title: Protein-losing enteropathy after the Fontan operation.
abstract: Patients were observed after the Fontan operation to determine the frequency and severity of protein-losing enteropathy. A total of 427 patients who survived for 30 days after the Fontan operation, performed between 1973 and January 1987, were analyzed and, thus far, protein-losing enteropathy has developed in 47 of 427. The cumulative risk for the development of protein-losing enteropathy by 10 years was 13.4% among 30-day survivors, and 5-year survival after the diagnosis was 46%. Hemodynamic studies done coincident with the diagnosis of protein-losing enteropathy have shown increased systemic venous pressure, decreased cardiac index, increased pulmonary vascular resistance, and increased ventricular end-diastolic pressure. Medical management of protein-losing enteropathy was only partially successful. Statistical analysis has shown that factors related to protein-losing enteropathy were ventricular anatomy, increased preoperative ventricular end-diastolic pressure, longer operative bypass time, increased length of hospital stay, and postoperative renal failure. This study suggests that scrupulous selection of cases for the Fontan operation is mandatory and that certain perioperative factors may predispose to this serious complication of the Fontan procedure.
authors.affiliation: Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minn 55905, USA.
authors.forename: R H
authors.initials: RH
authors.lastname: Feldt
authors.forename: D J
authors.initials: DJ
authors.lastname: Driscoll
authors.forename: K P
authors.initials: KP
authors.lastname: Offord
authors.forename: R H
authors.initials: RH
authors.lastname: Cha
authors.forename: J
authors.initials: J
authors.lastname: Perrault
authors.forename: H V
authors.initials: HV
authors.lastname: Schaff
authors.forename: F J
authors.initials: FJ
authors.lastname: Puga
authors.forename: G K
authors.initials: GK
authors.lastname: Danielson
mesh_terms: D000367:Age Factors; D002303:Cardiac Output, Low / Q000209:etiology; D002315:Cardiopulmonary Bypass; D002648:Child; D002675:Child, Preschool; D003971:Diastole; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007431:Intraoperative Complications; D007902:Length of Stay; D008168:Lung / Q000098:blood supply; D008297:Male; D018579:Patient Selection; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000209:etiology*; D051437:Renal Insufficiency / Q000209:etiology; D012307:Risk Factors; D015996:Survival Rate; D014655:Vascular Resistance; D014690:Venous Pressure; D017725:Ventricular Pressure