Preoperative Exchange Transfusion for Sickle Cell Disease Patients Undergoing Open-Heart Surgery: An Exception to the Rule

Abstract

Preoperative exchange transfusion is a routine practice in patients with sickle cell disease having elevated sickle cell hemoglobin levels (>40%) undergoing open-heart surgery on cardiopulmonary bypass. A new approach toward acceptance and management of sickle cell disease patients with high sickle cell hemoglobin levels for open-heart surgery without preoperative exchange transfusion of blood is presented.

Catastrophic sickling crisis in patient undergoing cardiac transplantation with sickle cell trait

“…There are many case reports/series describing the use of exchange transfusion pre‐ and perioperatively in patients with SCD undergoing cardiac surgery with the aim of reducing HbS concentration and therefore risk of sickling and vaso‐occlusive events, with some suggesting <30% to be the target . However, there are as many case reports/series describing patients undergoing cardiac surgery without any specific modification to the pre‐ and perioperative management experiencing no sickle cell complications, even in patients with SCD and HbS concentration >30% . It is our practice to reserve full exchange transfusion for SCD and perform partial exchange in SCT patients with expected extremely low core body temperature, profound acidosis, or prolonged episodes of stasis and hypoxia during the surgery, such as in pulmonary thromboendarterectomy .…”

Paediatric cardiac anaesthesia in sickle cell disease: a case series

“…1,[4][5][6][7] A limitation of retrospective data collection from patient folders is that key information is often not available. For instance, it is not clear whether perioperative HbA/HbS concentrations were monitored, or when erythrocyte transfusions were performed.…”

“…To avoid sickling of coronary blood, the use of initial warm crystalloid cardioplegia (26-32°C) to flush out residual erythrocytes, followed by cold cardioplegia solution, is advocated. [3][4][5][6] The concerns of cooling and cardioplegia should be considered on a case-to-case basis, and are best discussed with the cardiothoracic surgeon and perfusionist prior to CPB. Depending on the cardiac lesion, the new technique of mild to moderate hypothermia and the use of blood cardioplegia may benefit SCD patients.…”

Management of sickle cell disease in patients undergoing cardiac surgery

“…Currently, exchange transfusion is recommended to reduce HbS levels to <30% in patients undergoing high‐risk procedures, including cardiac surgery . Adhikari et al have also advocated for preoperative exchange transfusion or simple transfusion when the duration of surgery is expected to be protracted, when baseline Hb is <10 g/dL, and when the patient has a history of sickling crises requiring hospital admission . The Preoperative Transfusion in Sickle Cell Disease Study Group alternatively demonstrated that a conservative transfusion approach, targeting a preoperative Hb level >10 g/dL, rather than a HbS fraction <30%, afforded similar surgical outcomes while reducing transfusion‐associated complications .…”

“…12,13 Adhikari et al have also advocated for preoperative exchange transfusion or simple transfusion when the duration of surgery is expected to be protracted, when baseline Hb is <10 g/dL, and when the patient has a history of sickling crises requiring hospital admission. 14…”