Aortic dissection in a young man with Loeys–Dietz syndrome

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Presentation transcript:

Aortic dissection in a young man with Loeys–Dietz syndrome Vojtech Melenovsky, MD, PhD, Marek Adamira, MD, Dana Kautznerova, MD, Ludek Voska, MD, Jiri Weichet, MD, PhD, Bart Loeys, MD, PhD, Jan Pirk, MD, PhD The Journal of Thoracic and Cardiovascular Surgery Volume 135, Issue 5, Pages 1174-1175.e1 (May 2008) DOI: 10.1016/j.jtcvs.2008.01.003 Copyright © 2008 The American Association for Thoracic Surgery Terms and Conditions

Figure 1 A, Computed tomographic angiogram at the presentation demonstrated extensive dissection of the aortic root. B, Histologic analysis of the aortic tissue (Sirius red stain) showed cystic medionecrosis. The Journal of Thoracic and Cardiovascular Surgery 2008 135, 1174-1175.e1DOI: (10.1016/j.jtcvs.2008.01.003) Copyright © 2008 The American Association for Thoracic Surgery Terms and Conditions

Figure 2 Postoperative magnetic resonance angiogram demonstrated diffuse arterial ellongations and tortuosities (arrows), most apparent in carotid + vertebral (A) and basilar (B) territories. The Journal of Thoracic and Cardiovascular Surgery 2008 135, 1174-1175.e1DOI: (10.1016/j.jtcvs.2008.01.003) Copyright © 2008 The American Association for Thoracic Surgery Terms and Conditions

Family tree presenting affected (dark) and unaffected relatives of the proband (arrow). Asterisks denote genotyped individuals. The Journal of Thoracic and Cardiovascular Surgery 2008 135, 1174-1175.e1DOI: (10.1016/j.jtcvs.2008.01.003) Copyright © 2008 The American Association for Thoracic Surgery Terms and Conditions