Quality of life and cognitive function in Fontan patients, a population-based study

Idorn, Lars; Jensen, Annette Schophuus; Juul, Klaus; Overgaard, Dorthe; Nielsen, Niels Peter; Sørensen, Keld E.; Reimers, Jørgen; Søndergaard, Lars

doi:10.1016/j.ijcard.2013.04.008

Cited by 60 publications

(58 citation statements)

References 39 publications

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“…Lower self-reported physical and psychosocial QOL is also consistent with parent-reports of functioning in these domains on the CHQ in this same patient group, 12 and with pediatric and adult self-reports in patients receiving Fontan previously reported in Denmark and the Netherlands, 4, 23 as well as in patients with other chronic health conditions. 7 Deficits in physical health or functional status 6, 24 and depression 24 have also been reported in the single ventricle population.…”

Section: Discussionsupporting

confidence: 89%

“…Past studies have often relied on parental report, are limited by small sample size/single center design, are restricted to a specific age-group, or use different measures across age-groups. 4–10 No longitudinal follow-up of QOL has been reported in patients with Fontan physiology as they move through childhood to young adulthood, perhaps in part reflecting the lack of an available self-report QOL measurement instrument that spans the age range of this growing population of survivors of Fontan.…”

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confidence: 99%

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

Uzark

Zak

Shrader

et al. 2016

The Journal of Pediatrics

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Objectives 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 (CHQ-87, age < 19years) or Short Form 36 (SF-36, 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<0.001). 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=0.004) and the emotional functioning score decreased by an average of 0.64 points (p=0.03). Among subjects ≥ 19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (p=0.02). PedsQL scale scores were significantly correlated with conceptually related CHQ-87 (p<0.001) and SF-36 scores (p<0.001). 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. Trials Registration ClinicalTrials.gov: NCT00132782.

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Section: Discussionsupporting

confidence: 89%

mentioning

confidence: 99%

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

Uzark

Zak

Shrader

et al. 2016

The Journal of Pediatrics

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“…The results of our cohort of adult subjects showed similar scores when compared with a recent study involving 54 Danish subjects with a Fontan circulation. 27 A recent systematic review of quality-of-life studies in adult congenital heart disease patients suggests that, in general, quality of life is compromised in the physical domains compared with norms and less so in relation to psychosocial and environmental/occupational domains. 28 These findings are supported by our results.…”

Section: Discussionmentioning

confidence: 99%

Survival Data and Predictors of Functional Outcome an Average of 15 Years after the Fontan Procedure: The Pediatric Heart Network Fontan Cohort

et al. 2014

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Objective Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort. Design Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care. Results Thirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9–13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow-up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF-36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1. Conclusions We found 95% interim transplant-free survival for Fontan survivors over an average of 7 years of follow-up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long-term outcomes and to improve functional health status.

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“…Physical functional status and physical quality-of-life measures have been shown to be reduced compared with healthy control populations 10 11. Furthermore, data from the ongoing Single Ventricle Trial group have very clearly shown poorer neurodevelopment as measured by the psychomotor development index, motor development index of the Bayley development scores at 14 months 12 13…”

Section: Introductionmentioning

confidence: 99%

Physical and neurodevelopmental outcomes in children with single-ventricle circulation

Davidson¹,

Gringras²,

Fairhurst³

et al. 2014

Arch Dis Child

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Quality of life and cognitive function in Fontan patients, a population-based study

Cited by 60 publications

References 39 publications

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

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

Survival Data and Predictors of Functional Outcome an Average of 15 Years after the Fontan Procedure: The Pediatric Heart Network Fontan Cohort

Physical and neurodevelopmental outcomes in children with single-ventricle circulation

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