Aortic valve replacement in patients with systemic mastocytosis.

Systemic mastocytosis is a hematologic disorder with important perioperative implications. A variety of stimuli and medications can cause severe anaphylaxis in these patients. We report successful preoperative, intraoperative, and postoperative management of a patient with systemic mastocytosis who underwent an aortic valve replacement and review the literature pertaining to cardiac surgery in these patients.