Impact of Congenital Heart Disease on Brain Development and Neurodevelopmental Outcome

Abstract

Advances in cardiac surgical techniques and perioperative intensive care have led to improved survival in babies with congenital heart disease (CHD). While it is true that the majority of children with CHD today will survive, many will have impaired neurodevelopmental outcome across a wide spectrum of domains. While continuing to improve short-term morbidity and mortality is an important goal, recent and ongoing research has focused on defining the impact of CHD on brain development, minimizing postnatal brain injury, and improving long-term outcomes. This paper will review the impact that CHD has on the developing brain of the fetus and infant. Neurologic abnormalities detectable prior to surgery will be described. Potential etiologies of these findings will be discussed, including altered fetal intrauterine growth, cerebral blood flow and brain development, associated congenital brain abnormalities, and risk for postnatal brain injury. Finally, reported neurodevelopmental outcomes after surgical repair of CHD will be reviewed.

Prevalence and Spectrum of In Utero Structural Brain Abnormalities in Fetuses with Complex Congenital Heart Disease

“…1,2 During the past decade, a growing body of evidence has demonstrated the high prevalence of preoperative brain imaging abnormalities in neonates with CHD, suggesting an antenatal origin in many cases. [3][4][5][6] More recently, our group has used quantitative MR imaging to demonstrate a progressive third-trimester deceleration in volumetric brain growth and metabolism in fetuses with CHD, 7 reduced volumes of gray (cortical and subcortical) and white matter, and significant delays in cortical gyrification and surface area in fetuses with hypoplastic left-heart syndrome (HLHS).…”

Changes in biometry and cerebroplacental hemodynamics in fetuses with congenital heart diseases

“…Prenatal counseling of CHD is challenging for fetal medicine specialists although the extended knowledge of these diseases together with cardiac surgery advancements are still related to critical survival rates and poor neurodevelopment later in life (23,24).…”

Developmentally Supportive Care in Congenital Heart Disease: A Concept Analysis

“…Improvements in recognition, surgical techniques, and perioperative management of infants and children with congenital heart disease (CHD) have led to significantly improved survival; yet, up to half of CHD survivors face some degree of neurodevelopmental delay (Donofrio & Massaro, 2010). These delays may result from preoperative microcephaly and structural brain immaturity, right-to-left intracardiac shunting that may result in decreased cerebral oxygen delivery, intraoperative or postoperative use of extracorporeal life support, or low cardiac output (Donofrio & Massaro, 2010;Licht et al, 2009;Marino et al, 2012;Wernovsky, 2006).…”

“…These delays may result from preoperative microcephaly and structural brain immaturity, right-to-left intracardiac shunting that may result in decreased cerebral oxygen delivery, intraoperative or postoperative use of extracorporeal life support, or low cardiac output (Donofrio & Massaro, 2010;Licht et al, 2009;Marino et al, 2012;Wernovsky, 2006). Exposure to these risk factors may be a single occurrence, or may continue throughout life in children who require staged palliation.…”