Abstract | BACKGROUND: METHODS: We enrolled 16 patients with TGFBR mutations identified by gene analysis in this study. Between 1993 and 2011, they underwent 41 aortic surgical procedures. Ten patients (group D: dissection group) underwent aortic repair for acute or chronic aortic dissection as a first surgical intervention, and 6 patients (group N: nondissection group) underwent surgical treatment for aortic root dilatation. The mean follow-up period was 103.7 ± 92.3 months (range, 2- 276 months). RESULTS: There were no in-hospital deaths. In group N, valve-sparing root replacement (VSRR) was performed in all patients. The residual aorta in 9 patients (90%) from group D required further repairs, 3 times on average. Moreover, in 4 patients (40%), the aorta was entirely replaced in serial procedures. In group N, aortic dissection occurred in only 1 patient (17%). The aortic event-free rates at 5 years were 40% in group D and 80% in group N, respectively (p = 0.819). One late death due to arrhythmia occurred 1 month after VSRR. The cumulative survival rates at 5 years were 100% in group D and 83% in group N, respectively (p = 0.197). CONCLUSIONS: Surgical outcomes for patients with LDS were satisfactory. Once aortic dissection occurred, the aorta expanded rapidly, requiring further operations. Therefore, early surgical intervention may improve prognosis by preventing a fatal aortic event.
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Authors | Yutaka Iba, Kenji Minatoya, Hitoshi Matsuda, Hiroaki Sasaki, Hiroshi Tanaka, Hiroko Morisaki, Takayuki Morisaki, Junjiro Kobayashi, Hitoshi Ogino |
Journal | The Annals of thoracic surgery
(Ann Thorac Surg)
Vol. 94
Issue 5
Pg. 1413-7
(Nov 2012)
ISSN: 1552-6259 [Electronic] Netherlands |
PMID | 22921234
(Publication Type: Journal Article)
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Copyright | Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved. |
Topics |
- Adult
- Aortic Aneurysm
(surgery)
- Female
- Humans
- Loeys-Dietz Syndrome
(pathology, surgery)
- Male
- Retrospective Studies
- Severity of Illness Index
- Young Adult
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