Impaired Neurocognitive Outcome in Children with Congenital Heart Disease (CHD) - What can we Currently Achieve and Effect in the Future? Case Report and Overview

Background: Neurocognitive Failure(NCF) is the most common morbidity or complication that significantly reduces the QoL of children with CHD after cardiac surgery. But is it the surgical intervention alone?

Methods: Case report and evidence-based literature research Medline, PubMed etc.

Results: Case report: The NCF is reported of a GUCH-patient with pulmonary valve atresia after cardiac surgery and PPVI (“Melody”-Valve). While children with mild AHF have normal CNS u. neuro-developmental outcomes.ed Children with complex AHF have a risk population with a significant incidence of adverse developmental outcomes. Current developmental evaluation techniques in neonates and infants are inaccurate predictors of subsequent outcomes. The evaluation of pre-school and school-age children shows patterns of neurodevelopmental disorders characterized by mild cognitive impairment, motor dysfunction, impaired visual-spatial and visual-motor functions, and attention(AD(H)D) and academic difficulties. There are significant problems with expressive speech and language and a high incidence of learning disabilities. The factors that lead to CNS damage and dysfunction in these children are diverse and so far incompletely understood. Developmental disorders result from a complex interaction between patient-specific factors (genetic susceptibility, CHD diagnosis, fetal development) and environmental factors (preoperative events, technical support during cardiac surgery, postoperative events, socioeconomic status). Currently, reported risk factors do not adequately explain pattern or incidence of CNS damage following cardiac surgery in infants, suggesting that other patient-specific factors may modulate the response to CHD and heart surgery, increasing the risk of adverse consequences of neurodevelopment. Children with complex CHD have risk for cerebral ischemia before, during and after cardiac surgery. Therefore, factors that interfere with CNS recovery after ischemia may be important determinants of the long-term neurological outcome. At the moment important studies are in progress. 1. to understand the developing brain in the fetus with complex CHD, 2. Identify modifiable risk factors in the operating room and on the ICU to maximize long-term neurological outcomes. 3. Develop strategies to improve family psychosocial health, child development and health-related QoL after hospital discharge.

Conclusion: Neurocognitive failure is the most common morbidity or complication, which usually significantly reduces QoL in children with severe and complex CHD after heart surgery. As already established with risk infants, these children should also be cared for in development diagnostics and neuropediatrics and (early) supported, for example in social pediatric centers. Furthermore, the identification of an early postoperative surrogate variable with good predictive validity for long-term results is crucial. MRI is very promising in this area as it reveals the early structural changes (especially in the white matter) in intermediate neurological developmental outcomes. Clinical trials should be performed to improve the neurocognitive outcome.