Elective root replacement increases the risk of type B dissection in patients with Marfan syndrome.

Objective: Marfan syndrome 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 noncompliant nature of Dacron.

Methods: All adult patients with a diagnosis of Marfan syndrome at a single academic center, excluding those with history of dissection or concomitant arch repair, were studied (n = 322). Student t test or Wilcoxon Mann-Whitney test was used for continuous variables; chi-square or Fisher exact test was used for categorical variables. Propensity matching used age, sex, hypertension, race, body mass index, family history of Marfan syndrome, and genetic mutational class. Differences in freedom from type B aortic dissection were determined using the log-rank test.

Results: A total of 124 patients underwent root replacement compared with 198 patients with no prior aortic surgery. Median follow-up time was 9.90 years. Male sex, weight, and hypertension prevalence were higher in the root replacement group (P < .05). Distribution of fibrillin-1 mutations was homogenous (P > .9). Type B aortic dissection frequency in the root replacement group was higher (21% [n = 20] vs 4.2% [n = 4], P < .001). Aortic-related mortality was higher in the root replacement group (11% [n = 14] vs 3.5% [n = 7], P < .01). Distal aortic intervention frequency was higher in the root replacement group (P = .009).

Conclusions: Patients with Marfan syndrome 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 patients with Marfan syndrome who undergo root replacement.