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

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. 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 patie