Ivemark's syndrome
- 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
- infants with Ivemark syndrome usually present during the neonatal period with cyanosis and respiratory distress resulting from complex cardiac anomalies
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