Neuropsychological performance of school-aged children after staged surgical palliation of hypoplastic left heart syndrome

OBJECTIVES: Despite advances in perioperative management during surgical treatment of hypoplastic left heart syndrome (HLHS), patients are still at risk for adverse neurodevelopmental sequelae including cognitive dysfunction. This study aimed to assess a neuropsychological profile of HLHS patients at school age who underwent the Norwood operation between 1996 and 2003 with deep hypothermic circulatory arrest (DHCA) or antegrade selective cerebral perfusion (ASCP), respectively. METHODS: Forty HLHS patients (DHCA: n = 11 and ASCP: n = 29) were compared with healthy controls (DHCA controls: n = 10 and ASCP controls: n = 24), recruited according to age, sex and socioeconomic status. Neuropsychological assessment included non-verbal intelligence (IQ norms) and raw score measures of visual and verbal short-and long-term memory as well as executive functions, processing speed and concentration. Neuropsychological data were correlated with bypass and circulatory arrest times. RESULTS: Compared with control subjects, both patient groups had average non-verbal intelligence scores [DHCA: 102 (72-112) and ASCP: 92 (70-127)], but showed reduced long-term memory capacities and decreased executive performance as well as reduced processing speed. DHCA patients, furthermore, had a reduced visual attention span, and ASCP patients scored less on the verbal learning task than controls. The duration of DHCA and cardiopulmonary bypass (CPB) was associated with visual executive functions and short-term memory. In the DHCA group, the duration of DHCA was correlated with the time to complete the Trail Making Test A measuring visual executive functions (Spearman rank correlation, r(S) = 0.867, P = 0.018). In patients provided with ASCP, the cumulated CPB duration was negatively correlated with the score of the block span test measuring visual short memory (r(S) = -0.476, P = 0.020) as well as with the copy score of the Rey Figure assessing visual executive functions (r(S) = -0.399, P = 0.032). CONCLUSIONS: School-age children with HLHS who underwent the Norwood procedure either with DHCA or ASCP show cognitive impairments compared with healthy controls. Our data indicate deficits in specific cognitive domains such as memory, executive functions and processing speed rather than basic intellectual dysfunction.