Successful Surgical Repair of Type A Acute Aortic Dissection in a Patient with Vascular Ehlers–Danlos Syndrome

Kousuke Mori; Hirohito Ishii; Shuhei Sakaguchi; Daichi Sakurahara; Ayaka Iwasaki; Koji Furukawa

doi:10.3400/avd.cr.21-00122

Abstract

Surgery for vascular complications of a patient with vascular Ehlers–Danlos syndrome (vEDS) is challenging due to the fragility of the associated tissues. In this study, we present a type A acute aortic dissection case in a patient with vEDS successfully treated via total arch replacement. A 42-year-old woman was transferred to our hospital 10 days after the onset of symptoms and underwent emergency surgery. Intraoperative findings revealed severe inflammatory changes without tissue fragility that is distinctive of vEDS. The postoperative course was uneventful except for left recurrent laryngeal nerve palsy, and 24 months after the operation, the patient has remained free from any arterial event.

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© 2022 The Editorial Committee of Annals of Vascular Diseases. This article is distributed under the terms of the Creative Commons Attribution License, which permits use, distribution, and reproduction in any medium, provided the credit of the original work, a link to the license, and indication of any change are properly given, and the original work is not used for commercial purposes. Remixed or transformed contributions must be distributed under the same license as the original.

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