A contemporary approach to a young female patient with Loeys-Dietz syndrome and an uncomplicated

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064173%3A_____%2F20%3AN0000081" target="_blank" >RIV/00064173:_____/20:N0000081 - isvavai.cz</a>

Result on the web

<a href="https://doi.org/10.1186/s13019-020-01274-0" target="_blank" >https://doi.org/10.1186/s13019-020-01274-0</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1186/s13019-020-01274-0" target="_blank" >10.1186/s13019-020-01274-0</a>

Result language

angličtina

Original language name

A contemporary approach to a young female patient with Loeys-Dietz syndrome and an uncomplicated

Original language description

BACKGROUND: Aortic dissection is a relatively uncommon, but often catastrophic disease that requires early and accurate diagnosis. It often presents in patients with congenital connective tissue disorders. The current aortic surgical techniques are related with serious early and late complications. This case report emphasizes the importance of early diagnosis of aortic root dilatation and the risk of dissection, especially in patients with congenital connective tissue disorders. We present an alternative, contemporary and multidisciplinary approach based on the present state of knowledge. CASE PRESENTATION: We present a rare case of a young female patient with Loeys-Dietz syndrome who was admitted with an uncomplicated aortic dissection (Stanford type B / DeBakey type III) and a dilated aortic root. After a period of close surveillance and extensive vascular imaging, thoracic endovascular aortic repair was deemed to be technically not possible. Medical treatment was optimized and our patient successfully underwent a personalised external aortic root support procedure (PEARS) as a contemporary alternative to existing aortic root surgical techniques. CONCLUSIONS: This case highlights the importance of interdisciplinary approach, close follow-up and multimodality imaging. The decision to intervene in a chronic type B aortic dissection is still challenging and should be made in experienced centers by an interdisciplinary team. However, if an acute complication occurs, thoracic endovascular aortic repair TEVAR is the method of choice. In all cases optimal medical treatment is important. There is increasing evidence that personalized external aortic root support procedure PEARS is effective in stabilizing the aortic root and preventing its dilatation and dissection not only in patients with Marfan syndrome, but also in other cases of aortic root dilation of other etiologies. Moreover, many publications have reported the additional benefit of reduction or even eradication of aortic regurgitation by improving coaptation of the aortic valve leaflets in dilated aortas.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30201 - Cardiac and Cardiovascular systems

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2020

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Journal of Cardiothoracic Surgery

ISSN

1749-8090

e-ISSN

—

Volume of the periodical

15

Issue of the periodical within the volume

August

Country of publishing house

GB - UNITED KINGDOM

Number of pages

6

Pages from-to

231

UT code for WoS article

000567929700001

EID of the result in the Scopus database

2-s2.0-85090106290