Objectives:Aortic dissection in patients with Marfan and related syndromes (HTAD) is a serious pathology whose treatment by thoracic endovascular repair (TEVAR) is still under debate. The aim of this study was to assess the results of the TEVAR for aortic dissection in patients with HTAD as compared to a young population without HTAD.Methods:The study received the proper ethical oversight. We performed an observational exposed (confirmed HTAD) vs. non-exposed (<65 years old) study of TEVAR-treated patients. The preoperative, 1 year, and last available CT scans were analyzed. The thoracic and abdominal aortic diameters, aortic length, and volumes were measured. The entry tears and false lumen (FL) status were assessed. The demographic, clinical, and anatomic data were collected during the follow-up.Results:Between 2011 and 2021, 17 patients were included in the HTAD group and 22 in the non-HTAD group. At 1 year, the whole aortic volume increased by +21.2% in the HTAD group and by +0.2% the non-HTAD groups,p= 0.005. An increase in the whole aortic volume > 10% was observed in ten cases (58.8%) in the HTAD group and in five cases (22.7%) in the non-HTAD group (p= 0.022). FL thrombosis was achieved in nine cases (52.9%) in the HTAD group vs. twenty (90.9%) cases in the non-HTAD group (p< 0.01). The risk factors for unfavorable anatomical evolution were male gender and the STABILISE technique. With a linear model, we observed a significantly different aortic volume evolution between the two groups (p< 0.01) with the STABILISE technique; this statistical difference was not found in the TEVAR subgroup. In the HTAD patients, there was a significant difference in the total aortic volume evolution progression between the patients treated with the STABILISE technique and the patients treated with TEVAR (+160.1 ± 52.3% vs. +47 ± 22.5%,p< 0.01 and +189.5 ± 92.5% vs. +58.6 ± 34.8%,p< 0.01 at 1 year and at the end of follow-up, respectively).Conclusions:TEVAR in the HTAD patients seemed to be associated with poorer anatomical outcomes at 1 year. This result was strongly related to the STABILISE technique which should be considered with care in these specific patients.