ICD-11 definition : A congenital malformation in which the internal thoraco-abdominal organs demonstrate
abnormal arrangement across the left-right axis of the body. By convention, heterotaxy
syndrome does not include patients with complete mirror-imaged arrangement of the
internal organs along the left-right axis also known as situs inversus totalis.;
ICD-11 synonym : Heterotaxy syndrome; Splenic syndromes; Abnormal arrangement of thoraco-abdominal organs; Heterotaxy; Heterotaxia syndrome; Situs ambiguous; Heterotaxia;
A congenital malformation in which the internal thoraco-abdominal organs demonstrate
abnormal arrangement across the left-right axis of the body. By convention, heterotaxy
syndrome does not include patients with complete mirror-imaged arrangement of the
internal organs along the left-right axis also known as situs inversus totalis.