Total Arch Replacement for Aortic Arch Aneurysm with Cold Agglutinin Disease after Rituximab and Plasmapheresis

Abstract Background: Cold agglutinin disease can lead to significant complications, specifically for patients undergoing arch repair requiring hypothermic circulatory arrest. Rituximab and plasmapheresis are treatments for cold agglutinin disease; therefore, we used them under consultation of hematologists before mild hypothermic aortic arch surgery maintaining the body temperature above thermal altitude. Case presentation: We describe an 86-year-old male with acute Type A aortic dissection who had been treated for rheumatoid arthritis and a 55-mm thoracic aortic aneurysm as an outpatient. The patient was scheduled to undergo urgent surgery for a Type A intramural hematoma and progressive aortic aneurysm; however, blood clotting was observed on a laboratory test and cold agglutinin was detected. Consequently, we rescheduled the urgent surgery. Under consultation of hematologists, rituximab was initiated 3 months before surgery, and plasmapheresis was performed 2 days before surgery for cold agglutinin disease. Then, total arch replacement under mild hypothermia with the frozen elephant trunk technique was performed by maintaining cerebral and lower body perfusion. The postoperative course was uneventful. On the 42nd postoperative day, the patient was discharged without any neurological deficits. Conclusions: We report a case of total arch replacement with mild hypothermia for aortic arch aneurysm with cold agglutinin disease after rituximab treatment and plasmapheresis.