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What is incidence of BAV?

Is there family history

1-2 % of population , autosomial dominant with reduced penetrance


What is the increased risk of acute aortic complications because of BAV

9 fold higher prevalence of aortic dissection

BAV associated with more severe medial remodeling of the aorta, which occureed earlier during the aneurysm course.

Aortic valve insufficiency and dilation of aortic root is a different phenotype and maybe at greater risk for post adverese event issues with the aorta


Describe the Fazel-Stanford classification of aortopathy

Type I: dilation at aortic root
Type II: tubular dilation of ascending aorta
Type III: tubular dilation of the ascending aorta and arch
Type IV: dilation of aortic root, ascending aorta, and arch.


What are long term outcomes of pts with BAV

series of 642 asymptomatic pts with BAV, most (63%) had normal or mildly abnormal valve function at baseline.

On average 9 year follow up--25% required surgery for symptomatic valve disease, left ventricular dysfunction, ascending aortic dilation, or endocarditis.

predictors for cardiovascular events, age > 30, moderate aortic valve dysfunction.


How do pts with BAV develop aortopathy

Histopathologic studies support underlying connective tissue disease process with
a.elastin fragmentation
b. irregularities in smooth muscle integrity
c. increased collagen deposition


What is survival of BAV

Cardiac morbidity with VAB is significant, overall life expectancy is not shortened relative to general population estimates

Olmstead County study, survival was 97% and 90% at 10 and 20 years

Similarly in a Toronto cohort, 10 year survival was 97%


What is most common fusion pattern for Bicuspid

Right and Left coronary cusp (Anterio-Posterior leaflet type)

56% of cases

Fusion of the Right and Non-Coronary cusp is the next most common (44%)


What is different about RL phenotype

More often associated with aortic dilation extending to the arch


What is difference of coronary anatomy in pts withe BAV

most have a dominant circumflex coronary system
Small right Coronary artery


List several connective tissue disorders that can cause AI

Ankolosing spondylititis
Osteogensis imperfecta
rheumatoid artthritis
Reiters syndrome


Name a medication that can cause AI

Anorexigenic drugs---Fenfluramine and phentermine


What is incidence of pts withe BAV requiring aortic valve and/or ascending aorta surgery and the total adverse cardiovascular events

27.4% and 42.5%


What is rate of growth for pts with BAV

BAV pts have higher rates of growth then tricuspid aortas and the rate of dilation of the asecending aorta is also higher (0.19cm vs 0.1cm)
Pt with AS have a higher risk of rupture, dissection, or death


What is the embrology of BAV

Both the aortic valve and the proximal aortic wall arise from neural crest cell. Stops in aortic arch


What is tissue morphology of BAV

Cystic medial necrosis
Altered smooth muscle cell alignment
loss of elastic fibers

risk of aortic dissection is 5 to 9x higher then general population


What is recommendation of screening of patient with BAV

First degree relatives of young patients with BAV should undergo further testing


Describe Seivers classification of BAV

Based on the number of Raphe and the location of fused raphe

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