Health-Related Quality of Life and Functional Status Are Associated with Cardiac Status and Clinical Outcome in Children with Cardiomyopathy

Abstract

Objectives To measure the health-related quality of life (HRQOL) and functional status of children with cardiomyopathy and to determine whether they are correlated with sociodemographics, cardiac status, and clinical outcomes. Study design Parents of children in the Pediatric Cardiomyopathy Registry completed the Child Health Questionnaire (CHQ; age ≥5 years) and Functional Status II (Revised) (age ≤18 years) instruments. Linear and Cox regressions were used to examine hypothesized associations with HRQOL. Results The 355 children evaluated at ≥5 years (median 8.6 years) had lower functioning (CHQ Physical and Psychosocial Summary Scores 41.7 ± 14.4 and 47.8 ± 10.7) than that of healthy historical controls. The most extreme CHQ domain score, Parental Impact-Emotional, was one SD below normal. Younger age at diagnosis and smaller left ventricular end-diastolic dimension z score were associated independently with better physical functioning in children with dilated cardiomyopathy. Greater income/education correlated with better psychosocial functioning in children with hypertrophic and mixed/other types of cardiomyopathy. In the age ≥5 year cohort, lower scores on both instruments predicted earlier death/transplant and listing for transplant in children with dilated and mixed/other types of cardiomyopathy (P < .001). Across all ages (n = 565), the Functional Status II (Revised) total score was 87.1 ± 16.4, and a lower score was associated with earlier death/transplant for all cardiomyopathies. Conclusions HRQOL and functional status in children with cardiomyopathy is on average impaired relative to healthy children. These impairments are associated with older age at diagnosis, lower socioeconomic status, left ventricular size, and increased risk for death and transplant. Identification of families at risk for functional impairment allows for provision of specialized services early in the course of disease. Trial registration ClinicalTrials.gov: NCT00005391.

The mental health of adolescents and pre-adolescents living with inherited arrhythmia syndromes: a systematic review of the literature

“…20 Although lower than normative data, the majority of parent-reported scores were within the normal range (−2SD to +2 SD). 20 Parents in two small studies (n = 20-26) indicated that their child's physical but not psychosocial scores were significantly (p = 0.003) below the mean for parent-reported population data. 27,30 Parent-reported subscales were also lower for physical functioning (p = 0.003), role/social-physical (p = 0.01), general health (p < .001), and self-esteem (p = 0.002).…”

“…– 19 Of the 11 studies that used quantitative measures, none reported effect size. Two studies, Sleeper et al 20 and Czosek et al, 21 addressed the clinical relevance of their findings by indicating the proportion of patients more than two standard deviations from the norm or whose assessment results were classified as “at risk”.…”

“…Research reporting quality of life among adolescents and pre-adolescents with inherited arrhythmia syndromes is limited. Patient self-reported assessments of quality of life used a diverse array of assessment procedures (Short Form Health Survey; 19 Pediatric Quality-of-Life Inventory, 21 , 23 , 29 , 31 Pediatric Cardiac Quality of Life Instrument, 21 , 23 , 29 Child Health Questionnaire; 20 , 27 , 30 semi-structured interview 28 ) making comparison of results difficult. Of the nine identified studies, three were analyses from the same database of patients 21 …”

“…In all, 11 identified studies included parent reports of the mental health (six studies) and/or quality of life (eight studies) of their children with inherited arrhythmia syndromes. Among parents, physical and psychosocial quality of life were reported to be lower than healthy peers, 20,20 and similar to patients with complex CHD. 20 Although lower than normative data, the majority of parent-reported scores were within the normal range (−2SD to +2 SD).…”

“…The retrospective chart review (Stefanelli et al 18 ) contained very limited data on mental health or quality of life, reporting only that one of 27 study All of the qualitative studies 18,19,22,26,28 gathered data using interviews. The quantitative studies 17,[19][20][21][23][24][25]27,29,30 used standardised survey instruments, except for two studies -Wojcicka et al 16 and Koopman et al 17 that used a survey specifically developed by Wojcicka et al 16 to assess worry among patients with an implantable cardioverter defibrillator. Nine studies were completed in North America, five in Europe, one in Australia, and one in South America.…”