XXXX and neurodevelopmental status in @entity299 before Fontan procedure.

@entity1 @entity75 @entity1699 @entity854 @entity299
OBJECTIVES: Neonates with @entity299 are at risk for @entity75 . Little is known about the further evolution of cerebral abnormalities until Fontan procedure. METHODS: Between August 2012 and July 2015, we conducted a prospective cross-sectional two centre study using cerebral magnetic resonance imaging (MRI) and neuro-developmental outcome assessed by the Bayley-III. Forty-seven @entity1 (31 male) were evaluated at a mean age of 25.9        3.4 months with @entity1699 (25) or other single ventricle (22). RESULTS: Cerebral MRI was abnormal in 17 @entity1 (36.2%) including liquor space enlargements (10), small grey (9) and minimal white (5) matter injuries. Eight of 17 individuals had combined lesions. Median (range) cognitive composite score (CCS) (100, 65-120) and motor composite score (MCS) (97, 55-124) were comparable to the reference data, while language composite score (LCS) (97, 68-124) was significantly lower (P   =   0.040). Liquor space enlargement was associated with poorer performance on all Bayley-III subscores (CCS: P   =   0.02; LCS: P   =   0.002; MCS: P   =   0.013). The number of re-operations [odds ratio (OR) 2.2, 95% confidence interval (CI) 1.1-4.3] (P   =   0.03) and re-interventions (OR 2.1, 95% CI 1.1-3.8) (P   =   0.03) was associated with a higher rate of @entity854 . CONCLUSIONS: @entity75 occur in more than one third of @entity1 with single ventricle, while the neuro-developmental status is less severely affected before Fontan procedure. Liquor space enlargement is the predominant MRI finding associated with poorer neuro-developmental status, warranting further studies to determine aetiology and further evolution until school-age.
