D2.364 - Perioperative Management and Safety of Bradykinin-Mediated Angioedema: A 9-Year Retrospective Study

Bradykinin-mediated angioedema (BK-AE) is characterized by recurrent swelling with potential life-threatening airway compromise, particularly during medical procedures. We aimed to evaluate the safety of surgical and obstetric procedures in patients with hereditary (HAE) or acquired angioedema (AAE) at a reference center.

We conducted a retrospective study (2015–2023) of patients with HAE or AAE-C1INH undergoing procedures under general (GA) or locoregional anesthesia (LA). Clinical characteristics, anesthetic management, and outcomes were analyzed.

The study included 31 patients (22 females; median age 49 years) comprising 22 HAE (14 C1-INH deficiency, 8 FXII mutation) and 9 AAE-C1INH, of whom one displayed anti C1INH antibodies. Fifteen patients (48%) had a history of upper airway attacks. A total of 34 procedures were performed, including 13 abdominal surgeries, 8 gynecological/obstetrical procedures, 7 endoscopies, and 5 ENT/maxillofacial surgeries. General anesthesia was performed in 23 cases (68%) and LA in 11. Airway management involved tracheal intubation in 15 cases, with no reported difficulties. Short-term prophylaxis with plasma derived C1INH (pdC1-INH) concentrate was administered in 30 procedures (88%) at a median dose of 25 UI/kg (IQR 24–26). No intraoperative complications occurred. Postoperatively, 3 moderate angioedema attacks were recorded (8.8%), but none occurred in patients receiving pdC1-INH prophylaxis. No laryngeal attacks were reported. The median hospital length of stay was 2 days.

Surgical procedures, including those requiring tracheal intubation, can be performed safely in HAE and AAE patients. Short-term prophylaxis with pdC1-INH is highly effective in preventing perioperative attacks. Given the absence of severe complications, outpatient management or reduced hospital stays may be feasible for selected patients.