Aortic and Pulmonary Root Aneurysms in a Child With Loeys-Dietz Syndrome Stefania Rizzo, MD, PhD, Giovanni Stellin, MD, Ornella Milanesi, MD, Massimo.

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Aortic and Pulmonary Root Aneurysms in a Child With Loeys-Dietz Syndrome Stefania Rizzo, MD, PhD, Giovanni Stellin, MD, Ornella Milanesi, MD, Massimo Padalino, MD, PhD, Luca A. Vricella, MD, Gaetano Thiene, MD, Duke E. Cameron, MD, Cristina Basso, MD, PhD, Vladimiro L. Vida, MD The Annals of Thoracic Surgery Volume 101, Issue 3, Pages 1193-1195 (March 2016) DOI: 10.1016/j.athoracsur.2015.05.045 Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions

Fig 1 Aneurysm of the ascending aorta. (A, B, C) Preoperative computerized tomography. Note the severely dilated aortic root (asterisk). (A) Posterior–anterior view. (B) Three-dimensional reconstruction with evidence of previous reconstruction with Dacron graft (arrow) with lack of coaptation of the aortic valve cusps. (asterisk). (C) Coronal view. (D, E, F, G) Surgical pathologic view of the resected aorta. The tunica media shows severe elastic disarray and fragmentation (D, and close-up, elastic Weigert Van-Gieson stain), and increased collagen deposition in the lamellar units (E, and close-up, Heidenhain trichrome stain). The smooth muscle cells of the lamellar units are reduced in number (F, smooth muscle actin immunostaining) and show increased nuclear pSmad2 (G). (D, E scale bar = 1 mm; F, G scale bar = 100 micron.) The Annals of Thoracic Surgery 2016 101, 1193-1195DOI: (10.1016/j.athoracsur.2015.05.045) Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions

Fig 2 Aneurysm of the pulmonary artery. (A) Severe cardiomegaly and prominence of the pulmonary artery are visible on preoperative chest roentgenogram(posterior–anterior view). (B, C) Preoperative computerized tomography shows enlargement of the main pulmonary artery (MPA) and dilatation of right ventricular (RV) infundibular portion (B) with lack of pulmonary valve cusps coaptation (C, asterisk and arrows). LV = left ventricle. (D, E, F, G) Surgical pathologic view of the resected pulmonary artery. The tunica media shows mild elastic disarray and fragmentation (D, and close-up, elastic Weigert Van-Gieson stain) and increased collagen deposition in the lamellar units (E, and close-up, Heidenhain trichrome stain). As in the aorta, note the reduced number of smooth muscle cells (F, smooth muscle actin immunostaining) and increased nuclear pSmad2 (G). (D, E scale bar = 1 mm; F, G scale bar = 100 micron.) The Annals of Thoracic Surgery 2016 101, 1193-1195DOI: (10.1016/j.athoracsur.2015.05.045) Copyright © 2016 The Society of Thoracic Surgeons Terms and Conditions