Heterotaxy Syndrome Associated With Left and Right Isomerism – An Overview.

Heterotaxy syndrome is a condition in which the internal organs are abnormally arranged in the chest and abdomen. Heterotaxy syndrome is also known as Ivemark syndrome. The term situs ambiguous is an abnormality in which the components of situs solitus and situs inversus are seen in the same person. Heterotaxy may also results from genetic changes (mutations); and the main feature of heterotaxy syndrome is the isomerism which is a symmetrical development of normally asymmetrical organs or organ systems. Left and right isomerisms are the two subtypes of heterotaxy. Patients with left isomerism have less severe cardiac malformations than with right isomerism. The management of complex congenital heart disease in patients with heterotaxy syndrome has steadily improved. The survival of morbidity and mortality rate is decreased in heterotaxy population. The surgical outcome of heterotaxy patients in fontan circulation and biventricular repair techniques were recent advances. In this article, we review about the isomerism of heterotaxy and its improvement in the current era. [ABSTRACT FROM AUTHOR]