ABSTRACT

Introduction The prevalence of congenital heart disease (CHD) is about 1 in every 100 live births, of which about one-third is in critical need of surgical intervention in neonatal or infant age (Figure 59.1).1 Since the 1980s, advanced diagnostic methods, neonatal cardiopulmonary bypass operations enabling early correction of complex congenital heart defects, and improvements in postoperative care have markedly increased life expectancy. Today, more than 90% of CHD patients survive into adulthood. At the same time, these patients are at significant risk of short-and long-term neurodevelopmental impairment with negative impact on psychosocial and academic performance as well as on quality of life and independence in adulthood.2-6

In this chapter, the causes and risk factors for neurodevelopmental disability are discussed, and groups of CHD children who are at high risk are identified. The phenotype of affected children in infant age and throughout childhood and adolescence is described. And, strategies for systematic screening, evaluation, and intervention are suggested.