Understanding the Neurological and Functional Development of Children with Critical Congenital Heart Disease

Critical congenital heart disease (cCHD) encompasses the most severe forms of congenital heart defects. To survive, infants with cCHD must undergo complex cardiac surgeries (CCS) in early life. Thanks to continuous improvements in the overall care of these children, survival rates have improved significantly, and now most children with cCHD can reach adulthood. By the late twentieth century, as the percentage of children surviving CCS increased, researchers began to wonder about their neurodevelopment. Today, after more than three decades of further studies, it is clear that children with cCHD are at high risk for developmental delays. Developmental challenges commonly include deficits in cognitive, language, motor, behavior and social interaction abilities. These challenges arise from the cumulative effect of multiple recognized pre- and post-natal factors, and contrary to what was previously thought, these do not strictly relate to the presence of hypoxemia or the cardiac surgery itself. By examining three issues not previously described in the literature, this thesis aims to improve the understanding of the neurological and functional developmental impact of CCS in children with cCHD. All three projects in this thesis include children with cCHD registered in the Western Canadian Complex Pediatric Therapies Follow-up Program (WCCPTFP). This inception cohort project identifies all infants born with cCHD who undergo early-life CCS at the Stollery Children’s Hospital (Edmonton, Alberta), and follows them prospectively across western Canada. As part of this program, developmental outcomes are determined by multidisciplinary assessment at approximately 8 months, 21 months and 4.5 years of age. The study presented in chapter 2 begins this work by assessing the frequency and presentation of chronic neuromotor disability (CND), including cerebral palsy and acute brain injury in kindergarten-aged children who have survived CCS. Results indicated CND affects 6% of CCS survivors and almost 10% of those who have required more than one CCS. Most children with CND could ambulate without the need of a mobility device, and often had a high frequency of associated developmental impairments. Older age in days at first CCS, highest plasma lactate before first CCS, and undergoing more than one CCS were predictors of CND. The thesis work continues in chapter 3, where a more specific group of kindergarten-aged children is examined: those who survive the Fontan operation. The aim of the study is to better understand the impact of this operation on the functional abilities of children, and its potential relation with stroke (an already recognized peri-operative complication). Overall, more than a quarter of children experienced deterioration of functional abilities following the Fontan operation. Both peri-operative stroke and older age at Fontan were predictors of decline of functional abilities. Finally, in chapter 4, the relationship between the need for gastrostomy tube feeding (GTF) any time before the 21-month multidisciplinary assessment and the presence of developmental delays is assessed. Findings suggested GTF identifies CCS survivors at risk for developmental delay who would benefit from early developmental intervention. Presence of chromosomal anomaly, single ventricle anatomy, number of post-operative days with open sternum and total number of hospital days at CCS were predictors of GTF requirements before the 21-month assessment. Together these three projects address key gaps in knowledge by describing outcomes not previously reported, all of which can have a significant impact on the participation of children in activities of everyday life. Efforts to prevent and to address these developmental difficulties should be undertaken.

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