Like Father like Daughter: Surgical Redo Thoracoabdominal Aneurysm Repairs in a Family With Loeys-Dietz Syndrome.
Autor: | Chen JR; Division of Cardiac Surgery, Thomas Jefferson University, Philadelphia, PA, USA., Shah VN; Division of Cardiac Surgery, Thomas Jefferson University, Philadelphia, PA, USA., Pritting C; Division of Cardiac Surgery, Thomas Jefferson University, Philadelphia, PA, USA., Nooromid M; Division of Vascular and Endovascular Surgery, Thomas Jefferson University, Philadelphia, PA, USA., Abai B; Division of Vascular and Endovascular Surgery, Thomas Jefferson University, Philadelphia, PA, USA., Plestis K; Division of Cardiac Surgery, Thomas Jefferson University, Philadelphia, PA, USA. |
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Jazyk: | angličtina |
Zdroj: | Vascular and endovascular surgery [Vasc Endovascular Surg] 2025 Jan; Vol. 59 (1), pp. 93-96. Date of Electronic Publication: 2024 Aug 29. |
DOI: | 10.1177/15385744241279139 |
Abstrakt: | Loeys Dietz Syndrome (LDS) is an autosomal dominant connective tissue disorder resulting from a mutation in the transforming growth factor beta receptor (TGFBR) family of genes. It is commonly associated with the development of aortic aneurysms and dissections. We report the successful open surgical management of thoracoabdominal aneurysms in a father and daughter with Loeys-Dietz Syndrome after failed endovascular repair. The daughter required stent graft explantation, while the stent graft remained in the father. These cases highlight the importance of early genetic testing of both patients and first-degree family members in those with a strong history of aortic disease, even when there is a lack of typical connective tissue disorder associated physical exam findings and open surgical index operations. Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Babak Abai: consultant for Endologix and Cook Medical. All other authors have no conflicts of interest to declare. |
Databáze: | MEDLINE |
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