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KMID : 0364020150480040272
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Korean Journal of Thoracic and Cardiovascular Surgery
2015 Volume.48 No. 4 p.272 ~ p.276
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Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome
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Sim Hyung-Tae
Seo Dong-Ju
Yu Jeong-Jin
Baek Jae-Suk
Goo Hyn-Woo
Park Jeong-Jun
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Abstract
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Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.
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KEYWORD
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Loeys-Dietz syndrome, Aortic root, Aortic valve, surgery, Aneurysm, Aorta
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