Open Access

ARTICLE

A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation

Christina E. Holbein¹, Nicholas D. Fogleman^1,2, Kevin Hommel¹, Silke Apers³, Jessica Rassart³, Philip Moons^3,4, Koen Luyckx³, Maayke A. Sluman⁵, Junko Enomoto⁶, Bengt Johansson⁷, Hsiao-Ling Yang⁸, Mikael Dellborg^4,9, Raghavan Subramanyan¹⁰, Jamie L. Jackson¹¹, Werner Budts^3,12, Adrienne H. Kovacs¹³, Stacey Morrison¹, Martha Tomlin¹, Kathy Gosney¹, Alexandra Soufi¹⁴, Katrine Eriksen¹⁵, Corina Thomet^3,16, Malin Berghammer^4,17, Luis Alday¹⁸, Edward Callus¹⁹, Susan M Fernandes²⁰, Maryanne Caruana²¹, Samuel Menahem²², Stephen C. Cook²³, Gwen R. Rempel²⁴, Kamila White²⁵, Paul Khairy²⁶, Shelby Kutty²⁷, Gruschen Veldtman¹

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 Umeå University, Umeå, 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, Trollhättan, Sweden
18 Hospital de Ninos, C ~ ordoba, 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

* Corresponding Author: Gruschen Veldtman, Adolescent and Adult Congenital Heart Program, 3333 Burnett Avenue, Cincinnati, Ohio. Email:

Congenital Heart Disease 2018, 13(3), 392-400. https://doi.org/10.1111/chd.12583

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 ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between 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 and 258 ASD/VSD) ages 18-83 years.
Outcome Measures: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively.
Results: Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p 5 <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association 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 of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

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