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  • Title: Perioperative management of sickle cell disease in paediatric cardiac surgery.
    Author: Bhatt K, Cherian S, Agarwal R, Jose S, Cherian KM.
    Journal: Anaesth Intensive Care; 2007 Oct; 35(5):792-5. PubMed ID: 17933172.
    Abstract:
    In sickle cell disease, cardiopulmonary bypass may induce red cell sickling. Partial exchange transfusion reduces the circulating haemoglobin S level. We report the management of a child with sickle cell disease who required surgical closure of a ventricular septal defect. Preoperative exchange transfusion of 50% of the total blood volume was performed with fresh packed red cells over three days. Further exchange transfusion was performed as cardiopulmonary bypass commenced. The haemoglobin S level was reduced from 76% to 37%. The blood removed from the patient during the exchanges was processed allowing storage and re-infusion of the patient's plasma and platelets. Combined preoperative and intraoperative exchange transfusions, instead of a single stage 50% volume exchange, was effective and potentially avoids larger haemodynamic effects. Cardiopulmonary bypass was conducted at normothermia and cold cardioplegia was avoided (fibrillatory arrest was used during the surgical repair).
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