Ravi R Thiagarajan1,2, Stephen J Roth3,4, Steven Margossian5,4, Andrew S Mackie3,4, Ellis J Neufeld5,4, Peter C Laussen3,4, Joseph M Forbess6,4, Elizabeth D Blume3,4. 1. Department of Cardiology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA, 02115, USA. ravi@cardio.chboston.org. 2. Departments of Pediatrics and Surgery, Dana Farber Cancer Institute, Harvard Medical School, Boston, MA, 02115, USA. ravi@cardio.chboston.org. 3. Department of Cardiology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA, 02115, USA. 4. Departments of Pediatrics and Surgery, Dana Farber Cancer Institute, Harvard Medical School, Boston, MA, 02115, USA. 5. Division of Hematology, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA, 02115, USA. 6. Department of Cardiovascular Surgery, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA, 02115, USA.
Abstract
OBJECTIVE: To report the use of extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation in a patient with hemophilia A and dilated cardiomyopathy. DESIGN: Case report. INTERVENTIONS AND RESULTS: During ECMO factor VIII concentrate was administered to maintain a factor VIII level close to 50% of normal control both to decrease the risk of bleeding and to allow standard anticoagulation with heparin. The patient developed an inhibitor to exogenous factor VIII during hospitalization, which was successfully managed with a high-dose continuous infusion of factor VIII during ECMO, the transplant operation, and the postoperative period. CONCLUSIONS: We report the successful use of ECMO as a bridge to cardiac transplantation in a patient with hemophilia A and low-level factor VIII inhibitors.
OBJECTIVE: To report the use of extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation in a patient with hemophilia A and dilated cardiomyopathy. DESIGN: Case report. INTERVENTIONS AND RESULTS: During ECMO factor VIII concentrate was administered to maintain a factor VIII level close to 50% of normal control both to decrease the risk of bleeding and to allow standard anticoagulation with heparin. The patient developed an inhibitor to exogenous factor VIII during hospitalization, which was successfully managed with a high-dose continuous infusion of factor VIII during ECMO, the transplant operation, and the postoperative period. CONCLUSIONS: We report the successful use of ECMO as a bridge to cardiac transplantation in a patient with hemophilia A and low-level factor VIII inhibitors.
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