Apical window demonstrating congenital mitral stenosis and a supravalvar mitral ring (SVMR). Note the prominent papillary muscle that extends further toward the annulus than in a normal apparatus, the accompanying shortened chordae and hypoplasia of the annulus. The membranous SVMR is seen as a thin projection from the atrial side of the leaflet, extending from the annulus into the supravalvar flow orifice. Color Doppler in the same imaging plane demonstrating flow acceleration beginning just prior to the annulus – a characteristic finding in SVMR, which if present should prompt the imager to undertake an extensive search for an unrecognized SVMR. Note the additional egress from the inflow directed medially via an abnormal intrachordal space. This type of complex inflow orifice makes accurate measurement of the flow orifice area particularly challenging (see text).

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