Abstract: Objective To investigate health-related quality of life (HRQOL) in pediatric patients with functional single ventricle (FSV), and to compare HRQOL between children with FSV and healthy pediatric population. Methods A cross-sectional survey was conducted to recruit 113 pediatric patients with FSV aged 2 to 18 years and their parents from 2002 to 2018 in Shanghai Children's Medical Center.Patients and their parents completed the Chinese version of the Pediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales and 3.0 cardiac module.Independent t-test was used to compare generic PedsQL scores between patients with FSV and healthy population.Generic PedsQL scores for parent proxy-report by age group were compared by ANOVA.Kruskal-Wallis H test was used to compare HRQOL by age group in the cardiac module. Results By self-report and parent proxy-report, generic PedsQL scores for patients with FSV were significantly lower than healthy population in the total scores, physical, psychosocial, emotional, social and school domains (self-report:t=6.823, 7.850, 5.825, 4.260, 4.817, 7.621; parent proxy-report:t=7.641, 8.589, 6.459, 5.823, 6.206, 7.381, P＜0.05).The PedsQL scores in preschool patients with FSV were significantly lower (F=4.090, P＜0.05).In the cardiac module, patients ≥8 years old reported lower scores for physical appearance (H=8.812, P＜0.05).Self-report and parent proxy-report PedsQL scores for treatment anxiety were significantly lower in patients ≤ 8 years old (H=6.045, 14.702, P＜0.05). Conclusions Overall, pediatric patients with FSV perceive poorer quality of life than healthy population.Assessment and interventions on pediatric patients' HRQOL are needed.

Key words: health-related quality of life, Pediatric Quality of Life Inventory, functional single ventricle

摘要： 目的 调查功能性单心室患儿的健康相关生存质量(HRQOL),比较其与健康人群之间的差异。方法 对2002-2018年在上海儿童医学中心诊断为功能性单心室的113名2～18岁患儿及其家长进行横断面分析。采用中文版儿童生存质量测定量表(PedsQL)4.0普适性量表和3.0心脏模块的自评表和家长报告表,通过独立样本t检验比较功能性单心室组与健康组的普适性量表得分,采用单因素方差分析比较不同年龄段普适性量表中家长报告表得分差异。通过Kruskal-Wallis H检验分析不同年龄段心脏模块差异。结果 功能性单心室组的自评表和家长报告表得分在总分、生理、心理社会、情感、社会和学校方面均低于健康组,差异均有统计学意义(自评表:t=6.823、7.850、5.825、4.260、4.817、7.621,家长报告:t＝7.641、8.589、6.459、5.823、6.206、7.381,P＜0.05),并且学龄前患儿的生存质量得分偏低(F=4.090, P＜0.05)。在心脏模块中,自评表的感知身体外貌方面,8岁以上患儿得分明显降低(H=8.812,P＜0.05)。在自评表和家长报告表的治疗焦虑方面,8岁以下患儿得分偏低(H=6.045、14.702,P＜0.05)。 结论 与健康儿童相比,功能性单心室患儿的HRQOL明显下降,因此需要对其HRQOL进行相关干预及评估。

关键词: 健康相关生存质量, 儿童生存质量测定量表, 功能性单心室