Ivemark Syndrome: Syndrome of Right Isomerism, a Case Report and Review of Literature

Abstract

Right isomerism (Ivemark syndrome) is a rare disorder associated with multiple congenital malformations. It is the result of embryological anatomical disturbances, characterized by absence of spleen, malformations of the heart and abnormal arrangement of the internal organs of the chest and abdomen. This case report describes a rare occurrence in a newborn, presented at 4 hours of age with bluish discoloration of the extremities and low oxygen saturation and diagnosed with complex cyanotic congenital heart defect with situs ambiguous, which was undetected in the antenatal period.