Lynn A Sleeper1, Jeffrey A Towbin2, Steven D Colan3, Daphne Hsu4, Endel J Orav5, Matthew S Lemler6, Sarah Clunie7, Jane Messere3, Darlene Fountain8, Tracie L Miller9, James D Wilkinson10, Steven E Lipshultz10. 1. New England Research Institutes, Watertown, MA. Electronic address: lynn.sleeper@cardio.chboston.org. 2. University of Tennessee Health Science Center, St. Jude Children's Research Hospital and Le Bonheur Children's Hospital, Memphis, TN. 3. Boston Children's Hospital, Boston, MA. 4. The Children's Hospital at Montefiore/Albert Einstein College of Medicine, Bronx, NY. 5. Department of Medicine, Harvard Medical School, Boston, MA. 6. University of Texas Southwestern Medical Center, Dallas, TX. 7. Texas Children's Hospital, Houston, TX. 8. Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, TN. 9. University of Miami Miller School of Medicine, Miami, FL. 10. Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit, MI.
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.
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.
Authors: M A Grenier; S K Osganian; G F Cox; J A Towbin; S D Colan; P R Lurie; L A Sleeper; E J Orav; S E Lipshultz Journal: Am Heart J Date: 2000-02 Impact factor: 4.749
Authors: Jeffrey A Towbin; April M Lowe; Steven D Colan; Lynn A Sleeper; E John Orav; Sarah Clunie; Jane Messere; Gerald F Cox; Paul R Lurie; Daphne Hsu; Charles Canter; James D Wilkinson; Steven E Lipshultz Journal: JAMA Date: 2006-10-18 Impact factor: 56.272
Authors: Ashley E Neal; Christian Stopp; David Wypij; David C Bellinger; Carolyn Dunbar-Masterson; David R DeMaso; Jane W Newburger Journal: J Pediatr Date: 2014-10-28 Impact factor: 4.406
Authors: Gemma Gatta; Giulia Zigon; Riccardo Capocaccia; Jan Willem Coebergh; Emmanuel Desandes; Peter Kaatsch; Guido Pastore; Rafael Peris-Bonet; Charles A Stiller Journal: Eur J Cancer Date: 2009-02-21 Impact factor: 9.162
Authors: Linda M Lambert; L LuAnn Minich; Jane W Newburger; Minmin Lu; Victoria L Pemberton; Ellen A McGrath; Andrew M Atz; Mingfen Xu; Elizabeth Radojewski; Darlene Servedio; Brian W McCrindle Journal: Pediatrics Date: 2009-10-19 Impact factor: 7.124
Authors: Brian W McCrindle; Richard V Williams; Paul D Mitchell; Daphne T Hsu; Stephen M Paridon; Andrew M Atz; Jennifer S Li; Jane W Newburger Journal: Circulation Date: 2006-02-20 Impact factor: 29.690
Authors: JonDavid Menteer; Virginia N Beas; Jennifer C Chang; Karen Reed; Jeffrey I Gold Journal: Pediatr Cardiol Date: 2012-09-06 Impact factor: 1.655
Authors: Malindi van der Mheen; Marijke H van der Meulen; Susanna L den Boer; Dayenne J Schreutelkamp; Jan van der Ende; Pieter Fa de Nijs; Johannes Mpj Breur; Ronald B Tanke; Nico A Blom; Lukas Aj Rammeloo; Arend Dj Ten Harkel; Gideon J du Marchie Sarvaas; Elisabeth Mwj Utens; Michiel Dalinghaus Journal: Eur J Cardiovasc Nurs Date: 2019-09-25 Impact factor: 3.908