Bergemann Annabell, Hansen Jan Hinnerk, Rotermann Ina, Voges Inga, Scheewe Jens, Otto-Morris Christine, Geiger Friedemann, Kramer Hans-Heiner
Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany.
Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany
Eur J Cardiothorac Surg. 2015 May;47(5):803-11. doi: 10.1093/ejcts/ezu299. Epub 2014 Aug 6.
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.
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.
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, rS = 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 (rS = -0.476, P = 0.020) as well as with the copy score of the Rey Figure assessing visual executive functions (rS = -0.399, P = 0.032).
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.
尽管在左心发育不全综合征（HLHS）手术治疗期间围手术期管理取得了进展，但患者仍有发生包括认知功能障碍在内的不良神经发育后遗症的风险。本研究旨在评估1996年至2003年间分别接受深低温停循环（DHCA）或顺行性选择性脑灌注（ASCP）的诺伍德手术的学龄期HLHS患者的神经心理学概况。
根据年龄、性别和社会经济状况招募了40例HLHS患者（DHCA组：n = 11；ASCP组：n = 29）与健康对照者（DHCA对照组：n = 10；ASCP对照组：n = 24）。神经心理学评估包括非言语智力（智商常模）以及视觉和言语短期及长期记忆、执行功能、处理速度和注意力的原始分数测量。神经心理学数据与体外循环和停循环时间相关。
与对照受试者相比，两组患者的非言语智力得分均处于平均水平[DHCA组：102（72 - 112）；ASCP组：92（70 - 127）]，但长期记忆能力降低，执行表现下降，处理速度减慢。此外，DHCA患者的视觉注意力广度降低，ASCP患者在言语学习任务上的得分低于对照组。DHCA和体外循环（CPB）的持续时间与视觉执行功能和短期记忆相关。在DHCA组中，DHCA的持续时间与完成测量视觉执行功能的连线测验A的时间相关（斯皮尔曼等级相关，rS = 0.867，P = 0.018）。在接受ASCP的患者中，累计CPB持续时间与测量视觉短期记忆的方块跨度测试得分呈负相关（rS = -0.476，P = 0.020），以及与评估视觉执行功能的雷氏图形临摹得分呈负相关（rS = -0.399，P = 0.032）。
与健康对照者相比，接受DHCA或ASCP诺伍德手术的学龄期HLHS儿童存在认知障碍。我们的数据表明在特定认知领域存在缺陷，如记忆、执行功能和处理速度，而非基本智力功能障碍。
