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

Michael V. Di Maria*, 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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)110-115
Number of pages6
JournalPediatric cardiology
Issue number1
StatePublished - Jan 15 2019


  • Fontan
  • Multidisciplinary clinic
  • Surveillance testing

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health


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