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Heart, Lung and Circulation

Surveillance of End-Organ Damage in Fontan Patients Prior to Transition to Adult Care: Are We There Yet?

  • Thomas G. Wilson
    Correspondence
    Corresponding author at: Department of Gastroenterology, The Royal Children’s Hospital, Melbourne, Flemington Rd, Parkville, Vic 3052.
    Affiliations
    Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Vic, Australia

    Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Vic, Australia

    Department of Gastroenterology, The Royal Children’s Hospital, Melbourne, Vic, Australia
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  • Ajay J. Iyengar
    Affiliations
    Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Vic, Australia

    Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand
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  • Dominica Zentner
    Affiliations
    Department of Cardiology, The Royal Melbourne Hospital, Vic, Australia

    Department of Medicine, The University of Melbourne, Vic, Australia
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  • Yves d’Udekem
    Affiliations
    Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Vic, Australia

    Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Vic, Australia

    Division of Cardiac Surgery, Children’s National Hospital, Washington, DC, USA
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Published:October 28, 2021DOI:https://doi.org/10.1016/j.hlc.2021.10.004

      Background

      Recently published guidelines and consensus statements have outlined recommended screening practices for monitoring of end-organ dysfunction in Fontan patients. We reviewed the current approach to end-organ screening in a local population of Fontan patients at the time of transition to adult care.

      Methods

      Patient data from the Australia and New Zealand Fontan Registry and patient medical records were used to review investigations performed in Fontan patients transitioned from The Royal Children’s Hospital Melbourne to an adult centre between 1 July 2015 and 30 June 2020.

      Results

      A total of 32 patients were referred for transition to an adult centre between 1 July 2015 and 30 June 2020 at a mean age of 18.5±0.7 years (12.7±2.5 years post-Fontan). Liver function tests were performed in 22 patients (69%) within 5 years prior to transition and were abnormal in 15 patients (68%). Liver ultrasound was performed in 13 patients (41%) within 5 years prior to the date of transition, of whom 10 (77%) had abnormal findings (features suggestive of hepatic fibrosis in seven [54%], cirrhosis in two [15%], and portal hypertension in three [23%]). Fourteen (14) patients (44%) had no record of a liver ultrasound being performed between the date of the Fontan procedure and the time of transition to adult care. Hepatocellular carcinoma was diagnosed in one patient at 18 months following transition. A total of 24 patients (75%) had a serum creatinine measured within the 5 years prior to transition, and two (8%) had an estimated glomerular filtration rate (eGFR) less than 90 mL/min/1.73 m2. No patient had a urine protein-creatinine ratio measured between the date of the Fontan procedure and the time of transition to adult care.

      Conclusions

      In this study we have identified that the majority of patients transitioned from a tertiary paediatric centre to an adult centre within the last 5 years did not undergo routine surveillance for end-organ dysfunction. Routine screening for end-organ complications of the Fontan circulation should be incorporated into clinical practice and is an important part of Fontan patient care both pre- and post-transition to adult services.

      Keywords

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