Intensive Care Medicine

, Volume 29, Issue 6, pp 985–988 | Cite as

Extracorporeal membrane oxygenation as a bridge to cardiac transplantation in a patient with cardiomyopathy and hemophilia A

  • Ravi R. Thiagarajan
  • Stephen J. Roth
  • Steven Margossian
  • Andrew S. Mackie
  • Ellis J. Neufeld
  • Peter C. Laussen
  • Joseph M. Forbess
  • Elizabeth D. Blume
Neonatal and Pediatric Intensive Care

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.

Keywords

Dilated cardiomyopathy Extracorporeal membrane oxygenation Hemophilia A Factor VIII inhibitor Cardiac transplantation 

Notes

Acknowledgements

The authors are grateful to the manufacturers of the following factor VIII products for donating factor VIII concentrate: Recombinate (Baxter Health Care, Round Lake, Ill., USA), Kogenate (Bayer Corporation, Elkart, Ind., USA), Monoclate-P (Aventis Behring, King of Prussia, Pa., USA), and Monarc-M (American Red Cross, Arlington, Va., USA). The authors also thank Carol Sweeney, RN, BSN, Department of Hematology, Brigham and Women's Hospital, Boston, Mass., for her efforts in procuring factor VIII concentrate from the above manufacturers.

References

  1. 1.
    Dalton HJ, Siewers RD, Fuhrman BP, del Nido P, Thompson AE, Shaver MG, Dowhy M (1993) Extracorporeal membrane oxygenation for cardiac rescue in children with severe myocardial dysfunction. Crit Care Med 21:1020–1028Google Scholar
  2. 2.
    Duncan BW, Hraska V, Jonas RA, Wessel DL, del Nido PJ, Laussen PC, Mayer JM, Wilson JM (1999) Mechanical circulatory support in children with cardiac disease. J Thorac Cardiovasc Surg 117:529–542Google Scholar
  3. 3.
    Duncan BW (2001) Extracorporeal membrane oxygenation for children with cardiac disease. In: Duncan BW (ed) Mechanical support for cardiac and respiratory failure in pediatric patients, 1st edn. Dekker, New York, pp 1–20Google Scholar
  4. 4.
    Graves P (1999) Management of anti-coagulation and blood products during ECMO. In: Van Meurs K (ed) ECMO specialist training manual, 2nd edn. Extracorporeal Life Support Organization, pp 85–100Google Scholar
  5. 5.
    Liu ML, Nakaya S, Thompson AR (2002) Non-inversion factor VIII mutations in 80 hemophilia A families including 24 with alloimmune responses. Thromb Haemost 87:273–276Google Scholar
  6. 6.
    Bontempo FA, Lewis JH, Spero JA, Ragni MV, Kiss JE, Petroski CJ, Thompson ME, Hardesty RL, Kormos RL, Griffith BP (1988) Heart transplant in a hemophiliac with an acquired factor VIII inhibitor: synthesis of factor VIII: C in the pericardial fluid. Transplant Proc 20 [Suppl 1]:790–791Google Scholar
  7. 7.
    Sheth S, Dimichele D, Lee M, Lamour J, Quaegebeur J, Hsu D, Addonizio L, Piomelli S (2000) Heart transplant in a factor VIII-deficient patient with a high titre inhibitor: perioperative management using a high-dose continuous factor VIII and recombinant factor VIIa. Haemophilia 7:227–232Google Scholar
  8. 8.
    Leggett PL, Doyle D, Byron Smith W, Culpepper III W, Cooper S, Ochsner JL (1984) Elective cardiac operation in a patient with severe and acquired factor VIII antibodies. J Thorac Cardiovasc Surg 87:556–560Google Scholar
  9. 9.
    Kulik TJ, Moler FW (2000) Pediatric cardiac support: physiology and techniques. In: Zwischenberger JB, Steinhorn RH, Bartlett RH (eds) Extracorporeal cardiopulmonary support in critical care, 2nd edn. Extracorporeal life support organization pp 563–576Google Scholar
  10. 10.
    Bacha EA, O'Brien P, Smoot L, del Nido PJ (2001) The use of mechanical circulatory support in pediatric cardiac transplantation. In: Duncan BW (ed) Mechanical support for cardiac and respiratory failure in pediatric patients, 1st edn. Dekker, New York, pp 221–232Google Scholar
  11. 11.
    Levi D, Marelli D, Plunkett M, Alejos J, Bresson J, Tran J, Eisenring C, Sadeghi A, Galindo A, Fazio D, Gupta A, Burch S, George B, Laks H (2002) Use of assist devices and ECMO to bridge pediatric patients with cardiomyopathy to transplantation. J Heart Lung Transplant 21:760–770Google Scholar
  12. 12.
    DiMichele D, Neufeld EJ (1998) Hemophilia. A new approach to an old disease. Hematol Oncol Clin North Am 12:1315–1344Google Scholar
  13. 13.
    Nuss R, Soucie JM, Evatt B (2001) Changes in the occurrence of and risk factors for hemophilia-associated intracranial hemorrhage. Am J Hematol 68:37–42Google Scholar
  14. 14.
    Hattersley PG (1966) Activated coagulation time of whole blood. JAMA 196:436–440Google Scholar
  15. 15.
    McManus ML, Kevy SV, Bower LK Hickey PR (1995) Coagulation factor deficiencies during initiation of extracorporeal membrane oxygenation. J Pediatr 126:900–904Google Scholar

Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • Ravi R. Thiagarajan
    • 1
    • 4
  • Stephen J. Roth
    • 1
    • 4
  • Steven Margossian
    • 2
    • 4
  • Andrew S. Mackie
    • 1
    • 4
  • Ellis J. Neufeld
    • 2
    • 4
  • Peter C. Laussen
    • 1
    • 4
  • Joseph M. Forbess
    • 3
    • 4
  • Elizabeth D. Blume
    • 1
    • 4
  1. 1.Department of CardiologyChildren's Hospital BostonBostonUSA
  2. 2.Division of HematologyChildren's Hospital BostonBostonUSA
  3. 3.Department of Cardiovascular SurgeryChildren's Hospital BostonBostonUSA
  4. 4.Departments of Pediatrics and Surgery, Dana Farber Cancer InstituteHarvard Medical SchoolBostonUSA

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