Heterotaxy with malrotation is a rare. The details of understanding the anatomy and embryology are challanging, even for many physicians.  A simplified explanation is that heterotaxy is a partial situs inversus.  In situs inversus, the abdominal organs are in a complete mirror image placement within the abdomen.  Partial reversal is termed “heterotaxy or heterotaxia.”  Heterotaxy may be associated with malrotation. The decision when to evaluate a patient with heterotaxy for malrotation is discussed in the PowerPoint presentation below.

The following pdf gives details of the disorder for medical professionals and includes surgical photos.