Perioperative considerations in a sickle cell patient undergoing cardiopulmonary bypass

Abstract

An 11-year-old child, a known case of sickle cell anaemia with a history suggestive of sickling crisis in the past was scheduled for surgical pulmonary valvotomy. Pre-operative blood transfusion and hydroxyurea were administered. Pre-operative blood transfusion is indicated in sickle cell disease patients to raise the haematocrit level and lower sickle haemoglobin (HbS) levels. Before the start of cardiopulmonary bypass (CPB), exchange transfusion was performed to reduce HbS level and raise adult haemoglobin level. Hypothermia was prevented by employing normothermic CPB.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass . In cardiac pulmonary bypass, some authors suggest a stricter HbS limit of <5% .…”

“…To avoid preoperative transfusions, introduction of HU therapy prior elective surgery is included in different protocols, with some authors, in case reports, suggesting HU as a possible “alternative.” Also, EPO is also mentioned in this context . Folic acid replacement is a standard in many protocols .…”

“…Therefore, detailed documentation of blood loss is an important part of the perioperative monitoring . The use of cell savers is controversial with authors recommending against its use, others having used such approach . In cardiac surgery, autologous platelet‐rich plasma is re‐used by many groups .…”

“…Complex and high‐risk procedures in the area of cardiac surgery are now seen as feasible and safe for patients with SCD. Cardiopulmonary bypass can be done in normothermic or hypothermic conditions . Cold vs warm cardioplegia have been used without consensus.…”

Severe Hypoxia and Compartment Syndrome in a Patient With Sickle Cell Trait After Redo Aortic Valve Replacement: A Case Report and Review of the Literature

“…Several case reports have examined the intraoperative management of SCD patients, including normothermia CPB, preoperative blood transfusion, intraoperative exchange transfusion, and use of hydroxyurea. [19][20][21] There is limited literature examining the use of these approaches in patients with SCT. However, strategies should be considered in patients with SCT undergoing redo surgeries, which could put these patients at increased risk of vaso-occlusive crisis.…”