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.

Figure 1

A, CHQ Parent Report Summary Scores (N = 343) by functional type of cardiomyopathy. Overall Summary Scores were 41.7±14.4 for Physical (raw score −0.83±1.44, median −0.40) and 47.8 ±10.7 for Psychosocial (raw score−0.22 ±1.07, median −0.05). B, CHQ Parent Report Scales: Median z scores, overall cardiomyopathy cohort. P values from signed rank test indicate whether the cardiomyopathy cohort median differs from the healthy norm of Z = 0.

Figure 2

Correlation between FSII(R) total score and A, CHQ Physical Summary Score (Spearman R = 0.52, 95% CI 0.43–0.59) and B, CHQ Psychosocial Summary Score (Spearman R = 0.55, 95% CI 0.47–0.62).

Figure 3

Freedom from clinical outcome in children with dilated cardiomyopathy with HRQOL assessment at age ≥5 years, all P < .001. Curves are truncated at 8 years. A, Time to death/cardiac transplantation by CHQ Physical Summary Score tertile. There were 14, 8, and 2 events in the lowest, middle, and highest tertile, respectively. B, Time to listing for cardiac transplantation by CHQ Physical Summary Score tertile. There were 15, 8, and 4 events in the lowest, middle, and highest tertile, respectively. C, Time to death/cardiac transplantation by FSII(R) Total Score tertile. There were 14, 9, and 2 events in the lowest, middle, and highest tertile, respectively. D, Time to listing for cardiac transplantation by FSII(R) Total Score tertile. There were 15, 10, and 3 events in the lowest, middle, and highest tertile, respectively.