title: Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation.
abstract: BACKGROUND
  Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.
  
  
  OBJECTIVE
  The aim of this study was to review outcomes in patients with PLE following the Fontan operation.
  
  
  METHODS
  From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively.
  
  
  RESULTS
  Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m(2) vs. 2.7 ± 0.7 l/min/m(2); p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]).
  
  
  CONCLUSIONS
  PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.
authors.affiliation: Division of Cardiology, Children's National Medical Center, George Washington University School of Medicine, Washington, DC; Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota. Electronic address: anjohn@cnmc.org.
authors.forename: Anitha S
authors.initials: AS
authors.lastname: John
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Johnson
authors.affiliation: Division of Cardiology, Children's National Medical Center, George Washington University School of Medicine, Washington, DC.
authors.forename: Munziba
authors.initials: M
authors.lastname: Khan
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: David J
authors.initials: DJ
authors.lastname: Driscoll
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Carole A
authors.initials: CA
authors.lastname: Warnes
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota; Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota. Electronic address: cetta.frank@mayo.edu.
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D018729:Fontan Procedure / Q000401:mortality* / Q000639:trends*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011504:Protein-Losing Enteropathies / Q000401:mortality* / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D055815:Young Adult
keywords: Fontan procedure; protein-losing enteropathy