1887

oa Southern African Journal of Anaesthesia and Analgesia - Paediatric cardiac anaesthesia in sickle cell disease : a case series : research

 

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 oncardiopulmonary 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.

Loading

Article metrics loading...

/content/medsajaa/22/3/EJC191197
2016-01-01
2016-12-06
This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error