Introduction: Hereditary Angioedema is a rare disease caused by C1 esterase inhibitor deficiency leading to diffuse and potentially life-threatening oedema formation. Preventing attacks is critical, particularly for patients undergoing cardiac surgery.
Case report: We report a case of a 71-years-old woman with a history of Hereditary Angioedema scheduled for open-heart surgery on Cardiopulmonary Bypass. Multidisciplinar teamwork and patient-targeted strategy were crucial to obtain a favorable outcome.
Discussion: Cardiac surgery is a major stressor for Angioedema attacks because of Complement cascade and inflammatory response activation leading to potential life-threatening oedema formation. In literature only few cases of complex open heart surgery under Cardiopulmonary Bypass are described.
Conclusion: Continuous updating and multidisciplinarity are key elements to manage patients with Hereditary Angioedema in cardiac surgery in order to reduce morbidity and mortality.
Keywords: C1 esterase inhibitor deficiency; cardiac surgery; cardiopulmonary bypass; hereditary angioedema; perioperative management.