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In our study population, >50% of the patients admitted for surgery had a bicuspid valve. Aortic aneurysm was more common in BAV than in TAV patients. Aortic stenosis and aortic regurgitation were equally common in TAV and BAV with normal aortic dimensions, while aortic regurgitation was predominant in TAV with dilated aortas and aortic stenosis in BAV with dilated aortas. Dilatation of the aorta was similarly distributed regardless of BAV leaflet morphology. These findings support the idea of an intrinsic mechanisms underlying dilatation of the aorta in BAV patients.

Objective-Thoracic aortic aneurysm is a common complication in patients with bicuspid aortic valve (BAV).Alternatively

Individuals with a bicuspid aortic valve (BAV) are at significantly higher risk of developing aortic complications than individuals with tricuspid aortic valves (TAV) and defective signaling during the embryonic development and/or life time exposure to abnormal hemodynamic have been proposed as underlying factors. However, an explanation for the molecular mechanisms of aortopathy in BAV has not yet been provided. We combined proteomics, RNA analyses, immunohistochemistry, and electron microscopy to identify molecular differences in samples of non-dilated ascending aortas from BAV (N = 62) and TAV (N = 54) patients. Proteomic analysis was also performed for dilated aortas (N = 6 BAV and N = 5 TAV) to gain further insight into the aortopathy of BAV. Our results collectively showed the molecular signature of an endothelial/epithelial-mesenchymal (EndMT/EMT) transition-like process, associated with instability of intimal cell junctions and activation of RHOA pathway in the intima and media layers of ascending aorta in BAV patients. We propose that an improper regulation of EndMT/EMT during the spatiotemporally related embryogenesis of semilunar valves and ascending aorta in BAV individuals may result in aortic immaturity and instability prior to dilation. Exasperation of EndMT/EMT state in post embryonic life and/or exposure to non-physiological hemodynamic could lead to the aneurysm of ascending aorta in BAV individuals.

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Introduction: Aortic valve calcification (AVC) may predict poor outcome. Bicuspid aortic valve (BAV) leads to several haemodynamic changes accelerating the progress of aortic valve (AV) disease. Aims: To compare the diagnostic accuracy of transoesophageal echocardiography (TEE) and transthoracic echocardiography (TTE) in the assessment of aortic valve phenotype and degree of AVC, with intra-operative evaluation as a reference. Methods: We examined 169 patients (median age 65 years, 51 women) without significant coronary artery disease undergoing AV and/or aortic root surgery. TTE was performed within a week prior to surgery and TEE at the time of surgery. Results: Compared with surgical AVC assessment, visual evaluation using a 5-grade scoring system and real-time images showed a higher correlation (TTE r = 0Á83 and TEE r = 0Á82) than visual (TTE r = 0Á64 and TEE 0Á63) or grey scale mean (GSMn) (TTE r = 0Á63 and TEE r = 0Á52) assessment of end-diastolic still frames. AVC assessment using real-time images showed high intraclass correlation coefficients (TTE 0Á94 and TEE 0Á93). With regard to BAV, TEE was superior to TTE with a higher interobserver agreement, sensitivity and specificity (0Á86, 92% and 94% versus 0Á57, 77% and 82%, respectively). Conclusion: Semi-quantitative AVC assessment of real-time cine loops from both TEE and TTE correlated well with intra-operative evaluation of AVC. Applying a predefined scoring system for AVC evaluation assures a high interobserver correlation. TEE was superior to TTE for evaluation of valve phenotype and should be considered when a diagnosis of BAV is clinically important.

IntroductionAortic valve calcification (AVC), even without haemodynamic significance, may be prognostically import as an expression of generalized atherosclerosis, but techniques for echocardiographic assessment are essentially unexplored.MethodsTwo-dimensional (2D) echocardiographic recordings (Philips IE33) of the aortic valve in short-axis and long-axis views were performed in 185 consecutive patients within 1 week before surgery for aortic stenosis (n = 109, AS), aortic regurgitation (n = 61, AR), their combination (n = 8) or dilation of the ascending aorta (n = 7). The grey scale mean (GSMn) of the aortic valve in an end-diastolic short-axis still frame was measured. The same frame was scored visually 1–5 as indicating that the aortic valve was normal, thick, or had mild, moderate or severe calcification. The visual echodensity of each leaflet was determined real time applying the same 5-grade scoring system for each leaflet, and the average for the whole valve was calculated. Finally, a similar calcification score for the whole valve based on inspection and palpation by the surgeon was noted.ResultsVisual assessment of real-time images using the proposed scoring system showed better correlation with the surgical evaluation of the degree of valve calcification (r = 0·83, P<0·001) compared to evaluation of stop frames by visual assessment (r = 0·66, P<0·001) or the GSMn score (r = 0·64, P<0·001). High inter- and intra-observer correlations were observed for real-time visual score (both intraclass correlation coefficient = 0·93).ConclusionReal-time evaluation of the level of AVC is superior to using stop frames assessed either visually or by dedicated computer grey scale measurement software.