title: Low pulmonary vascular compliance predisposes post-Fontan patients to protein-losing enteropathy.
abstract: BACKGROUND
  Protein-losing enteropathy (PLE) is a life-threatening and poorly understood complication after the Fontan operation. We sought to determine the pre-operative risk factors for PLE which developed after the extracardiac conduit Fontan operation.
  
  
  METHODS
  Two hundred thirty-five patients who underwent the extracardiac conduit Fontan operation as an initial Fontan type procedure (median age at operation: 3.5 years) were enrolled in this cross-sectional retrospective study. Pre-operative and peri-operative variables were surveyed through a review of medical records.
  
  
  RESULTS
  Within the median follow-up duration of 5 years, 12 patients developed PLE (12/234, 5.1%) at a median interval of 2.2 years after the Fontan procedure, and 4 died of PLE at a median interval of 1.2 years (range 0.21-7.62) after diagnosis. Factors found to be related to the time to the development of PLE on univariate analysis were pulmonary vascular compliance (Cpv) (p=0.0019), central venous pressure at postoperative 12 hours (p=0.0026), days of ICU stay (P=0.0449), days of hospitalization (p=0.0135), and days of chest tube indwelling (p=0.0493). Multivariate analysis, however, showed that only Cpv (p=0.0367) remained significant. The range of Cpv was 8.8-26.1 mm(2)/m(2)/mmHg (median 17.9) in patients with PLE, and 6.6-122.3 mm(2)/m(2)/mmHg (median 26.8) in patients without PLE.
  
  
  CONCLUSIONS
  Low pulmonary vascular compliance is associated with the development of PLE after the extracardiac conduit Fontan operation.
authors.affiliation: Division of Pediatric Cardiology, College of Medicine, University of Ulsan, Seoul, South Korea.
authors.forename: Jeong Jin
authors.initials: JJ
authors.lastname: Yu
authors.forename: Tae-Jin
authors.initials: TJ
authors.lastname: Yun
authors.forename: Sung-Cheol
authors.initials: SC
authors.lastname: Yun
authors.forename: Yu Mi
authors.initials: YM
authors.lastname: Im
authors.forename: Seung Chul
authors.initials: SC
authors.lastname: Lee
authors.forename: Hong Ju
authors.initials: HJ
authors.lastname: Shin
authors.forename: Hong Ki
authors.initials: HK
authors.lastname: Ko
authors.forename: Jeong-Jun
authors.initials: JJ
authors.lastname: Park
authors.forename: Dong-Man
authors.initials: DM
authors.lastname: Seo
authors.forename: Young-Hwue
authors.initials: YH
authors.lastname: Kim
authors.forename: Jae-Kon
authors.initials: JK
authors.lastname: Ko
authors.forename: In-Sook
authors.initials: IS
authors.lastname: Park
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis* / Q000453:epidemiology*; D011504:Protein-Losing Enteropathies / Q000175:diagnosis* / Q000453:epidemiology*; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000502:physiology*; D055815:Young Adult