Heterotaxy syndromes

A spectrum of disorders where the normal left-right arrangement of organs in the body is disturbed (“errors of lateralisation”).

Situs inversus is complete mirror image arrangement – there are no health consequences as a result (other than iatrogenic eg delayed diagnosis of appendicitis).

Many genetic causes. Primary cilial dyskinesia (Kartagener syndrome) is one.

Heart – IVC can be interrupted, requiring azygos veins to drain lower body vessels back to heart. Many variations of valves and connections seen. Congenital heart block often seen.

Gut – malrotation, biliary atresia

Asplenia or polysplenia.

Horseshoe or dysplastic kidneys.