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Congenital: Tetralogy of Fallot| Volume 165, ISSUE 6, P2169-2180.e3, June 2023

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Long-term outcomes of staged repair of tetralogy of Fallot

  • Xin Tao Ye
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia

    Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
    Search for articles by this author
  • Edward Buratto
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia

    Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
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  • Antonia Schulz
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia
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  • Samuel J. Macalister
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia

    Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
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  • Robert G. Weintraub
    Affiliations
    Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia

    Department of Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia

    The Melbourne Centre for Cardiovascular Genomics and Regenerative Medicine, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
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  • Christian P. Brizard
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia

    Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia

    The Melbourne Centre for Cardiovascular Genomics and Regenerative Medicine, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
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  • Igor E. Konstantinov
    Correspondence
    Address for reprints: Igor E. Konstantinov, MD, PhD, FRACS, Royal Children's Hospital, Flemington Rd, Parkville, Victoria 3029, Australia.
    Affiliations
    Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia

    Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia

    Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia

    The Melbourne Centre for Cardiovascular Genomics and Regenerative Medicine, Murdoch Children's Research Institute, Melbourne, Victoria, Australia
    Search for articles by this author
Published:August 04, 2022DOI:https://doi.org/10.1016/j.jtcvs.2022.07.026

      Abstract

      Background

      The optimal management strategy for symptomatic young infants with tetralogy of Fallot (TOF) is yet to be determined. We aimed to evaluate the long-term outcomes of a staged approach with initial shunt palliation followed by complete repair.

      Methods

      Between January 1993 and July 2021, 160 children with TOF underwent a systemic-to-pulmonary shunt at our institution, including 65 neonates (41%). The mean duration of follow-up was 12.3 ± 8.1 years.

      Results

      Hospital mortality was 3% (4 of 160), all occurring in patients with a shunt size-to-weight ratio ≥1.2 mm/kg. Composite morbidity—defined as cardiac arrest, postoperative mechanical circulatory support, or unplanned reoperation—occurred in 21% (33 of 160). On multivariable analysis, a shunt size-to-weight ratio ≥1.2 mm/kg and prematurity were independent predictors of composite morbidity. Interstage mortality was 3% (4 of 156). A limited transannular patch was used in 75% (113 of 150) of TOF repairs. Actuarial survival at 20 years after shunt was 90% (95% confidence interval [CI], 79%-95%). Actuarial freedom from reinterventions at 20 years after TOF repair was 40% (95% CI, 28%-52%). Neonates had comparable composite morbidity, mortality, and late risk of reinterventions to older children.

      Conclusions

      Staged repair of TOF in symptomatic young infants results in low mortality but high rates of reinterventions at long-term follow-up. A shunt size-to-weight ratio ≥1.2 mm/kg is a significant risk factor for mortality and morbidity prior to complete repair. Neonates undergoing shunt insertion have comparable outcomes to older children.

      Key Words

      Abbreviations and Acronyms:

      BTS (Blalock–Taussig shunt), CI (confidence interval), CPB (cardiopulmonary bypass), IQR (interquartile range), MBTS (modified Blalock–Taussig shunt), PA (pulmonary artery), PAPR (pulmonary annulus-preserving repair), PR (pulmonary regurgitation), PTFE (polytetrafluoroethylene), PVA (pulmonary valve annulus), RV (right ventricle), RVOT (right ventricular outflow tract), RVOTO (right ventricular outflow tract obstruction), TAP (transannular patch), TOF (tetralogy of Fallot)
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