Abstract

GONZALEZ, Madelón  and  DIBARBOURE, Luis. Heterotaxy syndrome: tomographic findings. Rev. argent. radiol. [online]. 2022, vol.86, n.3, pp.179-189.  Epub Oct 05, 2022. ISSN 1852-9992.  http://dx.doi.org/10.24875/rar.m22000031.

The objective of this brief communication is to characterize the relevant tomographic aspects in the heterotaxy syndrome, by means of 4 patients that exemplify the most frequent findings in this pathology. Situs solitus is the usual arrangement of organs and blood vessels and situs inversus refers to the mirror image of situs solitus. When the arrangement of the organs is indeterminate and unpredictable and does not correspond to situs solitus or the situs inversus, we are facing the situs ambiguus or heterotaxy syndrome, abnormal spectrum of anomalies in the relations of the thoracoabdominal organs. It may be accompanied by right isomerism or left isomerism. Attempts to classify it into two subgroups are usually difficult since none of these has unique and pathognomonic findings, but rather there is broad overlap. Both are of poor prognosis, in the cases of left isomerism 5-10% reach adulthood, with a worse prognosis being the cases of right isomerism due to the fact that they have immunodepression secondary to asplenia and more severe congenital cardiopathies. Each case should be analyzed in an individualized and detailed manner to establish the diagnosis, determine the lesional association and establish those patients that present a higher risk of complications.

Keywords : Heterotaxy syndrome; Asplenia syndrome; Polysplenia syndrome; Isomerism; Situs ambiguous.

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