Paediatric cardiac anaesthesia in sickle cell disease: a case series

Abstract

Sickle cell disease (SCD) is the most common inherited haematological disorder, producing a mutation of the haemoglobin molecule known as haemoglobin S (HbS). The presence of HbS in the erythrocyte makes it prone to sickling — a process that may lead to vaso-occlusive injury, haemolysis and a hypercoagulable state. Sickling is precipitated by dehydration, hypoxia, hypothermia, acidosis and low flow situations. Over time, multi-organ damage develops with significant morbidity and mortality. Paediatric patients with SCD and congenital heart defects may require anaesthesia for corrective cardiac surgery on cardiopulmonary bypass (CPB). During the perioperative period these high-risk patients may suffer significant complications when exposed to the conditions that favour erythrocyte sickling. This case series details experience of four paediatric patients with SCD who underwent corrective cardiac surgery at Red Cross War Memorial Children’s Hospital. The pathophysiology is discussed and the perioperative management of transfusion, cardiopulmonary bypass and temperature regulation is highlighted.