Ivemark's syndrome

Last reviewed 01/2018

• 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