Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study

Background To characterize global and health‐related quality of life (QOL) among adults with Fontan physiology enrolled in the Australian and New Zealand Fontan Registry (ANZFR), and identify sociodemographic, clinical, psychological, and relational factors associated with outcomes. Methods and Resu...

Full description

Saved in:
Bibliographic Details
Main Authors: Kate H. Marshall, Yves d'Udekem, David S. Winlaw, Diana Zannino, David S. Celermajer, Robert Justo, Ajay Iyengar, Rachael Cordina, Gary F. Sholler, Susan R. Woolfenden, Nadine A. Kasparian
Format: Article
Language:English
Published: Wiley 2024-08-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.123.033818
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850146662309167104
author Kate H. Marshall
Yves d'Udekem
David S. Winlaw
Diana Zannino
David S. Celermajer
Robert Justo
Ajay Iyengar
Rachael Cordina
Gary F. Sholler
Susan R. Woolfenden
Nadine A. Kasparian
author_facet Kate H. Marshall
Yves d'Udekem
David S. Winlaw
Diana Zannino
David S. Celermajer
Robert Justo
Ajay Iyengar
Rachael Cordina
Gary F. Sholler
Susan R. Woolfenden
Nadine A. Kasparian
author_sort Kate H. Marshall
collection DOAJ
description Background To characterize global and health‐related quality of life (QOL) among adults with Fontan physiology enrolled in the Australian and New Zealand Fontan Registry (ANZFR), and identify sociodemographic, clinical, psychological, and relational factors associated with outcomes. Methods and Results Using a cross‐sectional survey design, 66 adults with Fontan physiology (58% women; mean age, 29.6±7.7 years; range, 18–50 years) completed validated self‐report measures. Health‐related QOL was assessed using the Pediatric Quality of Life Inventory, and global QOL was assessed using a visual analog scale (0–10). Participants reported lower total health‐related QOL (P<0.001), as well as lower physical (P<0.001) and social (P=0.002) functioning compared with normative data. Median global QOL was 7.0 (interquartile range: 5.0‐8.0) and most participants (71%) rated their QOL ≥6. For health‐related QOL, age, sex, university education, and length of hospital stay in the past 12 months explained 27% of the variance in scores, while general psychological stress, medical traumatic stress, communication problems, and access to emotional support explained a further 44% of variance (final model: 71% of variance explained). For global QOL, sociodemographic and clinical factors explained 20% of the variance in scores, while psychological stress and sense of coherence explained a further 24% (final model: 44% of variance explained). Conclusions Adults with Fontan physiology reported lower overall health‐related QOL compared with community‐based norms. Variance in QOL outcomes were predominantly attributable to psychological and relational factors. Tailored screening and assessment to identify Fontan patients at greatest risk of lower QOL, and a proactive approach to supportive care, are needed.
format Article
id doaj-art-32f120b78db047b894fcb8e597f3ccbb
institution OA Journals
issn 2047-9980
language English
publishDate 2024-08-01
publisher Wiley
record_format Article
series Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
spelling doaj-art-32f120b78db047b894fcb8e597f3ccbb2025-08-20T02:27:46ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802024-08-01131510.1161/JAHA.123.033818Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life StudyKate H. Marshall0Yves d'Udekem1David S. Winlaw2Diana Zannino3David S. Celermajer4Robert Justo5Ajay Iyengar6Rachael Cordina7Gary F. Sholler8Susan R. Woolfenden9Nadine A. Kasparian10Heart Centre for Children The Sydney Children’s Hospitals Network Sydney NSW AustraliaDivision of Cardiac Surgery Children’s National Hospital Washington DC United StatesHeart Center, Ann &amp; Robert H. Lurie Children’s Hospital Chicago IL United StatesClinical Epidemiology and Biostatistics Unit Murdoch Children’s Research Institute Melbourne VIC AustraliaSydney Medical School The University of Sydney NSW AustraliaQueensland Pediatric Cardiac Service Queensland Children’s Hospital Brisbane QLD AustraliaPediatric and Congenital Cardiac Service Starship Children’s Hospital Auckland New ZealandSydney Medical School The University of Sydney NSW AustraliaHeart Centre for Children The Sydney Children’s Hospitals Network Sydney NSW AustraliaSydney Medical School The University of Sydney NSW AustraliaHeart and Mind Wellbeing Center Heart Institute and Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine Cincinnati OH United StatesBackground To characterize global and health‐related quality of life (QOL) among adults with Fontan physiology enrolled in the Australian and New Zealand Fontan Registry (ANZFR), and identify sociodemographic, clinical, psychological, and relational factors associated with outcomes. Methods and Results Using a cross‐sectional survey design, 66 adults with Fontan physiology (58% women; mean age, 29.6±7.7 years; range, 18–50 years) completed validated self‐report measures. Health‐related QOL was assessed using the Pediatric Quality of Life Inventory, and global QOL was assessed using a visual analog scale (0–10). Participants reported lower total health‐related QOL (P<0.001), as well as lower physical (P<0.001) and social (P=0.002) functioning compared with normative data. Median global QOL was 7.0 (interquartile range: 5.0‐8.0) and most participants (71%) rated their QOL ≥6. For health‐related QOL, age, sex, university education, and length of hospital stay in the past 12 months explained 27% of the variance in scores, while general psychological stress, medical traumatic stress, communication problems, and access to emotional support explained a further 44% of variance (final model: 71% of variance explained). For global QOL, sociodemographic and clinical factors explained 20% of the variance in scores, while psychological stress and sense of coherence explained a further 24% (final model: 44% of variance explained). Conclusions Adults with Fontan physiology reported lower overall health‐related QOL compared with community‐based norms. Variance in QOL outcomes were predominantly attributable to psychological and relational factors. Tailored screening and assessment to identify Fontan patients at greatest risk of lower QOL, and a proactive approach to supportive care, are needed.https://www.ahajournals.org/doi/10.1161/JAHA.123.033818congenital heart diseaseFontanpsychological healthquality of life
spellingShingle Kate H. Marshall
Yves d'Udekem
David S. Winlaw
Diana Zannino
David S. Celermajer
Robert Justo
Ajay Iyengar
Rachael Cordina
Gary F. Sholler
Susan R. Woolfenden
Nadine A. Kasparian
Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
congenital heart disease
Fontan
psychological health
quality of life
title Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study
title_full Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study
title_fullStr Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study
title_full_unstemmed Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study
title_short Quality of Life and Well‐Being in Adults With Fontan Physiology: Findings From the Australian and New Zealand Fontan Registry Quality of Life Study
title_sort quality of life and well being in adults with fontan physiology findings from the australian and new zealand fontan registry quality of life study
topic congenital heart disease
Fontan
psychological health
quality of life
url https://www.ahajournals.org/doi/10.1161/JAHA.123.033818
work_keys_str_mv AT katehmarshall qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT yvesdudekem qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT davidswinlaw qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT dianazannino qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT davidscelermajer qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT robertjusto qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT ajayiyengar qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT rachaelcordina qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT garyfsholler qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT susanrwoolfenden qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy
AT nadineakasparian qualityoflifeandwellbeinginadultswithfontanphysiologyfindingsfromtheaustralianandnewzealandfontanregistryqualityoflifestudy