This study states that Despite a shared degenerative vascular phenotype, Marfan syndrome (MFS), Loeys-Dietz syndrome, and other genetically distinct connective tissue diseases (CTDs) have unique extravascular pathologic changes that affect the outcomes of aortic replacement. The aim of our study was to investigate the impact of CTD genotype on postoperative outcomes and branch patency in open thoracoabdominal aortic replacement in a large institutional cohort.

All patients undergoing open branched thoracoabdominal aortic replacement at a single academic center from 2006 to 2020 were included and classified as CTD or non-CTD on the basis of the presence of genotypic documentation. Outcomes were compared using analysis of variance and χ2 testing for continuous and discrete variables, respectively. Kaplan-Meier curves were used to examine patency of graft branches over time. Overall, 172 patients were included with a mean follow-up of 30.5 ± 34.9 months. CTD was present in 45 patients (26%), and 32 had MFS specifically. CTD patients had more type II aneurysms (40% vs 15%), more reconstructed branches.

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