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Canadian Journal of Cardiology

Exercise Training for People Living With Fontan Circulation: An Underutilized Intervention

  • Michael Khoury
    Correspondence
    Corresponding author: Dr Michael Khoury, Department of Pediatrics, Stollery Children’s Hospital, 8440-112th Street NW, Edmonton, Alberta T6G 2B7, CanadaTel.: +1-780-407-8361; fax: +1-780-407-3954.
    Affiliations
    Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
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  • Rachael Cordina
    Affiliations
    Sydney Medical School, University of Sydney, Sidney, New South Wales, Australia

    Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
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Published:January 15, 2022DOI:https://doi.org/10.1016/j.cjca.2022.01.008

      Abstract

      Surgical repair for children born with single-ventricle congenital heart disease, culminating in the Fontan operation, has resulted in dramatic improvements in survival; children born with these lesions are now typically expected to survive well into adulthood. Most, but not all, patients with Fontan circulation have reduced exercise capacity compared with the general population that, in turn, is associated with increased cardiovascular morbidity and mortality. The cause of reduced exercise capacity is multifactorial. A significant contributor is the absence of a subpulmonary ventricular pump, which limits preload and appropriate cardiac output augmentation to meet the increased metabolic demands that occur with exercise. Although in its infancy relative to adults with acquired heart disease, the evidence to date suggests that exercise interventions to improve exercise capacity and Fontan physiology in children and adults with Fontan circulation are safe, effective, and well tolerated. However, many knowledge gaps remain, including a detailed understanding of the unique physiological adaptations that occur, the optimal approach to exercise in this population, and the effectiveness of home-based interventions using telemedicine and remote physiological monitoring technologies. Furthermore, the long-term impact of such interventions on the Fontan-cardiovascular system, physical activity levels, health-related quality of life, and late cardiovascular morbidity and mortality are not well characterized. In this review, we outline the factors associated with reduced exercise capacity in persons with Fontan circulation, review the experience to date of dedicated interventions to improve exercise capacity, and highlight the current knowledge gaps in the field and priorities for further study.

      Résumé

      La réparation chirurgicale pédiatrique des cœurs univentriculaires, dont l’intervention de Fontan constitue la dernière étape, a donné lieu à une amélioration spectaculaire de la survie : de nos jours, l’espérance de vie des enfants présentant ces malformations cardiaques congénitales s’étend généralement bien au-delà du début de l’âge adulte. Chez la plupart des patients, la circulation de Fontan limite la tolérance à l’effort comparativement à la population générale, ce qui se trouve associé à une augmentation de la morbidité et de la mortalité cardiovasculaires. Plusieurs facteurs contribuent à limiter la tolérance à l’effort dans ce contexte. Un des principaux facteurs en cause est l’absence de pompe ventriculaire sous-pulmonaire, qui limite la précharge et l’augmentation du débit cardiaque nécessaire pour répondre aux demandes métaboliques accrues survenant à l’effort. Bien qu’elles soient relativement plus récentes que celles visant les adultes atteints de cardiopathies acquises, les données probantes recueillies à ce jour chez les enfants et les adultes ayant subi l’intervention de Fontan donnent à penser que les interventions de rééducation par l’exercice visant à améliorer la tolérance à l’effort et les caractéristiques physiologiques de la circulation de Fontan sont sûres, efficaces et bien tolérées. Toutefois, nos connaissances demeurent grandement lacunaires, notamment au chapitre de la compréhension fine des adaptations physiologiques uniques observées chez les sujets ayant subi l’intervention de Fontan, de l’approche optimale à adopter en rééducation par l’exercice chez ces derniers et de l’efficacité des interventions à domicile réalisées grâce à la télémédecine et aux technologies de télésurveillance physiologique. En outre, l’effet à long terme de telles interventions sur l’appareil cardiovasculaire, les niveaux d’activité physique, la qualité de vie liée à la santé, la morbidité et la mortalité cardiovasculaires tardives ne sont pas bien caractérisés en l’occurrence. Dans le présent article de synthèse, nous décrivons les facteurs associés à la limitation de la tolérance à l’effort chez les sujets ayant subi l’intervention de Fontan, nous passons en revue l’expérience acquise à ce jour au chapitre des interventions axées sur l’amélioration de la tolérance à l’effort et nous soulignons les lacunes actuelles dans nos connaissances en la matière et les aspects qui devraient être abordés en priorité dans le cadre des études à venir.
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