Open-heart surgery in a patient with heterozygous alpha 2-antiplasmin deficiency. Perioperative strategies in the first reported case.

Abstract

alpha 2-Antiplasmin deficiency is a serious coagulation disorder that results in unrestrained fibrinolytic activity. Clinically, it is manifested by instability of the fibrin hemostatic plug and prolonged or delayed bleeding, which is more serious in patients who are homozygous for this trait. A patient scheduled for aortic valve replacement and coronary bypass presented with a history of repeated episodes of postoperative bleeding. Hemostatic laboratory evaluation revealed that the patient had the heterozygous form of alpha 2-antiplasmin deficiency with a serum concentration of 52 percent (normal, greater than 65 percent of the activity of pooled plasma). He underwent preoperative plasmapheresis with administration of 3,000 ml of fresh frozen plasma, which resulted in an increase in the preoperative level of alpha 2-antiplasmin to 78 percent. Although postoperative blood loss was greater than normal, it was easily managed. Preoperative identification of this rare coagulation abnormality permitted appropriate treatment and probably prevented a postoperative death from hemorrhage.