Protein-losing enteropathy in patients with Fontan circulation: Is it triggered by infection?

Dominik Lenz, Jörg Hambsch, Peter Schneider, Hans Jürgen Häusler, Ursula Sauer, John Hess, Attila Tárnok

Research output: Contribution to journalReview articlepeer-review

49 Scopus citations


Introduction. Protein-losing enteropathy (PLE) is a recognised complication of the Fontan circulation. Its pathogenesis is not fully understood, however, and it is unclear why its onset occurs months or even years after Fontan surgery. Patients. We report a 4.5-year-old girl with Fontan circulation who developed PLE almost 1 year after surgery. At the time of onset the patient had rotavirus enteritis and streptococcal tonsillitis. We have reviewed the records of seven other patients with longstanding PLE. In six of these patients we identified infections at the onset of symptoms. None of our patients had evidence of opportunistic infection. Discussion and conclusion. The immune system of patients with PLE is compromised, but reports on recurrent opportunistic infections are rare. The present observations suggest that infection and inflammation may be associated with the onset of PLE. The mechanism of how infection may trigger PLE warrants further investigation.

Original languageEnglish
Pages (from-to)185-190
Number of pages6
JournalCritical Care
Issue number2
StatePublished - Apr 2003
Externally publishedYes


  • Fontan type surgery
  • Immunodeficiency
  • Infection
  • Inflammation


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