Mitral Valve Replacement in Sickle C Disease Using Intraoperative Exchange Transfusion

Abstract

Sickle C (SC) disease is a relatively uncommon hematologic disorder that poses special challenges when the patient requires a major surgical procedure. In particular, those who have a history of hemolytic crises require some type of intervention, usually homologous transfusion, to decrease the level of circulating hemoglobin S (HbS) and prevent intraoperative sickle crisis. We describe a 25-year-old man with SC disease and a history of multiple sickle cell crises who underwent mitral valve replacement using intraoperative exchange transfusion to decrease his HbS level from 53% to 7%.

Open Heart Surgery in Patients With Sickle Cell Hemoglobinopathy

“…2,3,[5][6][7][8][9][10][11][12][13][14][15][16] On the other hand, CPB itself is also a system that facilitates exchange transfusion, and using blood for priming the circuits can reconcile the aim. 2,6,14,15 Blood transfusion is one of the major issues during any kind of surgery. Generally, autologous transfusion is preferred, but preoperative phlebotomy or the use of cell-saver systems during surgery could reduce the need for homologous blood transfusion.…”

“…8 -11 Although the risks accompanying sickle cell pathology before high-risk surgery, including orthopedic and cholecystectomy, have been well stated, 9 the literature contains only small series or case reports on sickle cell patients having cardiac surgery. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] It should be noted that above-mentioned predisposing conditions are more common in patients undergoing cardiac surgery. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Especially during the operation, CPB itself, as well as aortic cross-clamping, low-flow states, topical or whole-body hypothermia, cold cardioplegia, and use of vasoconstrictive agents, may predispose to the crisis state.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Such conditions lead to potassium efflux, causing formation of insoluble globin polymers. These molecules increase the viscosity of blood and lead to vasoocclusive phenomena, which include cell sickling, adherence of sickle cells to the endothelium, and vasoocclusion.…”

“…[3][4][5][6][7][8] With advances in diagnostic and surgical techniques and improvements in anesthetic management skills, the number of patients with SCD who require cardiac surgery is increasing considerably. Nevertheless, literature on the evaluation and specific management of these patients remains limited, and further studies are strongly recommended.…”

“…This can be avoided by applying "topical-only" cooling or achieving moderate hypothermia, but totally warm CPB is another valid option mentioned frequently in the literature. 2,3,[5][6][7]14,15 In coronary surgery, the endomyocardial capillaries are at particular risk of occlusion by the sickling phenomena when exposed to cold cardioplegia. This can be prevented with the initial institution of warm cardioplegia to wash out the existing blood in coronary arteries, followed by cold or designated cardioplegia according to the protocols of the operating team.…”