Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Ghonem, Mohammed E, Yuan, Xun, Mitsis , Andreas, Nienaber, Christoph A
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2018
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
_version_ 1783341404464873472
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
work_keys_str_mv AT ghonemmohammede interventionalrepairofavascularaneurysminapatientwithmarfansyndrome
AT yuanxun interventionalrepairofavascularaneurysminapatientwithmarfansyndrome
AT mitsisandreas interventionalrepairofavascularaneurysminapatientwithmarfansyndrome
AT nienaberchristopha interventionalrepairofavascularaneurysminapatientwithmarfansyndrome