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J Thorac Cardiovasc Surg 2009;138:502-503
© 2009 The American Association for Thoracic Surgery

Brief Technique Report

Aortic decision-making in the Loeys–Dietz syndrome: Aortic root aneurysm and a normal-caliber ascending aorta and aortic arch

^a Cardiothoracic Section, Anesthesiology and Critical Care, University of Pennsylvania School of Medicine, Philadelphia, Pa
^b Quantitative Echocardiographic Laboratory, Division of Cardiology, Department of Medicine, University of Pennsylvania Medical Center, Philadelphia, Pa
^c Division of Cardiothoracic Surgery, Department of Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pa

Received for publication May 14, 2008; revisions received June 4, 2008; accepted for publication June 15, 2008.

^_* Address for reprints: John G. T. Augoustides, MD, FASE, Cardiothoracic Section, Anesthesiology and Critical Care, Dulles 680, HUP, 3400 Spruce St, Philadelphia, PA 19104-4283. (Email: yiandoc@hotmail.com).

The first 20% of the full text of this article appears below.

	Introduction

 
Loeys–Dietz syndrome (LDS) is a recently described autosomal dominant connective tissue disorder characterized by premature aggressive aortic aneurysm and dissection.¹ Thoracic aortic dissection is the leading cause of death in this lethal vascular syndrome.² We present a case of an aortic root aneurysm in a young man with LDS who underwent elective reconstruction of his aortic root, ascending aorta, and aortic arch. This aggressive surgical approach was both curative (aortic root aneurysm) and preventive (future high risk of type A dissection). This aggressive surgical management is presented to describe a preemptive approach to defend patients with LDS from devastating type A dissection.³

	Clinical Summary

 
A 15-year-old boy with LDS was referred for aortic reconstruction caused by an aortic root aneurysm. Serial assessment of the aneurysm diameter by means of computed axial tomographic analysis over the previous 2 years had documented a growth rate of more . . . [Full Text of this Article]

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Aortic decision making in Loeys–Dietz syndrome

Christophe Jayle, Jamil Hajj-Chahine, and Pierre Corbi
J. Thorac. Cardiovasc. Surg. 2010 139: 1088-1089. [Extract] [Full Text] [PDF]

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				J. J. B. Edelman, F. Ramponi, P. G. Bannon, and R. Jeremy Familial aortic aneurysm and dissection due to transforming growth factor-{beta} receptor 2 mutation Interact CardioVasc Thorac Surg, May 1, 2011; 12(5): 863 - 865. [Abstract] [Full Text] [PDF]

				C. Jayle, J. Hajj-Chahine, and P. Corbi Aortic decision making in Loeys-Dietz syndrome. J. Thorac. Cardiovasc. Surg., April 1, 2010; 139(4): 1088 - 1089. [Full Text] [PDF]

				J. G.T. Augoustides and J. E. Bavaria Reply to the editor. J. Thorac. Cardiovasc. Surg., April 1, 2010; 139(4): 1089 - 1089. [Full Text] [PDF]