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J Thorac Cardiovasc Surg 1998;115:1063-1073
© 1998 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

Protein-losing enteropathy after the Fontan operation: An international multicenter study

Luc Mertens, MD, PhDa, Donald J. Hagler, MDb, Ursula Sauer, MDc, Jane Somerville, MDd, Marc Gewillig, MD, PhDa

Sponsored in part by The Belgian Foundation for Research in PediatricCardiology.

From the Department of Pediatric Cardiology, UZ Leuven, Leuven, Belgiuma;the Department of Pediatric Cardiology, Mayo Clinic, Rochester, Minn.b;Deutsches Herzzentrum, München, Germanyc; and Grown UpCongenital Heart Unit, Royal Brompton and National Heart Hospital, London,United Kingdom.d

Received for publication July 16, 1997. Revisions requested August 25, 1997. Revisions received Nov. 17, 1997. Accepted for publication Nov. 20, 1997. Address for reprints: Marc Gewillig, MD, Pediatric Cardiology,University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium.

Objective: This multicenter studyretrospectively analyzes the data on 114 patients with protein-losingenteropathy after Fontan-type surgery. Special attention was given to thedifferent treatment strategies used and their effect on outcome.
Methods and results: In 35 participating centers 3029Fontan operations were performed. The incidence of protein-losing enteropathy inthe survivors was 3.7%. The median age at Fontan-type surgery was 8.2years (range: 0.6 to 32.9 years). Median age at diagnosis of protein-losingenteropathy was 11.7 years with a median time interval between surgery anddiagnosis 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 atrialpressure 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 improvementin 29%, and death in 46%. Surgical treatment (n =52) was associated with relief of protein-losing enteropathy in 19%, noimprovement in 19%, and death in 62%. In 13 patients 16percutaneous interventions were performed. This resulted in symptomaticimprovement after 12 interventions and no improvement after 4 interventions.
Conclusions: We conclude that the currenttreatment of protein-losing enteropathy after Fontan operation is associatedwith a very high mortality and morbidity rate. Preventive strategies and newtherapeutic approaches are necessary. (J Thorac Cardiovasc Surg1998;115:1063-73)




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