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Double orifice mitral valve (DOMV) is a rare congenital anomaly of the subvalvar mitral valve apparatus (chordae tendinae and papillary muscles) consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices. The size of the orifices is balanced in only 15%. Anatomically and functionally, the mitral leaflets are essentially normal in most cases, but they can be regurgitant or stenotic. DOMV rarely occurs as an isolated anomaly, but is most commonly associated with a variety of other cardiac anomalies such as atrioventricular septal defects, coarctation of the aorta, bicuspid aortic valve, sinus venosus atrial septal defect, ventricular septal defect, patent ductus arteriosus, hypoplastic left heart syndrome, double orifice tricuspid valve, tetralogy of Fallot, and Ebstein's anomaly. Acquired DOMV may be present after surgical correction of a mitral valve prolapse where the free edge of the middle portion of the prolapsing anterior or posterior leaflet is anchored to the facing edge of the opposite leaflet (“edge to edge” or “double orifice repair” technique described by Alfieri).
We describe the echocardiographic finding of a 19 year old woman with “multiple left heart obstructions” (Shone's complex) and surgically corrected ventricular septal defect and coarctation of the aorta, who was admitted for treatment of recoarctation. Echocardiography at admission surprisingly revealed a previously undiagnosed balanced type DOMV without significant obstruction or insufficiency. The figure shows a short view of a diastolic frame at the level of the mitral leaflet tips (asterisks indicate DOMV).
This case shows that DOMV may also exist with Shone's complex.
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