Complete Aortic Replacement in a Patient With Loeys-Dietz Syndrome. (November 2022)

• Record Type: Journal Article
• Authors: Darigny, Sandrine; Astarci, Parla; Elens, Maxime
• Is Part Of: Vascular & endovascular surgery. Volume 56:Number 8(2022)
• Page Start: 767
• Page End: 771
• Publication Date: 2022-11
• Subjects: Loeys-Dietz syndrome -- aorta replacement -- endovascular aortic repair -- aneurysm -- aortic dissection; Blood-vessels -- Surgery -- Periodicals; Blood-vessels -- Endoscopic surgery -- Periodicals; Vascular Surgical Procedures -- Periodicals; Angioplasty -- Periodicals; Surgical Procedures, Minimally Invasive -- Periodicals; Vascular Diseases -- surgery -- Periodicals; Vaisseaux sanguins -- Chirurgie -- Périodiques; Vaisseaux sanguins -- Chirurgie endoscopique -- Périodiques; 617.41
• DOI: 10.1177/15385744221109037
• Languages: English
• ISSNs: 1538-5744

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.