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CC BY 4.0 · Aorta (Stamford)
DOI: 10.1055/s-0044-1795131
Case Report

Rapid Aneurysmal Degeneration and Repair of Thoracic Aortic Aneurysm in a Patient with Concomitant Vascular Ehlers–Danlos and Loeys–Dietz Syndromes

Arjune S. Dhanekula
1   Division of Cardiothoracic Surgery, Department of Surgery, University of Washington Medical Center, Seattle, Washington
,
Sara Zettervall
1   Division of Cardiothoracic Surgery, Department of Surgery, University of Washington Medical Center, Seattle, Washington
,
Ioannis Dimarakis 
1   Division of Cardiothoracic Surgery, Department of Surgery, University of Washington Medical Center, Seattle, Washington
,
Scott Deroo
1   Division of Cardiothoracic Surgery, Department of Surgery, University of Washington Medical Center, Seattle, Washington
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Abstract

Vascular Ehlers–Danlos (vEDS) and Loeys–Dietz syndrome 3 (LDS3) are connective tissue disorders with diverse systemic manifestations. Most notable in these disorders, though, are their aggressive aortopathies, often presenting early in life with aneurysmal disease or dissection. Herein we present the case of a 33-year-old patient, previously lost to follow-up, who underwent complex reoperative arch replacement after ascending and hemiarch replacement for Type A aortic dissection 6 years prior. Postoperative genetic testing revealed both vEDS and LDS, a unique genotype that has not been described before in the literature.

Keywords

Loeys–Dietz syndrome - Ehlers–Danlos syndrome - cardiac surgery - outcomes


Publication History

Received: 08 August 2023

Accepted: 04 September 2024

Article published online:
26 November 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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