Predictors of ascending aortic dilatation with bicuspid aortic valve: a wide spectrum of disease expression☆

Abstract

BAV patients constitute an importantly heterogeneous population in terms of risk and features of aortic disease. The most common condition is an ectasia of the mid-ascending tract, with unaffected or mildly involved root. If further confirmed, this could suggest that surgical approach may spare the root in most BAV patients. Mid-ascending dilatation is proportional to stenosis severity, suggesting a post-stenotic causative mechanism. Root dilatation is rarer, mostly observed in younger men, and unrelated to the presence and severity of stenosis. The two different aortic dilatation phenotypes (mid-ascending and root) may be subtended by different pathogeneses.

Clinical and Genetic Aspects of Bicuspid Aortic Valve: A Proposed Model for Family Screening Based on a Review of Literature

“…Dilatation of the aortic root is described to be relatively rare in BAV and is proposed to be the form of bicuspid aortopathy most likely to be associated with a genetic cause. 3,13,20,24,[31][32][33] The other studies reported percentages of TAA in the first-degree TAV relatives of 3-4%. 54,[65][66][67][68] This is around population risk since 2.3% of the general population, by definition, is expected to have z scores >2.…”

“…This root phenotype has been proposed to be the form of bicuspid aortopathy most likely to be associated with a genetic cause. 3,13,20,24,[31][32][33] Although rare, the most feared complication in BAV patients is thoracic aortic dissection, which has been reported at young age. 34 Whereas the lifetime risk of aortic dissection in BAV patients was initially reported around 5%, recent studies show a lifetime risk of aortic dissection of less than 1% in BAV patients and a normal life expectancy.…”

“…16 Dilatation of the thoracic aorta, most commonly located in the ascending aorta, has been reported in 35-80% of adult BAV patients and has rarely been observed as early as childhood. [11][12][13][14][20][21][22][23][24][25] In adults TAA is defined as an aortic diameter with a z-score of ≥2, corresponding to an observed value >1.96 standard deviations above the predicted value for age, gender, and body surface area (BSA). 26 An aortic root >4.0 cm in adults is considered to be dilated irrespective of age, gender or BSA.…”

Novel Phenotypes in Bicuspid Aortic Valve Disease

“…1). The association between BAV stenosis and asymmetric mid-ascending aortic dilatation has been proposed to be pathogenetic (Della Corte et al, 2007). It has been proved that stenosis of the BAV, which is geometrically asymmetrical, produces an eccentric turbulent transvalvular blood flow that results in asymmetrical wall stress distribution in the ascending aorta ( Fig.…”

Bicuspid Aortic Valve Disease: New Insights

“…Several factors are associated with progressive aortic dilatation and include age, hypertension, male gender, aortic valve disease, and valve morphologies. 8,16,17 Evaluation of the aortic dimensions in BAV patients is mandatory. In a prospective community study from Olmsted County, Minnesota, USA, aortic aneurysm (an ascending aorta ≥45 mm) was identified in 7.7% of BAV patients at baseline with a mean age of 55 years.…”