Aortic and pulmonary root aneurysms in a child with loeys-dietz syndrome

Stefania Rizzo; Giovanni Stellin; Ornella Milanesi; Massimo Padalino; Luca A. Vricella; Gaetano Thiene; Duke E. Cameron; Cristina Basso; Vladimiro L. Vida

doi:10.1016/j.athoracsur.2015.05.045

Aortic and pulmonary root aneurysms in a child with loeys-dietz syndrome

Stefania Rizzo, Giovanni Stellin, Ornella Milanesi, Massimo Padalino, Luca A. Vricella, Gaetano Thiene, Duke E. Cameron, Cristina Basso, Vladimiro L. Vida

School of Medicine

Research output: Contribution to journal › Article › peer-review

3 Scopus citations

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.

Original language	English (US)
Pages (from-to)	1193-1195
Number of pages	3
Journal	Annals of Thoracic Surgery
Volume	101
Issue number	3
DOIs	https://doi.org/10.1016/j.athoracsur.2015.05.045
State	Published - Mar 1 2016

ASJC Scopus subject areas

Surgery
Pulmonary and Respiratory Medicine
Cardiology and Cardiovascular Medicine

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title = "Aortic and pulmonary root aneurysms in a child with loeys-dietz syndrome",

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.",

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T1 - Aortic and pulmonary root aneurysms in a child with loeys-dietz syndrome

AU - Rizzo, Stefania

AU - Stellin, Giovanni

AU - Milanesi, Ornella

AU - Padalino, Massimo

AU - Vricella, Luca A.

AU - Thiene, Gaetano

AU - Cameron, Duke E.

AU - Basso, Cristina

AU - Vida, Vladimiro L.

PY - 2016/3/1

Y1 - 2016/3/1

N2 - 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.

AB - 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.

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Aortic and pulmonary root aneurysms in a child with loeys-dietz syndrome

Abstract

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