Left ventricular structures in atrioventricular septal defect associated with isomerism of atrial appendages compared with similar features with usual atrial arrangement.

In patients with isomeric atrial appendages, regurgitation of atrioventricular valves is recognized clinically as one of the risk factors that militate against successful achievement of definitive repairs. To determine whether this reflected anatomic features, we investigated 91 specimens with atrioventricular septal defect that had a common atrioventricular valve and biventricular atrioventricular connections. Of these specimens, 35 had isomeric right appendages, 23 showed isomeric left appendages, and 33 had usual atrial arrangement. We measured either the size or location of the supporting papillary muscles and the circumference of the mural leaflet within the morphologically systemic ventricle, as well as the length of outlet, inlet, and so-called scooped dimensions of the muscular ventricular septum. Presence of a solitary papillary muscle, or deviation of the attachments of the papillary muscles, was more frequent in hearts with isomeric right appendages. Values for the diameter and lengths of the papillary muscles were significantly smaller in hearts with isomeric right appendages compared with those with usual atrial arrangement (p < 0.001), as were the distances between the papillary muscles (p < 0.002) and the circumference of the mural leaflet (p < 0.001). The proportional length of ventricular outlet was longer in the setting of isomeric right appendages than in the other groups (p < 0.001), whereas the extent of septal scooping showed no differences among these three groups. We conclude that these structural features could be factors in the known insufficiency of the common atrioventricular valve and the ventricular dysfunction in patients with isomeric right appendages.