The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings
Introduction Advances in the care of patients with single-ventricle congenital heart disease have led to a new generation of individuals living with a Fontan circulation. For people with Fontan physiology, physical, psychological and neurodevelopmental challenges are common. The objective of this st...
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BMJ Publishing Group
2022-09-01
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| Series: | BMJ Open |
| Online Access: | https://bmjopen.bmj.com/content/12/9/e065726.full |
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| author | Daniel S J Costa Kim Dalziel Yves d’Udekem David S Winlaw David S Celermajer Nadine A Kasparian Diana Zannino Rachel Bishop Kate H Marshall Susan R Woolfenden Gary F Sholler |
| author_facet | Daniel S J Costa Kim Dalziel Yves d’Udekem David S Winlaw David S Celermajer Nadine A Kasparian Diana Zannino Rachel Bishop Kate H Marshall Susan R Woolfenden Gary F Sholler |
| author_sort | Daniel S J Costa |
| collection | DOAJ |
| description | Introduction Advances in the care of patients with single-ventricle congenital heart disease have led to a new generation of individuals living with a Fontan circulation. For people with Fontan physiology, physical, psychological and neurodevelopmental challenges are common. The objective of this study is to describe and develop a deeper understanding of the factors that contribute to quality of life (QOL) among children, adolescents and adults living with a Fontan circulation across Australia and New Zealand, their parents and siblings.Methods and Analysis This article presents the protocol for the Australian and New Zealand Fontan Registry (ANZFR) QOL Study, a cross-sectional, population-based study designed to examine QOL among people of all ages with a Fontan circulation, their parents and siblings. Study eligibility criteria includes (1) individuals with a Fontan circulation aged ≥6 years, at least 12 months post-Fontan procedure and enrolled in the ANZFR; (2) parents of individuals enrolled in the ANZFR; and (3) siblings aged ≥6 years of an individual enrolled in the ANZFR. A novel, online research platform is used to distribute personalised assessments tailored to participant age and developmental stage. A suite of validated psychometric self-report and parent-proxy report instruments capture potential correlates and predictors of QOL, including symptoms of psychological distress, personality attributes, coping and cognitive appraisals, family functioning, healthcare experiences and costs, access to emotional support and socioeconomic factors. Clinical characteristics are captured via self-report and parent-proxy report, as well as the ANZFR. Descriptive analyses and multilevel models will be used to examine QOL across groups and to investigate potential explanatory variables.Ethics and Dissemination Approval has been obtained from all relevant Human Research Ethics Committees (HRECs), including the Sydney Children’s Hospitals Network and the Royal Children’s Hospital Melbourne HRECs. Study findings will be published in peer-reviewed journals and presented at national and international meetings and seminars. |
| format | Article |
| id | doaj-art-fa4351430c4644ee82d11d1c2aff9635 |
| institution | Kabale University |
| issn | 2044-6055 |
| language | English |
| publishDate | 2022-09-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | BMJ Open |
| spelling | doaj-art-fa4351430c4644ee82d11d1c2aff96352025-02-01T19:20:10ZengBMJ Publishing GroupBMJ Open2044-60552022-09-0112910.1136/bmjopen-2022-065726The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblingsDaniel S J Costa0Kim Dalziel1Yves d’Udekem2David S Winlaw3David S Celermajer4Nadine A Kasparian5Diana Zannino6Rachel Bishop7Kate H Marshall8Susan R Woolfenden9Gary F Sholler10School of Psychology, The University of Sydney, Sydney, New South Wales, AustraliaHealth Economics Unit, Centre for Health Policy, University of Melbourne, Melbourne, Victoria, AustraliaDepartment of Cardiac Surgery, Royal Children’s Hospital, Melbourne, Victoria, AustraliaCardiothoracic Surgery, The Heart Institute, Cincinnati Children’s Hospital Medical Center and the University of Cincinnati College of Medicine, Cincinnati, Ohio, USADepartment of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, AustraliaDiscipline of Paediatrics, School of Women`s and Children`s Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, AustraliaClinical Epidemiology and Biostatistics Unit, Murdoch Children`s Research Institute, Parkville, Victoria, AustraliaAustralian and New Zealand Fontan Registry, Melbourne, Victoria, AustraliaHeart Centre for Children, The Sydney Children`s Hospitals Network, Sydney, New South Wales, AustraliaDiscipline of Paediatrics and Child Health, School of Clinical Medicine, University of New South Wales, Sydney, New South Wales, AustraliaHeart Centre for Children, The Sydney Children`s Hospitals Network, Sydney, New South Wales, AustraliaIntroduction Advances in the care of patients with single-ventricle congenital heart disease have led to a new generation of individuals living with a Fontan circulation. For people with Fontan physiology, physical, psychological and neurodevelopmental challenges are common. The objective of this study is to describe and develop a deeper understanding of the factors that contribute to quality of life (QOL) among children, adolescents and adults living with a Fontan circulation across Australia and New Zealand, their parents and siblings.Methods and Analysis This article presents the protocol for the Australian and New Zealand Fontan Registry (ANZFR) QOL Study, a cross-sectional, population-based study designed to examine QOL among people of all ages with a Fontan circulation, their parents and siblings. Study eligibility criteria includes (1) individuals with a Fontan circulation aged ≥6 years, at least 12 months post-Fontan procedure and enrolled in the ANZFR; (2) parents of individuals enrolled in the ANZFR; and (3) siblings aged ≥6 years of an individual enrolled in the ANZFR. A novel, online research platform is used to distribute personalised assessments tailored to participant age and developmental stage. A suite of validated psychometric self-report and parent-proxy report instruments capture potential correlates and predictors of QOL, including symptoms of psychological distress, personality attributes, coping and cognitive appraisals, family functioning, healthcare experiences and costs, access to emotional support and socioeconomic factors. Clinical characteristics are captured via self-report and parent-proxy report, as well as the ANZFR. Descriptive analyses and multilevel models will be used to examine QOL across groups and to investigate potential explanatory variables.Ethics and Dissemination Approval has been obtained from all relevant Human Research Ethics Committees (HRECs), including the Sydney Children’s Hospitals Network and the Royal Children’s Hospital Melbourne HRECs. Study findings will be published in peer-reviewed journals and presented at national and international meetings and seminars.https://bmjopen.bmj.com/content/12/9/e065726.full |
| spellingShingle | Daniel S J Costa Kim Dalziel Yves d’Udekem David S Winlaw David S Celermajer Nadine A Kasparian Diana Zannino Rachel Bishop Kate H Marshall Susan R Woolfenden Gary F Sholler The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings BMJ Open |
| title | The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings |
| title_full | The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings |
| title_fullStr | The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings |
| title_full_unstemmed | The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings |
| title_short | The Australian and New Zealand Fontan Registry Quality of Life Study: Protocol for a population-based assessment of quality of life among people with a Fontan circulation, their parents, and siblings |
| title_sort | australian and new zealand fontan registry quality of life study protocol for a population based assessment of quality of life among people with a fontan circulation their parents and siblings |
| url | https://bmjopen.bmj.com/content/12/9/e065726.full |
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