Congenital pulmonary stenosis
A congenital pulmonary stenosis (CPS) broadly refers to a congenital narrowing along any segment of the pulmonary arterial system.
The estimated incidence is at ~ 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
- sub valvular - infundibular
Supra-valvular stenoses have been traditionally classified in to four types.
Generally occurs as an isolated feature and associations are rare 3. They include:
- Noonan syndrome
- Williams syndrome (supravalvular)
- tetralogy of Fallot
- in utero rubella exposure
- Down syndrome
- Ehlers Danlos syndrome
- 22q deletion syndrome
Non 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.
CT / CTA
Direct visualisation of stenotic segment +/- post stenotic dilatation of distal arterial segments.
MRI / MRA
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
Doppler ultrasound / echocardiography
May show a high flow jet with turbulent flow through the pulmonary valve or narrowed segment.
- 1. Gupta H, Mayo-smith WW, Mainiero MB et-al. Helical CT of pulmonary vascular abnormalities. AJR Am J Roentgenol. 2002;178 (2): 487-92. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Didier D, Ratib O, Lerch R et-al. Detection and quantification of valvular heart disease with dynamic cardiac MR imaging. Radiographics. 20 (5): 1279-99. Radiographics (full text) - Pubmed citation
- 3. Ryan R, Abbara S, Colen RR et-al. Cardiac valve disease: spectrum of findings on cardiac 64-MDCT. AJR Am J Roentgenol. 2008;190 (5): W294-303. doi:10.2214/AJR.07.2936 - Pubmed citation
- 4. Shah R, Cestone P, Mueller C. Case 2. Congenital multiple peripheral pulmonary artery stenosis (Pulmonary branch stenosis or supravalvular pulmonary stenosis). AJR Am J Roentgenol. 2000;175 (3): 854. - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Pulmonary stenosis : congenital||✗|
|Congenital pulmonary arterial stenosis||✓|
|Congenital pulmonary stenosis (CPS)||✗|