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Does functional health status predict health-related quality of life in children after Fontan operation?

  • Karolijn Dulfer (a1), Sjoerd S. M. Bossers (a2), Elisabeth M. W. J. Utens (a1), Nienke Duppen (a2), Irene M. Kuipers (a3), Livia Kapusta (a4) (a5), Gabrielle van Iperen (a6), Michiel Schokking (a4), Arend D. J. ten Harkel (a7), Tim Takken (a8) and Willem A. Helbing (a2)...



It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status – medical history and present medical status – on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents.


We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years.

Functional health status was assessed as medical history – that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures – and present medical status – assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form.


In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains.


Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.


Corresponding author

Correspondence to: W. A. Helbing, Department of Pediatric Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Sp-2429, PO Box 2060, 3000 CB Rotterdam, the Netherlands. Tel: +31 10 7036264; Fax: +31 10 7036772; E-mail:


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Both authors contributed equally.



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Does functional health status predict health-related quality of life in children after Fontan operation?

  • Karolijn Dulfer (a1), Sjoerd S. M. Bossers (a2), Elisabeth M. W. J. Utens (a1), Nienke Duppen (a2), Irene M. Kuipers (a3), Livia Kapusta (a4) (a5), Gabrielle van Iperen (a6), Michiel Schokking (a4), Arend D. J. ten Harkel (a7), Tim Takken (a8) and Willem A. Helbing (a2)...


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