Supplementation of C1-esterase inhibitor concentrates for a patient suffering from hereditary angioedema undergoing complex open-heart surgery.

Hereditary angioedema (HAE) is an autosomal dominantly inherited deficiency of C1-inhibitor, and it is an extremely rare condition. During surgery, oedema can be induced by a variety of stresses, and a high mortality rate has been reported. Since open-heart surgery involves cardiopulmonary bypass, the inflammatory response and complement activity are increased, meaning that an even greater risk can be anticipated. Perhaps for this reason, the only reports to date of cases of open-heart surgery have been cases of short-term cardiopulmonary bypass or off-pump coronary artery bypass grafting (CABG). We provide the first report of long-term cardiopulmonary bypass (longer than 5h) for open-heart surgery in a patient with HAE that did not result in any postoperative decline in respiratory function, systemic oedema, laryngeal oedema or similar complications, and a favourable outcome was obtained.