Heterotaxy- visceral- X-linked


Heterotaxy ('heter' meaning 'other' and 'taxy' meaning 'arrangement'), or situs ambiguus, is a developmental condition characterized by randomization of the placement of visceral organs, including the heart, lungs, liver, spleen, and stomach. The organs are oriented randomly with respect to the left-right axis and with respect to one another (Srivastava, 1997). 30 PubMed Neighbors Heterotaxy is a clinically and genetically heterogeneous disorder. See also HTX2 (605376), caused by mutation in the CFC1 gene (605194) on chromosome 21; HTX3 (606325), which maps to 6q21; and HTX4, caused by mutation in the ACVR2B gene (602730).