Neurocognitive Outcomes at Kindergarten Entry After Surgical Repair of Total Anomalous Pulmonary Venous Connection in Early Infancy

The objective of this study was to determine neurocognitive outcomes 4.5 years after surgery for TAPVC in infancy and predictors of these outcomes. A cohort having TAPVC repair at age ≤6 weeks between 1998 and 2007 were followed by the Complex Pediatric Therapies Follow-up Program at 4.5 years. Outcomes include mortality, full-scale intelligence quotient (FSIQ), verbal IQ (VIQ), performance IQ (PIQ), visual motor integration (VMI), and general adaptive composite of the Adaptive Behavior Assessment System (GAC). There were 51 infants with simple TAPVC [4 year mortality 4 (8 %)], and 16 with complex TAPVC [4 year mortality 7 (44 %)], hazard ratio (HR) 7.02 (95 % CI 2.05–24.07, p = 0.002). Of the 47 survivors after simple TAPVC, FSIQ (SD) was 92 (17), VIQ 92 (17), PIQ 94 (15), VMI 92 (15), and GAC 92 (15). Independent predictors of neurocognitive outcome included father’s socioeconomic status, mother’s years of schooling, gender, post-operative base deficit, and deep hypothermic circulatory arrest (DHCA) time. Complex TAPVC was associated on univariate analysis only with PIQ [81.9 (10.2) vs. 93.6 (15.4); p = 0.012] and FSIQ [80.7 (10.1) vs. 92.0 (17.7); p = 0.017]. Original peoples accounted for 25/51 (49 %) of simple and 3/16 (19 %) of complex TAPVC. Original peoples race was associated with 4-year mortality [HR 6.85 (95 % CI 2.15, 21.76, p = 0.001)]. Survivors of TAPVC repair in early infancy have encouraging neurocognitive outcomes. Few independent predictors of neurocognitive outcome were found, with post-operative acidosis and DHCA time being potentially modifiable. Original peoples account for an unexpected proportion of patients (42 %) and have a higher mortality.