Heart Centre for Children, The Sydney Children's Hospitals Network, Sydney, NSW, Australia; School of Clinical Medicine, University of New South Wales, Sydney, NSW, Australia.
Division of Cardiac Surgery, Children's National Hospital, Washington, DC.
J Pediatr. 2024 Oct;273:114156. doi: 10.1016/j.jpeds.2024.114156. Epub 2024 Jun 17.
To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes.
Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the Australia and New Zealand Fontan Registry.
Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes.
Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population.
评估 Fontan 生理学患儿和青少年的健康相关生活质量（HRQOL）和整体生活质量（QOL），并确定影响这些结果的关键预测因素。
对澳大利亚和新西兰 Fontan 注册中心的 73 名年龄在 6-17 岁（Fontan 手术后至少 12 个月）的儿童和青少年进行横断面分析。评估包括儿科生活质量量表（PedsQL）用于 HRQOL 和针对整体 QOL 的发展性量身定制的视觉模拟量表（0-10），以及经过验证的社会人口统计学、临床、心理、关系和父母措施。临床数据由澳大利亚和新西兰 Fontan 注册中心提供。
参与者（平均年龄：11.5±2.6 岁，62%为男性）报告整体 HRQOL 较低（P<.001），并且所有 HRQOL 领域的评分均较低（所有 P<.0001），与参考数据相比。全球 QOL 的中位数评分为 7.0（IQR 5.8-8.0），大多数参与者（79%）将其全球 QOL 评为≥6。分别有 21%和 26%的参与者报告存在焦虑和抑郁症状，需要进行临床评估。年龄、性别和对先天性心脏病严重程度的感知解释了 HRQOL 评分变化的 15%，而抑郁症状和与治疗相关的焦虑解释了另外的 37%（最终模型：解释了 52%的方差）。对于全球 QOL，社会人口统计学和临床因素解释了评分变化的 13%，而抑郁症状解释了进一步的 25%（最终模型：解释了 38%的方差）。父母因素与儿童 QOL 结果无关。
尽管报告整体 QOL 尚可，但 Fontan 生理学患儿和青少年的 HRQOL 低于基于社区的正常值。心理因素主要影响 QOL 结果，这表明加强心理健康的策略可能会提高该人群的 QOL。
