right atrial isomerism

A visceral heterotaxy that is characterized by complete atrioventricular septal defect with a common atrium and univentricular AV connection, total anomalous pulmonary drainage, and transposition or malposition of the great arteries and that has_material_basis

A visceral heterotaxy that is characterized by complete atrioventricular septal defect with a common atrium and univentricular AV connection, total anomalous pulmonary drainage, and transposition or malposition of the great arteries and that has_material_basis_in homozygous mutation in the GDF1 gene on chromosome 19p12.

Signs and symptoms

• Common atrium
• Total anomalous pulmonary venous return
• Asplenia
• Transposition of the great arteries
• Complete atrioventricular canal defect
• Abnormal lung lobation
• Single ventricle
• Agenesis of corpus callosum
• Abdominal situs ambiguus
• Ventricular septal defect

Also known as: Ivemark syndrome; asplenia with cardiovascular anomalies