scholarly journals Bicuspid aortic valve fusion length correlates with maximum aortic diameter and heamodynamic abnormalities: a 4D flow CMR study

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Guala ◽  
G Teixido-Tura ◽  
L Dux-Santoy ◽  
A Ruiz-Munoz ◽  
F Valente ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Guala A. received funding from the Spanish Ministry of Science, Innovation and Universities. Background Bicuspid aortic valve (BAV), a congenital heart defect, is associated with ascending aorta dilation, possibly via alteration of aortic blood flow [1]. In BAV abnormal flow condition have been associated with aortic extracellular matrix dysregulation and elastic fiber degeneration [2]. Current morphological classification of BAV patients with aortic valve with a single fusion between two adjacent leaflets does not allow for risk stratification. Purpose This research work tested whether the extent of fusion between leaflets is related to AAo diameter and flow alterations. Methods Ninety BAV patients free from moderate and severe aortic valve disease and with no previous aortic or aortic valve surgery or replacement were prospectively enrolled. A comprehensive magnetic resonance protocol comprised a stack of double-oblique 2D balanced steady-state free-precession (bSSFP) cine CMR of the aortic valve, which was used to measure the length of the fusion between leaflets, a cine CMR at the level of the pulmonary bifurcation to assess aortic diameter and 4D flow MRI sequence to assess flow characteristics and regional stiffness [3]. Jet angle and flow radial displacement, quantifying the extent of flow eccentricity, and systolic flow reversal ratio (SFRR), assessing the relative amount of backward flow during systole, were computed at 8 equidistant planes in the ascending aorta and 4 equidistant planes in the aortic arch [4]. A two-tailed p-value < 0.05 was considered statistically significant. Results The length of leaflet fusion varied widely (median 7.7 mm, inter-quartile range [5.5; 10.2]), Table 1). In bivariate analysis, fusion length was also associated to ascending aortic diameter (R = 0.391, p < 0.001), age (R = 0.313, p = 0.005) and body surface area (R = 0.396, p < 0.001). It was also positively related to flow abnormalities: like displacement in the proximal and distal ascending aorta, jet angle in the mid ascending aorta, and SFRR in the ascending aorta and the aortic arch (see Figure 1). The association between fusion length and ascending aorta diameter persisted in multivariate analysis after correction for age (p = 0.006). Conclusions Bicuspid aortic valve fusion extent varies greatly and it is associated with aortic diameter, possibly through flow alterations. Prospective longitudinal studies are needed to establish whether fusion length may allow for risk stratification in bicuspid aortic valve patients.

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
A Guala ◽  
L Galian ◽  
G Teixido Tura ◽  
L Dux-Santoy ◽  
A Ruiz Munoz ◽  
...  

Abstract Introduction Bicuspid aortic valve (BAV) is the most common congenital valve defect. It consists in the fusion of two aortic valve leaflets, and it is associated with a high prevalence of proximal aorta dilation. Dilation is highly prevalent (around 30%) in BAV patient relatives with a tricuspid valve (TAV) identified by echocardiography. However, the presence of partial aortic valve leaflet fusion (also called mini-raphe or forme fruste BAV, see figure 1A) is easily missed by echocardiography. A recent study reported that 44% of patients from a small cohort of BAV patient relatives with aortic dilation followed by CT showed mini-raphe. Purpose We aimed to use 4D flow CMR to assess if the presence of mini-raphe is associated with aortic flow alterations, which may be concurs in the etiology of aortic dilation in BAV patient relatives. Methods Twenty BAV patients first-degree relatives with partial fusion (<50%) of aortic valve leaflets and proximal aorta dilation were identified by CT or cine CMR and prospectively included. One-hundred twenty-five BAV and 95 patients with TAV from our prospective dataset of 4D flow CMR were included for comparison. Propensity score matching was used throughout the study to correct the comparisons between mini-raphe and BAV and mini-raphe and TAV patients for differences in age, maximum aortic diameter, sex, height, weight, proximal aortic pulse wave velocity and, only for BAV, fusion pattern. The hemodynamic parameters previously related to aortic dilation were computed. They were jet angle, normalized flow displacement and systolic flow reversal ratio (SFRR, identifying through-plane vortexes) were computed and compared in the ascending aorta and in the aortic arch. Results The presence of mini-raphe was statistically-significantly associated with increase in jet angle (Figure 1B), flow displacement (Figure 1C) and vortexes (Figure 1D) in most of the ascending aorta and aortic arch when mini-raphe patients were compared with TAV patients. The severity of flow asymmetry found in mini-raphe patients was lower than the one characteristic of BAV patients, but vortexes were even higher in a small region at the distal ascending aorta. Figure 1 Conclusion Partial fusion of the aortic valve leaflets is related to increase in proximal aorta flow eccentricity and vorticity. These flow abnormalities are not as marked as those associated with BAV. Data regarding prevalence of mini-raphe as evaluated with CT or cine CMR are needed, especially in familiar of BAV patients. Acknowledgement/Funding European FP7/People 267128; Spanish Ministry of Economy and Competitiveness RTC-2016-5152-1 and Instituto de Salud Carlos III PI14/0106


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
G Goudot ◽  
T Mirault ◽  
C Cheng ◽  
M Gruest ◽  
J Amoah ◽  
...  

Abstract Background Magnetic resonance imaging allows evaluation of aortic stiffness by the maximum rate of systolic distension (MRSD) a new prognosis factor of aortic dilatation in patients with bicuspid aortic valve (BAV). MRSD requires a continuous monitoring of the aortic diameter during the cardiac cycle, not accessible to conventional echocardiography contrary to ultrafast ultrasound imaging (UF). Purpose To develop specific aortic sequences in ultrafast ultrasound imaging (UF) to provide access to the aortic MRSD Methods Tissue Doppler allowed a precise estimation of the movement of each wall and the fine variation of the aortic diameter. To automatically track the anterior and posterior aortic walls during the cardiac cycle, we developed in the laboratory a specific interface (Figure). MRSD was the maximum of the derivative of the diameter chande over time. To assess this new technique, 24 patients (10 BAV patients and 14 controls, mean age 45.8 vs. 40.7 years, p=0.464, respectively) were consecutively included at a reference center for BAV. The ascending aorta was evaluated at the sinus of Valsalva, the tubular aorta and the aortic arch with a phased array probe (Supersonic Imagine) and dedicated sequences at 2000 frames/s. Results The lab-made interface allowed to track the aortic diameter and to calculate the MRSD from the UF acquisitions for each patient. We found lower MRSD at the sinus of Valsalva in case of BAV in accordance with previously demonstrated higher stiffness at this segment by our team (Table). Table 1. UF Aortic parameters for BAV patients and controls BAV patients Controls p (Mann Whitney) Sinus of Valsalva Diameter (mm) 26.2 [22.4–32.5] 27.09 [23.5–29.5] 0.796 MRSD (s–1) 1.05 [0.73–1.19] 1.51 [1.28–1.99] 0.023 Tubular ascending aorta Diameter 31.4 [29.4–32.2] 28.9 [22.6–31.5] 0.328 MRSD 0.94 [0.59–1.27] 1.09 [0.87–1.41] 0.353 Aortic arch Diameter 24.2 [23.7–24.8] 24.2 [18.9–24.5] 0.673 MRSD 0.57 [0.35–1.07] 0.85 [0.76–1.02] 0.257 Results are median [25th–75th percentile]. Figure 1 Conclusion UF allows evaluation of aortic stiffness by MRSD using dedicated sequence and interface. As echocardiography, UF is easily accessible and therefore deserves attention from cardiologists taking care of BAV patients to evaluate the segmental aortic remodeling associated with BAV.


Author(s):  
Lydia Dux-Santoy ◽  
Andrea Guala ◽  
Julio Sotelo ◽  
Sergio Uribe ◽  
Gisela Teixidó-Turà ◽  
...  

Objective: To assess the relationship between regional wall shear stress (WSS) and oscillatory shear index (OSI) and aortic dilation in patients with bicuspid aortic valve (BAV). Approach and Results: Forty-six consecutive patients with BAV (63% with right-left-coronary-cusp fusion, aortic diameter ≤ 45 mm and no severe valvular disease) and 44 healthy volunteers were studied by time-resolved 3-dimensional phase-contrast magnetic resonance imaging. WSS and OSI were quantified at different levels of the ascending aorta and the aortic arch, and regional WSS and OSI maps were obtained. Seventy percent of BAV had ascending aorta dilation. Compared with healthy volunteers, patients with BAV had increased WSS and decreased OSI in most of the ascending aorta and the aortic arch. In both BAV and healthy volunteers, regions of high WSS matched regions of low OSI and vice versa. No regions of both low WSS and high OSI were identified in BAV compared with healthy volunteers. Patients with BAV with dilated compared with nondilated aorta presented low and oscillatory WSS in the aortic arch, but not in the ascending aorta where dilation is more prevalent. Furthermore, no regions of concomitant low WSS and high OSI were identified when BAV were compared according to leaflet fusion pattern, despite the well-known differences in regional dilation prevalence. Conclusions: Regions with low WSS and high OSI do not match those with the highest prevalence of dilation in patients with BAV, thus providing no evidence to support the low and oscillatory shear stress theory in the pathogenesis of proximal aorta dilation in the presence of BAV.


2021 ◽  
Author(s):  
Tie Zheng ◽  
Shijie Lu ◽  
Shuai Zhu ◽  
Jiafu Ou ◽  
Jun-Ming Zhu

Abstract Objective: Aim of this study is to investigate the influence of aortic diameter on hemodynamic environment characteristics in patient with the bicuspid aortic valve (BAV) and dilated ascending aorta (AAo) .Methods: In this study, an MRI of one BAV patient with 4.5 cm AAo was collected and numerical model was constructed. Based on the images,the other three numerical models were constructed with different ascending aortic size with 4.0cm, 5.0cm and 5.5cm respectively while the size and the geometry of other parts were fixed. Then hemodynamics in these four models was simulated numerically and the flow patterns and loading distributions were investigated.Results: Hemodynamics environments in the AAo were simulated with different aortic size. As the aortic diameter increases, we find: 1. the blood flow becomes more disturbing;2.the wall pressure at ascending aortic is higher; 3. the wall shear stress at the ascending aortic decreases; 4.oscillatory shear index of the outer part on the proximal AAo increases;5. all these hemodynamic parameters described above are asymmetrically distributed in dilated AAo and more parts of aorta would be affected as the AAo dilatation progresses.Conclusions: The study revealed that the diameter of ascending aortic can significantly influence the magnitude and distribution of the dynamics. There are altered flow patterns, pressure difference, WSS and OSI distribution features in bicuspid aortic valve patients with vascular dilatation. As the extent of aortic dilatation increases especially exceed 5.5cm,this study support the recent guideline that aortic replacement should be considered .


2020 ◽  
Vol 23 (4) ◽  
pp. E435-E440
Author(s):  
Fei Li ◽  
Qi Gao ◽  
En Qiao ◽  
Gang Yin ◽  
Ru-Jiao Zhang ◽  
...  

Purpose: The dilation of proximal arch (PArc) was suspected to develop as a result of valve-related hemodynamics or in consequence to the upward extension of the ascending aorta (AAo) dilation. We aimed to investigate the one that could be the possible contributing factor in patients with bicuspid aortic valve (BAV). Methods: All enrolled BAV patients underwent four-dimensional flow magnetic resonance imaging. Contour-averaged circumferential wall shear stress (WSScirc,avg) and the diameter of the middle of AAo (mid-AAo) were compared between the patients with and without PArc dilation. Additionally, we analyzed the correlation between WSScirc,avg and aortic diameter at PArc section, as well as the correlation between the diameter of mid-AAo and that of PArc. Results: No significant difference was observed in WSScirc,avg at PArc section between the patients with and without PArc dilation (P = .621). However, the diameter of mid-AAo in the patients who suffered PArc dilation was higher than those without it (P = .007). In addition, the aortic diameter did not correlate with the WSScirc,avg at PArc level (R = -0.068, P = .701). The correlation was observed between PArc diameter and mid-AAo diameter (R = 0.521, P = .002). Conclusion: Hemodynamics may not contribute to the development of PArc dilation. PArc diameter correlated with mid-AAo diameter, indicating PArc dilation may be secondary to the upward extension of AAo dilation. The influence of AAo dilation extending upward could be treated by AAo replacement; hence, a selective approach to transverse PArc replacement might be appropriate.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Belkadi ◽  
O Milleron ◽  
L Eliahou ◽  
F Arnoult ◽  
G Delorme ◽  
...  

Abstract Background Aortic dissection during pregnancy is uncommon, however, the risk of aortic dissection is increased if there is underlying aortopathy. Bicuspid aortic valve (BAV) is common in the general population and is associated with the presence of an aortic aneurysm, but this condition is mostly asymptomatic and ignored in women of childbearing age. Data on pregnancy in patients with BAV are scarce, and guidelines on this topic are based on the consensus opinion of experts. The risk of occurrence of aortic dissection as a function of aortic diameter during pregnancy remains poorly known in women with BAV. Purpose To investigate demographic and echocardiographic characteristics and aortic events associated with pregnancy in women with BAV and to estimate ascending aortic diameter at the time of pregnancy. Methods We performed a retrospective study using data from our tertiary centre. All women seen at our centre between 1996 and 2020 with BAV, at least 1 pregnancy, and no genetic syndrome were included. We have collected data from echocardiograms performed in and out of our centre and aortic events. Assuming from the literature an annual aortic dilation rate of 0.2 mm at the sinus of Valsalva and 0.4 mm at the tubular ascending aorta, we estimated ascending aortic size and Z-score at the time of pregnancy. Results We identified 47 women with BAV with occurrence of 103 pregnancies. The median age of BAV diagnosis was 43 years. The aorta was measured at a median of 13.3 years since the last delivery. At BAV diagnosis, the median largest ascending aortic diameter was 44mm, and the median Z-score was +4.3. Ascending aortic diameter was ≥40mm in 37/47 (79%) and Z-score ≥2 in 44/47 (94%). No aortic dissection was observed during pregnancy and postpartum in all 103 pregnancies. At the time of pregnancy, the estimated median diameter of the ascending aorta was 37mm and the estimated median Z-score was +3.3. The largest aortic diameter during pregnancy was estimated to be ≥40mm in 36/103 pregnancies, ≥45mm in 13/103, and ≥50mm in 1/103; Z-score was estimated to be ≥2 in 81/103 and ≥4 in 40/103. Type A aortic dissection occurred in 1 woman, 13 years after pregnancy, and type B aortic dissection in 1 woman, 14 years after pregnancy. Planned surgery was performed in 8 women at a median of 17.5 years after the last pregnancy: 1 isolated aortic valve replacement and 7 prophylactic aortic surgeries associated with aortic valve surgery. Conclusions In our population of women with BAV, pregnancy is not associated with the occurrence of aortic dissection even though, when estimating aortic diameter at the time of pregnancy, the rate of aortic dilation was high (Z-score ≥2 in 81/103 pregnancies). Prospective studies of a large population of women with BAV are needed to assess the risk of aortic complication during pregnancy according to aortic diameter. FUNDunding Acknowledgement Type of funding sources: None.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Geeraert ◽  
F Jamalidinan ◽  
M Bristow ◽  
C Lydell ◽  
A.G Howarth ◽  
...  

Abstract Introduction We use 4D Flow MRI to (1) investigate the effects of bicuspid aortic valve (BAV) disease on downstream pressure drop (PD), wall shear stress (WSS), and viscous energy loss (EL) in the ascending aorta (AAo) and (2) explore the associations between AAo diameter and PD, WSS, and EL. Hypothesis BAV patients show increased PD, WSS, and EL in the AAo compared to age-matched controls Methods 32 healthy controls (41±15 y, 10 female) and 53 BAV patients (44±16 y, 19 female) underwent cardiac MRI at 3T, inclusive of cine imaging and 4D flow. Cross sections were placed along segmented aortas at the: left ventricular outflow tract (LVOT), sinuses of Valsalva (SOV), mid-ascending aorta (MAA), and proximal to first aortic branch (AA1). Locations were analyzed for (i) net flow, (ii) aortic diameter (normalized to BSA), (iii) systolic PD (referenced to LVOT), (iv) systolic EL (measured within LVOT-AA1 volume and normalized by LVOT net flow; mW/mL), and (v) systolic WSS. Sub-vectors of WSS, axial (WSSax) and circumferential (WSScirc), were also analyzed. Results In comparison to controls, BAV patients showed greater PD (e.g. MAA: 9.5±8.0 vs. 2.8±2.4mmHg; p&lt;0.01), EL (0.09±0.05 vs. 0.04±0.01 mW/mL; p≤0.01), and WSScirc (e.g. MAA: 0.3±0.1 vs. 0.2±0.06 Pa; p≤0.01) throughout the AAo (Table 1 and Fig. 1). BAV patients exhibited significantly lower WSS and WSSax only at the SOV. In univariate analyses, AAo diameter was inversely correlated with WSS (R=−0.32, p&lt;0.01) and WSSax (R=−0.51, p≤0.01). In multivariate analyses, AAo diameter was associated with WSS (β=−0.36, p&lt;0.01) and WSSax (β=−0.26, p&lt;0.01). Conclusions BAV patients demonstrate significantly increased PD, EL, and WSScirc in the AAo, and an inverse association between AAo diameter and WSS measures. Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): Alberta Health Services


2020 ◽  
Vol 9 (1) ◽  
pp. 276 ◽  
Author(s):  
Evaldas Girdauskas ◽  
Niklas Neumann ◽  
Johannes Petersen ◽  
Tatiana Sequeira-Gross ◽  
Shiho Naito ◽  
...  

Objective: Aortic size-based criteria are of limited value in the prediction of aortic events, while most aortic events occur in patients with proximal aortic diameters < 50 mm. Serological biomarkers and especially circulating microRNAs (miRNAs) have been proposed as an elegant tool to improve risk stratification in patients with different aortopathies. Therefore, we aimed to evaluate the levels of circulating miRNAs in a surgical cohort of patients presenting with bicuspid aortic valve disease and distinct valvulo-aortic phenotypes. Methods: We prospectively examined a consecutive cohort of 145 patients referred for aortic valve surgery: (1) Sixty three patients (mean age 47 ± 11 years, 92% male) with bicuspid aortic valve regurgitation and root dilatation (BAV-AR), (2) thirty two patients (mean age 59 ± 11 years, 73% male) with bicuspid aortic valve stenosis (BAV-AS), and (3) fifty patients (mean age 56 ± 14 years, 55% male) with tricuspid aortic valve stenosis and normal aortic root diameters (TAV-AS) who underwent aortic valve+/-proximal aortic surgery at a single institution. MicroRNAs analysis included 11 miRNAs, all published previously in association with aortopathies. Endpoints of our study were (1) correlation between circulating miRNAs and aortic diameter and (2) comparison of circulating miRNAs in distinct valvulo-aortic phenotypes. Results: We found a significant inverse linear correlation between circulating miRNAs levels and proximal aortic diameter in the whole study cohort. The strongest correlation was found for miR-17 (r = −0.42, p < 0.001), miR-20a (r = −0.37, p < 0.001), and miR-106a (r = −0.32, p < 0.001). All miRNAs were significantly downregulated in BAV vs. TAV with normal aortic root dimensions Conclusions: Our data demonstrate a significant inverse correlation between circulating miRNAs levels and the maximal aortic diameter in BAV aortopathy. When comparing miRNAs expression patterns in BAV vs. TAV patients with normal aortic root dimensions, BAV patients showed significant downregulation of analyzed miRNAs as compared to their TAV counterparts. Further multicenter studies in larger cohorts are needed to further validate these results.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Hanigk ◽  
E Burgstaller ◽  
H Latus ◽  
N Shehu ◽  
J Zimmermann ◽  
...  

Abstract Introduction Bicuspid aortic valve (BAV) disease leads to deviant complex helical flow patterns in the aorta - especially in the mid-ascending (AoA) part. In association with congenital BAV, aortic wall alterations such as aortic dilatation and dissection may occur. Among others, wall shear stress (WSS) could be one parameter to contribute to the prediction of the long-term outcome of patients with BAV. 4D-flow in cardiovascular magnetic resonance has been established as a valid method to estimate WSS. Purpose The aim of this study is to reevaluate WSS and comparing it to values generated in the same patient cohort with bicuspid aortic valve disease in 2008. No one of the above had aortic dilation in 2008 but proven helical flow pattern. The long term follow-up study might show changes in WSS over the period of ten years. Methods Ten complete 4D flow datasets of patients (age at follow-up: median 34.5 years; range 19–41 years) with bicuspid aortic valve disease without enlargement of the aorta were obtained in 2008 and reevaluated in 2018/2019 in the same patient collective. Mean WSS values were calculated with identical specific software tools. All data were analyzed by two experienced investigators. Results Aortic diameters at the level of the mid AoA did not change significantly in the 10-year period. The WSS values were lower in 2018 at all levels of the ascending aorta (Table 1). Indexed aortic diameters at the level of the mid ascending aorta did not change, median difference 0.06 cm/m2 (range −0.1 cm/m2 to 0.2 cm/m2; p=0.28), absolute values of indexed AoA diameters in 2018/2019 ranged from 1.27 cm/m2 to 2.2 cm/m2 (median 1.76 cm/m2). Table 1 WSS magnitudinal [N/m2] 2008 (n=10) 2018 (n=10) Median difference p-value median range median range Level aortic bulb 0.95 0.80–1.46 0.42 0.34–0.82 −0.53 <0.01 Level mid ascending aorta 0.72 0.40–0.98 0.39 0.34–0.59 −0.33 <0.01 Level brachiocephalic trunc (BCT) 0.71 0.38–1.03 0.40 0.37–0.61 −0.31 <0.01 Conclusions Indexed AoA diameters in BAV disease did not change significantly over a 10-year period. WSS of AoA was less compared to values generated in 2008. This might be explained by a slight alteration in hemodynamic flow patterns by the aging aorta, but not by changes of the aortic diameters. Possibly a drop of WSS in BAV could serve as a marker for a benign long term course.


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