• Dominik Lenz, Jörg Hambsch, Peter Schneider, Hans-Jürgen Häusler, Ursula Sauer, John Hess, and Attila Tárnok.
• Department of Paediatric Cardiology, Cardiac Centre Leipzig, University Hospital, Leipzig, Germany.
• Crit Care. 2003 Apr 1; 7 (2): 185-90.

IntroductionProtein-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.PatientsWe 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 ConclusionThe 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.

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