The acute chest syndrome of sickle cell disease following aortic valve replacement

The acute chest syndrome (ACS) of sickle cell disease (SCD) is a leading cause of death in SCD, with a high incidence following surgery, though only one case has been reported following cardiac surgery. We present a case of ACS in an adult undergoing aortic valve replacement (AVR) despite instituting established peri-operative optimization measures to prevent sickling. Early diagnosis of this condition in our patient as a distinct clinical entity facilitated appropriate, specific therapy and a good subsequent postoperative recovery. Greater recognition of this syndrome in the growing number of adult sickle cell patients presenting for cardiac surgery may help improve their outcome.