Repair of type A aortic dissection in a patient with sickle cell disease

We describe a patient with homozygous HbSS sickle cell anemia and end stage renal disease who presented to our medical center with a Stanford type A aortic dissection. His dissection was successfully managed with a hemiarch repair and concomitant bio-Bentall aortic root replacement. Intraoperatively, he received exchange transfusion with omittance of cell saver. Postoperatively, he remained free of sickling events with a very low HbS fraction. His fluid status was managed by continuation of hemodialysis. His hospital course was otherwise uncomplicated, and he was discharged to home on postoperative Day 6.