HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Francis D. Pagani Steven F. Bolling Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Pagani, F. D. Articles by Bolling, S. F. Search for Related Content PubMed PubMed Citation Articles by Pagani, F. D. Articles by Bolling, S. F.

Ann Thorac Surg 1996;61:1841-1843
© 1996 The Society of Thoracic Surgeons

---

Case Report

Mitral Valve Reconstruction in Sickle Cell Disease

Section of Thoracic Surgery, Department of Surgery, University of Michigan Hospitals, Ann Arbor, Michigan

Accepted for publication December 12, 1995.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
As survival improves in patients with sickle cell anemia, the prospects of performing cardiac surgical procedures on older patients with this genetic defect increase. We describe the successful management of a 52-year-old patient with sickle cell disease (homozygous for hemoglobin S) and a history of multiple sickle crisis undergoing cardiopulmonary bypass for mitral valve repair. Preoperative partial exchange transfusion followed by total exchange transfusion at the time of operation was performed to reduce the level of hemoglobin S to 5.4% during bypass. Other management strategies included high-flow normothermic bypass with aortic cross-clamping, topical hypothermia, and cold crystalloid cardioplegia.

	Introduction

Top Footnotes Abstract Introduction Comment References

 
A 52-year-old African-American woman with sickle cell disease presented to the University of Michigan Hospitals with symptoms of progressive dyspnea, orthopnea, and fatigue. Her past medical history was significant for numerous hospitalizations for sickle cell crisis and cholecystectomy at age 38 years. Chest radiograph demonstrated cardiomegaly. Further evaluation including transesophageal echocardiogram demonstrated severe mitral insufficiency, with prolapse of the anterior mitral valve leaflet, accompanied by annular dilatation and significant left atrial and left ventricular enlargement. Left ventricular internal dimensions were 66 mm and 51 mm during diastole and systole, respectively. Catheterization demonstrated a pulmonary artery pressure of 43/18 mm Hg and a left ventricular end-diastolic pressure of 20 mm Hg. The ventriculogram noted global hypokinesis with an ejection fraction of 0.44. Coronary artery anatomy was normal. The patient was referred for mitral valve repair.

Approximately 2 weeks before the operation the patient underwent therapeutic phlebotomy with exchange transfusion as an outpatient. Baseline hemoglobin and hematocrit at that time were 7.2 g/dL and 20%, respectively. After the exchange transfusion, hemoglobin and hematacrit were raised to 12.1 g/dL and 35%, respectively. Hemoglobin electrophoresis after exchange transfusion demonstrated 35% of hemoglobin S. At the time of operation a total blood exchange transfusion was performed to further decrease the level of hemoglobin S. The cardiopulmonary circuit was constructed using an Avecor-Scimed (Plymouth, MN) silicone membrane oxygenator and hard-shell cardiotomy venous reservoir with a Medtronic-Biomedicus (Minneapolis, MN) centrifugal pump and Bard (Tewksbury, MA) arterial line filter. A Cardiovascular Devices Inc model 300 was used for in-line monitoring of arterial blood gasses and blood temperature. The cardiopulmonary bypass circuit was primed with 8 units of cross-matched O-positive, kell negative blood, 5 units of fresh frozen plasma, and 1,200 mL of Normosol-R (Abbott, Chicago, IL), along with 500 mL of Hespan (McGaw, Irvine, CA), 1.5 mg of fentanyl, 10 mg of pancuronium, 5 mg of midazolam, 500 mg of calcium chloride, 1,000,000 units (140 mg) of aprotonin, 150 mEq of sodium bicarbonate, 30,000 units of heparin, and 50 g of 12.5% mannitol. The prebypass level of hemoglobin S was 25%. After the administration of heparin, ascending aortic arterial and bicaval venous cannulation was performed. On the connectors to each of the bicaval cannulas were x -inch straight fittings with Luer-Lok (Baxter, Irvine, CA) adaptors. To each of the Luer-Lok fittings, x -inch adaptors were connected to -inch lines that were in turn connected to two 3,000-mL cardiotomy reservoirs of an Electromedics (Parker, CO) AT1000 autotransfusion cell-saving device. At the initiation of cardiopulmonary bypass, the initial 4,000 mL of the venous return volume was diverted to these cardiotomy reservoirs by clamping the venous line distal to the adapters while the patient was simultaneously transfused with the warm blood prime. After this, the lines to the cardiotomy reservoirs were clamped and removed, and high-flow, normothermic, alkalotic, high oxygen saturation cardiopulmonary bypass was resumed. The hemoglobin S level 10 minutes after initiation of cardiopulmonary bypass was 5.4%. The hematocrit was maintained between 22% and 25% during bypass. Mean arterial pressure was maintained between 60 and 80 mm Hg, and mean cardiac index was maintained at 3.2 L•min^-1•m^-2 during bypass. Plasmapheresis of the exsanguinated sickled red blood cells in the cell-saving device produced 1,500 mL of platelet-rich plasma, which was administered to the patient at the conclusion of cardiopulmonary bypass. Crystalloid cardioplegia solution, at 8°C, was administered to obtain myocardial arrest.

The mitral valve was approached through the interatrial groove. Inspection of the valve demonstrated myxomatous degeneration and prolapse of the anterior and posterior leaflets. Repair was performed with chordal shortening and an annuloplasty ring. After completion of the mitral valve repair, the patient was weaned from cardiopulmonary bypass without difficulty. Postrepair intraoperative transesophageal echocardiogram demonstrated no mitral insufficiency. The hemoglobin S level 2 hours after weaning from cardiopulmonary bypass was 4%. The patient was subsequently discharged from the hospital on postoperative day 7 after an uneventful course. At 18 months after the operation, the patient remains alive and is doing well. Echocardiogram at 1 year of follow-up demonstrated only mild mitral insufficiency.

	Comment

Top Footnotes Abstract Introduction Comment References

 
Successful reports of cardiac surgical procedures performed with cardiopulmonary bypass in patients with sickle cell anemia have been described previously. However, the majority of cases involve only those with sickle cell trait (heterozygous for hemoglobin S) in the pediatric age group or patients with sickle cell disease (homozygous for hemoglobin S) without a significant history of sickle crisis [1–4]. We have described the successful management of an older patient with sickle cell disease and a significant history of sickle crisis.

The prevention of sickling of red blood cells and its sequelae of hemolysis and vascular occlusion during cardiac surgical procedures with cardiopulmonary bypass focuses on a strategy of (1) reducing the level of hemoglobin S before operation and (2) avoiding predisposing factors during the operation that elicit sickling of red blood cells, including hypoxia (oxygen saturation <85%), low-flow states, hypovolemia, acidosis, hypothermia, and increased concentrations of 2,3-diphosphoglyceric acid. Although the absolute safe level of hemoglobin S in sickle cell disease (homozygous for hemoglobin S) during operations with cardiopulmonary bypass has not been well defined, current recommendations suggest the level of hemoglobin S be reduced to 5% at the time of operation [5]. This level of hemoglobin S can be obtained by simple transfusion over several weeks preoperatively, partial exchange transfusion in the preoperative period, complete exchange transfusion using the cardiopulmonary bypass circuit, or a combination of these techniques [6–10]. The value of preoperative exchange transfusions is an increase in the percentage of hemoglobin A relative to hemoglobin S in patients who are anemic and, importantly, suppression of further production of hemoglobin S. The majority of reported patients with sickle cell disease undergoing cardiac operations with cardiopulmonary bypass have received preoperative exchange transfusion [11].

The relative importance of other measures employed to potentially reduce the occurrence of sickling during cardiopulmonary bypass are debatable. The use of hypothermia during cardiopulmonary bypass in patients with sickle cell disease or trait (heterozygous for hemoglobin S) remains controversial. Hypothermia has been demonstrated by in vitro studies to slow polymerization of hemoglobin S and delay the onset of sickling of the red blood cell. However, this benefit of hypothermia must be weighed against its potential adverse effects on increasing capillary transit time in vivo, from vasoconstriction and slugging of red blood cells. One also must weigh whether patients have sickle trait or disease. The latter group is far more prone to a sickle crisis. A number of studies have demonstrated successful use of hypothermia in patients with sickle cell trait and disease [2–4]. We, as others, have chosen in our patient to avoid the potential complications of hypothermia [7]. Other points of unresolved controversy include the use of crystalloid versus blood cardioplegia and the degree of hemodilution during bypass.

Finally, although there are reports of successful mitral valve replacement in patients with sickle cell disease, there are several benefits of performing mitral valve repair as compared with replacement. First, avoiding the use of a prosthesis eliminates the risk of hemolysis. Second, long-term anticoagulation in a patient with chronic anemia can be avoided, and third, reoperation for bioprosthetic degeneration is avoided. In summary, cardiopulmonary bypass with cardiac surgical procedures can be performed safely in older patients with sickle cell disease and a history of multiple sickle crisis.

	Footnotes

Top Footnotes Abstract Introduction Comment References

 
Address reprint requests to Dr Bolling, Section of Thoracic Surgery, University of Michigan Hospitals, Taubman Health Care Center, 2120, Box 0344, Ann Arbor, MI 48109.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Baxter MRN, Bevan JC, Esseltine DW, Bernstein M. The management of two pediatric patients with sickle cell trait and sickle cell disease during cardiopulmonary bypass. J Cardiothorac Anesth 1989;3:577–80.
2. Fox MA, Abbott TR. Hypothermic cardiopulmonary bypass in a patient with sickle cell trait. Anaesthesia 1984;39:1121–3.[Medline]
3. Hudson I, Davidson IA, McGregor CGA. Mitral valve replacement using cold cardioplegia in a patient with sickle cell trait. Thorax 1981;36:151–2.
4. Balasundaram S, Duran CG, Al-Halees Z, Kassay M. Cardiopulmonary bypass in sickle cell anemia. Report of five cases. J Cardiovasc Surg 1991;32:271–4.[Medline]
5. Robinson JA. Apheresis in cardiac surgery. In: Pifarre R, ed. Anticoagulation, hemostatis, and blood preservation in cardiovascular surgery. 1st ed. Philadelphia: Hanley & Belfus, 1993:225–36.
6. Chun PKC, Flannery EP, Bowen TE. Open-heart surgery in patients with hematologic disorders. Am Heart J 1983;105:835–42.[Medline]
7. Szentpetery S, Robertson L, Lower RR. Complete repair of tetralogy associated with sickle cell anemia and G-6-PD deficiency. J Thorac Cardiovasc Surg 1976;72:276–9.[Abstract]
8. Craenen J, Kilman J, Hosier DM, Weinberger M. Mitral valve replacement in a child with sickle cell anemia. J Thorac Cardiovasc Surg 1972;63:797–9.[Medline]
9. Black HA, Dearing JP. Exchange transfusion prior to cardiopulmonary bypass in sickle cell anemia. J Extra-Corpor Technol 1980;12:82–5.
10. Parrish JM, Page PA, Cohen D, et al. Prebypass pheresis and red blood cell exchange in a patient with homozygous SS sickle cell disease undergoing cardiopulmonary bypass: a case report. J Extra-Corpor Technol 1994;26:143–51.
11. Metras D, Coulibaly AO, Ouattara K. Open heart surgery in sickle cell hemoglobinopathies: report of 15 cases. Thorax 1982;37:486–91.[Abstract/Free Full Text]

This article has been cited by other articles:

				K. A. Bocchieri, S. J. Scheinerman, and L. M. Graver Exchange Transfusion Before Cardiopulmonary Bypass in Sickle Cell Disease Ann. Thorac. Surg., July 1, 2010; 90(1): 323 - 324. [Abstract] [Full Text] [PDF]

				S. M. Yousafzai, M. Ugurlucan, O. A. Al Radhwan, A. L. Al Otaibi, and C. C. Canver Open Heart Surgery in Patients With Sickle Cell Hemoglobinopathy Circulation, January 5, 2010; 121(1): 14 - 19. [Abstract] [Full Text] [PDF]

				K. E Al-Ebrahim Cardiac Surgery and Sickle Cell Disease Asian Cardiovasc Thorac Ann, December 1, 2008; 16(6): 479 - 482. [Abstract] [Full Text] [PDF]

				M. M Maddali, M. C Rajakumar, J. Fahr, M. J Albahrani, and M. A Amna Cardiopulmonary Bypass Without Preoperative Exchange Transfusion in Sicklers Asian Cardiovasc Thorac Ann, February 1, 2006; 14(1): 51 - 56. [Abstract] [Full Text] [PDF]

				Y. Naito, M. Nakajima, H. Inoue, and K. Tsuchiya Successful CABG in a patient with paroxysmal nocturnal hemoglobinuria Eur J Cardiothorac Surg, March 1, 2004; 25(3): 468 - 470. [Abstract] [Full Text] [PDF]

				G. N. Djaiani, D. C. H. Cheng, J. A. Carroll, M. Yudin, and J. M. Karski Fast-Track Cardiac Anesthesia in Patients with Sickle Cell Abnormalities Anesth. Analg., September 1, 1999; 89(3): 598 - 598. [Abstract] [Full Text] [PDF]

				K. Frimpong-Boateng, A. G. B. Amoah, H.-M. Barwasser, and C. Kallen Cardiopulmonary bypass in sickle cell anaemia without exchange transfusion Eur J Cardiothorac Surg, November 1, 1998; 14(5): 527 - 529. [Abstract] [Full Text] [PDF]

				G. Shulman, C. McQuitty, R. A. Vertrees, and V. R. Conti Acute Normovolemic Red Cell Exchange for Cardiopulmonary Bypass in Sickle Cell Disease Ann. Thorac. Surg., May 1, 1998; 65(5): 1444 - 1446. [Abstract] [Full Text] [PDF]

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Francis D. Pagani Steven F. Bolling Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Pagani, F. D. Articles by Bolling, S. F. Search for Related Content PubMed PubMed Citation Articles by Pagani, F. D. Articles by Bolling, S. F.