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
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