Abstract

Introduction: Neurocognitive disorders (NCD) are a growing concern in congenital heart disease (CHD) patients. We hypothesized that early life cardiopulmonary bypass (CBP) as well as age at intervention (AAI) were risk factors for NCDs on long-term follow-up in CHD patients. Methods: Data source was the Quebec CHD database. We defined NCD based on ICD 9 and 10 codes including autism spectrum disorder, global developmental disorder, specific developmental disorder, attention deficit hyperactive disorder, intellectual disability or specific learning deficit. We compared NCD risk from 1983-2010 in 3 cohorts of patients undergoing procedures in childhood: percutaneous atrial septal defect (ASD) closure (no CBP group), surgical ASD closure (short CBP group), and complex CHD lesion repair surgeries (long CBP group). Patients were followed from intervention until NCD diagnosis, death, or administrative censoring whichever came first. Results: By 27 years of follow-up, the cumulative risk of NCD was significantly higher in the complex surgery group compared to the ASD surgery group (crude, 24.9% and 13.4%; p<0.0001) and with adjustments for AAI and sex (Cox regression, p=0.043). By 12 years of follow-up, the cumulative risk of NCD was significantly higher in the complex surgery group compared to the other two groups where the risk was almost identical. Cox models with different follow-up lengths identified younger AAI and male sex being significantly associated with increased risk in NCD. Conclusions: In long-term follow-up of CHD patients, younger AAI of CBP and male sex were associated with increased risk of NCD. Our findings suggest that CBP duration in a wide range of CHD lesions and cohort effects are important predictors of NCD. <!--EndFragment-->

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