Pregnancy, delivery, and the postpartum period in women with hereditary angioedema with C1 inhibitor deficiency: a review of current management approaches and analysis of survey data from Belarusian patien

Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) requires particular attention during pregnancy because of the unpredictable effects of hormonal changes on disease activity, the risk of potentially severe attacks, and the need to select treatment that is safe for both the mother and the fetus. This article summarizes current evidence from the literature and up-to-date recommendations for the management of women with C1-INH-HAE during pregnancy, delivery, and breastfeeding. The available evidence demonstrates that HAE is not a contraindication to pursuing reproductive plans. In addition, we present the results of a survey of 8 Belarusian women with C1-INH-HAE who had a total of 19 pregnancies, 14 of which resulted in delivery. Disease course in this cohort was highly variable: an increase in attack frequency was reported in 6/14 (42,9%) of pregnancies, near-complete cessation of attacks in 5/14 (35.7%), and no substantial change in 3/14 (21,4%); moreover, the course of the disease could differ between successive pregnancies. In one case, an abdominal HAE attack was misdiagnosed as an acute surgical condition, resulting in appendectomy at 26 weeks of gestation, followed by pregnancy loss. Most deliveries occurred vaginally (12/14, 85,7%), while HAE attacks within the first 72 hours after delivery occurred in 3/14 cases (21,4%). The current approaches to the treatment and prophylaxis of C1-INH-HAE presented in this article may serve as a practical guide for healthcare professionals involved in the management of patients with HAE, facilitating timely diagnosis and treatment of attacks, rational selection of therapy, and improvement of patients’ quality of life.