Abstract
Children, adolescents and adults living with Fontan circulation face numerous neurological and developmental challenges. As the population with complex CHD increases thanks to outstanding improvement in medical and surgical care, the long-term developmental and mental health sequelae have become a public health priority in pediatric and congenital cardiology. Many patients with a Fontan circulation experience difficulty in areas of cognition related to attention and executive functioning, visual spatial reasoning and psychosocial development. They are also at high risk for mental health morbidities, particularly anxiety disorders and depression. Several hemodynamic risk factors, beginning during the fetal period, may influence outcomes and yield to abnormal brain growth and development. Brain injury such as white matter lesions, stroke or hemorrhage can occur before, during, or after surgery. Other sociodemographic and surgical risk factors such as multiple catheterizations and surgeries and prolonged hospital stay play a detrimental role in patients' neurodevelopmental prognosis. Prevention and intervention to optimize long-term outcomes are critical in the care of this vulnerable population with complex CHD.