An uncommon case of postpartum venous thrombosis in a patient with hereditary angioedema. Patient from the ITACA Cohort (Italian Network for Hereditary and Acquired Angioedema)

Abstract

Hereditary angioedema (HAE) is a rare genetic condition characterized by episodes of cutaneous or submucosal edema, most commonly affecting the skin, the abdomen, and the upper respiratory tract. The most common cause of HAE is either a deficiency (type 1) or dysfunction (type 2) of the C1-inhibitor, leading to the overproduction of bradykinin and activation of bradykinin B2 receptors. This increases vascular permeability and results in angioedema attacks. Anatomic, physiological, and hormonal changes during pregnancy can have an impact on the manifestations of the disease and therefore its treatment. Here, we describe the case of a 30-year-old woman who experienced a significant worsening in both the number and severity of angioedema attacks during pregnancy. The cesarean section was complicated by thrombosis of the ovarian vein and inferior vena cava.

Keywords

• Hereditary angioedema
• postpartum venous thrombosis
• C1-inhibitor
• pregnancy