Gastrointestinal neuroendocrine tumors in Fontan patients

Corey Vural, Priya Skaria, Louis P. Dehner, Geetika Khanna

Research output: Contribution to journalArticlepeer-review


The Fontan procedure used for palliation of single ventricle physiology is associated with multisystemic morbidity. With improving survival and increased use of surveillance imaging in this patient population, long-term complications associated with Fontan circuits are commonly encountered by radiologists. One interesting observation is the apparent increased risk of paragangliomas and pheochromocytomas in this group of patients and perhaps a pathogenetic role of chronic hypoxia. We report 2 cases of gastrointestinal neuroendocrine tumors (NET) in the setting of Fontan circuit. The first is a 20-year-old female with history of hypoplastic left heart and Fontan palliation who presented with hematochezia and was diagnosed with a jejunal grade 2 NET. The second case is of a 12-year-old boy with history Fontan palliation for single ventricle physiology, incidentally found to have a well-differentiated pancreatic NET during precardiac transplant workup. These cases should alert the radiologists to be aware of the apparent association between Fontan procedure and NET.

Original languageEnglish
Pages (from-to)2153-2156
Number of pages4
JournalRadiology Case Reports
Issue number11
StatePublished - Nov 2020


  • Fontan procedure
  • Jejunum
  • Neuroendocrine tumors
  • Pancreas

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