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Unicuspid Aortic Valve with Mixed Stenosis and Regurgitation

Imaging findings

In a 13-year-old with a known aortic valve problem, cardiac MRI shows a thickened aortic valve with only a single discernible cusp and no separation into normal leaflets, with turbulent antegrade and regurgitant flow on 4D flow imaging; the regurgitant jet reaches a peak velocity of approximately 120 cm/s. There is no significant left ventricular hypertrophy to suggest long-standing severe stenosis.

Key takeaways

A thickened, single-cusp aortic valve with turbulent flow and significant regurgitation but without left ventricular hypertrophy indicates a unicuspid aortic valve with a predominantly regurgitant rather than stenotic physiology, and 4D flow MRI is a useful non-invasive tool for grading the degree and velocity of regurgitation in these congenitally abnormal valves.

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