The cardiac malformations: Souble inlet left ventricle and corrected transposition explained as deviations in the normal development of the interventricular septum

Abstract

To examine the hypothesis that malpositions of cardiac ventricles could be explained by altered development of the interventricular septum, we studied hearts from The Johns Hopkins Hospital autopsy files with double inlet left ventricle (16 cases) or corrected transposition (nine cases). In double inlet left ventricle both atrioventricular valves connect normally developed and positioned right and left atria to a posterior morphologic left ventricle. In hearts with corrected transposition the atria are normally positioned and the morphologic right ventricle is on the left and is continuous with the anteriorly positioned aorta. The morphologic left ventricle is on the right, connected to the posteriorly positioned pulmonary trunk. Normal ventricular septation may be understood as arising from the mechanics of a spiral fold in the primary heart tube produced by the left interventricular sulcus. The ventral limb of the spiral induces the muscular interventricular septum while the dorsal limb becomes a component of the crista supraventricularis. We propose that double inlet left ventricle and corrected transposition are the result of minor deviations in the position of the interventricular sulcus on the primary heart tube. Double inlet left ventricle may develop from the formation of a closed unspiraled ring around the interventricular canal. Corrected transposition may result from a left interventricular sulcus whose ventral limb gives rise to a left sided crista supraventricularis, which determines in part the right ventricular morphology of the left sided ventricle. The dorsal limb spirals toward the atrioventricular canal, gives rise to a malpositioned interventricular septum, and displaces the embryonic trabeculated right ventricle to the left. The concept presented accounts for the morphologic findings characteristic of double inlet left ventricle and corrected transposition.