<abstract xmlns="http://www.w3.org/1999/xhtml"><p> The major structural proteins of the vascular wall are collagen and elastin. Genetic connective tissue diseases lead to degeneration, aneurysms and spontaneous dissection or rupture of arteries. The best-known are Marfan syndrome, vascular Ehlers-Danlos syndrome (type IV), Loeys-Dietz syndrome and familial aortic aneurysms and dissections. Objective. This review addresses the current status of endovascular treatment options for major connective tissue diseases. The treatment of choice for patients who are mostly affected at a young age is primarily conservative or open repair. There is only limited evidence in favour of endovascular aortic repair (EVAR) of abdominal aneurysms or thoracic endovascular aortic repair (TEVAR) because disease progression and dilation leads to secondary endoleaks and high reintervention rates with uncertain long-term results. There is therefore consensus that EVAR and TEVAR should be limited to exceptional cases and emergency situations in patients with genetic aortic diseases.</p></abstract>

The major structural proteins of the vascular wall are collagen and elastin. Genetic connective tissue diseases lead to degeneration, aneurysms and spontaneous dissection or rupture of arteries. The best-known are Marfan syndrome, vascular Ehlers-Danlos syndrome (type IV), Loeys-Dietz syndrome and familial aortic aneurysms and dissections. Objective. This review addresses the current status of endovascular treatment options for major connective tissue diseases. The treatment of choice for patients who are mostly affected at a young age is primarily conservative or open repair. There is only limited evidence in favour of endovascular aortic repair (EVAR) of abdominal aneurysms or thoracic endovascular aortic repair (TEVAR) because disease progression and dilation leads to secondary endoleaks and high reintervention rates with uncertain long-term results. There is therefore consensus that EVAR and TEVAR should be limited to exceptional cases and emergency situations in patients with genetic aortic diseases.

Familial aneurysms of great vessels in young people

The EuroBiotech Journal

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

{"article-title":"Familial aneurysms of great vessels in young people"}

The major structural proteins of the vascular wall are collagen and elastin. Genetic connective tissue diseases lead to degeneration, aneurysms and spontaneous...

Familial aneurysms of great vessels in young people

Published Online: Dec 28, 2017

Page range: 119 - 121

DOI: https://doi.org/10.24190/ISSN2564-615X/2017/S2.02

Keywords
aneurysm, young people, dissection, thoracic, abdominal

© 2018

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Familial aneurysms of great vessels in young people

Published Online: Dec 28, 2017

Page range: 119 - 121

DOI: https://doi.org/10.24190/ISSN2564-615X/2017/S2.02

Keywordsaneurysm, young people, dissection, thoracic, abdominal

© 2018

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Keywords
aneurysm, young people, dissection, thoracic, abdominal