Anesthesia and Perioperative Management in Bentall Operation and Ascending Aorta Replacement in Patients with Marfan Syndrome Complicated with Cold Agglutinin Disease

Abstract

A-34-year old female patient with Marfan syndrome with elevated cold agglutinins was scheduled to recieve Bentall and replacement of the ascending aorta and aortic arch under total cardio-pulmonary bypass with deep hypothermia and retrograde cold cardioplesia. Preoperatively, the patient recieved double filtration-plasmapheresis (DFPP) to reduce plasma cold agglutinin titers. No adverse effects due to cold agglutinin appeared during perioperative course, nevertheless the patient suffered from subdural hemorrhage and mediastinitis postoperatively. Reduction of fibrinogen and immunogloblin by DFPP was suspected as one of the causes of these complications.