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Aortopathies: From Etiology to the Role of Arterial Stiffness

Journal

JOURNAL OF CLINICAL MEDICINE
Volume 12, Issue 12, Pages -

Publisher

MDPI
DOI: 10.3390/jcm12123949

Keywords

aorta; aortopathies; genetics; heritable connective tissue disorders; arterial stiffness; pulse wave velocity

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The aorta and aortic wall are complex biological systems that are closely associated with arterial stiffness, which has been linked to aortopathies and cardiovascular risk. Pulse-wave velocity (PWV) is considered the gold standard for assessing arterial stiffness and can be used to identify high-risk patients and evaluate therapeutic strategies.
The aorta and aortic wall have a complex biological system of structural, biochemical, biomolecular, and hemodynamic elements. Arterial stiffness could be considered a manifestation of wall structural and functional variations, and it has been revealed to have a strong connection with aortopathies and be a predictor of cardiovascular risk, especially in patients affected by hypertension, diabetes mellitus, and nephropathy. Stiffness affects the function of different organs, especially the brain, kidneys, and heart, promoting remodeling of small arteries and endothelial dysfunction. This parameter could be easily evaluated using different methods, but pulse-wave velocity (PWV), the speed of transmission of arterial pressure waves, is considered the gold standard for a good and precise assessment. An increased PWV value indicates an elevated level of aortic stiffness because of the decline in elastin synthesis and activation of proteolysis and the increase in fibrosis that contributes to parietal rigidity. Higher values of PWV could also be found in some genetic diseases, such as Marfan syndrome (MFS) or Loeys-Dietz syndrome (LDS). Aortic stiffness has emerged as a major new cardiovascular disease (CVD) risk factor, and its evaluation using PWV could be very useful to identify patients with a high cardiovascular risk, giving some important prognostic information but also being used to value the benefits of therapeutic strategies.

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