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Aortic and Pulmonary Root Aneurysms in a Child With Loeys-Dietz Syndrome.
- Source :
-
The Annals of thoracic surgery [Ann Thorac Surg] 2016 Mar; Vol. 101 (3), pp. 1193-5. - Publication Year :
- 2016
-
Abstract
- We report the case of an 11-year-old boy with Loeys-Dietz syndrome, with both aortic and pulmonary aneurysms requiring cardiac operation because of progressive valve incompetence resulting from loss of coaptation of the cusps. Arterial medial changes, consisting of disarray of elastic fibers and increased collagen deposition, were observed in surgical specimens from both the aorta and the pulmonary artery of our patient, and the strong pSmad2 nuclear staining of smooth muscle cells of both aortic and pulmonary tunica media are the best evidence of transforming growth factor-β pathway activation in Loeys-Dietz syndrome.<br /> (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)
- Subjects :
- Aortic Dissection diagnostic imaging
Aortic Aneurysm, Thoracic diagnostic imaging
Aortic Aneurysm, Thoracic etiology
Aortography methods
Biopsy, Needle
Child
Follow-Up Studies
Humans
Immunohistochemistry
Loeys-Dietz Syndrome diagnosis
Male
Pulmonary Artery diagnostic imaging
Pulmonary Artery pathology
Tomography, X-Ray Computed methods
Treatment Outcome
Tunica Media pathology
Vascular Surgical Procedures methods
Aortic Dissection surgery
Aortic Aneurysm, Thoracic surgery
Cardiac Surgical Procedures methods
Imaging, Three-Dimensional
Loeys-Dietz Syndrome complications
Pulmonary Artery 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 :
- 26897209
- Full Text :
- https://doi.org/10.1016/j.athoracsur.2015.05.045