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Loeys-Dietz syndrome as a cause of aortic dissection type A.

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Author(s): Jany Rodríguez Londres | Roger Ravelo Dopico | Gretell Medina Guirola | Lisbeth González González | Ailema Alemán Fernández

Journal: Revista Cubana de Cardiología y Cirugía Cardiovascular
ISSN 0864-2168

Volume: 17;
Issue: 4;
Start page: 361;
Date: 2011;
Original page

Keywords: Loeys-Dietz syndrome | aneurysm | aortic dissection

ABSTRACT
Loeys-Dietz syndrome is considered a genetic disorder with autosomic dominant transmission and defined by a characteristic triad: generalized arterialtortuosity and aneurysms, hypertelorism and cleft palate or split uvula. The arterial affection, especially aortic dissection and dilatation, is the main cause ofdeath in those patients. It is presented a clinic case with mentioned characteristics, being carried out the diagnosis of this entity so not very frequent andinteresting, like cause of dissection aortic type A.
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