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Canadian Journal of Cardiology

Quality of Life and Other Patient-Reported Outcomes Across the Life Span Among People With Fontan Palliation

  • Nadine A. Kasparian
    Correspondence
    Corresponding author: Dr Nadine Kasparian, Cincinnati Children’s Center for Heart Disease and Mental Health, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue (MLC 7039), Cincinnati, Ohio 45229, USA. Tel.: +1-617-838-1202.
    Affiliations
    Center for Heart Disease and Mental Health, Heart Institute and the Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA

    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
    Search for articles by this author
  • Adrienne H. Kovacs
    Affiliations
    Equilibria Psychological Health, Toronto, Ontario, Canada
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      Abstract

      Traditional congenital heart disease (CHD) outcomes include mortality (survival to adulthood and life expectancy) as well as cardiac and noncardiac morbidity. Strategies to identify and manage sequelae have primarily focused on objective data obtained though invasive and noninvasive diagnostic approaches. In contrast, patient-reported outcomes (PROs) provide subjective information, using standardized measures, about patients’ health and well-being as reported directly by patients, without interpretation, interference, or assumptions made by clinicians or others. Selection of PRO measures entails thoughtful consideration of who the individuals being surveyed are, why assessment is occurring (eg, what are the domains of interest; clinical vs research), and what processes are in place for acquisition, administration, interpretation, and response. In this review, we focus on 3 domains of PROs for pediatric and adult patients with Fontan physiology: physical health status, psychological functioning, and quality of life. Infants, children, adolescents, and adults with CHD face a spectrum of challenges that might influence PROs across the life span. In general, patients with Fontan palliation tend to have lower physical health status, experience more psychological distress, and have equivalent or reduced quality of life compared with healthy peers. Herein, we provide an overview of PROs among people with Fontan circulation as a group, yet simultaneously emphasize that the optimal way to understand the experiences of any individual patient is to ask and listen. We also offer clinical and research initiatives to improve the adoption and utility of PROs in CHD settings, which show commitment to capturing, understanding, and responding to the patient voice.

      Résumé

      L’issue classique des cardiopathies congénitales comprend le décès (survie jusqu’à l’âge adulte et espérance de vie écourtées) ainsi que la morbidité cardiaque et non cardiaque. Les stratégies de dépistage et de prise en charge des séquelles s’articulent essentiellement autour de données objectives obtenues par le biais d’approches diagnostiques invasives et non invasives. En revanche, les données d’autodéclaration des patients fournissent des informations subjectives sur la santé et le bien-être de ces derniers, au moyen de mesures normalisées, de façon directe et sans interprétation, intervention ni hypothèses de la part de cliniciens ou d’autres personnes. La sélection des paramètres d’autodéclaration des patients doit être mûrie et nécessite que l’on sache qui sont les répondants, pourquoi l’évaluation est effectuée (notamment en ce qui concerne la nature des domaines abordés, dans une optique clinique ou de recherche) et quels processus sont en place en matière de collecte, d’administration, d’interprétation et de réaction. Dans le présent article de synthèse, nous nous concentrons sur trois domaines d’autodéclaration des patients pédiatriques et adultes ayant subi l’intervention de Fontan : état de santé physique, bien-être psychologique et qualité de vie. Les nourrissons, les enfants, les adolescents et les adultes atteints de cardiopathies congénitales doivent composer avec un large éventail d’enjeux susceptibles d’influer sur les données d’autodéclaration tout au long de leur vie. Généralement, les patients qui ont subi l’intervention de Fontan tendent à présenter un moins bon état de santé physique, à éprouver plus de détresse psychologique et à avoir une qualité de vie équivalente ou inférieure comparativement à des pairs en bonne santé. Nous donnons ici un aperçu des données d’autodéclaration de personnes ayant subi l’intervention de Fontan, tous cas confondus. Nous soulignons également que la façon optimale de comprendre ce que vit un patient consiste à interroger et à écouter ce dernier. Nous proposons aussi des initiatives à l’intention des cliniciens et des chercheurs en vue d’accroître l’adoption et l’utilité des méthodes d’autodéclaration touchant les cardiopathies congénitales, dans l’optique d’une volonté affichée d’écoute, de compréhension et de réactivité à l’endroit du patient.
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