ASPLENIA SYNDROME - RISK-FACTORS FOR EARLY UNFAVORABLE OUTCOME

As medical and surgical management of congenital heart disease in infancy continues to improve, severe and complex defects are increasingly recognized as the source of mortality and long-term morbidity. The asplenia syndrome has a very poor prognosis; indeed, in 1962, Roberts and associates1 stated that "the finding of asplenia and situs inversus in a patient with congenital heart disease virtually precludes...corrective or even palliative surgical procedures." As part of a study of embryologic timing of the syndrome,2 we reviewed 519 autopsy cases in an attempt to define risk factors associated with early unfavorable outcome. © 1994.