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A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation
Authors:Christina E. Holbein PhD  Nicholas D. Fogleman MS  Kevin Hommel PhD  Silke Apers PhD  Jessica Rassart MSc  Philip Moons PhD  RN  Koen Luyckx PhD  Maayke A. Sluman MD  Junko Enomoto PhD  Bengt Johansson MD  PhD  Hsiao‐Ling Yang PhD  RN  Mikael Dellborg MD  PhD  Raghavan Subramanyan MD  Jamie L. Jackson PhD  Werner Budts MD  PhD  Adrienne H. Kovacs PhD  Stacey Morrison PsyD  Martha Tomlin Cnp  Kathy Gosney Mssw  Alexandra Soufi MD  Katrine Eriksen MSc  Corina Thomet MSc  Malin Berghammer PhD  Luis Alday MD  Edward Callus PhD  Susan M Fernandes PA‐C  Maryanne Caruana MD  Samuel Menahem MD  Stephen C. Cook MD  Gwen R. Rempel PhD  RN  Kamila White PhD  Paul Khairy MD  PhD  Shelby Kutty MD  Gruschen Veldtman MBChB  Frcp  APPROACH‐IS consortium  the International Society for Adult Congenital Heart Disease
Affiliation:1. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA;2. University of Louisville, Louisville, Kentucky, USA;3. KU Leuven, Leuven, Belgium;4. University of Gothenburg, Gothenburg, Sweden;5. Amsterdam Medical Center, Amsterdam, The Netherlands;6. Chiba Cardiovascular Center, Chiba, Japan;7. Ume? University, Ume?, Sweden;8. National Taiwan University, Taipei, Taiwan;9. Adult Congenital Heart Unit, Sahlgrenska University Hospital, Gothenburg, Sweden;10. Frontier Lifeline Hospital, Dr. K. M. Cherian Heart Foundation, Chennai, India;11. Nationwide Children's Hospital, Columbus, Ohio, USA;12. University Hospitals Leuven and Department of Cardiovascular Sciences, Leuven, Belgium;13. Oregon Health & Science University, Portland, Oregon, USA;14. Hospital Louis Pradel, Lyon, France;15. Oslo University Hospital, Oslo, Norway;16. University Hospital Bern, University of Bern, Bern, Switzerland;17. University West, Trollh?ttan, Sweden;18. Hospital de Ni?os, Córdoba, Argentina;19. IRCCS Policlinico San Donato Hospital, Milan, Italy;20. Stanford University, Palo Alto, California, USA;21. Mater Dei Hospital, Msida, Malta;22. Monash Medical Center, Melbourne, Australia;23. Helen DeVos Children's Hospital, Grand Rapids, Michigan, USA;24. University of Alberta, Edmonton, Canada;25. Washington University and Barnes Jewish Heart & Vascular Center, University of Missouri, Saint Louis, Missouri, USA;26. Montreal Heart Institute, Montreal, Canada;27. Children's Hospital & Medical Center, Omaha, Nebraska, USA
Abstract:Objective: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricularseptal defects [VSD]). Second, to explore illness perceptions as a mediator of the associationbetween congenital heart disease (CHD) diagnosis and QOL.
Design: Cross-sectional observational study.
Setting: Twenty-four cardiology centers from 15 countries across five continents.
Patients: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and258 ASD/VSD) ages 18-83 years.
Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With LifeScale and the Brief Illness Perceptions Questionnaire, respectively.
Results: Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p 5 <.001) andmore negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients withASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York HeartAssociation functional class, path analyses revealed a significant mediation model, αβ = 0.15,p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions.
Conclusions: The Fontan sample’s more negative perceptions of CHD were likely a reflection oflife with a more complex defect. Illness perceptions appear to account for unique differences inQOL between groups of varying CHD complexity. Psychosocial screening and interventions may beimportant treatment components for patients with CHD, particularly those with Fontan circulations.
Keywords:congenital heart disease  Fontan circulation  illness perceptions  quality of life
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