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July 1973

Ivemark Syndrome With Aortic Atresia

Author Affiliations

Milwaukee, Wis
From the departments of pediatrics, pathology, and surgery. The Medical College of Wisconsin, and the Milwaukee Children's Hospital.

Am J Dis Child. 1973;126(1):106-108. doi:10.1001/archpedi.1973.02110190094020

The first case of Ivemark syndrome (agenesis of the spleen with associated congenital heart disease) with aortic valve atresia is reported. No conotruncal abnormalities were noted. This finding brings into question the proposed etiology of this syndrome, namely an unknown teratogenic agent operating coincidently with the development of the spleen and the conotruncal region of the heart. It does not invalidate the flow hypothesis to explain the etiology of aortic atresia.

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