Anesthetic management of a surgical patient with an acute aortic dissection complicated by hemoglobin Kansas

BACKGROUND: Hemoglobin Kansas (Hb Kansas) is a rare disease with cyanosis. We report a case of anesthetic management for a patient with an acute aortic dissection complicated by Hb Kansas. CASE PRESENTATION: We encountered a 62-year-old male, surgical patient with an acute aortic dissection complicated by postoperative Hb Kansas. During anesthesia, his arterial oxygen saturation was low, while the partial pressure of arterial oxygen was within the normal range. The patient underwent ascending aortic replacement under hypothermic circulation arrest with a bladder temperature of 22 °C after introducing cardiopulmonary bypass. The patient was then referred to the hematology department for detailed examination and was diagnosed as having Hb Kansas through genetic analysis at 2 months after surgery. CONCLUSIONS: Except for apparent cyanosis, Hb Kansas causes no clinical problems because the delivery of oxygen to peripheral tissues may be enhanced for such patients. When we perform anesthetic management for cyanosis patients with unknown causes, it is necessary to consider the oxygen supply-demand balance.