Marfan syndrome is an autosomal dominant heritable connective tissue disorder which involves primarily the skeletal, ocular and cardiovascular system. The incidence of MS is on average 1: 10000 with 25-30% of cases caused by sporadic mutations

Czerny, M

Fleck, T

Grabenwoger, M

Wolner, E

PubMed

Marfan syndrome is an autosomal dominant heritable connective tissue disorder
which involves primarily the skeletal, ocular and cardiovascular system. The
incidence of MS is on average 1: 10000 with 25-30% of cases caused by sporadic
mutations.
The leading cause of premature death in these patients is progressive dilatation and
subsequent dissection of the ascending thoracic aorta resulting in cardiac
tamponade, and left ventricular failure due to aortic regurgitation. Life expectancy is
primarily determined by the severity of cardiovascular involvement, and has improved
substantially over the last 20 years due to the advances in surgical and medical
management.
The optimum management of Marfan patients includes a lifelong surveillance with
particular emphasis placed on aortic behaviour. Preventive replacement of various
portions of the aorta has been a major contribution for improved life expectancy in
these patients. The different surgical and interventional treatment options currently
available will be further outlined in this review.peer-reviewe

Fleck, Tatiana

Wolner, E.

Grabenwoger, M.

Czerny, M.

OAR@UM

English

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Interventional treatment methods in patients with Marfan Syndrome

https://www.um.edu.mt/library/oar/bitstream/handle/123456789/3989/Interventional%20treatment%20methods%20in%20patients%20with%20Marfan%20Syndrome.pdf?sequence=1&isAllowed=y

Interventional treatment methods in patients with Marfan Syndrome

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