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Interventional repair of a vascular aneurysm in a patient with Marfan syndrome
Marfan syndrome is a heritable connective tissue disorder affecting skeletal, ocular and cardiovascular systems. Cardiovascular manifestations comprise aneurysmal dilatation of aortic root, aortic dissection and rupture; peripheral arterial aneurysms have been reported in femoral, iliac and subclavi...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056777/ https://www.ncbi.nlm.nih.gov/pubmed/30046448 http://dx.doi.org/10.1177/2050313X18788448 |
Sumario: | Marfan syndrome is a heritable connective tissue disorder affecting skeletal, ocular and cardiovascular systems. Cardiovascular manifestations comprise aneurysmal dilatation of aortic root, aortic dissection and rupture; peripheral arterial aneurysms have been reported in femoral, iliac and subclavian arteries with surgical reconstruction as the first-line therapeutic option. We report a Marfan patient with a symptomatic aneurysm of left subclavian artery in the intrathoracic retro-clavicular space; instead of open surgical resection, an endovascular solution was successfully applied by use of a flexible self-expanding stent-graft (W.L Gore(®) Viabahn(®) Endoprosthesis 9 × 100 mm) to exclude the aneurysm. This case exemplifies a modern option to manage vascular pathology even in patients with Marfan syndrome. Follow-up over 1 year proved very reassuring with complete remodelling and resolution of the aneurysm; long-term follow-up is certainly warranted, considering the potential of recurrence or initial reactive hyperplasia. |
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