Hereditary Angioneurotic Edema: Report of a Case Undergoing Open-Heart Surgery— A Case Report

Abstract

Hereditary angioneurotic edema is a familial disease due to the absence or to the functional deficiency of the inhibitor of the activated first component of complement. It is characterized by recurrent attacks of localized edema affect ing skin and mucosal membranes; edema of the laryngeal mucosa may be fatal. Because trauma and surgical intervention may become a trigger of attacks, a pitfall for surgeons or physicians is always present. We recently encountered a patient with hereditary angioneurotic edema who successfully underwent open- heart surgery following short-term prophylaxis with danazol and fresh frozen plasma transfusion.