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

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• 作者：Lynn A. Sleeper ; ScD¹ ; ^{lynn.sleeper@cardio.chboston.org" class="auth_mail" title="E-mail the corresponding author} ; Jeffrey A. Towbin ; MD² ; Steven D. Colan ; MD³ ; Daphne Hsu ; MD⁴ ; Endel J. Orav ; PhD⁵ ; Matthew S. Lemler ; MD⁶ ; Sarah Clunie ; RN⁷ ; Jane Messere ; RN³ ; Darlene Fountain ; RN⁸ ; Tracie L. Miller ; MD ; SM⁹ ; James D. Wilkinson ; MD ; MPH¹⁰ ; Steven E. Lipshultz ; MD¹⁰ ; on behalf of the Pediatric Cardiomyopathy Registry Investigators^&lowast ; ; Steven E. Lipshultz ; MD ; Michelle A. Grenier ; MD ; Amy Giantris ; Lina Rossetti ; Rachel Rossetti ; Kristina McCoy ; RN ; Mabel Meloche ; William Sullivan ; MS ; Eran Muto ; Kristen Lewis ; Renee O&rsquo ; Brien ; Michael Gurell ; Christy Cianfrini ; Marcie Keesler ; Melissa McDonald ; MPH ; Steven E. Lipshultz ; MD ; James D. Wilkinson ; MD ; MPH ; Paolo Rusconi ; MD ; Danielle Dauphin ; Jorge A. Alvarez ; MD ; PhD ; J. Czachor ; J. Alvarez ; Lynn Sleeper ; ScD ; Lisa Cafferata ; Kirsten Noonan ; April Lowe ; MS ; Minmin Lu ; MS ; Rebecca Orfaly ; Linda Gilroy ; Foss Tighe ; MPH ; Patti Arsenault ; MBA ; Neena Pophali ; Lisa Schiavoni ; Stavroula Osganian ; MD ; ScD ; Leigha Cuniberti ; MPH ; Tara McKee ; Elizabeth Rauch ; Endel J. Orav ; PhD ; Paul Lurie ; MD ; Steven E. Lipshultz ; MD ; James D. Wilkinson ; MD ; MPH ; Dolores Tamer ; MD ; Grace Wolfe ; MD ; Paolo Rusconi ; MD ; Samuel Gidding ; MD ; Elfriede Pahl ; MD ; Sherrie Rodgers ; MSN ; Steven Colan ; MD ; Gerald Cox ; MD ; PhD ; Jane Messere ; RN ; Charles Canter ; MD ; Vernat Exil ; MD ; Arnold Strauss ; MD ; Daphne Hsu ; MD ; Carl Steeg ; MD ; Steven E. Lipshultz ; MD ; Michelle A. Grenier ; MD ; Carol Buzzard ; MD ; Susie Truesdell ; PAC ; Mary Ann Rees ; David Wilk ; MPH ; Seema Shaikh ; BS ; Daphne Hsu ; MD ; Darlene Fountain ; RN ; Rosalind Korsin ; RN ; Linda Addonizio ; MD ; Shilpi Epstein ; MD ; Allison Hill-Edgar ; Beth Kaufman ; MD ; Maryanne Chrisant ; MD ; Joseph Rossano ; MD ; MS ; Stephen Webber ; MBChB ; MRCP ; Jeffrey Towbin ; MD ; Sarah Clunie ; RN ; John L. Jefferies ; MD ; MPH ; Matthew Lemler ; MD ; Hollie Carron ; RN ; David Fixler ; MD ; M. Everitt ; MD ; Robert Shaddy ; MD ; Paul Kantor ; MBBCh ; Lee Benson ; MD ; Alice Newman ; MSc
• 关键词：CHQ ; Child Health Questionnaire ; DCM ; Dilated cardiomyopathy ; EDD ; End-diastolic dimension ; FSII(R) ; Functional Status II (Revised) ; HCM ; Hypertrophic cardiomyopathy ; HR ; Hazard ratio ; HRQOL ; Health-related quality of life ; LV ; Left ventricular ; PCMR ; Pediatric Cardiomyopathy Registry
• 刊名：The Journal of Pediatrics
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：170
• 期：Complete
• 页码：173-180.e4
• 全文大小：890 K

文摘

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.