A 57-year-old man presented with exertional dyspnea. Cardiac examination was normal. Transthoracic echocardiogram showed an asymmetrical septal hypertrophy with maximum thickness of 22 mm at the basal septum (Figure 1). There was no significant left ventricular outflow tract obstruction at rest. Parasternal short axis (PSAX) view at the level of the mitral valve (MV) leaflets demonstrated a double orifice mitral valve (DOMV), separated by an incomplete bridge with two asymmetrical orifices (Figure 2A, Movie 1). PSAX view at the level of the MV annulus showed a normal MV opening with an indentation on the anterior leaflet characterizing the “lovely heart” sign (Figure 2B).1) Apical four-chamber (A4-C) view demonstrated a separate subvalvular apparatus for each orifice (Figure 3). A4-Cview with color flow Doppler demonstrated two regurgitant jets through the two asymmetrical orifices of the DOMV amounting to mild mitral regurgitation (Figure 4). There was no significant gradient across the MV.
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