title: Risk factors and outcome of Fontan-associated plastic bronchitis: a case-control study.
abstract: BACKGROUND
  The onset of plastic bronchitis (PB) can be debilitating in survivors of Fontan surgery. The rarity of this complication makes designing studies to understand risk factors for PB challenging. This 2-center case-control study aimed to describe patient outcomes and to assess the association of antecedent patient factors with PB development.
  
  
  RESULTS
  Using center registries, PB patients (n=25) were matched 1:2 to non-PB Fontans (n=43) by date of Fontan surgery and center. The groups were compared for baseline characteristics. Association of patient characteristics with PB was assessed using logistic regression and of potential risk factors with onset of PB using time-to-event analyses. The median time from Fontan to PB diagnosis was 2.5 years. Overall, 12/25 PB patients died or underwent heart transplant; the median transplant-free survival was 8.3 years after diagnosis. Factors associated with developing PB included post-surgical chylothorax (44% PB versus 10% control; odds ratio [OR] 7.3; P=0.003), chest tube (CT) duration at stage 2 (P=0.04) and Fontan (P=0.004), and postoperative ascites (36% PB versus 12% control; OR 4.2; P=0.003). CT drainage >13 days at Fontan was associated with earlier PB onset (P=0.04). Early-onset PB was associated with an increased risk of death (OR 5.0; P=0.002).
  
  
  CONCLUSIONS
  PB is a life-threatening disorder. A longer duration of CT drainage after surgery, chylothorax, and development of ascites are all associated with developing PB. Understanding the pathophysiology of peri-operative complications in individual patients and using targeted interventions may delay the onset of the PB phenotype.
authors.affiliation: University of Michigan C.S. Mott Children's Hospital, Ann Arbor, MI.
authors.forename: Kurt R
authors.initials: KR
authors.lastname: Schumacher
authors.forename: Tajinder P
authors.initials: TP
authors.lastname: Singh
authors.forename: Joseph
authors.initials: J
authors.lastname: Kuebler
authors.forename: Kayla
authors.initials: K
authors.lastname: Aprile
authors.forename: Molly
authors.initials: M
authors.lastname: O'Brien
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Blume
mesh_terms: D001201:Ascites / Q000209:etiology; D001900:Boston; D001991:Bronchitis / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D016022:Case-Control Studies; D015505:Chest Tubes; D002675:Child, Preschool; D002916:Chylothorax / Q000209:etiology; D004322:Drainage / Q000009:adverse effects / Q000295:instrumentation; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D008824:Michigan; D016017:Odds Ratio; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome
keywords: Fontan procedure; pediatrics; risk factors
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