Genetic Considerations

Loci for isolated thoracic aortic aneurysm have been mapped to chromosomes 11,5, and 3. Thoracic aortic aneurysms and dissections are mainly associated with medial necrosis in which there is degeneration of elastic fibers, a loss of smooth muscle cells, and an accumulation of basophilic ground substance. Medial necrosis and thoracic aortic aneurysm/dissection are known to occur in certain heritable connective tissue diseases such as Marfan syndrome and vascular (type IV) Ehlers-Danlos syndrome. Medial necrosis has also been seen to cluster in families in the absence of a clearly identifiable syndrome.

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