Partial arch replacement for type A aortic dissection with cold agglutinin disease after sutimlimab

Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia that can lead to significant complications due to hemolysis during aortic surgery, requiring hypothermic circulatory arrest. Sutimlimab, a humanized monoclonal IgG4 antibody that binds to and inactivates the complement protein C1s, is a complement inhibitor used for the treatment of CAD. However, there are no reports of its use in aortic surgery that required hypothermic circulatory arrest. We describe the case of an 80-year-old female with acute type A aortic dissection and a 55-mm ascending aortic aneurysm. The patient was scheduled to undergo urgent surgery for type A aortic dissection and aortic aneurysm; however, CAD was detected. Under consultation with hematologists, sutimlimab was initiated three days before surgery for CAD. Partial arch replacement was performed by using the elephant trunk technique under mild hypothermic circulatory arrest with cerebral perfusion and cold cardioplegia. The postoperative course was uneventful. On postoperative day 18, the patient was discharged without any hemolysis-related deficits. Herein, we report a case of partial arch replacement with mild hypothermic circulatory arrest for type A aortic dissection and an aortic aneurysm with CAD after sutimlimab treatment.

There are no established methods for the perioperative management of patients with preexisting cold agglutination disease undergoing cardiovascular surgery requiring hypothermic circulatory arrest. Sutimlimab, an anti-complement (C1s) monoclonal antibody, is relatively easy to administer and may help avoid postoperative hemolytic complications.