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

• Published in: Annals of Vascular Diseases Vol. 15; no. 1; pp. 58 - 61
• Main Authors: Mori, Kousuke, Ishii, Hirohito, Sakaguchi, Shuhei, Sakurahara, Daichi, Iwasaki, Ayaka, Furukawa, Koji
• Format: Journal Article
• Language: English
• Published: Japan The Editorial Committee of Annals of Vascular Diseases 25.03.2022; Japanese College of Angiology / The Japanese Society for Vascular Surgery / Japanese Society of Phlebology
• Subjects: Case Report; total arch replacement; type A acute aortic dissection; vascular Ehlers–Danlos syndrome; total arch replacement; vascular Ehlers–Danlos syndrome; type A acute aortic dissection
• ISSN: 1881-641X; 1881-6428
• DOI: 10.3400/avd.cr.21-00122

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.