Thoracic aortic diseases that involve progressive enlargement, acute dissection, or rupture
are influenced by the hemodynamic loads and mechanical properties of the wall. We have …

Thoracic aortic aneurysms (TAA) increase the risk of aortic dissection or rupture and
represent an important source of morbidity and mortality. Inherited forms of the disease …

Background: Suspected genetic causes for extracellular matrix (ECM) dysregulation in the
ascending aorta in patients with bicuspid aortic valves (BAV) have influenced strategies and …

Marfan syndrome (MFS) is a heritable connective tissue disorder caused by mutations in
FBN1, which encodes the extracellular matrix protein fibrillin-1. To investigate the …

Background—Acute aortic dissection (AAD) is a life-threatening vascular disease without
effective pharmaceutical therapy. Matrix metalloproteinases (MMPs) are implicated in the …

who has some but not enough features for a clinical diagnosis and no or an uncertain family
history, molecular analysis is of minimal help. The real benefit of DNA analysis arises when …

Thoracic aortic dissection (TAD) is a highly lethal vascular disease. In many patients with
TAD, the aorta progressively dilates and ultimately ruptures. Dissection formation …

Elastic fibres are insoluble components of the extracellular matrix of dynamic connective
tissues such as skin, arteries, lungs and ligaments. They are laid down during development …

The stiffness and structural integrity of the arterial wall depends primarily on the organization
of the extracellular matrix and the cells that fashion and maintain this matrix. Fundamental to …

β-Blockers, eg, atenolol, are the cornerstone therapy for thoracic aortic aneurysm (TAA) in
patients with Marfan syndrome; however, continued aortic dilatation has been reported. We …