Key result
Two-stage total aortic replacement successfully treated aortic root, arch, and thoracoabdominal pathology in a young man with Loeys-Dietz syndrome.
Demonstrates the feasibility of two-stage total aortic replacement for extensive aortopathy in a patient with Loeys-Dietz syndrome.
Case report adds limited evidence for staged aortic surgery in Loeys-Dietz syndrome; leaves open generalizability and durability.
Loeys-Dietz syndrome (LDS) is a recently described connective tissue disorder characterized by generalized arterial tortuosity and aggressive aortopathy that untreated leads to early death even at aortic dimensions as small as 4 cm. We report the case of a young man with LDS successfully treated for aortic root, arch, and thoracoabdominal pathology.
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Williams et al. (2010) studied this question. Two-stage total aortic replacement successfully treated aortic root, arch, and thoracoabdominal pathology in a young man with Loeys-Dietz syndrome.
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