Neurodevelopmental outcomes in children after the Fontan operation

Background-Previous studies of patients after the Fontan operation have rep orted IQ scores lower than population norms. In the past decade, changes ha ve occurred both in surgical methods used and in the patient population und ergoing Fontan palliation. The present study examined the impact of these c hanges on neurodevelopmental outcomes after Fontan. Methods and Results-Neuropsychological tests were administered to 27 five-y ear-old children after Fontan. Mean age at repair was 2 years 4 months. The present sample was compared with an earlier Fontan group (EFG) of 133 pati ents who underwent surgery in the 1970s and 1980s. Mean age at repair for t he EFG was 7 years 3 months. Compared with EFG, the present study sample wa s younger at Fontan (P=0.0001) and more likely to have undergone a Norwood procedure (P=0.02), a pre-Fontan bidirectional cavopulmonary anastomosis (P <0.001), and Fontan fenestration (P=0.001). Although mean full-scale, verb al, and performance IQ scores were within 1 SD (15 points) of the populatio n mean of 100 (93 +/- 16, 95 +/- 15, and 91 +/- 17, respectively), mean ful l-scale and performance IQ scores were significantly lower than this popula tion mean (P=0.03 and P=0.01, respectively). Conclusions-Compared with a historical cohort of Fontan patients from this institution, a staged approach to Fontan earlier in life is not detrimental to neurodevelopmental outcome. Neurodevelopmental outcomes in children aft er Fontan are in the normal range, but performance remains lower than the g eneral population.