CTICasesAortic valve stenosis in Morquio syndrome
Cite this case
[...]. Aortic valve stenosis in Morquio syndrome. CTICases. Published November 16, 2018. https://cticases.com/c/z6s3u
Imaging findings
Gated cardiac CT demonstrates severe aortic valve stenosis and thick calcification of the aortic valve leaflets, along with mild diffuse airway thickening, in a young female patient with Morquio syndrome.
Key takeaways
Morquio syndrome (mucopolysaccharidosis type IV) is a lysosomal storage disease. Excess glycosaminoglycan deposition in cardiac tissues leads to progressive valvular thickening, calcification, and severe aortic stenosis, often at a young age.
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