Straddling mitral and tricuspid valves: morphologic differences and developmental backgrounds

The anatomy of 25 hearts with a straddling mitral or tricuspid valve, or both, is described. Malalignment of atrial and ventricular septa is an essential feature of a straddling tricuspid valve, creating an inlet septal defect. Across this defect, the tricuspid valve straddles into the opposite (left ventricular) chamber, where it is separated from the mitral valve by a posterior muscular ridge, the posteromedial muscle. A straddling mitral valve requires an infundibular septal defect, predominantly of the malalignment type, in which the anterior part of the ventricular septum deviates to the left of the infundibular septum. The mitral valve straddles into the opposite (right ventricular) chamber, anterior to the trabecula septomarginalis. From normal developmental stages, it is concluded that valve formation takes place only after completion of ventricular septation. Any malformation of the valves is therefore considered to be superimposed on a primary malformation of the septum. The ventricular septum itself develops from three different components. Malseptation in the inlet portion of the embryonic heart lead to the characteristic septal malformation seen in straddling tricuspid valve. Malseptation in the outlet portion may lead to the septal malformation that characterizes straddling mitral valve.