title: Perioperative management of sickle cell disease in paediatric cardiac surgery.
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).
authors.affiliation: Department of Cardiac Anaesthesiology, Frontier Lifeline, Chennai, Mogappair, India.
authors.forename: K
authors.initials: K
authors.lastname: Bhatt
authors.forename: S
authors.initials: S
authors.lastname: Cherian
authors.forename: R
authors.initials: R
authors.lastname: Agarwal
authors.forename: S
authors.initials: S
authors.lastname: Jose
authors.forename: K M
authors.initials: KM
authors.lastname: Cherian
mesh_terms: D000755:Anemia, Sickle Cell / Q000097:blood / Q000601:surgery / Q000628:therapy*; D002315:Cardiopulmonary Bypass; D002648:Child; D005078:Exchange Transfusion, Whole Blood / Q000379:methods*; D006343:Heart Septal Defects / Q000601:surgery; D006451:Hemoglobin, Sickle / Q000032:analysis; D006801:Humans; D008297:Male; D019990:Perioperative Care
chemical_list: D006451:Hemoglobin, Sickle