The differences in surgical long-term outcomes between Marfan syndrome and Loeys–Dietz syndrome

ABSTRACT Objectives The aim of this study was to disclose the differences of surgical outcomes between Marfan syndrome (MFS) with mutations in fibrillin-1 gene (FBN1) and Loeys-Dietz syndrome (LDS) with mutations in transforming growth factor-beta receptor (TGFBR) 1 and TGFBR2. Methods We reviewed 368 patients Results Freedom from all causes of mortality rate was similar between the two groups (p=0.40, log-rank). The cumulative incidence of reintervention was significantly lower in the MFS group than the LDS group (p = 0.016, Gray). The cumulative incidence of first aortic arch reoperation for aortic arch aneurysm was significantly lower in the MFS group than the LDS group (p Conclusion LDS with mutations in TGFBR1 and TGFBR2 revealed higher rates of reoperation and more specifically the arch reoperation was higher in LDS than MFS. Aggressive arch surgery in initial operation on proximal aorta is recommendable in LDS to avoid additional aortic event. In MFS, that is controversial in patients without dissection for low possibility to expand.