Double-orifice mitral valve associated with atrioventricular canal defects

Abstract

A 4 year-old male presented with effort dyspnea, and was diagnosed as atrioventricular canal defects. This finding was confirmed by open heart surgery, and a congenital double orifice mitral valve was discovered. The septal defect was closed but the double orifice mitral valve was respected because of the absence of hemodynamic disturbance. We report this case with review of literature.

Double orifice mitral valve: rare disease with a wide range of presentation

“…The pathophysiology of DOMV is complex, and it may involve a combination of hemodynamic abnormalities, such as regurgitation, stenosis, and turbulence, as well as structural abnormalities, such as prolapse, redundancy, and fibrosis, of the valve leaflets and the chordae tendineae. It is commonly associated with other congenital anomalies, most commonly atrioventricular septal defects, patent ductus arteriosus or coarctation of aorta ( 3 , 4 ). Thus, a thorough evaluation is needed if DOMV is detected.…”