Cold agglutinin disease complicating management of aortic dissection

Background: Cold agglutinin disease is characterized by acrocyanosis, hemolytic anemia, and occasionally, frank hemoglobinuria. Although cold agglutinins are commonly detected, they are rarely clinically significant due to subphysiologic temperatures at which agglutination occurs. Cardiovascular surgical procedures requiring hypothermia present a unique challenge for these patients, requiring modification of the conduct of cardiopulmonary bypass and cardioplegia.

Case report: Herein we report a case of a patient with a prior history of symptomatic cold agglutinin disease and type A aortic dissection, presenting with dilation of his known diseased ascending aorta, requiring semi-urgent repair. The patient underwent plasma exchange on two successive days preceding surgery to reduce the cold agglutinin titre. A modified Bentall procedure and replacement of ascending aorta and hemiarch under deep hypothermic circulatory arrest was performed without complication.

Conclusions: This case demonstrates the efficacy of employing plasma exchange in preparation for cardiac surgery with deep hypothermic circulatory arrest in a patient with clinically significant cold agglutinin disease. Plasma exchange alone may be sufficient in preparing patients with cold agglutinin disease for procedures requiring significant hypothermia when the delayed onset of action of alternative therapies is not acceptable. Choice of replacement fluid is critical in ensuring maintenance of coagulation proteins perioperatively and minimizing complement activation.