Procedure-specific risk of acute attacks in hereditary angioedema: A 410-procedure cohort analysis

Hereditary angioedema (HAE) is characterized by recurrent, potentially life-threatening attacks often triggered by surgical procedures. Standard risk assessments frequently overlook the invasiveness of procedures and tissue trauma, limiting individualized perioperative management.

To evaluate the procedure-specific risk of acute HAE attacks and assess the effectiveness of prophylactic strategies across diverse surgical and interventional categories.

This retrospective cohort study analyzed 410 procedures in 58 patients with C1-inhibitor-deficient HAE. Interventions were categorized as major surgery, minor surgery, diagnostic, obstetric, or minimally invasive cosmetic procedures based on invasiveness and airway involvement. Attacks within 72 hours postprocedure were recorded.

Overall, procedure-related attacks occurred in 12.9% (53 of 410) of cases. Major surgery carried the highest risk (34.7%, 17 of 49), with attack rates reaching 47.8% (11 of 23) without prophylaxis vs 23.1% (6 of 26) with prophylaxis. Minor surgery showed an 8.9% (4 of 45) attack rate; notably, no attacks occurred in patients receiving prophylaxis (0 of 12) compared with 13.0% (3 of 23) without it. For circumcision, the rate was 13.3% overall. Diagnostic procedures had an 18.18% (2 of 11) attack rate, occurring only without prophylaxis. Minimally invasive cosmetic procedures posed the lowest risk (1.94%, 5 of 258). Statistical analysis confirmed that the risk with major surgery significantly exceeded that with minor surgery (P = .023) and cosmetic procedures (P < .001). In addition, cosmetic procedures carried a lower risk than minor (P = .046) and diagnostic interventions (P = .043).

The HAE attack risk follows an invasiveness-dependent gradient. Major surgeries and obstetric procedures carry the highest risk, whereas minor and aesthetic interventions demonstrate significantly lower rates, emphasizing the need for stratified prophylactic protocols.