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

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 m...

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Bibliographic Details
Main Authors: Jany Rodríguez Londres, Roger Ravelo Dopico, Gretell Medina Guirola, Lisbeth González González, Ailema Alemán Fernández
Format: Article
Language:Spanish
Published: ECIMED 2011-01-01
Series:Revista Cubana de Cardiología y Cirugía Cardiovascular
Subjects:
Online Access:http://www.revcardiologia.sld.cu/index.php/revcardiologia/article/view/113/153
Description
Summary: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.
ISSN:0864-2168
1561-2937