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.

Additional Metadata
Keywords congenital heart disease, Fontan circulation, quality of life
Persistent URL dx.doi.org/10.1017/S1047951115000426, hdl.handle.net/1765/81351
Journal Cardiology in the Young
Dulfer, K, Bossers, S.S.M, Utens, E.M.W.J, Duppen, N, Kuipers, I.M, Kapusta, L, … Helbing, W.A. (2016). Does functional health status predict health-related quality of life in children after Fontan operation?. Cardiology in the Young, 26(3), 459–468. doi:10.1017/S1047951115000426