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Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey

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Abstract

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500–2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3–4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

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Authors and Affiliations

  1. Section of Pediatric Cardiology, Department of Pediatrics, Heart Institute, Children’s Hospital Colorado, University of Colorado School of Medicine, 13123 E. 16th Ave. Box 100, Aurora, CO, 80045, USA

    Michael V. Di Maria & Adel K. Younoszai

  2. Department of Cardiology, Boston Children’s Hospital, Boston, MA, USA

    David W. Brown

  3. Department of Pediatrics, Harvard Medical School, Boston, MA, USA

    David W. Brown

  4. Division of Pediatric Cardiology & Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA

    Frank Cetta

  5. Departments of Pediatrics and Internal Medicine, Medical College of Wisconsin, Children’s Hospital of Wisconsin, Milwaukee, WI, USA

    Salil Ginde

  6. Division of Pediatric Cardiology, and Cardiac Center at The Children’s Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, USA

    David Goldberg & Jack Rychik

  7. Division of Pediatric Cardiology, Primary Children’s Hospital, University of Utah, Salt Lake City, UT, USA

    Shaji C. Menon

  8. Sibley Heart Center Cardiology/Children’s Healthcare of Atlanta/Emory University School of Medicine, Atlanta, GA, USA

    Heather M. Phelps

  9. Congenital Heart Center, C.S. Mott Children’s Hospital, University of Michigan, Ann Arbor, MI, USA

    Kurt R. Schumacher

  10. Division of Pediatric Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Philip Thrush

  11. Adult Congenital Heart Program, Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 45229, USA

    Gruschen Veldtman

  12. Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA

    Gail Wright

Authors
  1. Michael V. Di Maria
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  2. David W. Brown
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  3. Frank Cetta
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  4. Salil Ginde
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  5. David Goldberg
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  6. Shaji C. Menon
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  7. Heather M. Phelps
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  8. Jack Rychik
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  9. Kurt R. Schumacher
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  10. Philip Thrush
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  11. Gruschen Veldtman
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  12. Gail Wright
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  13. Adel K. Younoszai
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Corresponding author

Correspondence to Michael V. Di Maria.

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All authors declare that they have no conflicts of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Di Maria, M.V., Brown, D.W., Cetta, F. et al. Surveillance Testing and Preventive Care After Fontan Operation: A Multi-Institutional Survey. Pediatr Cardiol 40, 110–115 (2019). https://doi.org/10.1007/s00246-018-1966-9

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  • DOI: https://doi.org/10.1007/s00246-018-1966-9

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