A case of near total aortic replacement in an adolescent with loeys-dietz syndrome.

Korean Circulation Journal
Yoon Jung SuhSung Sup Park

Abstract

Loeys-Dietz syndrome is a recently described autosomal dominant disorder caused by mutations in the genes for transforming growth factor-beta receptor type 1 or 2 (TGF-ßR 1/2). The syndrome predisposes patients to aortic aneurysm and dissections, along with craniofacial and musculoskeletal abnormalities. Here we report the case of an adolescent who underwent serial near total aortic replacement, from the aortic valve to the descending aorta. Loeys-Dietz syndrome was confirmed in this case by the detection of a mutation in the TGF-ßR 2 gene.

References

Aug 25, 2006·The New England Journal of Medicine·Bart L LoeysHarry C Dietz
Jan 30, 2007·The Annals of Thoracic Surgery·Jason A WilliamsDuke E Cameron
May 6, 2008·The Journal of Thoracic and Cardiovascular Surgery·Vojtech MelenovskyJan Pirk
May 26, 2009·The Journal of Thoracic and Cardiovascular Surgery·Melanie D EverittAnji T Yetman
Mar 23, 2010·The Journal of Thoracic and Cardiovascular Surgery·Christophe JaylePierre Corbi
May 25, 2010·Journal of Cardiac Surgery·Matthew L WilliamsG Chad Hughes
Sep 15, 2010·Current Opinion in Cardiology·Christine Van HemelrijkBart Loeys
Nov 26, 2010·The Annals of Thoracic Surgery·Eugenio NeriCarmelo Ricci
Mar 30, 2011·Journal of Cardiac Surgery·Judson B WilliamsG Chad Hughes

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Citations

Mar 1, 2014·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Gretchen MacCarrickHarry C Dietz
Aug 21, 2015·The Korean Journal of Thoracic and Cardiovascular Surgery·Hyung-Tae SimJeong-Jun Park
Jan 11, 2018·Radiographics : a Review Publication of the Radiological Society of North America, Inc·William W LoughboroughMark C K Hamilton

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Methods Mentioned

BETA
dissection

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