ABSTRACT
Congenital heart disease (CHD) affects 8 to 10 children out of every 1,000 live births. Of these, 2.2% have an abnormality associated with the spleen. Many previous studies have associated CHD and asplenia or polysplenia syndrome. We report a case of a 12-year-old girl with complex CHD who was noted to have Howell-Jolly bodies on her peripheral blood smear at birth, but disappeared at 2 months of age and radiographic evidence of splenic tissue with situs inversus. Unfortunately, she presented with overwhelming sepsis at 20 months of age. In this paper, we discuss multiple tests available for evaluating splenic function in children with CHD and heterotaxia. Hepatoiminodiacetic acid (HIDA) scan in conjunction with radiocolloid scan is the best available choice today to document the presence or absence of a functional spleen in children with heterotaxia and CHD.
