Hereditary angioedema (HAE) is a rare, potentially life-threatening genetic disorder caused by dysregulation of the kallikrein-kinin pathway and excessive bradykinin generation. Although advances in targeted therapies have substantially improved disease control and prognosis, patients with HAE remain vulnerable to procedure-related attacks triggered by tissue trauma, psychological stress, and airway manipulation. As the HAE population ages and cardiovascular comorbidities become increasingly prevalent, interventional cardiologists are increasingly likely to encounter patients requiring coronary angiography and percutaneous coronary intervention (PCI).

The periprocedural management of HAE in the catheterization laboratory presents unique challenges. Invasive vascular procedures may potentially precipitate angioedema attacks, while urgent coronary revascularization must not be delayed because of concerns related to HAE. Current evidence is largely derived from expert consensus, perioperative recommendations, and limited clinical experience rather than dedicated cardiovascular studies.

This review summarizes the current understanding of HAE pathophysiology relevant to invasive cardiology, discusses available prophylactic and on-demand treatment strategies, and proposes a practical management algorithm for patients undergoing coronary angiography and PCI. Particular emphasis is placed on risk stratification, short-term prophylaxis, procedural planning, airway management, and multidisciplinary collaboration. Adoption of standardized institutional protocols may improve procedural safety and clinical outcomes in this unique patient population.

The review also incorporates the recommendations of the recently published 2025 World Allergy Organization Guidelines, providing an updated framework for individualized periprocedural management in contemporary invasive cardiology practice.