Complete Aortic Replacement in a Patient With Loeys-Dietz Syndrome.

Abstract

Background: LDS is an autosomal dominant connective tissue disease. It is a rare multi-systemic disorder with serious vascular impact. Case report: We report a case of a 38-year-old male with Loeys-Dietz syndrome (LDS) suffering from major aortic complications. The disease initially manifested itself as a type A aortic dissection, successfully treated by open ascending aorta replacement. Ten days later, the patient developed an uncomplicated type B dissection. During follow up, the patient became symptomatic in both legs (rest pain) due to major true lumen compression. A thoracic endovascular aortic repair was performed with immediate improvement of the symptoms. During follow up, a computer tomography angiogram, showed a persistence false lumen perfusion and an aortic diameter increase. Multiple additional endovascular procedures and a final open thoracoabdominal aortic replacement were needed to exclude completely the false lumen. Conclusion: Open surgical repair is still the gold standard therapy for patients with connective tissue disease. However, with the nowadays progress, hybrid procedures could be a better option.

• Authors: Sandrine Darigny, Parla Astarci, Maxime Elens
• Journal: Vascular and endovascular surgery (Vasc Endovascular Surg) Vol. 56 Issue 8 Pg. 767-771 (Nov 2022) ISSN: 1938-9116 [Electronic] United States
• PMID: 35705511 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Aortic Dissection (diagnostic imaging, etiology, surgery)
• Aorta (surgery)
• Aorta, Thoracic (surgery)
• Aortic Aneurysm, Thoracic (diagnostic imaging, etiology, surgery)
• Humans
• Loeys-Dietz Syndrome (complications, diagnostic imaging, surgery)
• Male
• Treatment Outcome