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Ann Thorac Surg 1998;65:1444-1446
© 1998 The Society of Thoracic Surgeons


Case Reports

Acute Normovolemic Red Cell Exchange for Cardiopulmonary Bypass in Sickle Cell Disease

Gerald Shulman, MDa, Christopher McQuitty, MDb, Roger A. Vertrees, CCPc, Vincent R. Conti, MDc

a Department of Pathology and Laboratory Medicine (Blood Bank Division),, The University of Texas Medical Branch at Galveston, Galveston, Texas USA
b Department of Anesthesiology and Internal Medicine (Cardiology Division), The University of Texas Medical Branch at Galveston, Galveston, Texas, USA
c Department of Surgery (Cardiothoracic Division), The University of Texas Medical Branch at Galveston, Galveston, Texas, USA

Accepted for publication November 18, 1997.

Address reprint requests to Dr Shulman, Blood Bank Division, Department of Pathology, The University of Texas Medical Branch, 301 University Blvd, Galveston, TX 77555-0717
e-mail: (gshulman{at}utmb.edu)


    Abstract
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 
A patient with sickle cell disease (hematocrit, 28.5%; hemoglobin S fraction, 79%), required mitral valve repair. Partial red cell removal and blood component sequestration with an autotransfusion device before cardiopulmonary bypass initially decreased the sickle red cell mass. This was followed by an acute one-volume whole blood exchange transfusion performed upon the initiation of cardiopulmonary bypass, resulting in a further reduction. Both techniques yielded fresh autologous plasma for use; sequestration yielded a plateletpheresis product. Adequate postbypass hemostasis was demonstrated.


    Introduction
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 
Patients with sickle cell disease undergoing cardiopulmonary bypass (CPB) require a reduction of hemoglobin S (HbS) fraction to less than 30% to avoid a sickling crisis potentially induced by hypothermia, hypoxia, or hypotension. A brief review reveals that HbS reduction has been accomplished by various techniques from preoperative outpatient therapeutic phlebotomy and exchange transfusion [1] to an acute whole blood volume exchange transfusion before cardiopulmonary bypass (CPB) [2]. The method described in this report realizes the benefits of both methods, avoids the cost and complexity of a previous hemapheresis, and reduces the HbS fraction to acceptable levels.

The patient was a 17-year-old, 66-kg male with homozygous sickle cell disease, autosplenectomy, and severemitral regurgitation who presented for mitral valve repair because of increasing fatigability. The preoperative values were hematocrit, 27.3%; HbS, 79%; HbA, 20%; and platelet count, 419,000/µL.

Normovolemia was maintained with one unit of allogeneic red blood cells and 1.6 L of Plasma-Lyte A (Baxter Healthcare Corp, Deerfield, IL), and blood was drawn into the autotransfusion device (ELMD 500; Medtronic Electromedics, Parker, CO) with an open line to the waste bag for air management. Venous access was via an 8.5F venous catheter in the left internal jugular vein, filling the bowl at 100 mL · min-1 and a centrifuge speed of 5,600 rpm. Citrated platelet-poor plasma was separated above packed red cells in the bowl. When plasma reached the outlet of the centrifuge bowl, the line to the first plasma bag was opened while simultaneously closing the line to the waste bag. Venous blood was drawn until the surface of the red cell pack reached the shoulder of the bowl, when the draw rate was reduced to 50 mL · min-1 and the centrifuge speed to 2,400 rpm. Platelet-rich plasma was collected by opening the line to the second plasma bag, simultaneously closing the line to the first plasma bag. To enrich the platelet content, collection of the platelet-rich plasma was continued for 60 seconds after the red cells started to enter the second plasma bag. Autologous platelet concentrate, packed red blood cells, and citrated platelet-poor plasma were separated during a 57-minute time period, not delaying the start of the operation.

The CPB prime consisted of 6 units of allogeneic red cells, 6 units of fresh frozen plasma, 500 mL of 5% human serum albumin, 1,200 mL of Plasma-Lyte A, and 12,500 units of heparin. Initial venous drainage equivalent to one patient blood volume was diverted into an additional cardiotomy reservoir. Heparinized platelet-poor plasma was recovered using the autotransfusion device for retransfusion after CPB. The patient’s red blood cells were discarded. Myocardial preservation was accomplished with both antegrade and retrograde cardioplegia at less than 10°C with a blood:crystalloid ratio of 4:1. Heparinized platelet-poor plasma was used for volume replacement during CPB; the autologous plateletpheresis product was retransfused after CPB. Total CPB time was 95 minutes, with 40 minutes at 28°C.

Thromboelastography (Haemoscope Corporation, Skokie, IL) was performed before sequestration, immediately after institution of bypass, before protamine administration, after protamine administration, after infusion of platelet-rich plasma, and 24 hours postoperative. Variables measured were initiation of coagulation (R time), clot stability (K time), clot formation rate (angle), and clot elasticity and fibrin polymerization (maximum amplitude [MA]) [3]. Heparin anticoagulation was monitored with activated clotting times. Retrospective hemoglobin electrophoresis was carried out to evaluate HbS content in samples collected before induction of the operation, during CPB, and 24 hours after the completion of the operation.


    Results
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 
Sequestration before CPB decreased the patient’s HbS concentration from 79% to 67% (autologous red blood cell mass reduced to 58% of initial volume), yielding 675 mL of packed red cells (hematocrit, 85%), which were discarded, and 900 mL of platelet-poor plasma and 300 mL of plateletpheresis (platelet content of 6.74 x 1011 µL), which were reinfused later. Upon initiation of CPB, the HbS concentration was further reduced to 21% by an exchange transfusion with the extracorporeal circuit. After termination of CPB, red cells were recovered from the bypass circuit and reinfused, followed by the citrated autologous platelet-poor plasma and the plateletpheresis product, increasing the platelet count from 96,000 to 243,000/µL. After reinfusion of the plateletpheresis product, the thromboelastograph showed hemodilution effect determined by a prolonged R time, decreased MA, and reduced angle (Table 1).


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Table 1. Coagulation Studies Test Resultsa

 
Cost considerations
The estimated cost for labor and supplies for performing red cell mass reduction, plasmapheresis, and plateletpheresis with the autotransfusion device is $323.36. After the whole-blood exchange at initiation of CPB, heparinized autologous plasma from the sequestered autologous blood was processed with the same autotransfusion device. Substantial additional savings accrued from reuse of the patient’s citrated and heparinized plasma.

Clinical follow-up
After 17 hours, total mediastinal chest tube drainage was 800 mL and total urine output was 3,493 mL; the hematocrit was 28% with HbA 79% and HbS 20%. The patient had an uneventful postoperative recovery and was discharged from the hospital on the sixth postoperative day.


    Comment
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 
Blood component sequestration before CPB and red blood cell exchange at the onset of CPB were used to enhance reduction of the HbS mass to obviate the need for a preliminary hemapheresis procedure. During sequestration, autologous fresh plasma and plateletpheresis were also collected for reinfusion. The HbS was replaced with HbA to protect against a sickling crisis; autologous plasma was provided to maintain volume and preserve coagulation factors. In anticipation of a significant loss of circulating platelets, plateletpheresis for reinfusion after CPB was collected before CPB [4]. Postoperative mediastinal chest tube drainage was similar to that in our uncomplicated cardiac cases [5]. Despite an excellent increase in the platelet count after transfusion of the plateletpheresis product, thromboelastography results unexpectedly suggested diminished qualitative functional platelet activity. The value of postCPB reinfusion of sequestered autologous plateletpheresis collected immediately before CPB [4] has been variable in previous studies [4, 6]. In this study, although clinical hemostasis was good, the value was not evident when thromboelastrography was used to assess platelet function. The entire method of management of our case accomplished excellent HbS reduction while maintaining good clinical hemostasis and cost effectiveness.


    Acknowledgments
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 
We thank Eileen Figueroa and Karen Martin for manuscript preparation.


    Footnotes
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 
We acknowledge Medtronic Electromedics Inc, Parker, Colorado, for financial support in performing the investigations of the study.


    References
 Top
 Footnotes
 Abstract
 Introduction
 Results
 Comment
 Acknowledgments
 References
 

  1. Pagani F.D., Polito R.J., Bolling S.F. Mitral valve reconstruction in sickle cell disease. Ann Thorac Surg 1996;61:1841-1843.[Abstract/Free Full Text]
  2. Balasundaram M.S., Duran C.G., Al-Halees Z., Kassay M. Cardiopulmonary bypass in sickle cell anemia. J Cardiovasc Surg 1991;32:271-274.[Medline]
  3. McNicol P.L., Liu G., Harley I.D., et al. Patterns of coagulopathy during liver transplantation: experience with the first 75 cases using thrombelastography. Anaesth Intens Care 1994;22:659-665.[Medline]
  4. Christenson J.T., Reuse J., Badel P., Simonet F., Schmuziger M. Plateletpheresis before redo CABG diminishes excessive blood transfusion. Ann Thorac Surg 1996;62:1373-1379.[Abstract/Free Full Text]
  5. Vertrees R.A., Conti V.R., Lick S.D., Zwischenberger J.B., McDaniel L.B., Shulman G. Adverse effects of postoperative infusion of shed mediastinal blood. Ann Thorac Surg 1996;62:717-723.[Abstract/Free Full Text]
  6. Triulzi D.J., Gilmor P.M., Ness W.A., Baumgartner W.A., Schultheis L.W. Efficacy of autologous fresh whole blood or platelet-rich plasma in adult cardiac surgery. Transfusion 1995;35:627-634.[Medline]



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