Advertisement
Canadian Journal of Cardiology

The Unique Clinical Phenotype and Exercise Adaptation of Fontan Patients With Normal Exercise Capacity

  • Adam W. Powell
    Correspondence
    Corresponding author: Dr Adam W. Powell, 3333 Burnett Ave, MLC 2003, Cincinnati, Ohio 45229-3026, USA. Tel.: +1-513-636-4432; fax: +1-513-636-6952.
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
  • Clifford Chin
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
  • Tarek Alsaied
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
  • Harry B. Rossiter
    Affiliations
    Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, California, USA

    Faculty of Biological Sciences, University of Leeds, Leeds, United Kingdom
    Search for articles by this author
  • Samuel Wittekind
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
  • Wayne A. Mays
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
  • Adam Lubert
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
  • Gruschen Veldtman
    Affiliations
    Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

    The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA
    Search for articles by this author
Published:November 15, 2019DOI:https://doi.org/10.1016/j.cjca.2019.11.006

      Abstract

      Background

      Exercise limitation is almost universal among Fontan patients. Identifying unique clinical features in the small fraction of Fontan patients with normal exercise capacity (high-capacity Fontan [HCF]) provides potential to inform clinical strategies for those with low exercise capacity (usual Fontan).

      Methods

      We performed a retrospective chart review of all patients with single-ventricle physiology palliated with a Fontan operation who underwent incremental cardiopulmonary exercise testing at Cincinnati Children’s Hospital Medical Center from 2013 to 2018. Comparison was between patients with peak oxygen uptake < vs ≥ 80% predicted.

      Results

      A total of 22 of 112 patients were classified as HCF (68% were female; aged 18 ± 7 years). During incremental exercise, peak oxygen uptake (86.1% ± 6.1% vs 62% ± 12.2% predicted; P < 0.001) was greater in HCF vs usual Fontan despite similar chronotropic impairment, resulting in a greater oxygen pulse in HCF. Pulmonary function, breathing reserve, and ventilatory equivalent for CO2 output slope were not different between groups. Those in the HCF group were more likely to self-report exercise ≥ 4 days/week for at least 30 minutes (77% vs 10%, P < 0.001), have normal systolic function (95% vs 74%, P = 0.003), have fewer postoperative complications (8% vs 36%, P = 0.04), and have shorter post-Fontan length of stay (8 ± 2.8 vs 12.4 ± 0.9 days, P = 0.04).

      Conclusions

      Approximately 1 in 5 Fontan patients who undergo cardiopulmonary exercise testing have normal exercise capacity despite chronotropic impairment. This implies a better preserved stroke volume, perhaps due to greater muscle pump-mediated preload. Additionally, a complicated perioperative Fontan course is associated with eventual impaired functional capacity.

      Résumé

      Contexte

      Les patients ayant subi une intervention de Fontan ont presque tous une capacité à l’effort réduite. L’étude des caractéristiques cliniques uniques de la petite proportion de patients ayant subi une telle intervention qui conservent une capacité à l’effort normale (Fontan associé à une capacité à l’effort supérieure, FCPS) pourrait éclairer les stratégies cliniques auprès des patients dont la capacité à l’effort est réduite (Fontan type, FT).

      Méthodologie

      Nous avons examiné rétrospectivement les dossiers médicaux de tous les patients présentant une physiologie univentriculaire à laquelle on a remédié par une intervention de Fontan et ayant subi une épreuve d’effort cardiopulmonaire avec augmentation progressive de la charge au centre médical du Children’s Hospital de Cincinnati entre 2013 et 2018. On a comparé les patients ayant une consommation maximale d’oxygène < 80 % de la valeur prédite à ceux dont la consommation était ≥ 80 %.

      Résultats

      Au total, 22 patients sur 112 ont été classés comme FCPS (68 % d’entre eux étaient de sexe féminin; l’âge moyen était de 18 ± 7 ans). Durant l’augmentation de la charge d’effort, la consommation maximale d’oxygène (86,1 % ± 6,1 % vs 62 % ± 12,2 % de la valeur prédite; p < 0,001) était plus élevée chez les patients FCPS que chez les patients FT malgré une insuffisance chronotropique comparable dans les deux groupes; le pouls d’oxygène était donc plus élevé chez les patients FCPS. La fonction pulmonaire, la réserve respiratoire et la pente de l’équivalent ventilatoire pour le CO2 étaient comparables dans les deux groupes. Les patients du groupe FCPS étaient plus susceptibles de déclarer faire de l’exercice ≥ 4 jours/semaine pendant au moins 30 minutes (77 % vs 10 %, p < 0,001), d’avoir une fonction systolique normale (95 % vs 74 %, p = 0,003), de subir moins de complications postopératoires (8 % vs 36 %, p = 0,04), et d’être hospitalisés moins longtemps après l’intervention de Fontan (8 ± 2,8 vs 12,4 ± 0,9 jours, p = 0,04).

      Conclusions

      Environ un patient ayant subi une intervention de Fontan sur cinq soumis à une épreuve d’effort cardiopulmonaire présente une capacité à l’effort normale malgré une insuffisance chronotropique. Ce résultat traduit un plus grand volume d’éjection systolique préservé, peut-être en raison d’une précharge cardiaque plus importante. Par ailleurs, les complications périopératoires liées à l’intervention de Fontan sont associées à une capacité fonctionnelle potentiellement réduite.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Gewillig M.
        • Brown S.C.
        The Fontan circulation after 45 years: update in physiology.
        Heart. 2016; 102: 1081-1086
        • Gewillig M.
        • Brown S.C.
        • Eyskens B.
        • et al.
        The Fontan circulation: who controls cardiac output?.
        Interact Cardiovasc Thorac Surg. 2010; 10: 428-433
        • Fredriksen P.M.
        • Veldtman G.
        • Hechter S.
        • et al.
        Aerobic capacity in adults with various congenital heart disease.
        Am J Cardiol. 2001; 87: 310-314
        • Kempny A.
        • Dimopoulos K.
        • Uebing A.
        • et al.
        • Reference values for exercise limitations among adults with congenital heart disease
        Relation to activities of daily life-single centre experience and review of the published data.
        Eur Heart J. 2012; 33: 1386-1396
        • Egbe A.C.
        • Driscoll D.J.
        • Khan A.R.
        • et al.
        Cardiopulmonary exercise test in adults with prior Fontan operation: the prognostic value of serial testing.
        Int J Cardiol. 2017; 235: 6-10
        • Cordina R.
        • Plessis K.
        • Tran D.
        • d’Udekem Y.
        Super-Fontan: is it possible?.
        J Thorac Cardiovasc Surg. 2018; 155: 1192-1194
        • Wasserman K.
        • Hansen J.E.
        • Sue D.Y.
        • Casaburi R.
        • Whipp B.J.
        Principles of Exercise Testing and Interpretation: Including Pathophysiology and Clinical Applications.
        3rd ed. Lippincott, Williams & Wilkins, Philadelphia, PA1999
        • Borg G.
        Borg’s Perceived Exertion and Pain Scales.
        Human Kinetics, Champaign, IL1998
        • Cooper D.M.
        • Weiler-Ravell D.
        • Whipp B.J.
        • Wasserman K.
        Aerobic parameters of exercise as a function of body size during growth in children.
        J Appl Physiol. 1984; 56: 628-634
        • Wilkoff B.L.
        • Corey J.
        • Blackburn G.
        A mathematical model of cardiac chronotropic response to exercise.
        J Electrophysiol. 1989; 3: 176-180
        • Beaver W.L.
        • Wasserman K.
        • Whipp B.J.
        A new method for detecting anaerobic threshold by gas exchange.
        J Appl Physiol. 1986; 60: 2020-2027
        • Goldman H.I.
        • Becklake M.R.
        Respiratory function tests: normal values at median altitudes and the prediction of normal results.
        Am Rev Tuberc. 1959; 79: 457-467
        • Campbell S.C.
        A comparison of the maximum voluntary ventilation with the forced expiratory volume in one second.
        J Occup Med. 1982; 24: 531-533
        • Wong J.
        • Pushparajah K.
        • de Vecchi A.
        • et al.
        Pressure-volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitation in cardiac output in children with hypoplastic left heart syndrome.
        Int J Cardiol. 2017; 230: 439-446
        • Diller G.P.
        • Dimopoulos K.
        • Okonko D.
        • et al.
        Heart rate response during exercise predicts survival in adults with congenital heart disease.
        J Am Coll Cardiol. 2006; 48: 1250-1256
        • Cordina R.
        • O’Meagher S.
        • Gould H.
        • et al.
        Skeletal muscle abnormalities and exercise capacity in adults with a Fontan circulation.
        Heart. 2013; 99: 1530-1534
        • Udholm S.
        • Aldweib N.
        • Hjortdal V.E.
        • Veldtman G.R.
        Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review.
        Open Heart. 2018; 5e000812
        • Resenthal M.
        • Bush A.
        • Deanfield J.
        • Redington A.
        Comparison of cardiopulmonary adaption during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures.
        Circulation. 1995; 91: 372-378
        • Shafer K.M.
        • Garcia J.A.
        • Babb T.G.
        • et al.
        The importance of the muscle and ventilatory pumps during exercise in patients without a subpulmonary ventricle (Fontan operation).
        J Am Coll Cardiol. 2012; 60: 2115-2121
        • Turquetto A.L.R.
        • Dos Santos M.R.
        • Sayegh A.L.C.
        • et al.
        Blunted blood supply and underdeveloped skeletal muscle in Fontan patients: the impact on function capacity.
        Int J Cardiol. 2018; 271: 54-59
        • Sandberg C.
        • Crenshaw A.G.
        • Elcadi G.H.
        • et al.
        Slower skeletal muscle oxygenation kinetics in adults with complex congenital heart disease.
        Can J Cardiol. 2019; 35: 1815-1823
        • Laohachai K.
        • Winlaw D.
        • Selvadurai H.
        • et al.
        Inspiratory muscle training is associated with improved muscle strength, resting cardiac output and the ventilatory efficiency of exercise in patients with a Fontan circulation.
        J Am Heart Assoc. 2017; 6
        • Downing T.E.
        • Allen K.Y.
        • Glatz A.C.
        • et al.
        Long-term survival after the Fontan operation: twenty years of experience at a single center.
        J Thorac Cardiovasc Surg. 2017; 154: 243-253
        • Wolff D.
        • van Melle J.P.
        • Dijksta H.
        • et al.
        The Fontan circulation and the liver: a magnetic resonance diffusion-weighted imaging study.
        Int J Cardiol. 2016; 202: 595-600
        • Paridon S.M.
        • Mitchell P.D.
        • Colan S.D.
        • et al.
        A cross sectional study of exercise performance during the first 2 decades of life after the Fontan operation.
        J Am Coll Cardiol. 2008; 52: 99-107
        • Navaratnam D.
        • Fitzsimmons S.
        • Grocott M.
        • et al.
        Exercise-induced systemic venous hypertension in the Fontan circulation.
        Am J Cardiol. 2016; 117: 1667-1671
        • Broda C.R.
        • Sriraman H.
        • Wadhwa D.
        • et al.
        Renal dysfunction is associated with higher central venous pressures in patients with Fontan circulation.
        Congenit Heart Dis. 2018; 13: 602-607
        • Laye M.J.
        • Rector R.S.
        • Borengasser S.J.
        • et al.
        Cessation of daily wheel running differentially alters fat oxidation capacity in liver, muscle and adipose tissue.
        J Appl Physiol. 2009; 106: 161-168
        • Yeh M.P.
        • Gardner R.M.
        • Adams T.D.
        • et al.
        “Anerobic threshold”: problems of determination and validation.
        J Appl Physiol Respir Environ Exerc Physiol. 1983; 55: 1178-1186