2017 年 1 巻 1 号 p. 28-36
In children with congenital heart disease (CHD), the chance of survival exceeds 80%. In this review, the impact of the neurodevelopmental challenges, risk factors, pathophysiology, and future directions of the condition is discussed. The overall outcomes of CHD are favorable. In particular, the rates of major disabilities, such as intellectual disability, sensory loss, and cerebral palsy were low. However, a characteristic pattern of feeding problems, mild motor and cognitive delays, executive dysfunction, impaired social interaction and communication skills, and behavior problems was observed to be common and may impact academic performance, employability, lifelong earnings, and quality of life. The risk factors for poor outcome include type of CHD; presence of genetic conditions; fetal and neonatal neuroimaging abnormalities; pre-, peri-, and postoperative factors associated with hypoxia and hemodynamic instability; prematurity; male sex; and family socioeconomic status and resilience. In utero, CHD may affect cerebral blood flow and oxygenation with resultant slower brain growth, delayed brain maturation, and white matter vulnerability. Pre- and peri-operative instability may cause brain injury, such as white matter injury, microhemorrhages, and stroke. Operative factors, such as deep hypothermic cardiac arrest and cardiopulmonary bypass, played a minor role in determining long-term outcomes. Postoperatively, prolonged hospital stay and severity of illness were predictors of worse outcome. Provision of a nurturing environment with good growth, loving touch, and supportive parents was less studied but probably very important. Future directions should focus on neurodevelopmental screening and surveillance according to existing guidelines; these include early intervention and neurorehabilitation of problems identified, neuromonitoring in the perioperative period to optimize cerebral blood flow and oxygenation, audit and quality improvement, and family education and support.
