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 categorized 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

Right ventricular dysfunction and right atrial enlargement may occur due to the chronic pressure overload on the right-sided circulation. Specific features depend on the etiology, which affects the level at which the obstruction to right ventricular outflow occurs:

  • valvular pulmonic stenosis
    • most common cause level of obstruction
    • thickened and/or structurally abnormal valve leaflets
    • leaflets often demonstrate systolic doming
  • subvalvular pulmonic stenosis
    • double-chambered right ventricle (DCRV) 8
      • right ventricle functional subdivision into a chamber upstream to and downstream of the stenosis
      • best demonstrated in modified apical five chamber or parasternal short axis view
      • color flow Doppler shows aliased flow within the RV
      • muscular band extending from the interventricular septum to the RV free wall
      • associated commonly with a VSD (especially membranous)
    • infundibular pulmonic stenosis
      • protrusion of the infundibular septum into the right ventricular outflow tract
      • often associated with high amplitude "fluttering" of the pulmonary valve leaflets
  • supravalvular pulmonic stenosis
    • parasternal short axis at the level of the aortic valve with a clockwise tilt should be utilized to visualize the main PA
    • luminal narrowing and/or intraluminal echogenic membrane above the pulmonary valvular annulus

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

Direct visualization 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 categorized, 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

  • 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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