Sickle cell anaemia and the consequences on the anaesthetic management of cardiac surgery.

A review of the available literature on genetics and pathophysiology of Sickle Cell Anaemia was performed with special emphasis on the intraoperative management during cardiac surgery. Hypoxia, acidosis and hypothermia have been identified as independent sickling provoking factors. Although no official guidelines on transfusion for Sickle Cell patients have been published, useful directives on preoperative transfusion could be derived from available data. Additionally, we bundled and reviewed the published expertise in the management of cardiopulmonary bypass and the necessity of hypothermia during cardiac surgery in Sickle Cell patients. Our conclusion is that the available data in case reports and case series on cardiac surgery in case of Sickle Cell Anaemia suggest a necessary preoperative or on bypass blood transfusion to guarantee an uncomplicated course of cardiopulmonary bypass and hypothermia.