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Unicuspid aortic valve

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Transoesophageal echocardiography showed a unicuspid unicommissural valve in a 16 year old boy with severe aortic stenosis. In systole, a posteriorly situated eccentric orifice extending to the annulus was seen (O, eccentric orifice; LA, left atrium, AO, aorta). In diastole there was one lateral commissural attachment to the aorta posteriorly. The single commissure showed fibrosis and thickening. An aortic valve replacement was done.

The aortic valve develops from three tubercles that are converted into thin valve cusps and sinuses of Valsalva by a process of excavation. A unicuspid aortic valve is formed by fusion of all the three cusps. It may have a central opening (no true commissure) or an eccentric orifice (unicommissural) as in our patient. The free edge of the single leaflet originates from the single attachment. It then proceeds across the orifice without additional contact with the aorta. Then it bends on itself and returns to the point of origin. It is usually stenotic at birth.