Complex Cyanotic Congenital Heart Disease: The Heterotaxy Syndromes
The hallmark feature of heterotaxy is abnormal positioning of internal organs, including liver, spleen, intestines, venae cavae, atria, ventricles, and great arteries. Clinical presentation varies significantly as cardiac anatomy ranges from normal to severely anomalous. Bilateral sidedness in heterotaxy indicates duplication of the right or left side of the body, thus loosing to a certain extent the normal asymmetry of the viscera. In bilateral right sidedness there is asplenia, while in bilateral left sidedness there is polysplenia. In either case, functional asplenia is common. Cyanosis is a frequent feature in most cases of heterotaxy.