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Title: Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group
Authors: Mertens, Luc ×
Hagler, D J
Sauer, U
Somerville, J
Gewillig, Marc #
Issue Date: May-1998
Series Title: Journal of Thoracic and Cardiovascular Surgery vol:115 issue:5 pages:1063-73
Abstract: 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.
ISSN: 0022-5223
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Pediatric Cardiology Section (-)
Cardiovascular Developmental Biology
× corresponding author
# (joint) last author

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