PROTEIN-LOSING ENTEROPATHY AFTER THE FONTAN OPERATION: AN INTERNATIONAL MULTICENTER STUDY.
Mertens, Luc MD, PhD a; Hagler, Donald J. MD b; Sauer, Ursula MD c; Somerville, Jane MD d; Gewillig, Marc MD, PhD a; On behalf of the PLE study group
[Miscellaneous Article] Journal of Thoracic & Cardiovascular Surgery. 115(5):1063-1073, May 1998.

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Objective: This multicenter study retrospectively analyzes the data on 114 patients with protein-losing enteropathy after Fontan-type surgery. Special attention was given to the different treatment strategies used and their effect on outcome.

Methods and results: In 35 participating centers 3029 Fontan operations were performed. The incidence of protein-losing enteropathy in the survivors was 3.7%. The median age at Fontan-type surgery was 8.2 years (range: 0.6 to 32.9 years). Median age at diagnosis of protein-losing enteropathy was 11.7 years with a median time interval between surgery and diagnosis of 2.7 years (range: 0.1 to 16.4 years). Most patients had edema (79%) and effusions (75%). Hemodynamic data revealed a mean right atrial pressure of 17 /- SD 5.3 mm Hg with a cardiac index of 2.4 /- 0.8 L/min/m2. Medical treatment only (n = 52) resulted in a complete resolution of symptoms in 25%, no improvement in 29%, and death in 46%. Surgical treatment (n = 52) was associated with relief of protein-losing enteropathy in 19%, no improvement in 19%, and death in 62%. In 13 patients 16 percutaneous interventions were performed. This resulted in symptomatic improvement after 12 interventions and no improvement after 4 interventions.

Conclusions: We conclude that the current treatment of protein-losing enteropathy after Fontan operation is associated with a very high mortality and morbidity rate. Preventive strategies and new therapeutic approaches are necessary.

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