Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation

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• 作者：Karen Uzark ; PhD ; CPNP¹ ; ^{karenu@med.umich.edu" class="auth_mail" title="E-mail the corresponding author} ; Victor Zak ; PhD² ; Peter Shrader ; MA² ; Brian W. McCrindle ; MD ; MPH³ ; Elizabeth Radojewski ; RN³ ; James W. Varni ; PhD⁴ ; Kaitlyn Daniels ; BSN ; RN⁵ ; Jill Handisides ; BSN⁶ ; Kevin D. Hill ; MD ; MS⁷ ; Linda M. Lambert ; MSN ; FNP⁸ ; Renee Margossian ; MD⁶ ; Victoria L. Pemberton ; RNC ; MS ; CCRC⁹ ; Wyman W. Lai ; MD¹⁰ ; Andrew M. Atz ; MD¹¹ ; on behalf of the Pediatric Heart Network Investigators^&lowast ; ; Gail Pearson ; MD ; ScD ; Mario Stylianou ; PhD ; Jonathan Kaltman ; MD ; Lynn Sleeper ; ScD ; Steven Colan ; MD ; Dianne Gallagher ; MS ; Lynn Mahony ; MD ; Jane Newburger ; MD ; MPH (Principal Investigator) ; Roger Breitbart ; MD ; Carolyn Dunbar-Masterson ; RN ; MS ; Lisa-Jean Buckley ; Bethany Trainor ; RN ; BSN ; Rosalind Korsin ; RN ; Robert Shaddy ; MD (Principal Investigator) ; J. William Gaynor ; MD (Principal Investigator) ; Stephen M. Paridon ; MD (Principal Investigator) ; Tonia Morrison ; MSM ; CCRC ; Nicole Mirarchi ; RN ; Jennifer S. Li ; MD (Principal Investigator) ; Piers Barker ; MD ; Mingfen Xu ; MSN ; J. Philip Saul ; MD (Principal Investigator) ; Patricia Infinger ; CCRP ; Ann Harvey Frampton ; BS ; LuAnn Minich ; MD (Principal Investigator) ; Richard Williams ; MD ; Linda Lambert ; MSN ; cFNP ; Brian McCrindle ; MD (Principal Investigator) ; Elizabeth Radojewski ; RN ; Svetlana Khaikin ; RN ; MPH ; Patricia Walter ; RN ; BScN ; Bradley Marino ; MD ; Michael Artman ; MD ; Timothy Feltes ; MD ; Julie Johnson ; PharmD ; Jeffrey Krischer ; PhD ; G. Paul Matherne ; MD ; Nakela Cook ; MD ; MPH ; John Kugler ; MD ; David Gordon ; MD ; PhD ; David J. Driscoll ; MD ; Mark Galantowicz ; MD ; Sally A. Hunsberger ; PhD ; Holly Taylor ; PhD ; Thomas J. Knight ; JD ; Catherine L. Webb ; MD
• 关键词：CHD ; Congenital heart disease ; CHQ-87 ; Child Health Questionnaire ; Fontan 1 ; Pediatric Heart Network Fontan Cross-Sectional Study ; Fontan 2 ; Fontan Follow-up Study ; PedsQL ; Pediatric Quality of Life Inventory ; QOL ; Quality of life ; SF-36 ; Short Form Health Survey
• 刊名：The Journal of Pediatrics
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：170
• 期：Complete
• 页码：166-172.e1
• 全文大小：174 K

文摘

To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan.

Study design

Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined.

Results

Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P = .004) and the emotional functioning score decreased by an average of 0.64 points (P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P < .001) and Short Form Health Survey scores (P < .001).

Conclusions

Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood.

Trial registration

ClinicalTrials.gov: NCT00132782.