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Long-term outcomes following Fontan takedown in Australia and New Zealand

  • Supreet P. Marathe
    Affiliations
    Queensland Paediatric Cardiac Services, Queensland Children's Hospital, Brisbane, Australia

    School of Clinical Medicine, Children's Health Queensland Clinical Unit, University of Queensland, Brisbane, Australia
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  • Ajay J. Iyengar
    Affiliations
    Royal Children's Hospital, Melbourne, Australia

    Green Lane Paediatric and Congenital Cardiac Service, Starship Hospital, Auckland, New Zealand

    Murdoch Children's Research Institute, Melbourne, Australia

    Faculty of Medicine, Department of Paediatrics, University of Melbourne, Melbourne, Australia
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  • Kim S. Betts
    Affiliations
    Department of Epidemiology, Institute for Social Science Research, University of Queensland, Brisbane, Australia
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  • Karin du Plessis
    Affiliations
    Murdoch Children's Research Institute, Melbourne, Australia

    Faculty of Medicine, Department of Paediatrics, University of Melbourne, Melbourne, Australia
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  • Gananjay G. Salve
    Affiliations
    Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia
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  • Robert N. Justo
    Affiliations
    Queensland Paediatric Cardiac Services, Queensland Children's Hospital, Brisbane, Australia

    School of Clinical Medicine, Children's Health Queensland Clinical Unit, University of Queensland, Brisbane, Australia
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  • Prem Venugopal
    Affiliations
    Queensland Paediatric Cardiac Services, Queensland Children's Hospital, Brisbane, Australia

    School of Clinical Medicine, Children's Health Queensland Clinical Unit, University of Queensland, Brisbane, Australia
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  • David S. Winlaw
    Affiliations
    Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia

    Faculty of Health and Medicine, Sydney Medical School, Discipline of Child and Adolescent Health, University of Sydney, Sydney, Australia

    Sydney Children's Hospital Network Cardiac Services, Sydney, Australia
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  • Yves d'Udekem
    Affiliations
    Royal Children's Hospital, Melbourne, Australia

    Murdoch Children's Research Institute, Melbourne, Australia

    Faculty of Medicine, Department of Paediatrics, University of Melbourne, Melbourne, Australia
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  • Nelson Alphonso
    Correspondence
    Address for reprints: Nelson Alphonso, FRACS, Cardiac Services, Clinical Directorate 7f, Queensland Children's Hospital, PO Box 3474, Stanley St, South Brisbane, 4101, Australia.
    Affiliations
    Queensland Paediatric Cardiac Services, Queensland Children's Hospital, Brisbane, Australia

    School of Clinical Medicine, Children's Health Queensland Clinical Unit, University of Queensland, Brisbane, Australia
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Published:October 05, 2020DOI:https://doi.org/10.1016/j.jtcvs.2020.09.074

      Abstract

      Objective

      Fontan takedown remains an option for the management of Fontan failure. We sought to evaluate early and late outcomes after Fontan takedown.

      Methods

      The Australia and New Zealand Fontan Registry was interrogated to identify all patients who had a Fontan takedown.

      Results

      Over a 43-year study period (1975-2018), 36 of 1540 (2.3%) had a Fontan takedown. The median age at takedown was 5.1 years (interquartile range [IQR], 3.7, 7.0). Nine (25%) patients had a takedown within 48 hours, 6 (16%) between 2 days and 3 weeks, 14 (39%) between 3 weeks and 6 months, whereas 7 (19%) had a late takedown (>6 months). Median interval to takedown was 26 days (IQR, 1.5, 127.5). Sixteen (44%) patients died at a median of 57.5 days (IQR, 21.8, 76.8). The greatest mortality occurred between 3 weeks and 6 months (<2 days: 1/9, 11%; 2 days to 3 weeks: 2/6, 33%; 3 weeks to 6 months: 11/14, 79%; >6 months: 2/7, 28%; P = .007). At median follow-up of 9.4 years (IQR, 4.5, 15.3), 11 (31%) patients were alive with an intermediate circulation (10 in New York Heart Association class I/II). Five (14%) patients underwent a successful second Fontan. Freedom from death/transplant after Fontan takedown was 59%, 56%, and 52% at 1, 5, and 10 years, respectively.

      Conclusions

      The incidence of Fontan takedown is low, but mortality is high. The majority of takedowns occurred within 6 months. Mortality was lowest when takedown occurred <2 days and highest between 3 weeks and 6 months. A second Fontan is possible in a small proportion of survivors.

      Graphical abstract

      Key Words

      Abbreviations and Acronyms:

      BCPS (bidirectional cavopulmonary shunt), CI (confidence interval), DORV (double outlet right ventricle), IQR (interquartile range), NYHA (New York Heart Association), PLE (protein-losing enteropathy)
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      Linked Article

      • Commentary: Fontan takedown: The journey off the beaten path
        The Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 3
        • Preview
          Marathe and colleagues1 queried the Australia and New Zealand Fontan Registry to evaluate early and late outcomes of patients who had undergone a Fontan takedown procedure.
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      • Commentary: Fontan challenges: Critical early surgical decisions maybe key
        The Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 3
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          The Fontan physiology since its inception was never meant to be ideal for the human heart. Nevertheless, it has become the final step in the path of palliating many patients with single-ventricle disease.1 Currently, the total cavopulmonary connection or Fontan procedure places the pulmonary circulation like a dam between the systemic venous return and the systemic ventricle, creating, like any dam, upstream congestion and downstream decreased flow. These 2 features are the root cause of all early and late Fontan complications.
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