Abstract
Ivemark syndrome belongs to a group of heterotaxes syndromes in which the right-left differentiation of organs in the thoracic and abdominal cavities is erroneously formed during embryonic development. There is no mirror deposition of the anatomically normal organs at the site inversus.
Heterotaxis syndromes are distinguished by right-sided and left-sided isomerism according to the cardiac structure. Atrial isomerism is always present when the heart has two right or left atria, with associated, different, heart defects.
Findings on other organs are related to right-sided or left-sided isomerism.