the pulmonary artery of the heart and out to the rest of the body. When the pulmonary valve is narrowed, the heart must work harder to pump the blood, causing the muscle to thicken and weaken over time.

Blood Flow in a Healthy Heart

blood flow of a healthy heart with pulmonary valve circled

What are the different types of Pediatric Pulmonary Stenosis?

Valvar pulmonary stenosis

The leaflets are thickened and/or narrowed.

Supravalvar pulmonary stenosis

The pulmonary artery just above the pulmonary valve is narrowed.

Subvalvar (infundibular) pulmonary stenosis

The muscle under the valve area is thickened.

Branch peripheral pulmonic stenosis

The right or left pulmonary artery is narrowed, or both may be narrowed.

What are the signs and symptoms of Pediatric Pulmonary Stenosis?

Symptoms in infants (birth to 1 year*)

  • Weak pulse
  • Rapid breathing
  • Decreased feeding
  • Sweating
  • Lethargy (exhaustion)
  • Irritability
  • In extreme cases, congestive heart failure

Symptoms in children (birth to 19-years old**)

  • Rapid heart rate
  • Shortness of breath
  • Heavy or rapid breathing
  • Fatigue (tiredness)
  • Swelling in the feet, ankles, abdomen or face

*Age limit of infants defined by the World Health Organization (WHO).
**Age limit of pediatric/child age group defined by the WHO.

What are the causes of Pediatric Pulmonary Stenosis?

In most cases, the cause of the narrowing of the pulmonary valve is unknown, but it is often congenital (present at birth). The level of blockage can vary and become worse over time.