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Comprehensive Imaging and Biomarker Assessment of Aortic Valve Stenosis
The purpose of this study is detection of relationship between biomarkers and the severity of AS (echocardiographic hemodynamics/CT-calcium load). Detection of relationship between biomarkers to the consequences of AS on the LV (echo remodeling parameters and speckle-tracking LV strain). Detection of differences on echocardiographic and CT-calcium load AS severity assessments between tricuspid and bicuspid valves. Detection of biomarker associations with AS hemodynamics, CT-calcium load, LV remodeling and LV strain between tricuspid and bicuspid phenotypes.
International Bicuspid Aortic Valve Consortium (BAVCon) (BAVCon)
Bicuspid aortic valve (BAV) disease is the most frequent congenital cardiac malformation, occurring in 0.5-1.2% of the US population. In young adults, it is generally a benign abnormality; but in older adults it is associated with thoracic aortic aneurysm or dissection in 20-30% of those with BAV. BAV is strongly associated with early development of aortic valve calcification or incompetence in >50% of BAV patients, and accounts for ~40% of the >30,000 aortic valve replacements (AVR) performed in the US each year. Yet, we know little of the etiology, cellular events and modifiers of progression of BAV to calcific aortic valve disease and we still do not understand the genetic cause(s) of BAV despite evidence for its high heritability.
The Specific Aims of this study are:
1. To identify the genetic causes of bicuspid aortic valve disease and its associated thoracic aortic disease.
2. To identify potential pathways to predict the clinical course of BAV disease and for treating human BAV disease.
To achieve these aims, we have created the International Bicuspid Aortic Valve Consortium (BAVCon), a consortium of institutions with cohorts of BAV patients and the expertise to fulfill the performance of these aims.