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Number 10-03: Unicuspid aortic valve and associated abnormalities

Case from: Marc Dweck, John Payne, David Northridge, Claire Malone, Fergus Perks, and Graeme McKillop
Edinburgh Heart Centre, Edinburgh, UK

Clinical history: A 24 year-old male underwent a subclavian flap repair for aortic coarctation at the age of 1. A recent echocardiogram had been reported as showing mild aortic regurgitation and a dilated aortic root. A cardiac MRI scan was requested primarily to image the thoracic aorta.


Movie 1 - right click mouse to enlarge
Movie 2
Movie 3

Cine CMR: The SSFP images showed that he had a unicuspid aortic valve (Movie 1 - right click mouse to enlarge). The aortic valve was opening well with only mild aortic regurgitation (regurgitant fraction 12% by flow velocity mapping). There was also severe aortic root dilatation with a maximum diameter of 51mm across the sinuses of Valsalva, and 50 mm at the level of the sinotubular junction, as well as at the level of the right pulmonary artery (Movies 2 and 3).

Movie 4
Movie 5

The repair of his coarctation was satisfactory (Movie 4). His left sub-clavian artery was absent in keeping with his flap repair (Movie 5).

The association of unicuspid valves with both coarctation and aortic root dilatation has been described before1,2. Its presence in this case raises several interesting clinical questions regarding potential surgical procedures. Should a lower threshold for aortic root replacement be considered, as is appropriate with bicuspid valves3? Should the unicuspid valve be replaced at the time of root surgery even though it is functioning well? 

Perspective: This case illustrates that CMR provides not only clear views of the structure of the aortic valve, but in addition, it also allows comprehensive assessment of valvular function and aortic structure.

1) Debl K, Djavidani B et al Unicuspid aortic valve disease: a magnetic resonance imaging study. Rofo. 2008 Nov;180(11):983-7.

2) Dursun M, Yilmaz S et al. Combination of unicuspid aortic valve, aortic coarctation and aberrant right subclavian artery in a child: MR imaging and CTA findings. Cardiovasc Intervent Radiol 2007;30:547-9.

3) Bonow et al. AACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease. J Am Coll Cardiol. 2006 Aug 1;48(3):e1-148.

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