Phenotypes of adults with congenital heart disease around the globe: a cluster analysis.


Callus E(1)(2), Pagliuca S(1), Boveri S(3), Ambrogi F(4), Luyckx K(5)(6), Kovacs AH(7)(8), Apers S(9), Budts W(10)(11), Enomoto J(12), Sluman MA(13)(14), Wang JK(15), Jackson JL(16), Khairy P(17), Cook SC(18), Chidambarathanu S(19), Alday L(20), Eriksen K(21), Dellborg M(22)(23)(24), Berghammer M(24)(25)(26), Johansson B(27), Mackie AS(28), Menahem S(29), Caruana M(30), Veldtman G(31), Soufi A(32), Fernandes SM(33), White K(34), Kutty S(35)(36), Moons P(37)(38)(39)(40); APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).
Author information:
(1)Clinical Psychology Service, IRCCS Policlinico San Donato, Milan, Italy.
(2)Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy.
(3)Scientific Directorate, IRCCS Policlinico San Donato, Milan, Italy.
(4)Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
(5)KU Leuven School Psychology and Development in Context, KU Leuven, Leuven, Belgium.
(6)UNIBS, University of the Free State, Bloemfontein, South Africa.
(7)Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, Canada.
(8)Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, USA.
(9)KU Leuven Department of Public Health and Primary Care, KU Leuven, Kapucijnenvoer 35, Box 7001, 3000, Leuven, Belgium.
(10)Division of Congenital and Structural Cardiology, University Hospitals Leuven, Leuven, Belgium.
(11)KU Leuven Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
(12)Department of Education, Toyo University, Tokyo, Japan.
(13)Coronel Institute of Occupational Health, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
(14)Department of Cardiology, Jeroen Bosch Hospital, 's Hertogenbosch, The Netherlands.
(15)Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
(16)Center for Biobehavioral Health, Nationwide Children's Hospital, Columbus, OH, USA.
(17)Adult Congenital Heart Center, Montreal Heart Institute, Université de Montréal, Montreal, Canada.
(18)Adult Congenital Heart Disease Center, Helen DeVos Children's Hospital, Grand Rapids, MI, USA.
(19)Pediatric Cardiology, Frontier Lifeline Hospital
(Dr. K. M. Cherian Heart Foundation), Chennai, India.
(20)Division of Cardiology, Hospital de Niños, Córdoba, Argentina.
(21)Adult Congenital Heart Disease Center, Oslo University Hospital - Rikshospitalet, Oslo, Norway.
(22)Adult Congenital Heart Unit, Sahlgrenska University Hospital/Östra, Gothenburg, Sweden.
(23)Institute of Medicine, The Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden.
(24)Centre for Person-Centred Care
(GPCC), University of Gothenburg, Gothenburg, Sweden.
(25)Department of Health Sciences, University West, Trollhättan, Sweden.
(26)Department of Paediatrics, Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden.
(27)Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
(28)Division of Cardiology, Stollery Children's Hospital, University of Alberta, Edmonton, Canada.
(29)Monash Heart, Monash Medical Centre, Monash University, Melbourne, Australia.
(30)Department of Cardiology, Mater Dei Hospital, Birkirkara Bypass, Malta.
(31)Adult Congenital Heart Disease Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
(32)Department of Congenital Heart Disease, Louis Pradel Hospital, Hospices civils de Lyon, Lyon, France.
(33)Adult Congenital HeartProgram at Stanford, Lucile Packard Children's Hospital Stanford and Stanford Health Care, Palo Alto, CA, USA.
(34)Adult Congenital Heart Disease Center, Washington University and Barnes Jewish Heart & Vascular Center, University of Missouri, Saint Louis, MO, USA.
(35)Adult Congenital Heart Disease Center University of Nebraska Medical Center/ Children's Hospital and Medical Center, Omaha, NE, USA.
(36)Taussig Heart Center, Johns Hopkins School of Medicine, Baltimore, USA.
(37)KU Leuven Department of Public Health and Primary Care, KU Leuven, Kapucijnenvoer 35, Box 7001, 3000, Leuven, Belgium. [Email]
(38)Centre for Person-Centred Care
(GPCC), University of Gothenburg, Gothenburg, Sweden. [Email]
(39)Institute of Health and Care Sciences, University of Gothenburg, Gothenburg, Sweden. [Email]
(40)Department of Paediatrics and Child Health, University of Cape Town, Cape Town, South Africa. [Email]


OBJECTIVE: To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). METHODS: This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013-2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0-100) measuring QoL. RESULTS: 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. CONCLUSIONS: This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care.