51. The differences in surgical long-term outcomes between Marfan syndrome and Loeys-Dietz syndrome.
- Author
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Seike Y, Matsuda H, Inoue Y, Sasaki H, Morisaki H, Morisaki T, and Kobayashi J
- Subjects
- Adolescent, Adult, Aorta surgery, Female, Humans, Male, Receptor, Transforming Growth Factor-beta Type I genetics, Receptor, Transforming Growth Factor-beta Type II genetics, Young Adult, Aortic Dissection, Loeys-Dietz Syndrome epidemiology, Loeys-Dietz Syndrome surgery, Marfan Syndrome complications, Marfan Syndrome genetics, Marfan Syndrome surgery
- Abstract
Objective: The aim of this study was to disclose the differences of surgical outcomes between Marfan syndrome with mutations in fibrillin-1 gene and Loeys-Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2., Methods: We reviewed 368 patients aged less than 50 years who underwent surgery for thoracic aortic diseases between 1988 and 2019, and 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)., Results: Freedom from all causes of mortality was similar between the 2 groups (P = .40, log-rank). The cumulative incidence of reintervention was significantly lower in the Marfan syndrome group than in the Loeys-Dietz syndrome group (P = .016, Gray). The cumulative incidence of first aortic arch reoperation for aortic arch aneurysm was significantly lower in the Marfan syndrome group than in the Loeys-Dietz syndrome group (P < .001, Gray). The cumulative incidence of first aortic root reoperation for aortic root aneurysm (P = .57, Gray) and first descending aorta reoperation for descending aortic aneurysm (P = .76, Gray) was similar between the 2 groups. The cumulative incidence of aortic dissection after initial surgery was significantly lower in Marfan syndrome than in Loeys-Dietz syndrome (P = .0059, Gray)., Conclusions: Loeys-Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2 revealed higher rates of reoperation, and more specifically the arch reoperation was higher in those with Loeys-Dietz syndrome than those with Marfan syndrome. Aggressive arch surgery in the initial operation on the proximal aorta is recommendable in Loeys-Dietz syndrome to avoid additional aortic events. In Marfan syndrome, this is controversial in patients without dissection because of a low possibility to expand., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)
- Published
- 2022
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