title: Lower incidence of procoagulant abnormalities during follow-up after creation of the Fontan circulation in children.
abstract: OBJECTIVE
  Children who undergo surgery for complex congenital cardiac disease are reported to be at increased thrombotic risk. Our aim was to evaluate long-term changes in the haemostatic system after surgery, to compare markers of activated coagulation in children having surgery with those in a healthy control population, and to relate them to adverse clinical outcome.
  
  
  METHODS
  We studied, prior to surgery, the coagulation profiles of a cohort of 28 children admitted for a modified Fontan operation, studying them again after a period of mean follow-up of 9.6 years. Median age at the time of final surgery was 18.5 months, with a range from 12 to 76 months. We compared generation of thrombin, and levels of the activated protein C-protein C inhibitor complex to controls at follow-up. Thrombophilia and clinical outcome were evaluated.
  
  
  RESULTS
  At long-term follow-up, a lower incidence of procoagulant abnormalities was observed compared to that before surgery. Of 27 patients, 3 (11%), but none of 45 controls, had levels of activated protein C-protein C inhibitor complex above the reference range. There were no significant differences in generation of thrombin between patients and controls. No thrombotic events were recorded, and the patients were generally in good clinical condition.
  
  
  CONCLUSIONS
  Overall, haemostasis appeared to be in balance, and less prothrombotic, after surgery. A subset of the cohort did show indications of activated coagulation. The current therapeutic approach seems to be sufficient to protect the majority of patient. New tests of global coagulation, nonetheless, may be helpful in improving identification of individuals at increased thrombotic risk.
authors.affiliation: Department of Paediatrics, Malmö University Hospital, Malmö, Sweden. olof.rask@med.lu.se
authors.forename: Olof
authors.initials: O
authors.lastname: Rask
authors.forename: Katarina
authors.initials: K
authors.lastname: Hanséus
authors.forename: Rolf
authors.initials: R
authors.lastname: Ljung
authors.forename: Karin
authors.initials: K
authors.lastname: Strandberg
authors.forename: Erik
authors.initials: E
authors.lastname: Berntorp
mesh_terms: D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000097:blood* / Q000601:surgery; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D011183:Postoperative Complications; D011379:Prognosis; D018046:Protein C Inhibitor / Q000097:blood*; D012307:Risk Factors; D013548:Sweden / Q000453:epidemiology; D013917:Thrombin / Q000378:metabolism*; D013923:Thromboembolism / Q000097:blood / Q000453:epidemiology* / Q000209:etiology; D013997:Time Factors
chemical_list: D018046:Protein C Inhibitor; D013917:Thrombin