Normothermic total arch replacement without hypothermic circulatory arrest to treat aortic distal arch aneurysm in a patient with cold agglutinin disease

Abstract

Cold agglutinin disease although rare, can lead to serious complications for patients undergoing cardio-thoracic surgery, especially when cardiopulmonary bypass is applied under hypothermic circulatory arrest. We describe normothermic total arch replacement without hypothermic circulatory arrest in a patient with cold agglutinin disease. The patient tolerated all procedures well and did not develop cerebral ischemia due to surgical maneuvers or thrombotic or haemolytic complications due to cold agglutinin disease. Although endovascular aortic repair is the first choice under such complex conditions, this method could also serve as an alternative strategy when endovascular aortic repair is precluded.

Cold agglutinin disease

“…5,6 Fatal complications have been reported, with exacerbations of hemolysis triggered by febrile illness, trauma, or surgery. [38][39][40][41][42][43][44][45] In addition to hemolysis, clinical manifestations include coldinduced circulatory symptoms; livedo reticularis, Raynaud disease, acrocyanosis and, rarely, cutaneous necrosis. 20,[46][47][48][49] Berentsen et al 5 reported cold-induced symptoms in more than 90% of patients.…”

Single Stage Aortic Arch Replacement Without Circulatory Arrest

“…However, it was ruled out for two reasons: 1) the lack of an adequate proximal landing zone for the deployment of the endovascular graft, and 2) the need for surgical resection of the diverticulum in order to resolve the dysphagia. Although some authors have described aortic arch replacement without DHCA [1,2], the traditional surgical approach for the treatment of this pathology requires CPB and DHCA and is associated with mortality and morbidity [3,4]. We ruled out this option in view of our patient's malnourishment and frail condition.…”

Single Stage Aortic Arch Replacement without Circulatory Arrest

“…Although some authors have described aortic arch replacement without DHCA [1,2], the traditional surgical approach for the treatment of this pathology requires CPB and DHCA and is associated with mortality and morbidity [3,4]. We ruled out this option in view of our patient's malnourishment and frail condition.…”