Miller Thomas A, Sharma Binu, Gongwer Russell, Trachtenberg Felicia L, Newburger Jane W, Goldberg Caren S, Gustafson Kathryn E, Gaynor J William, Votava-Smith Jodie K, Lambert Linda M, Sananes Renee, Kral Mary C, Tsang Rocky, Heinrich Kimberley P, Cnota James, Shah Amee, Ilardi Dawn
Department of Pediatrics, Maine Medical Center, Portland (T.A.M.).
Department of Pediatrics, University of Utah, Salt Lake City (T.A.M.).
Circulation. 2025 Jul 17. doi: 10.1161/CIRCULATIONAHA.125.074523.
Neurodevelopmental and functional impairments are among the most consequential morbidities for survivors of hypoplastic left heart syndrome after staged single ventricle surgical palliation. The SVRIII trial (Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type) enrolled adolescents, who were randomized to different surgical shunt types at the time of Norwood procedure as neonates, for multifaceted in-person evaluation. This study reports their neurodevelopmental outcomes.
Transplant-free survivors from SVRIII were invited to complete an in-person comprehensive neurodevelopmental evaluation in early adolescence. Outcomes across domains of cognition, academics, learning, memory, and attention, as well as social, emotional, behavioral, adaptive, and executive function, were compared with those of normative populations. Associations with demographic and medical covariates, including Norwood shunt type, were also assessed.
Among 549 participants enrolled in the SVR trial (Single Ventricle Reconstruction), 200 of the 237 SVRIII participants (84%) completed a neurodevelopmental evaluation at a mean age of 11 years (range, 10 to 14 years). SVRIII participants who did versus did not undergo evaluation were more likely to be male (63% versus 51%), to be White (87% versus 76%), and to have a higher Childhood Opportunity Index score (61±26 versus 46±39). Full-scale intelligence quotient (88±18) was significantly lower than in the normative population, with 39% >1 and 15% >2 SD below the normative mean. Similar patterns were seen for reading (38% >1 SD and 16% >2 SD below the normative mean) and math (38% >1 SD and 19% >2 SD below the normative mean) scores. Attention, executive functioning, social development, visual memory, and adaptive functioning were all more impaired than in the normative population. Measures of socioeconomic status, number of medical complications, and requirement for a gastrostomy tube were each independent predictors of neurodevelopment, with socioeconomic status the most consistently significant factor across models. Group differences by shunt type were inconsistent across neurodevelopmental domains without a clear benefit of one surgical strategy.
In early adolescence, transplant-free survivors of surgical palliation for hypoplastic left heart syndrome show concerning impairments across all domains of neurodevelopment. The distribution of affected outcomes is broad and associated with demographic, medical, and, most frequently, socioeconomic factors. Our findings support recommendations for neurodevelopmental evaluation during adolescence to guide individualized interventions to promote educational success and psychosocial well-being.
URL: https://www.clinicaltrials.gov; Unique identifier: NCT02455531.
对于经分期单心室手术姑息治疗的左心发育不全综合征幸存者而言，神经发育和功能障碍是最为严重的发病情况之一。SVRIII试验（左心发育不全综合征患儿的长期预后及诺伍德分流类型的影响）纳入了青少年，这些青少年在新生儿期接受诺伍德手术时被随机分配至不同的手术分流类型，并接受多方面的现场评估。本研究报告了他们的神经发育结局。
邀请SVRIII试验中未接受心脏移植的幸存者在青春期早期完成一次现场综合神经发育评估。将认知、学业、学习、记忆和注意力等领域以及社交、情感、行为、适应和执行功能方面的结局与正常人群进行比较。还评估了与人口统计学和医学协变量（包括诺伍德分流类型）之间的关联。
在参与SVR试验（单心室重建）的549名参与者中，237名SVRIII参与者中的200名（84%）在平均年龄11岁（范围为10至14岁）时完成了神经发育评估。接受评估与未接受评估的SVRIII参与者相比，更有可能为男性（63%对51%）、为白人（87%对76%），且儿童机会指数得分更高（61±26对46±39）。全量表智商（88±18）显著低于正常人群，39%的人比正常均值低1个标准差以上，15%的人比正常均值低2个标准差以上。阅读（38%比正常均值低1个标准差以上，16%比正常均值低2个标准差以上）和数学（38%比正常均值低1个标准差以上，19%比正常均值低2个标准差以上）得分也呈现类似模式。注意力、执行功能、社交发展、视觉记忆和适应功能均比正常人群受损更严重。社会经济地位指标、医疗并发症数量和胃造口管需求均为神经发育的独立预测因素，其中社会经济地位是各模型中最一致的显著因素。不同分流类型的组间差异在各神经发育领域并不一致，没有一种手术策略具有明显优势。
在青春期早期，接受手术姑息治疗的左心发育不全综合征未接受心脏移植的幸存者在神经发育的所有领域均表现出令人担忧的损害。受影响结局的分布广泛，且与人口统计学、医学因素相关，最常见的是与社会经济因素相关。我们的研究结果支持在青少年期进行神经发育评估的建议，以指导个体化干预，促进教育成功和心理社会福祉。
