Syndrome of right isomerism: Ivemark syndrome

Abstract

Heterotaxy Syndrome is a complex syndrome that occurs when the axes of the body fail to rotate correctly when developing in the uterus. This can result in many different organ systems being affected. Cardiac malformations are a major component of heterotaxy syndrome, resulting in significant morbidity and mortality. Abnormal cardiac development typically leads to atrial appendage isomerism, resulting in either bilateral paired right atria (right atrial isomerism or paired left atria (left atrial isomerism. Here we present a one and a half-month-old female child with right isomerism.

Keywords

• Asplenia
• Ivemark syndrome
• multiple cardiac defects