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Objectives To evaluate the mid-term outcomes, and the aortic remodeling in Marfan syndrome (MFS) patients with type B dissection that were treated with endovascular repair. Background MFS is a relative contraindication to thoracic endovascular aortic repair (TEVAR). Mid-term aortic outcomes data in MFS after TEVAR are limited, and the occurrence of late events remains unclear. Methods Of 89 patients that underwent TEVAR between September 2002 and February 2011, 10 patients with mid-term follow-up fulfilled the Ghent criteria for MFS and complicated type B dissection. High risk for open surgery was documented in 90%. Results The mean age was 35.1 ± 9.4 years and all patients presented with acute aortic syndrome complicating a chronic type B dissection (DeBakey type IIIb). Five patients underwent a Bentall surgical procedure previous to endovascular repair, and in four patients initial TEVAR was followed by surgery of the ascending aorta. Treatment was limited to endovascular repair in only one patient. In-hospital mortality was 10%. At a mean follow-up of 59.6 ± 38.9 months, the cumulated mortality was of 20% and late mortality 11.1%. The rate of secondary endoleak was 44.4%, and late reintervention of 33.3%. Survival freedom from cardiovascular death at 8 years was 80.0%, and positive remodeling was documented in 37.5% of patients. Conclusions Our results suggest that TEVAR is feasible, safe, and associated with a high reintervention rate and reduced rate of positive aortic remodeling in patients with Marfan syndrome. Survival at 8 years was comparable to contemporary series of open repair. © 2012 Wiley Periodicals, Inc.