Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease

Abstract

Over a decade ago, I co-authored a review in Cardiology in the Young regarding neurological outcomes following surgery for congenital cardiac disease.1In that review, I placed much emphasis on the conduct of cardiopulmonary bypass, and its role in neurodevelopmental disabilities. Much has been learned in the intervening years regarding the multifactorial causes of abnormal school-age development, in particular, the role of prenatal, perioperative, socioeconomic, and genetic influences. In this update, I will highlight some of the recent advances in our understanding of the protean causes of neurological, behavioral, and developmental abnormalities in children and young adults with complex forms of congenital cardiac disease. In addition, I will summarize the current data on patients at particular high-risk for adverse neurodevelopmental outcomes, specifically those with a functionally univentricular heart who have had staged reconstruction with ultimate conversion to the Fontan circulation.