Cohen Scott, Gurvitz Michelle, Burns Kristin M, Wheaton Olivia, Umfleet Laura G, Brown Nicole, Silversides Candice K, Dugan Lauryn, Stumpf Mary E, Cotts Timothy B, Ermis Peter R, Jameson Susan M, Gaydos Stephanie, Hoskoppal Arvind, Lindsay Ian, Markham Larry W, Fuller Stephanie, Rodriguez Fred H, Ranganathan Gayatri, Trachtenberg Felicia, Zaidi Ali N
Departments of Medicine and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
J Am Coll Cardiol. 2025 Sep 2;86(9):640-655. doi: 10.1016/j.jacc.2025.06.051.
Adults with congenital heart disease (ACHD) undergo repeated cardiac interventions, develop cardiac complications including atrial fibrillation, heart failure, and stroke, and may have genetic vulnerabilities for neurocognitive deficits (NCDs). However, NCDs have not been well studied in this population.
The aim of this study was to examine the prevalence and risk factors associated with NCDs in young adults with moderate and severe complex congenital heart disease (CHD).
This was a prospective study of objective (cognitive battery of the National Institutes of Health [NIH] Toolbox) and subjective (NeuroQOL Item Bank v2) measures of neurocognitive function in ACHD patients (ages 18-30 years) from 14 North American ACHD centers. Multivariable regression was used to examine predictors of neurocognitive function.
Overall, 467 ACHD participants were enrolled (82 dextro-transposition of the great arteries, 123 tetralogy of Fallot, 132 single ventricle, and 130 other CHD). The mean age was 24.5 years, 44% were male, 81% were White, and 78% had NYHA functional class I. For the total cohort, 34% scored at least 1 standard deviation below the normative mean on the NIH Toolbox Total Composite Score and 22% scored at least 1 standard deviation below the normative mean on the NeuroQOL. Education and NYHA functional class were independently associated with NIH Toolbox scores. Education, body mass index, atrial arrhythmias, depression, and anxiety were independently associated with NeuroQOL scores.
NCDs are prevalent in young adults with moderate and severely complex CHD, with approximately one-third having objective NCDs and one-fifth subjective NCDs. These findings emphasize the importance of integrating neurocognitive assessments into routine ACHD care.
患有先天性心脏病的成年人(ACHD)需接受多次心脏干预,会出现包括心房颤动、心力衰竭和中风在内的心脏并发症,并且可能存在神经认知缺陷(NCD)的遗传易感性。然而,该人群中的神经认知缺陷尚未得到充分研究。
本研究旨在调查患有中度和重度复杂性先天性心脏病(CHD)的年轻成年人中神经认知缺陷的患病率及相关危险因素。
这是一项前瞻性研究,对来自北美14个先天性心脏病中心的ACHD患者(年龄18 - 30岁)进行神经认知功能的客观(美国国立卫生研究院[NIH]工具箱认知测试组合)和主观(NeuroQOL项目库第2版)测量。采用多变量回归分析神经认知功能的预测因素。
共纳入467名ACHD参与者(82例大动脉转位、123例法洛四联症、132例单心室和130例其他CHD)。平均年龄为24.5岁,44%为男性,81%为白人,78%的心功能分级为纽约心脏协会(NYHA)I级。在整个队列中,34%的患者在NIH工具箱总综合评分上至少比正常均值低1个标准差,22%的患者在NeuroQOL上至少比正常均值低1个标准差。教育程度和NYHA心功能分级与NIH工具箱评分独立相关。教育程度、体重指数、房性心律失常、抑郁和焦虑与NeuroQOL评分独立相关。
神经认知缺陷在患有中度和重度复杂性先天性心脏病的年轻成年人中很普遍,约三分之一的人存在客观神经认知缺陷,五分之一的人存在主观神经认知缺陷。这些发现强调了将神经认知评估纳入ACHD常规护理的重要性。