• Ryan Thomas, Arjune S Dhanekula, Peter Byers, Rachel Flodin, Scott DeRoo, Sherene Shalhub, and Christopher R Burke.
• University of Washington School of Medicine, Seattle, WA.
• J. Thorac. Cardiovasc. Surg. 2024 Sep 24.

ObjectiveMarfan syndrome (MFS) is a genetic disorder with increased risk of aortic dissection. Currently, type A aortic dissection risk is mitigated by aortic root replacement with Dacron. It is unclear if root replacement increases the risk of distal aortic disease given the non-compliant nature of Dacron.MethodsAll adult patients with a diagnosis of MFS at a single academic center, excluding those with history of dissection or concomitant arch repair, were studied (n=322). Student's t-test or Wilcoxon-Mann-Whitney test were used for continuous variables; Chi-squared or Fisher's exact test for categorical variables. Propensity matching used age, sex, hypertension, race, BMI, family history of MFS, and genetic mutational class. Differences in freedom from type B aortic dissection (TBAD) were determined using the log-rank test.Results124 patients underwent root replacement (RR) compared to 198 patients with no prior aortic surgery (NRR). Median follow-up time was 9.90 years. Male sex, weight, and hypertension prevalence was higher in the RR group (p<0.05). Distribution of fibrillin-1 mutations was homogenous (p>0.9). TBAD frequency in the RR group was higher (21% (n=20) vs 4.2% (n=4), p<0.001). Aortic-related mortality was higher in the RR group (11% (n=14) vs. 3.5% (n=7), p<0.01). Distal aortic intervention frequency was higher in the RR group (p=0.009).ConclusionsMarfan syndrome patients who undergo elective aortic root replacement appear to have a higher incidence of subsequent type B aortic dissection, independent of other risk factors. Careful consideration must be made to the management of the distal aorta in MFS patients who undergo root replacement.Copyright © 2024. Published by Elsevier Inc.

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