PEDIATRICS Vol. 48 No. 2 August 1971, pp. 277-280
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FUNCTIONAL HYPOSPLENIA IN CYANOTIC CONGENITAL HEART DISEASE

Howard A. Pearson M.D.1, Gerold L. Schiebler M.D., Ph.D.1, and Richard P. Spencer M.D.1

1 Department of Pediatrics, Yale University School of Medicine, and The Yale-New Haven Hospital, New Haven, Connecticut; The Department of Pediatrics, University of Florida College of Medicine, and The Shands Teaching Hospital, Gainesville, Florida; and The Division of Nuclear Medicine, Yale University School of Medicine

Hematologic abnormalities suggesting decreased splenic function (Howell-Jolly and Heinz bodies ) were found in the blood of two infants with cyanotic congenital heart disease. Since both of these infants were shown to possess anatomically normal spleens, "functional hyposplenia" is considered to be the basis for the abnormal hematologic findings.

The congenital asplenia syndrome is usually associated with severe, complex, and uncorrectable forms of cardiac malformation. This has discouraged diagnostic and therapeutic measures in children in whom the diagnosis has been made. Since similar blood changes may occur in children with anatomically normal spleens, this bad prognosis may not always be valid. Functional hyposplenia might, on occasion, contribute to the morbidity and mortality of infants with cyanotic congenital heart disease.

Submitted on October 30, 1970
Accepted on March 1, 1971