Despite a shared degenerative vascular phenotype, Marfan syndrome (MFS), Loeys-Dietz syndrome (LDS) and other genetically distinct connective tissue diseases (CTD) have unique extravascular pathologies that impact the outcomes of aortic replacement. The aim of our study was to investigate the association of CTD genotype with postoperative outcomes and branch patency following open thoracoabdominal aortic replacement in a large institutional cohort.
All patients undergoing open branched thoracoabdominal aortic replacement at a single academic center from 2006-2020 were included and classified as CTD or non-CTD based on the presence of genotypic documentation. Outcomes were compared using ANOVA and χ testing for continuous and discrete variables, respectively. Kaplan-Meier curves were utilized 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%); specifically, 32 had MFS, 5 had LDS and 8 had another CTD. CTD patients had more extent II thoracoabdominal aneurysms (40% vs 15%), more reconstructed branches (3.5 vs 1.8), more frequently reconstructed visceral branches (86.7% vs. 22.7%) and higher intraoperative blood loss (13.3L vs. 6.8L, all p<0.05) compared to non-CTD patients. MFS patients were more frequently systemically anticoagulated preoperatively (50% vs 5%) and demonstrated higher rates of postoperative DVT/PE compared to non-CTD patients (9% vs 2%, both p<0.05). 5-year renal branch patency was decreased among all patients compared to visceral branches (87.3% vs. 95.6%, p=0.05), but there were no individual branch patency differences between CTD and non-CTD patients (p=0.086). Overall branch patency at 1 and 5 years was significantly higher in MFS than in non-CTD patients (98.9% vs 89.1% at 5 years); there were no significant patency differences between non-CTD patients and any other CTD subgroup, mostly due to early patency loss.
Open thoracoabdominal reconstruction in CTD is technically challenging, associated with increased transfusion and postoperative thromboembolic events when compared with non-CTD patients. Technical outcomes of the procedure are excellent and are differentially associated with genotype, with MFS patients experiencing significantly improved branch patency over both non-CTD patients and other CTD patients, a finding which has multifactorial drivers.

Copyright © 2021. Published by Elsevier Inc.