Martin Billie-Jean, De Villiers Jonker I, Joffe Ari R, Bond Gwen Y, Acton Bryan V, Ross David B, Robertson Charlene M T, Rebeyka Ivan M, Atallah Joseph
Department of Surgery, University of Alberta, Edmonton, Canada.
Department of Pediatrics, University of Alberta, Edmonton, Canada.
Pediatr Cardiol. 2017 Jun;38(5):922-931. doi: 10.1007/s00246-017-1598-5. Epub 2017 Mar 24.
There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database. Developmental outcomes were assessed at 2 years of age using the Bayley Scales of Infant and Toddler Development (Bayley-III). Survival was assessed using Kaplan-Meier analysis. Baseline characteristics, survival, and neurodevelopmental outcomes were compared between those with HLHS and those with non-HLHS anatomy (non-HLHS). The study comprised 126 infants (75 male), 87 of whom had HLHS. Five-year survival was the same for subjects with HLHS and those with non-HLHS (HLHS 71.8%, non-HLHS 76.9%; p = 0.592). Ninety-three patients underwent neurodevelopmental assessment including Bayley-III scores. The overall mean cognitive composite score was 91.5 (SD 14.6), language score was 86.6 (SD 16.7) and overall mean motor composite score was 85.8 (SD 14.5); being lower than the American normative population mean score of 100 (SD 15) for each (p-value for each comparison, <0.0001). None of the cognitive, language, or motor scores differed between those with HLHS and non-HLHS (all p > 0.05). In the generalized linear models, dominant right ventricle anatomy (present in 117 (93%) of patients) was predictive of lower language and motor scores. Comparative analysis of the HLHS and non-HLHS groups undergoing single ventricle palliation including a Norwood-Sano, during the same era, showed comparable 2-year survival and neurodevelopmental outcomes.
有证据表明，接受诺伍德手术治疗非左心发育不全综合征（HLHS）解剖结构的患者死亡率可能低于经典HLHS，但尚未评估神经发育结局的差异。我们的目标是在一个大型、详细描述的队列中比较同一手术时代的生存率和神经发育结局。纳入了2005年至2014年间接受诺伍德-佐野手术的所有受试者。随访的临床、神经学和发育数据来自加拿大西部复杂儿科治疗随访项目数据库。使用贝利婴幼儿发育量表（贝利-III）在2岁时评估发育结局。使用Kaplan-Meier分析评估生存率。比较HLHS患者和非HLHS解剖结构（非HLHS）患者的基线特征、生存率和神经发育结局。该研究包括126名婴儿（75名男性），其中87名患有HLHS。HLHS患者和非HLHS患者的五年生存率相同（HLHS为71.8%，非HLHS为76.9%；p = 0.592）。93名患者接受了神经发育评估，包括贝利-III评分。总体平均认知综合评分为91.5（标准差14.6），语言评分为86.6（标准差16.7），总体平均运动综合评分为85.8（标准差14.5）；均低于美国正常人群的平均得分100（标准差15）（每项比较的p值均<0.0001）。HLHS患者和非HLHS患者的认知、语言或运动评分均无差异（所有p>0.05）。在广义线性模型中，优势右心室解剖结构（117名（93%）患者存在）可预测较低的语言和运动评分。对同一时期接受包括诺伍德-佐野手术在内的单心室姑息治疗的HLHS组和非HLHS组进行的比较分析显示，两组2年生存率和神经发育结局相当。
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