Ivemark Syndrome BT - Atlas of Genetic Diagnosis and Counseling

Abstract

Ivemark syndrome consists of visceroatrial heterotaxia associated with asplenia (Ivemark 1955). It is also known as asplenia syndrome, heterotaxy syndrome, cardiosplenic syndrome, right atrial isomerism (two morphologically right atrial appendages), or bilateral right-sidedness. The incidence of the syndrome is estimated to be 1/10,000–1/40,000 live births (Rose et al. 1975; Noack et al. 2002). Ivemark syndrome accounts for 1–3 % of all congenital heart defects (Cesko et al. 1997).