Background: Aortic dissection with malperfusion necessitates emergency surgery and is associated with poor outcomes. Therefore, in this study, we analyzed patients' treatment courses from the initial management to hospital discharge in cases of acute aortic dissection (AAD) with malperfusion and investigated the risk factors associated with mortality., Methods: We evaluated cases of AAD with malperfusion treated at our institution over a 16-year period from 2007 to 2022. The primary endpoint was in-hospital mortality. The study's primary outcome measure was mortality during hospitalization. We collected and analyzed data encompassing preoperative patient characteristics, Stanford classification, sites of preoperative malperfusion, surgical techniques employed, and postoperative complications. These variables were examined to identify factors associated with in-hospital mortality., Results: During the study period, 366 patients were admitted with AAD, 102 of whom had malperfusion. There were 62 men (61%) and 40 women (39%), with a mean age of 64 ± 13 years (range: 28-87 years). According to the Stanford classification, 75 (74%) and 27 (26%) patients had type A and B aortic dissection, respectively, and 29 patients (28%) presented with shock. Preoperative malperfusion sites included the brain, coronary arteries, abdominal viscera, limbs, and spinal cord in 40 (39%), 10 (10%), 34 (33%), 52 (51%), and six (6%) patients, respectively. Eleven (11%) patients required immediate intervention in the emergency department. The treatments administered to the patients were as follows: ascending aortic replacement, 30 (29%) patients; aortic arch replacement, 34 (33%) patients; root replacement, three (3%) patients; thoracic endovascular aortic repair (TEVAR), 12 (12%) patients; non-anatomic bypass, five (5%) patients; and conservative management, five (5%) patients. In-hospital mortality occurred in 23 (23%) patients. Multivariate logistic regression analysis identified preoperative coronary malperfusion as an independent risk factor of mortality., Conclusion: Preoperative coronary malperfusion is an independent risk factor for in-hospital mortality in patients with AAD presenting with malperfusion., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nakajima et al.)