Complete Aortic Replacement in a Patient With Loeys-Dietz Syndrome
| Autor: | Sandrine Darigny, Parla Astarci, Maxime Elens |
|---|---|
| Přispěvatelé: | UCL - SSS/IREC/CARD - Pôle de recherche cardiovasculaire, UCL - (SLuc) Service de chirurgie cardiovasculaire et thoracique |
| Rok vydání: | 2022 |
| Předmět: | |
| Zdroj: | Vascular and Endovascular Surgery, Vol. 138, no.1, p. 153857442211090 (2022) |
| ISSN: | 1938-9116 1538-5744 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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