Abstract 9979: Health-Related Quality of Life in Children With Marfan Syndrome
| Autor: | Reed E. Pyeritz, Timothy J. Bradley, Elizabeth Radojewski, Kathleen A. Mussatto, Felicia L. Trachtenberg, Karen Uzark, Larry W Markham, Aaron K Olson, Mary Ella M Pierpont, Michelle S Hamstra, Meghan K Mac Neal, Danielle Hollenbeck-Pringle, Mingfen Xu, Sylvia De Nobele, Georgeann Groh, Victoria L. Pemberton, Jami C. Levine, Rosalind Korsin, Kathryn Waitzman, Teresa W Atz, Jill C Handisides, Elizabeth Sparks, Tonia Morrison, Michelle Robinson, Ronald V. Lacro, Mary J. Roman |
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| Rok vydání: | 2015 |
| Předmět: | |
| Zdroj: | Circulation. 132 |
| ISSN: | 1524-4539 0009-7322 |
| Popis: | Background: Marfan syndrome (MFS) is an autosomal dominant disorder that affects the heart, aorta, eyes, skeleton, lungs, and other organs. Objective: To assess quality of life (QL) in a large multicenter cohort of children with MFS. Methods: The Pediatric Quality of Life Inventory (PedsQL) was administered to 256 subjects with MFS ages 5-18 years as an ancillary study to the Pediatric Heart Network’s Marfan Trial, which compared the effects of atenolol vs. losartan on aortic root growth. PedsQL scores were compared to population norms by one-sample t-tests. Scores > 1 SD below the population sample mean represent at-risk status for impaired health-related QL. The impact of treatment arm (atenolol vs. losartan), severity of clinical features, and patient-reported symptoms on QL was assessed by general linear models. Results: The subjects had a mean age of 11.8±3.9 years and were 62% male, 84% white, and 88% non-Hispanic. Mean PedsQL scores for MFS subjects were significantly lower than population norms. Overall, scores were in the impaired range for physical QL and psychosocial QL in 34% and 27% of subjects, respectively. QL across multiple domains correlated negatively with frequency of patient-reported symptoms (r=0.32-0.40, p Conclusions: Children with MFS are at risk for impaired QL. Higher number of patient-reported symptoms had the greatest negative impact on QL, rather than treatment arm or severity of cardiac, skeletal, or ocular findings. Future interventions to address patient symptoms and neurodevelopmental disorders could improve QL for children with MFS. |
| Databáze: | OpenAIRE |
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