Pediatric Cardiology

, Volume 40, Issue 1, pp 110–115 | Cite as

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

  • Michael V. Di MariaEmail author
  • David W. Brown
  • Frank Cetta
  • Salil Ginde
  • David Goldberg
  • Shaji C. Menon
  • Heather M. Phelps
  • Jack Rychik
  • Kurt R. Schumacher
  • Philip Thrush
  • Gruschen Veldtman
  • Gail Wright
  • Adel K. Younoszai
Original Article


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.


Fontan Surveillance testing Multidisciplinary clinic 



There were no sources of funding for this work.

Compliance with Ethical Standards

Conflict of interest

All authors declare that they have no conflicts of interest.

Ethical Approval

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.

Informed Consent

Not applicable.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Michael V. Di Maria
    • 1
    Email author
  • David W. Brown
    • 2
    • 3
  • Frank Cetta
    • 4
  • Salil Ginde
    • 5
  • David Goldberg
    • 6
  • Shaji C. Menon
    • 7
  • Heather M. Phelps
    • 8
  • Jack Rychik
    • 6
  • Kurt R. Schumacher
    • 9
  • Philip Thrush
    • 10
  • Gruschen Veldtman
    • 11
  • Gail Wright
    • 12
  • Adel K. Younoszai
    • 1
  1. 1.Section of Pediatric Cardiology, Department of Pediatrics, Heart Institute, Children’s Hospital ColoradoUniversity of Colorado School of MedicineAuroraUSA
  2. 2.Department of CardiologyBoston Children’s HospitalBostonUSA
  3. 3.Department of PediatricsHarvard Medical SchoolBostonUSA
  4. 4.Division of Pediatric Cardiology & Department of Cardiovascular DiseasesMayo ClinicRochesterUSA
  5. 5.Departments of Pediatrics and Internal Medicine, Medical College of WisconsinChildren’s Hospital of WisconsinMilwaukeeUSA
  6. 6.Division of Pediatric Cardiology, and Cardiac Center at The Children’s Hospital of PhiladelphiaThe Perelman School of Medicine at the University of PennsylvaniaPhiladelphiaUSA
  7. 7.Division of Pediatric Cardiology, Primary Children’s HospitalUniversity of UtahSalt Lake CityUSA
  8. 8.Sibley Heart Center Cardiology/Children’s Healthcare of Atlanta/Emory University School of MedicineAtlantaUSA
  9. 9.Congenital Heart Center, C.S. Mott Children’s HospitalUniversity of MichiganAnn ArborUSA
  10. 10.Division of Pediatric Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of ChicagoNorthwestern University Feinberg School of MedicineChicagoUSA
  11. 11.Adult Congenital Heart Program, Heart InstituteCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  12. 12.Division of Pediatric Cardiology, Department of PediatricsStanford University School of MedicinePalo AltoUSA

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