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

Published online by Cambridge University Press:  23 April 2015

Karolijn Dulfer ,
Sjoerd S. M. Bossers ,
Elisabeth M. W. J. Utens ,
Nienke Duppen ,
Irene M. Kuipers ,
Livia Kapusta ,
Gabrielle van Iperen ,
Michiel Schokking ,
Arend D. J. ten Harkel  and
Tim Takken
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Karolijn Dulfer
Affiliation:
Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Centre–Sophia Children’s Hospital, Rotterdam, the Netherlands
Sjoerd S. M. Bossers
Affiliation:
Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Rotterdam, the Netherlands
Elisabeth M. W. J. Utens
Affiliation:
Department of Child and Adolescent Psychiatry/Psychology, Erasmus Medical Centre–Sophia Children’s Hospital, Rotterdam, the Netherlands
Nienke Duppen
Affiliation:
Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Rotterdam, the Netherlands
Irene M. Kuipers
Affiliation:
Department of Paediatrics, Division of Cardiology, Academic Medical Centre, Amsterdam, the Netherlands
Livia Kapusta
Affiliation:
Department of Paediatrics, Division of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands Department of Paediatrics, Pediatric Cardiology Unit, Tel-Aviv Sourasky Medical Centre, Tel Aviv, Israel
Gabrielle van Iperen
Affiliation:
Department of Paediatrics, Division of Cardiology, University Medical Centre Utrecht – Wilhelmina Children’s Hospital, Utrecht, the Netherlands
Michiel Schokking
Affiliation:
Department of Paediatrics, Division of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands
Arend D. J. ten Harkel
Affiliation:
Department of Paediatrics, Division of Cardiology, Leiden University Medical Centre, Leiden, the Netherlands
Tim Takken
Affiliation:
Child Development and Exercise Centre, University Medical Centre Utrecht – Wilhelmina Children’s Hospital, Utrecht, the Netherlands
Willem A. Helbing*
Affiliation:
Department of Paediatrics, Division of Cardiology, Erasmus Medical Centre – Sophia Children’s Hospital, Rotterdam, the Netherlands
*
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: w.a.helbing@erasmusmc.nl
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Abstract

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.

Keywords

Fontan circulationcongenital heart diseasequality of life
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Supplement 3 , March 2016 , pp. 459 - 468
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Copyright
© Cambridge University Press 2015 

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Footnotes

*

Both authors contributed equally.

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