Kastrup M. von Heymann C. Hotz H. Konertz WF. Ziemer S. Kox WJ. Spies C. , Recombinant factor VIIa (rFVIIa) after aortic valve replacement in a patient with osteogenesis imperfecta. Annals of Thoracic Surgery 74(3):910-2, 2002.
A 26-year-old man with osteogenesis imperfecta and severe aortic regurgitation was scheduled for aortic valve replacement. A 23-mm aortic valve was implanted under normothermic cardiopulmonary bypass. Because of the friability of the aortic tissue, 3.0 mL of fibrin sealant was used to stop bleeding from the aortic root. Transfusion of packed red blood cells (RPBC) or fresh frozen plasma (FFP) was not required during surgery. After ICU admission, the average chest tube drainage was more than 150 mL per hour for the first 6 hours, and 3 U RPBC, 4 U FFP, and 2 U platelets were transfused. A continuous infusion of aprotinin at 100,000 IU/h was started immediately after admission but did not reduce the amount of bleeding. The systolic blood pressure was kept between 80 and 100 mm Hg with a continuous infusion of nitroglycerin and normal coagulation tests. After the blood loss from the mediastinal chest drain exceeded 1,000 mL without any reduction in bleeding rFVIIa was administered at a dose of 40 micrograms/kg and bleeding stopped immediately. No further blood products were administered. The patient was extubated on the morning of the first postoperative day. The coronary catheterization, which was performed immediately on the same day, showed good aortic prosthesis function. No alterations in the coronary arteries could be identified. The patient was discharged from the intensive care unit on the second day after surgery in stable clinical condition.