The trabecula septomarginalis (Leonardo’s cord) in abnormal ventriculo-arterial connections: anatomic and morphogenetic implications

BACKGROUND: The abnormal ventriculo-arterial connections in atrio-ventricular concordance and situs solitus with two well developed ventricles include the range from tetralogy of Fallot throughout the different forms of double outlet right ventricle to transposition of great arteries. The infundibular septum and the trabecula septomarginalis are the fundamental anatomical landmarks for the segmental analysis. In these abnormalities there is a pathological progressive counter-clockwise rotation of the infundibular septum which divorces from the antero-superior limb of the trabecula septomarginalis and achieves his identity. Is there any anatomical evidence of a simultaneous abnormal counter-clockwise rotation of the trabecula septomarginalis? METHODS: Malposition of great arteries is a generic term since all relationships have to be expected. We present specimens with anatomical evidence of a progressive counter-clockwise rotation from 0° to about 180°of the plane passing throughout the trabecula septomarginalis’s limbs. RESULTS: We can observe sequentially: 1. Malformations in which the posterior limb of the trabecula septomarginalis is committed to the ventriculo infundibular fold: (tetralogy of Fallot, double outlet right ventricle with sub-aortic ventricular septal defect, truncus arteriosus and doubly committed ventricular septal defect); 2. Malformations in which the posterior limb of the trabecula septomarginalis is committed to the infundibular septum (double outlet right ventricle with sub-pulmonary ventricular septal defect, transposition of great arteries). CONCLUSIONS: 1. The sequential-segmental analysis identify all the morphologies. 2. The trabecula septomarginalis plane presents a progressive counter-clockwise twist on the long axis. 3. Since the trabeculated portions of the ventricles are the oldest developmental components, our observations support the hypothesis that the abnormal ventriculo-arterial connections could be in relation with a pathological myocardial process during early cardio-genesis. We are promoting new studies to investigate our anatomical observations.