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

The Journal of Thoracic and Cardiovascular Surgery Sep 08, 2020

Seike Y, Matsuda H, Inoue Y, et al. - Surgical outcomes of Marfan syndrome with mutations in fibrillin-1 gene vs Loeys–Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2 were determined. From 368 reviewed patients (aged less than 50 years) who underwent surgery for thoracic aortic diseases between 1988 and 2019, they enrolled 99 patients with Marfan syndrome (26.9%; 57 men, 33 ± 7.5 years) and 24 patients with Loeys–Dietz syndrome (6.5%; 13 men, 28 ± 11 years). Findings revealed higher rates of reoperation among Loeys-Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2; more specifically, those with Loeys–Dietz syndrome had higher arch reoperation than those with Marfan syndrome. They recommend performing aggressive arch surgery in the initial operation on the proximal aorta in Loeys–Dietz syndrome to avoid additional aortic events. In Marfan syndrome, this is controversial in patients without dissection as there is a low possibility for expansion.

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