title: Ventricular assist device as a bridge to transplant, and extracorporeal membrane oxygenation for primary graft failure in a child with hemophilia A.
abstract: OBJECTIVE
  To report the management of hemophilia in a patient with dilated cardiomyopathy during application of the Berlin-Heart biventricular assist.
  
  
  METHODS
  Case report.
  
  
  RESULTS
  The Berlin-Heart biventricular assist was successfully used as a bridge to heart transplantation (178 days) in a child with hemophilia A; post-transplant extracorporeal membrane oxygenation was implanted until the patient recovered from primary graft failure. Anticoagulant therapy varied as a function of patient status: 1) postoperative bleeding was treated by partial replacement of deficient factors (VII and VIII) and hemoderivatives in order to maintain factor VIII at 50% of normal levels; 2) once the bleeding had stopped, the effect of the hemophilia itself maintained the degree of anticoagulation required by the ventricular assist device; and 3) transplant surgery was followed by complete replacement of factor VIII and intravenous heparinization (a simple way of preventing clot formation in the device and to ensure proper scarring of surgical wounds).
  
  
  CONCLUSIONS
  Selection of anticoagulant therapy as a function of patient status in terms of bleeding and surgical-wound scarring progress is vital for the proper functioning of support techniques (Berlin-Heart biventricular assist and extracorporeal membrane oxygenation) in hemophiliac patients. Collagen dressings placed on surgical wounds achieved good functional and aesthetic results, as well as mechanically isolating the scars from the exterior.
authors.affiliation: Pediatric Intensive Care Unit and Hematology Service , University Hospital Reina Sofía. mafri@ono.com
authors.forename: Manuel Ángel
authors.initials: MÁ
authors.lastname: Frías
authors.forename: Susana
authors.initials: S
authors.lastname: Jaraba
authors.forename: Ignacio
authors.initials: I
authors.lastname: Ibarra
authors.forename: María José
authors.initials: MJ
authors.lastname: Arroyo
authors.forename: María José
authors.initials: MJ
authors.lastname: Velasco
authors.forename: Esther
authors.initials: E
authors.lastname: Ulloa
authors.forename: Francisco
authors.initials: F
authors.lastname: Velasco
authors.forename: Juan Luis
authors.initials: JL
authors.lastname: Pérez-Navero
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D002675:Child, Preschool; D015199:Extracorporeal Membrane Oxygenation; D005167:Factor VII / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D006333:Heart Failure / Q000601:surgery; D016027:Heart Transplantation; D006353:Heart-Assist Devices; D006467:Hemophilia A; D006801:Humans; D008297:Male; D019106:Postoperative Hemorrhage / Q000188:drug therapy; D055031:Primary Graft Dysfunction
chemical_list: D000925:Anticoagulants; D005167:Factor VII; D005169:Factor VIII