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Ivemark syndrome

Background

Ivemark syndrome: Isomerism of the Right Atrial Appendages, Heterotaxy syndrome, Asplenia syndrome

Ivemark syndrome is now largely a historical term for the association of congenital asplenia (absence of a spleen) with malformations of the cardiovascular system (which transports blood through the body via the hearts, lungs and veins) and abnormal arrangement of the chest and abdominal (thoraco-abdominal) organs.

Ivemark Syndrome is part of a group of disorders known as heterotaxy syndromes which includes both right (Ivemark) and left isomerism. In these conditions there is a failure of the normal lateralisation of internal organs (i.e. left and right sided) resulting in mirror imaging of certain structures on both sides of the body.

What are the symptoms? View What are the symptoms?

Medical text written February 2008 by Dr Jonathan Forsey, Specialist Registrar Paediatric Cardiology and Dr Robin Martin, Consultant Paediatric Cardiologist, Bristol Royal Hospital for Children, Bristol, UK

 

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