Impact of connective tissue disease on the surgical outcomes of aortic dissection in patients with cystic medial necrosi
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RESEARCH ARTICLE
Open Access
Impact of connective tissue disease on the surgical outcomes of aortic dissection in patients with cystic medial necrosis Toshiki Fujiyoshi4*, Kenji Minatoya1, Yoshihiko Ikeda2, Hatsue Ishibashi-Ueda2, Takayuki Morisaki3, Hiroko Morisaki3 and Hitoshi Ogino4
Abstract 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. Keywords: Aortic dissection, Surgery, Genetically diagnosed connective tissue disease, Cystic medial necrosis
Background Cystic medial necrosis (CMN) or degeneration is found in surgical specimens of aortic dissection, and tends to be associated with higher risks of various aortic complications including aortic dissection and dilatation [1–3]. In addition, CMN is also considered as one of the histological markers for connective tissue diseases (CTD), including Marfan syndrome, Loeys-Dietz syndrome, and Ehlers-Danlos syndrome. Although some differences are found in the degree of loss of elastic fibers, there are no significant differences in the histopathological patterns within the aortic wall between CTD and non-CTD patients, especially in patients who are of advanced age and have systemic hypertension for a long time [4, 5].
Further genetic examinations are then highly recommended to diagnose CTD more precisely. However, for every patient with aortic dissection, there are difficulties in performing routine genetic examinations which are generally carried out when association with CTD is suspected, based on bodily features and family histories. On the other hand, there have been few published studies looking at the adverse effects and relationships of CTD and CMN to the surgical outcomes of aortic dissection. Under these circumstances, we wanted to determine the imp
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