title: Guide to performing cardiac surgery in patients with hereditary bleeding disorders.
abstract: OBJECTIVE
  This study presents the experience of a large Australian hospital in performing cardiac surgery on patients with hereditary bleeding disorders (HBDs) and provides a suggested approach for their perioperative management.
  
  
  METHODS
  Medical records of patients with HBDs who underwent cardiac surgery from January 1997 to December 2013 were reviewed.
  
  
  RESULTS
  Seventeen patients were included in this study, 13 with Hemophilia A, one symptomatic Hemophilia A carrier, one with Hemophilia B, and two with von Willebrand Disease. Cardiac surgical procedures performed included 10 coronary artery bypass graft (CABG) operations, two aortic valve replacements, two mitral valve repairs, two aortic root replacements, and one combined aortic valve replacement and CABG. Perioperative management centered on factor substitution to maintain normal factor levels. Perioperative outcomes including length of hospital stay, mortality, and return to the operating room for bleeding were recorded. Two patients returned to the operating room for bleeding, one patient on the first postoperative day and one patient at day 20 postoperatively with pericardial bleeding.
  
  
  CONCLUSIONS
  Most patients demonstrated excellent hemostasis, suggesting that cardiopulmonary bypass surgery in HBD patients may be safely performed with careful planning.
authors.affiliation: Ronald Sawers Haemophilia Centre, The Alfred Hospital, Melbourne, Australia.
authors.forename: Prachi
authors.initials: P
authors.lastname: Bhave
authors.forename: David
authors.initials: D
authors.lastname: McGiffin
authors.forename: James
authors.initials: J
authors.lastname: Shaw
authors.forename: Megan
authors.initials: M
authors.lastname: Walsh
authors.forename: Penny
authors.initials: P
authors.lastname: McCarthy
authors.forename: Huyen
authors.initials: H
authors.lastname: Tran
authors.forename: Amanda Katherine
authors.initials: AK
authors.lastname: Davis
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006348:Cardiac Surgical Procedures; D002315:Cardiopulmonary Bypass; D005260:Female; D006331:Heart Diseases / Q000150:complications* / Q000601:surgery*; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D019990:Perioperative Care / Q000379:methods*; D019106:Postoperative Hemorrhage / Q000517:prevention & control; D017410:Practice Guidelines as Topic; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000150:complications*