The term heterotaxia is the name given to a series of abnormalities related to the internal arrangement of body organs, which would be located in a different position than usual; for example, the liver would be on the left side, etc. This term was created by Geoffroy Saint-Hilaire. This causes, for example the Situs inversus, or the Situs ambiguus terms that are used to describe structures reversed in mirror image of internal organs. There are cases of familial heterotaxia, in which several individuals in a family have the abnormality, but there are also isolated cases. One of these abnormalities is caused by a gene on the X chromosome, called Zic3. Even so the factors involved in these malformations are unknown or not fully understood.
In individuals with dextrocardia, which is one of the most common forms (even if it is also a very rare disease) of heterotaxia, the process of heart formation does not occur properly causing a laterality disorder. This usually results in complex heart disease because the left or right chambers are often misaligned with normal blood flow (arteries and veins).
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