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Background  A retrospective analysis was performed to determine the impact of genetically diagnosed connective tissue disease (CTD) on the early and late outcomes of surgical treatment for aortic dissection in patients having aortic pathology associated with cystic medial necrosis (CMN).    Methods  Between 2003 and 2013, a total of 43 patients (37 ± 12.8 years old, 23 men, 20 women) who had undergone surgery for aortic dissection associated with CMN in the aortic wall underwent genetic examinations. Subsequently, there were 30 patients with CTD (CTD group) and 13 without CTD (non-CTD group).    Results  There were no early or late deaths (the follow-up rate was 100% for 57.1 ± 43.0 months). The median age was significantly lower in the CTD group ( p  = 0.030). The rate of elastic fiber loss was significantly higher in the CTD group ( p  = 0.014). In the long-term follow-up, there were no significant differences in the incidences of re-dissection ( p  = 0.332). However, re-operations were required more frequently in the CTD group ( p  = 0.037).    Conclusions  In patients with CTD as well as CMN, the onset of aortic dissection tends to be earlier, which would result in higher rates of re-operation, compared with the non-CTD group. Closer and stricter follow-up with medication and adequate surgical treatments with appropriate timing are mandatory for such high-risk patients.

Impact of connective tissue disease on the surgical outcomes of aortic dissection in patients with cystic medial necrosis