title: Coagulation factor abnormalities after the Fontan procedure and its modifications.
abstract: OBJECTIVE
  Recently we reported the prevalence of thromboembolism in patients who underwent the Fontan procedure and its modifications. Although hemodynamic factors may well contribute to thromboembolism, recent evidence suggests that coagulation factor abnormalities may also play a role. We therefore set out to investigate the coagulation status in a group of patients who had undergone the Fontan procedure.
  
  
  METHODS
  The study population consists of 20 children who had undergone the Fontan procedure and its modifications. They were examined for coagulation factor abnormalities. Concentrations of serum albumin, total protein, and liver enzymes were also measured. The median age at the time of the operation was 6.2 years (17 months to 8 years) with a male/female ratio of 2.3:1. The median time from the Fontan repair was 4.9 years (18 to 76 months).
  
  
  RESULTS
  Protein C (p < 0.001), protein S (p < 0.02), and factor VII (p < 0.001) were significantly lower than the normal range. The changes in serum albumin and total protein and factors II, IX, and X were not significant.
  
  
  CONCLUSIONS
  It is possible that deficiency in protein C, protein S, and factor VII partly account for the prevalence of thromboembolism after Fontan-type repairs. The risk of long-term anticoagulation should be weighed against the best palliative procedure for these patients. We suggest that reduced protein C, protein S, and factor VII levels in this group of patients should be regarded as risk factors and that such patients should be treated with anticoagulants.
authors.affiliation: Department of Paediatric Cardiology and Cardiac Surgery, Royal Brompton Hospital, London, United Kingdom.
authors.forename: M
authors.initials: M
authors.lastname: Jahangiri
authors.forename: D
authors.initials: D
authors.lastname: Shore
authors.forename: V
authors.initials: V
authors.lastname: Kakkar
authors.forename: C
authors.initials: C
authors.lastname: Lincoln
authors.forename: E
authors.initials: E
authors.lastname: Shinebourne
mesh_terms: D002648:Child; D005168:Factor VII Deficiency / Q000150:complications*; D005260:Female; D018729:Fontan Procedure / Q000379:methods; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D011183:Postoperative Complications / Q000097:blood*; D011486:Protein C; D020151:Protein C Deficiency; D018455:Protein S Deficiency / Q000150:complications*; D012307:Risk Factors; D013923:Thromboembolism / Q000097:blood / Q000209:etiology*
chemical_list: D011486:Protein C