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

Karolijn Dulfer, Sjoerd S M Bossers, Elisabeth M W J Utens, Nienke Duppen, Irene M. Kuipers, Livia Kapusta, Gabrielle van Iperen-Schutte, Michiel Schokking, Arend D J ten Harkel, Tim Takken, Willem A. Helbing*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Purpose: 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. Methods: 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. Results: 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. Conclusions: 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.

Original languageEnglish
Pages (from-to)459–468
JournalCardiology in the Young
Issue number3
Publication statusPublished - Mar 2016


  • congenital heart disease
  • Fontan circulation
  • quality of life


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