UNASSIGNED: Bicuspid aortic valve (BAV) is the most common congenital heart disease with an increased risk of infective endocarditis (IE). Few data are available on isolated native BAV-IE. The aim of this study was to compare patients with tricuspid aortic valve (TAV) IE and BAV-IE in terms of characteristics, management and prognosis.
UNASSIGNED: We included 728 consecutive patients with IE on isolated native aortic valve in 3 centres: Amiens and Marseille Hospitals in France and Salerno Hospital in Italy. We studied in hospital and long-term mortality before and after matching for age, sex and comorbidity index. Median follow-up was 67.2 [IQR: 19-120] months.
UNASSIGNED: Of the 728 patients, 123 (16.9%) had BAV. Compared with patients with TAV-IE, patients with BAV-IE were younger, had fewer co-morbidities and were more likely to be male. They presented more major neurological events and perivalvular complications (both p < 0.05). Early surgery (<30 days) was performed in 52% of BAV-IE cases vs. 42.8% for TAV-IE (p = 0.061). The 10-year survival rate was 74 ± 5% in BAV-IE patients compared with 66 ± 2% in TAV-IE patients (p = 0.047). After propensity score matching (for age, gender and comorbidities), there was no difference in mortality between the two groups, with an estimated 10-year survival of 73 ± 5% vs. 76 ± 4% respectively (p = 0.91).
UNASSIGNED: BAV is a frequent finding in patients with isolated aortic valve IE and is associated with more perivalvular complications and neurological events. The differences in survival with TAV-IE are probably related to the age and comorbidity differences between these two populations.

OBJECTIVE: Patients with bicuspid aortic valve (BAV) and aortic regurgitation have higher rate of aortic complications compared with patients with BAV and stenosis, as well as BAV without valvular disease. Aortic regurgitation alters blood haemodynamics not only in systole but also during diastole. We therefore sought to investigate wall shear stress (WSS) during the whole cardiac cycle in BAV with aortic regurgitation.
RESULTS: Fifty-seven subjects that underwent 4D flow cardiovascular magnetic resonance imaging were included: 13 patients with BAVs without valve disease, 14 BAVs with aortic regurgitation, 15 BAVs with aortic stenosis, and 22 normal controls with tricuspid aortic valve. Peak and time averaged WSS in systole and diastole and the oscillatory shear index (OSI) in the ascending aorta were computed. Student\'s t-tests were used to compare values between the four groups where the data were normally distributed, and the non-parametric Wilcoxon rank sum tests were used otherwise. BAVs with regurgitation had similar peak and time averaged WSS compared with the patients with BAV without valve disease and with stenosis, and no regions of elevated WSS were found. BAV with aortic regurgitation had twice as high OSI as the other groups (P ≤ 0.001), and mainly in the outer mid-to-distal ascending aorta.
CONCLUSIONS: OSI uniquely characterizes altered WSS patterns in BAVs with aortic regurgitation, and thus could be a haemodynamic marker specific for this specific group that is at higher risk of aortic complications. Future longitudinal studies are needed to verify this hypothesis.

Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Although the etiology is complex, altered flow dynamics is thought to play an important role. Blood speckle tracking (BST) allows for visualization and quantification of complex flow, which could be useful in identifying patients at risk of root dilation and could aid in surgical planning. The aims of this study were to assess and quantify flow in the aortic root and left ventricle using BST in children with bicuspid aortic valves.
A total of 38 children <10 y of age were included (24 controls, 14 with BAV). Flow dynamics were examined using BST in the aortic root and left ventricle. Children with BAV had altered systolic flow patterns in the aortic root and higher aortic root average vorticity (25.9 [23.4-29.2] Hz vs. 17.8 [9.0-26.2] Hz, p < 0.05), vector complexity (0.17 [0.14-0.31] vs. 0.05 [0.02-0.13], p < 0.01) and rate of energy loss (7.9 [4.9-12.1] mW/m vs. 2.7 [1.2-7.4] mW/m, p = 0.01). Left ventricular average diastolic vorticity (20.9 ± 5.8 Hz vs. 11.4 ± 5.2 Hz, p < 0.01), kinetic energy (0.11 ± 0.05 J/m vs. 0.04 ± 0.02 J/m, p < 0.01), vector complexity (0.38 ± 0.1 vs. 0.23 ± 0.1, p < 0.01) and rate of energy loss (11.1 ± 4.8 mW/m vs. 2.7 ± 1.9 mW/m, p < 0.01) were higher in children with BAV.
Children with BAV exhibit altered flow dynamics in the aortic root and left ventricle in the absence of significant aortic root dilation. This may represent a substrate and potential predictor for future dilation and diastolic dysfunction.

Bicuspid aortic valve (BAV) patients have an increased risk to develop thoracic aortic complications. Little is known about the prevalence and severity of atherosclerosis in the BAV ascending aortic wall. This study evaluates and compares the prevalence of thoracic aortic atherosclerosis in BAV and tricuspid aortic valve (TAV) patients.
Atherosclerosis was objectified using three diagnostic modalities in two separate BAV patient cohorts (with and without an aortic dilatation). Within the first group, atherosclerosis was graded histopathologically according to the modified AHA classification scheme proposed by Virmani et al. In the second group, the calcific load of the ascending aorta and coronary arteries, coronary angiographies and cardiovascular risk factors were studied. Patients were selected from a surgical database (treated between 2006-2020), resulting in a total of 128 inclusions.
Histopathology showed atherosclerotic lesions to be more prevalent and severe in all TAV as compared to all BAV patients (OR 1.49 (95%CI 1.14 - 1.94); p = 0.003). Computed tomography showed no significant differences in ascending aortic wall calcification between all BAV and all TAV patients, although a tendency of lower calcific load in favor of BAV was seen. Coronary calcification was higher in all TAV as compared to all BAV (OR 1.30 (95%CI 1.06 - 1.61); p = 0.014).
Ascending aortic atherosclerotic plaques were histologically more pronounced in TAV as compared to the BAV patients, while CT scans revealed equal amounts of calcific depositions within the ascending aortic wall. This study confirms less atherosclerosis in the ascending aortic wall and coronary arteries of BAV patients as compared to TAV patients. These results were not affected by the presence of a thoracic aortic aneurysm.

暂无摘要。

UNASSIGNED: Non-invasive assessment of aortic hemodynamics using four dimensional (4D) flow magnetic resonance imaging (MRI) provides new information on blood flow patterns and wall shear stress (WSS). Aortic valve stenosis (AS) and/or bicuspid aortic valves (BAV) are associated with altered aortic flow patterns and elevated WSS. Aim of this study was to investigate changes in aortic hemodynamics over time in patients with AS and/or BAV with or without aortic valve replacement.
UNASSIGNED: We rescheduled 20 patients for a second 4D flow MRI examination, whose first examination was at least 3 years prior. A total of 7 patients received an aortic valve replacement between baseline and follow up examination (=operated group = OP group). Aortic flow patterns (helicity/vorticity) were assessed using a semi-quantitative grading approach from 0 to 3, flow volumes were evaluated in 9 planes, WSS in 18 and peak velocity in 3 areas.
UNASSIGNED: While most patients had vortical and/or helical flow formations within the aorta, there was no significant change over time. Ascending aortic forward flow volumes were significantly lower in the OP group than in the NOP group at baseline (NOP 69.3 mL ± 14.2 mL vs. OP 55.3 mL ± 1.9 mL p = 0.029). WSS in the outer ascending aorta was significantly higher in the OP group than in the NOP group at baseline (NOP 0.6 ± 0.2 N/m2 vs. OP 0.8 ± 0.2 N/m2, p = 0.008). Peak velocity decreased from baseline to follow up in the aortic arch only in the OP group (1.6 ± 0.6 m/s vs. 1.2 ± 0.3 m/s, p = 0.018).
UNASSIGNED: Aortic valve replacement influences aortic hemodynamics. The parameters improve after surgery.

Bicuspid aortic valve (BAV) disease is associated with increased risk of aortopathy. In addition to current intervention guidelines, BAV mediated changes in aortic 3D hemodynamics have been considered as risk stratification measures. We aimed to evaluate the association of 4D flow cardiovascular magnetic resonance (CMR) derived voxel-wise aortic reverse flow with aortic dilation and to investigate the role of aortic valve regurgitation (AR) and stenosis (AS) on reverse flow in systole and diastole.
510 patients with BAV (52 ± 14 years) and 120 patients with trileaflet aortic valve (TAV) (61 ± 11 years) and mid-ascending aorta diameter (MAAD) > 35 mm who underwent CMR including 4D flow CMR were retrospectively included. An age and sex-matched healthy control cohort (n = 25, 49 ± 12 years) was selected. Voxel-wise reverse flow was calculated in the aorta and quantified by the mean reverse flow in the ascending aorta (AAo) during systole and diastole.
BAV patients without AS and AR demonstrated significantly increased systolic and diastolic reverse flow (222% and 13% increases respectively, p < 0.01) compared to healthy controls and also had significantly increased systolic reverse flow compared to TAV patients with aortic dilation (79% increase, p < 0.01). In patients with isolated AR, systolic and diastolic AAo reverse flow increased significantly with AR severity (c = - 83.2 and c = - 205.6, p < 0.001). In patients with isolated AS, AS severity was associated with an increase in both systolic (c = - 253.1, p < 0.001) and diastolic (c = - 87.0, p = 0.02) AAo reverse flow. Right and left/right and non-coronary fusion phenotype showed elevated systolic reverse flow (> 17% increase, p < 0.01). Right and non-coronary fusion phenotype showed decreased diastolic reverse flow (> 27% decrease, p < 0.01). MAAD was an independent predictor of systolic (p < 0.001), but not diastolic, reverse flow (p > 0.1).
4D flow CMR derived reverse flow associated with BAV was successfully captured even in the absence of AR or AS and in comparison to TAV patients with aortic dilation. Diastolic AAo reverse flow increased with AR severity while AS severity strongly correlated with increased systolic reverse flow in the AAo. Additionally, increasing MAAD was independently associated with increasing systolic AAo reverse flow. Thus, systolic AAo reverse flow may be a valuable metric for evaluating disease severity in future longitudinal outcome studies.

To evaluate the prognostic value of aortic distensibility measured by cardiovascular magnetic resonance (CMR) as predictors of prophylactic aortic valve or aortic surgery in patients with bicuspid aortic valve (BAV). 110 patients with BAV were included. Distensibility of middle ascending aorta (AscAo) and proximal descending aorta (DescAo) at baseline was determined using CMR. The association between aortic distensibility and primary endpoint of aortic valve and/or aortic surgery was investigated with Cox proportional hazard regression analyses. The receiver operating characteristics curves (ROC) of the area under receiver-operator (AUC) and DeLong test were used to evaluate and compare the performance of different models. During a median follow-up of 66.5 months [IQR 13-75 months], 42 patients experienced surgical treatments. After adjusting for traditional risk factors, aortic distensibility (P = 0.003) and severe valve dysfunction (P < 0.001) were found significantly associated with aortic valve and/or aortic surgery. The model 2 (aortic distensibility and severe valve dysfunction) is slightly better in predicting primary endpoint than the model 1 (aortic diameter and severe valve dysfunction) (AUC: 0.893 vs. 0.842, P = 0.106). In BAV patients, aortic distensibility and severe valve dysfunction are valuable predictors for final aortic valve and/or aortic surgery.

UNASSIGNED: To assess the impact of conventional transcatheter heart valve (THV) commissural alignment techniques on THV/coronary overlap and coronary access (CA) after transcatheter aortic valve replacement (TAVR) in bicuspid aortic valve (BAV).
UNASSIGNED: Specific Evolut Pro/Pro + and Acurate Neo2 THV orientations are associated with reduced neo-commissural overlap with coronary ostia in tricuspid aortic anatomy. Whether standard orientation techniques are effective also in the setting of BAV anatomy has not been studied.
UNASSIGNED: The DA VINCI (Definition of trAnscatheter aortic Valve orIeNtation in biCuspId aortic valve) pilot study is a prospective registry enrolling consecutive patients with severe BAV stenosis undergoing TAVR with last generation supra-annular tall-frame THVs implanted with a cusp overlap view-based commissural alignment. Patients underwent pre- and post-TAVR computed tomography (CT) and coronary angiography. The study endpoint was the rate of favorable THV/coronary overlap, defined as an angle > 40° between the THV commissural post and coronary ostia. Other endpoints were the rates of successful THV alignment with respect to the raphe and of selective CA after TAVR. Moreover, different virtual THV alignment models were tested to identify which one would produce the lower degree of THV/coronary overlap.
UNASSIGNED: Thirty-four patients with type 1 BAV with right-left raphe undergoing TAVR (23 with Evolut Pro/Pro + and 11 with Acurate Neo2) were included. At pre-TAVR CT, moderate/severe cusp asymmetry was found in 50% of patients, severe coronary ostia eccentricity was observed in 47.1% for the RCA vs. 8.8% for the LCA (P < 0.007). Correct TVH orientation was achieved in 29 cases. At post-TAVR CT, optimal THV alignment/mild misalignment to the raphe was observed in 86.2%, but a moderate/severe overlap with the coronaries was seen in 13.7% for the RCA and 44.8% for the LCA (P = 0.019). After TAVR, selective RCA cannulation was possible in 82.8% vs. 75.9% for the LCA (P = 0.74), while combined selective CA of both coronaries was possible in less than two-thirds of the patients. Virtual THV alignment in the coronary ostia overlap view assuming a hypothetical circular THV expansion would produce an optimal THV/coronary overlap in almost 90% of cases.
UNASSIGNED: Given cusp asymmetry and coronary ostia eccentricity of BAV combined with potential THV asymmetrical expansion, conventional commissural alignment techniques are associated with higher rates of THV misalignment and of moderate/severe neo-commissure overlap with the coronary ostia as compared to tricuspid aortic stenosis, resulting in lower rates of selective CA after TAVR. A modified THV orientation technique based on the coronary ostia overlap view might be preferable in BAV patients.

To determine the relationship between aortic distensibility and left ventricular (LV) remodeling, myocardial strain and blood biomarkers in patients with stenotic bicuspid aortic valve (BAV) and preserved ejection fraction (EF) by cardiovascular magnetic resonance (CMR). 43 stenotic BAV patients were prospectively selected for 3.0 T CMR. Patients were divided into LV remodeling group (LV mass/volume ≥ 1.15, n = 21) and non-remodeling group (LV mass/volume < 1.15, n = 22). Clinical characteristics, biochemical data including cardiac troponin T(cTNT), N-terminal pro-B type natriuretic peptide (NT-proBNP) and creatine kinase isoenzyme (CK-MB) were noted. Distensibility of middle ascending aorta (mid-AA) and proximal descending aorta, LV structural and functional parameters, global and regional myocardial strain were measured. Compared to non-remodeling group, LV remodeling group had significantly decreased LV global strain (radial: 26.04 ± 8.70% vs. 32.92 ± 7.81%, P = 0.009; circumferential: - 17.20 ± 3.38% vs. - 19.65 ± 2.34%, P = 0.008; longitudinal: - 9.13 ± 2.34% vs. - 11.63 ± 1.99%, P < 0.001) and decreased mid-AA distensibility (1.22 ± 0.24 10-3 mm/Hg vs 1.60 ± 0.41 10-3 mm/Hg, P = 0.001). In addition, mid-AA distensibility was independently associated with LV remodeling (β =  - 0.282, P = 0.003), and it was also significantly correlated with LV global strain (radial: r = 0.392, P = 0.009; circumferential: r =  - 0.348, P = 0.022; longitudinal: r =  - 0.333, P = 0.029), cTNT (r =  - 0.333, P = 0.029) and NT-proBNP (r =  - 0.440, P = 0.003). In this cohort with stenotic BAV and preserved EF, mid-AA distensibility is found significantly associated with LV remolding, which encouraging to better understand mechanism of ventricular vascular coupling.