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
- subvalvular: infundibular
Supravalvular stenoses have been traditionally classified into four types.
Generally, occurs as an isolated feature and associations are rare 3. They include:
- Noonan syndrome
- Williams syndrome (supra valvular)
- tetralogy of Fallot
- in utero rubella exposure
- Down syndrome
- Ehlers-Danlos syndrome
- 22q deletion syndrome
- single ventricle
- Alagille syndrome
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.
- 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
- 5. Castaneda-Zuniga W, Formanek A, Amplatz K. Radiologic diagnosis of different types of pulmonary stenoses. Cardiovasc Radiol. 1978;1 (1): 45-57. Read relevant article. Accessed on 27/05/2016
- 6. Saremi F, Gera A, Ho SY et-al. CT and MR imaging of the pulmonary valve. Radiographics. 2014;34 (1): 51-71. doi:10.1148/rg.341135026 - Pubmed citation
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.
congenital heart disease
- normal relationship between chambers and valves
- atrioventricular valves
- outflow tract
- great vessels
- venous inflow
- anomalous valves
- abnormal relationship of chambers and valves
- atrioventricular abnormality
- great vessel connection abnormality
- conotruncal cardiac anomalies
- pentalogy of Cantrell
- Shone syndrome
- congenital heart disease - chest x-ray approach
surgical repairs (mnemonic)
- arterial switch procedure
- Blalock-Taussig shunt
- double switch procedure
- Fontan procedure
- Glenn procedure
- Mustard repair
- Norwood procedure
- Pott shunt
- pulmonary artery banding
- Rastelli procedure
- Sano shunt
- Senning repair
- total repair of tetralogy of Fallot (TOF)
- unifocalisation procedure
- Waterston shunt