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Ivemark's syndrome

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

  • this syndrome is the association of ostium primum atrial septal defects (+/- pulmonary valve stenosis or atresia) and congenital asplenia

Ivemark syndrome, also referred to as asplenia syndrome

  • most common cause of congenital asplenia
  • visceral heterotaxy is present with bilateral right-sidedness
  • more common in males than in females
  • most patients die in their first year of life due to cardiovascular complications
  • right-sided organs are duplicated, and organs that are normally present on the left side are absent
    • infants with Ivemark syndrome usually present during the neonatal period with cyanosis and respiratory distress resulting from complex cardiac anomalies
      • transposition of the great arteries with pulmonary stenosis or atresia and total anomalous venous drainage are common
      • accompanying malformations may involve the GI system secondary to aberrant mesenteric attachments and renal anomalies
        • liver tends to be symmetrical and transverse
        • stomach may be in the midline and hypoplastic

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