Isolated double-orifice mitral valve: an extremely rare and interesting anomaly

Incidental finding of an isolated double-orifice mitral valve in an asymptomatic man

“…This anomaly is characterized by the presence of a single mitral annulus with two orifices, each having independent subvalvular apparatus and papillary muscles. [134] DOMV is an extremely rare condition, usually associated with other congenital heart anomalies (above all, atrioventricular septal defect, coarctation of aorta, interrupted aortic arch, patent ductus arteriosus, and ventricular septal defect). [1] It generally presents with symptoms related to mitral regurgitation or stenosis, but it can also present as an incidental finding.…”

“…[134] DOMV is an extremely rare condition, usually associated with other congenital heart anomalies (above all, atrioventricular septal defect, coarctation of aorta, interrupted aortic arch, patent ductus arteriosus, and ventricular septal defect). [1] It generally presents with symptoms related to mitral regurgitation or stenosis, but it can also present as an incidental finding. [15] Three specific types of DOMV can be described at two-dimensional echocardiography: (1) Complete bridge type (about 15% of DOMVs), in which both openings are visible from the leaflet edge and both orifices appear circular (equal or unequal in size) with normal subvalvular apparatus and papillary muscles; (2) incomplete bridge type, in which the connection is seen only at the leaflet edge; and (3) hole type (the most frequent) characterized by a small accessory orifice situated at either the posteromedial or anterolateral commissure and identifiable only at the mid-leaflet level.…”

“…[1] Interestingly, this cardiac abnormality rarely presents as an isolated finding, preferentially associated with valvular regurgitation rather than stenosis. [1] The etiology of DOMV has been recognized in the abnormal fusion of the endocardial cushions and abnormal development of the mitral valve during the delamination process.…”

“…[1] Interestingly, this cardiac abnormality rarely presents as an isolated finding, preferentially associated with valvular regurgitation rather than stenosis. [1] The etiology of DOMV has been recognized in the abnormal fusion of the endocardial cushions and abnormal development of the mitral valve during the delamination process. [2] Transthoracic echocardiography is the modality of choice for the characterization of this anomaly; in this respect, the parasternal short-axis view allows a full morphologic characterization of the anomaly and should always be performed in the case of a meaningful clinical suspect.…”

“…[2] Transthoracic echocardiography is the modality of choice for the characterization of this anomaly; in this respect, the parasternal short-axis view allows a full morphologic characterization of the anomaly and should always be performed in the case of a meaningful clinical suspect. [1]…”