Absent right atrioventricular connexion with a straddling left atrioventricular valve connecting the left atrium to a ventricular mass with a dominant left ventricle.

We present clinical and postmortem examination of a child who presented in life with severe obstruction to the left ventricular outflow tract. The heart was right-sided with usual atrial arrangement. The right atrioventricular connexion was absent. There was straddling of the left atrioventricular valve which had a double orifice. Each orifice was connected to a well-differentiated ventricle, the left ventricle being dominant and right-sided (left-hand topology). An inlet ventricular septal defect was restricted by the leaflet tissue of the straddling valve.