Summary and Future Directions Although children with mild types of CHD (those who have not had any surgical or catheter-based procedures) appear to have normal CNS and neurodevelopmental and psychosocial outcomes,220,234 children with cCHD constitute a high-risk population with a significant incidence of adverse neurodevelopmental, behavioral, and emotional outcomes Current techniques for developmental evaluation in neonates and infants are imprecise predictors of late outcomes Evaluation of preschool- and school-aged children reveals a pattern of neurodevelopmental dysfunction characterized by mild cognitive impairment, motor dysfunction, impaired visual-spatial and visual-motor skills, and attention and academic difficulties in many of the children There are more significant problems with expressive speech and language in some, and a high incidence of learning differences The factors resulting in CNS injury (most commonly WMI) and developmental dysfunction in these children are multiple, interactive, and incompletely understood Developmental dysfunction results from a complex relationship between patient-specific factors (genetic susceptibility, cardiac diagnosis, fetal development) and environmental factors (preoperative events, techniques of support during surgical repair, postoperative events, SES, family functioning) Currently, reported risk factors incompletely explain the patterns or prevalence of CNS injury following cardiac surgery in infants, suggesting that as-yet-unidentified prenatal, perioperative, and patientspecific factors may modulate the response to CHD and cardiac surgery, increasing the risk of adverse neurodevelopmental and psychosocial consequences Currently, important research investigations are underway to (1) understand and improve the developing brain in the fetus with cCHD82,235–237; (2) identify modifiable risk factors in the operating room and ICU to maximize long-term neurodevelopmental outcomes2,238; and (3) develop strategies after hospital discharge to improve family psychosocial health, childhood development, longterm psychosocial outcomes, and health-related QOL Crucial in this effort is the identification of an early postoperative surrogate variable (e.g., biomarkers and newer brain physiologic and imaging assessments) with good predictive validity for long-term outcomes.43,239–240 MRI in particular is showing great promise in this area, with correlations now being seen with early structural changes, particularly in the white matter, with intermediate neurodevelopmental outcomes.49–50,241 If an appropriate surrogate variable for long-term outcomes can be identified and measured relatively early after surgical intervention for cCHD, reliable clinical trials can be undertaken to improve upon current neurodevelopmental and psychosocial outcomes References Mahle WT, Newburger JW, Matherne GP, et al Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the AHA and AAP Pediatrics 2009;124:823–836 Wernovsky G Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease Cardiol Young 2006;16(suppl 1):92–104 Marino BS, Lipkin PH, Newburger JW, et al On behalf of the American Heart Association congenital heart defects committee of the council on cardiovascular disease in the young, council on cardiovascular nursing, and stroke council Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association Circulation 2012;126:1143–1172 Marino BS, Cassedy A, Drotar D, Wray J The impact of neurodevelopmental and psychosocial outcomes on health-related quality of life in survivors of congenital heart disease J Pediatr ... Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the AHA and AAP Pediatrics 2009;124:823–836 Wernovsky G Current insights regarding neurological and developmental abnormalities in