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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 |
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author | Ghonem, Mohammed E Yuan, Xun Mitsis , Andreas Nienaber, Christoph A |
author_facet | Ghonem, Mohammed E Yuan, Xun Mitsis , Andreas Nienaber, Christoph A |
author_sort | Ghonem, Mohammed E |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-6056777 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-60567772018-07-25 Interventional repair of a vascular aneurysm in a patient with Marfan syndrome Ghonem, Mohammed E Yuan, Xun Mitsis , Andreas Nienaber, Christoph A SAGE Open Med Case Rep Case Report 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. SAGE Publications 2018-07-23 /pmc/articles/PMC6056777/ /pubmed/30046448 http://dx.doi.org/10.1177/2050313X18788448 Text en © The Author(s) 2018 http://www.creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Case Report Ghonem, Mohammed E Yuan, Xun Mitsis , Andreas Nienaber, Christoph A Interventional repair of a vascular aneurysm in a patient with Marfan syndrome |
title | Interventional repair of a vascular aneurysm in a patient with Marfan
syndrome |
title_full | Interventional repair of a vascular aneurysm in a patient with Marfan
syndrome |
title_fullStr | Interventional repair of a vascular aneurysm in a patient with Marfan
syndrome |
title_full_unstemmed | Interventional repair of a vascular aneurysm in a patient with Marfan
syndrome |
title_short | Interventional repair of a vascular aneurysm in a patient with Marfan
syndrome |
title_sort | interventional repair of a vascular aneurysm in a patient with marfan
syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056777/ https://www.ncbi.nlm.nih.gov/pubmed/30046448 http://dx.doi.org/10.1177/2050313X18788448 |
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