Congenital pulmonary stenosis

Congenital pulmonary stenosis (CPS) refers to congenital narrowing of the right ventricular outflow tract, pulmonary valve or pulmonary artery.

See pulmonary valve stenosis for a general discussion about this valvulopathy.

The estimated incidence is 1 in 2000 births.

Can be morphologically categorised depending on the relationship to the pulmonary valve 3:

  • supra valvular: distal to the valve: commonest ~60% 4
  • valvular  
  • subvalvular: infundibular

Supravalvular stenoses have been traditionally classified into four types.  

Generally, occurs as an isolated feature and associations are rare 3. They include:

Findings on chest radiographs are not specific. Can have a normal heart size or may show evidence of right ventricular hypertrophy. May also show evidence of a dilated pulmonary trunk or a main pulmonary artery. Pulmonary vascularity is often normal in mild cases. In some cases of pulmonary valvular stenosis, the flow of blood through the stenotic valve preferentially enter the left pulmonary artery which may result to an enlarged left pulmonary artery and slightly increased left lung vascularity, known as Chen sign

May show a high flow jet with turbulent flow through the pulmonary valve or narrowed segment.

Direct visualisation of stenotic segment +/- post stenotic dilatation of distal arterial segments.

Direct visualisation of stenotic segment +/- associated features. Velocity encoded phase contrast (VEC) cine sequences can assist assessing the severity of the stenosis by allowing measurement of blood flow velocities and volumes 2.

Congenital heart disease

There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.

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Article information

rID: 7438
Synonyms or Alternate Spellings:
  • Pulmonary stenosis : congenital
  • Congenital pulmonary arterial stenosis
  • Congenital pulmonary stenosis (CPS)

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Cases and figures

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    Case 1
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    Pulmonary valve s...
    Case 2
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    Case 3
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    Case 4: subvalvular
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    Infundibular sten...
    Case 5: subvalvular
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    Case 6: subvalvular
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