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Adult surgical experience with Loeys-Dietz syndrome.

Publication ,  Journal Article
Williams, JA; Hanna, JM; Shah, AA; Andersen, ND; McDonald, MT; Jiang, Y-H; Wechsler, SB; Zomorodi, A; McCann, RL; Hughes, GC
Published in: Ann Thorac Surg
April 2015

BACKGROUND: Loeys-Dietz syndrome (LDS) results from mutations in receptors for the cytokine transforming growth factor-β leading to aggressive aortic pathology sometimes accompanied by specific phenotypic features including bifid uvula, hypertelorism, cleft palate, and generalized arterial tortuosity. We reviewed our adult surgical experience with LDS in order to validate current recommendations regarding management of this newly described disease. METHODS: All adult (≥ 18 years old) patients with LDS undergoing surgical treatment at a single referral institution from September 1999 to May 2013 were retrospectively reviewed. RESULTS: Eleven adult LDS patients were identified by clinical criteria and genotyping. Seven (64%) experienced acute type A dissection at some point in their lives. All eventually required aortic root replacement, and 73% required multiple vascular surgical interventions. Over a mean follow-up of 65 ± 49 months, 2.8 cardiovascular procedures per patient were performed. In patients with type A dissection, a mean of 3.4 operations were performed versus 1.8 operations for patients without dissection. Total aortic replacement was required in 5 patients (45%) and 2 (18%) required neurosurgical intervention for cerebrovascular pathology. There was 1 late death from infectious complications, and no deaths from vascular catastrophe. CONCLUSIONS: These results confirm the aggressive nature of LDS aortic pathology. However, the improved survival compared with earlier LDS reports suggest that aggressive treatment strategies may alter outcomes and improve the natural history of this syndrome.

Duke Scholars

Published In

Ann Thorac Surg

DOI

EISSN

1552-6259

Publication Date

April 2015

Volume

99

Issue

4

Start / End Page

1275 / 1281

Location

Netherlands

Related Subject Headings

  • Young Adult
  • Vascular Surgical Procedures
  • Treatment Outcome
  • Survival Rate
  • Severity of Illness Index
  • Risk Assessment
  • Retrospective Studies
  • Respiratory System
  • Reoperation
  • Prognosis
 

Citation

APA
Chicago
ICMJE
MLA
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Williams, J. A., Hanna, J. M., Shah, A. A., Andersen, N. D., McDonald, M. T., Jiang, Y.-H., … Hughes, G. C. (2015). Adult surgical experience with Loeys-Dietz syndrome. Ann Thorac Surg, 99(4), 1275–1281. https://doi.org/10.1016/j.athoracsur.2014.11.021
Williams, Jason A., Jennifer M. Hanna, Asad A. Shah, Nicholas D. Andersen, Marie T. McDonald, Yong-Hui Jiang, Stephanie Burns Wechsler, Ali Zomorodi, Richard L. McCann, and G Chad Hughes. “Adult surgical experience with Loeys-Dietz syndrome.Ann Thorac Surg 99, no. 4 (April 2015): 1275–81. https://doi.org/10.1016/j.athoracsur.2014.11.021.
Williams JA, Hanna JM, Shah AA, Andersen ND, McDonald MT, Jiang Y-H, et al. Adult surgical experience with Loeys-Dietz syndrome. Ann Thorac Surg. 2015 Apr;99(4):1275–81.
Williams, Jason A., et al. “Adult surgical experience with Loeys-Dietz syndrome.Ann Thorac Surg, vol. 99, no. 4, Apr. 2015, pp. 1275–81. Pubmed, doi:10.1016/j.athoracsur.2014.11.021.
Williams JA, Hanna JM, Shah AA, Andersen ND, McDonald MT, Jiang Y-H, Wechsler SB, Zomorodi A, McCann RL, Hughes GC. Adult surgical experience with Loeys-Dietz syndrome. Ann Thorac Surg. 2015 Apr;99(4):1275–1281.
Journal cover image

Published In

Ann Thorac Surg

DOI

EISSN

1552-6259

Publication Date

April 2015

Volume

99

Issue

4

Start / End Page

1275 / 1281

Location

Netherlands

Related Subject Headings

  • Young Adult
  • Vascular Surgical Procedures
  • Treatment Outcome
  • Survival Rate
  • Severity of Illness Index
  • Risk Assessment
  • Retrospective Studies
  • Respiratory System
  • Reoperation
  • Prognosis