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Total Aortic Replacement for a 9-Year-Old Boy With Loeys-Dietz Syndrome.
- Source :
-
The Annals of thoracic surgery [Ann Thorac Surg] 2016 Mar; Vol. 101 (3), pp. 1185-8. - Publication Year :
- 2016
-
Abstract
- Loeys-Dietz syndrome (LDS) is a recently identified rare connective tissue disorder caused by mutations of the transforming growth factor-β receptors and first described in 2005. It is an autosomal dominant syndrome with 2 different phenotypic expressions-LDS I and II. LDS is characterized by the triad of arterial tortuosity and aneurysm, hypertelorism, and a bifid uvula or cleft palate. We present a case of a 9-year-old boy diagnosed with LDS who underwent urgent thoracoabdominal aortic aneurysm repair followed by total arch replacement and aortic valve-sparing root replacement (AVSRR).<br /> (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)
- Subjects :
- Aortic Dissection diagnostic imaging
Aortic Aneurysm, Thoracic etiology
Aortography methods
Blood Vessel Prosthesis
Cardiopulmonary Bypass methods
Child
Follow-Up Studies
Humans
Loeys-Dietz Syndrome complications
Loeys-Dietz Syndrome diagnosis
Loeys-Dietz Syndrome etiology
Male
Rare Diseases
Risk Assessment
Thoracotomy methods
Tomography, X-Ray Computed methods
Treatment Outcome
Aortic Dissection surgery
Aortic Aneurysm, Thoracic diagnostic imaging
Aortic Aneurysm, Thoracic surgery
Blood Vessel Prosthesis Implantation methods
Imaging, Three-Dimensional
Loeys-Dietz Syndrome diagnostic imaging
Loeys-Dietz Syndrome surgery
Subjects
Details
- Language :
- English
- ISSN :
- 1552-6259
- Volume :
- 101
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- The Annals of thoracic surgery
- Publication Type :
- Academic Journal
- Accession number :
- 26897206
- Full Text :
- https://doi.org/10.1016/j.athoracsur.2015.05.071