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Surgical experience with aggressive aortic pathologic process in Loeys-Dietz syndrome
Yutaka Iba1, Kenji Minatoya, Hitoshi Matsuda
1Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Osaka, Japan. iba@hsp.ncvc.go.jp
The Annals of Thoracic Surgery
|August 28, 2012
Summary
Surgical outcomes for Loeys-Dietz syndrome (LDS) patients were satisfactory, but aortic dissection requires further repairs. Early intervention may prevent fatal aortic events in LDS patients with TGFBR mutations.
Area of Science:
- Cardiovascular Surgery
- Genetics
- Connective Tissue Disorders
Background:
- Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder caused by mutations in TGFBR1 and TGFBR2.
- Surgical outcomes for aortic repair in LDS patients are not well-established.
Purpose of the Study:
- To evaluate the surgical outcomes of aortic repair in patients with Loeys-Dietz syndrome.
- To compare outcomes between patients with and without aortic dissection as the initial presentation.
Main Methods:
- Retrospective review of 16 LDS patients with TGFBR mutations who underwent 41 aortic procedures between 1993 and 2011.
- Patients were divided into a dissection group (n=10) and a non-dissection group (n=6).
- Follow-up averaged 103.7 months.
Main Results:
- No in-hospital deaths occurred. All non-dissection patients received valve-sparing root replacement (VSRR).
- 90% of the dissection group required re-operations, with 40% undergoing complete aortic replacement.
- Five-year aortic event-free rates were 40% for the dissection group and 80% for the non-dissection group.
Conclusions:
- Surgical outcomes for LDS patients are generally satisfactory.
- Aortic dissection in LDS patients leads to rapid expansion and necessitates further interventions.
- Early surgical intervention is recommended to prevent fatal aortic events and improve prognosis.
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