Hereditary angioedema in cardiac surgery: Perioperative management considerations for a rare disease

Author:

Adami Enrica Chiara1ORCID,Magri Federica2,Plotti Chiara2,Renzi Stefania2,Chiarini Giovanni2ORCID,De Cicco Giuseppe3

Affiliation:

1. Cardiothoracic Intensive Care Unit, Cardiothoracic Department, ASST Spedali Civili di Brescia, Brescia, Italy

2. Division of Anesthesiology, Intensive Care and Emergency Medicine, University of Brescia, Brescia, Italy

3. Cardiac Surgery Division, Cardiothoracic Department, ASST Spedali Civili di Brescia, Brescia, Italy

Abstract

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.

Publisher

SAGE Publications

Subject

Advanced and Specialized Nursing,Cardiology and Cardiovascular Medicine,Safety Research,Radiology, Nuclear Medicine and imaging,General Medicine

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