Anomalous mitral arcade variant with accessory mitral leaflet and chordae presenting for the first time with acute decompensated heart failure in an adult.

The anomalous mitral arcade is a rare congenital malformation of the mitral valve and its tensor apparatus. It is characterized by enlarged papillary muscles connected to each other and to the free edge of the anterior mitral leaflet by a bridge of fibrous tissue. We report a rare variant of anomalous mitral arcade that was associated with accessory mitral leaflet in subaortic area and accessory chordae. Our patient was asymptomatic till the age of 18 years, when he presented for the first time in acute decompensated heart failure secondary to severe mitral regurgitation and left ventricular dysfunction. The patient had rapid deterioration with fatal outcome.