477Partial fusion of two aortic valve leaflets is related to alterations in ascending aorta flow: 4D flow CMR study

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 20 (Supplement_2) ◽  
Author(s):  
A Guala ◽  
L Galian ◽  
G Teixido Tura ◽  
A Ruiz Munoz ◽  
C Granato ◽  
...  

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 &lt; 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 &lt; 0.001), age (R = 0.313, p = 0.005) and body surface area (R = 0.396, p &lt; 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.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Guala ◽  
A Evangelista ◽  
L La Mura ◽  
G Teixido-Tura ◽  
L Dux-Santoy ◽  
...  

Abstract Background Aortic dilation in bicuspid aortic valve (BAV) patients has been related to altered flow patterns, which contribute to aortic wall degeneration. However, preventive aortic replacement is currently based on a diameter threshold. Several studies on excised BAV reported wide variability of fusion extent. Purpose To unveil whether leaflet fusion extent can be quantified by CMR and is related to aortic dilation and flow abnormalities in non-dysfunctional BAV. Methods One hundred and twenty adults with non-dysfunctional BAV and no previous aortic or aortic valve surgery and 28 healthy volunteers underwent double-oblique cine and 4D flow CMR. BAV patients with two sinuses of Valsalva or left and non-coronary cusps fusion were excluded. Peak systolic circumferential wall shear stress (WSSc) and pulse wave velocity (PWV) in the ascending aorta (AAo) were assessed by 4D flow CMR. Fusion length between leaflets was measured using a stack of double-oblique cine CMR images of the aortic valve. Results The length of the fusion was effectively measured in 112/120 (93%) patients with good reproducibility (ICC = 0.826) and showed great variability (range 2.3–15.4 mm, 7.8±3.2 mm and tertiles cut-off points 6 and 9.3 mm). In multivariate analysis adjusted for clinical and demographic characteristics and PWV, fusion length was independently associated with the diameter at the sinus of Valsalva (p=0.002) and the AAo (p=0.02) (Table). WSSc progressively increased with larger fusion length (Figure), with statistical significance (p&lt;0.05) in the right and outer regions of the proximal and mid AAo. Conclusions Bicuspid aortic leaflet fusion length varies considerably, and it is independently associated with AAo and aortic root dilation, possibly through flow alterations. Figure 1. Maps of circumferential WSS Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study has been partially funded by Instituto Carlos III, Spanish Ministry of Science and Innovation (PI17/00381). Guala A. has received funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I).


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.


VASA ◽  
2005 ◽  
Vol 34 (3) ◽  
pp. 181-185 ◽  
Author(s):  
Westhoff-Bleck ◽  
Meyer ◽  
Lotz ◽  
Tutarel ◽  
Weiss ◽  
...  

Background: The presence of a bicuspid aortic valve (BAV) might be associated with a progressive dilatation of the aortic root and ascending aorta. However, involvement of the aortic arch and descending aorta has not yet been elucidated. Patients and methods: Magnetic resonance angiography (MRA) was used to assess the diameter of the ascending aorta, aortic arch, and descending aorta in 28 patients with bicuspid aortic valves (mean age 30 ± 9 years). Results: Patients with BAV, but without significant aortic stenosis or regurgitation (n = 10, mean age 27 ± 8 years, n.s. versus control) were compared with controls (n = 13, mean age 33 ± 10 years). In the BAV-patients, aortic root diameter was 35.1 ± 4.9 mm versus 28.9 ± 4.8 mm in the control group (p < 0.01). The diameter of the ascending aorta was also significantly increased at the level of the pulmonary artery (35.5 ± 5.6 mm versus 27.0 ± 4.8 mm, p < 0.001). BAV-patients with moderate or severe aortic regurgitation (n = 18, mean age 32 ± 9 years, n.s. versus control) had a significant dilatation of the aortic root, ascending aorta at the level of the pulmonary artery (41.7 ± 4.8 mm versus 27.0 ± 4.8 mm in control patients, p < 0.001) and, furthermore, significantly increased diameters of the aortic arch (27.1 ± 5.6 mm versus 21.5 ± 1.8 mm, p < 0.01) and descending aorta (21.8 ± 5.6 mm versus 17.0 ± 5.6 mm, p < 0.01). Conclusions: The whole thoracic aorta is abnormally dilated in patients with BAV, particularly in patients with moderate/severe aortic regurgitation. The maximum dilatation occurs in the ascending aorta at the level of the pulmonary artery. Thus, we suggest evaluation of the entire thoracic aorta in patients with BAV.


2020 ◽  
Vol 22 (Supplement_N) ◽  
pp. N142-N145
Author(s):  
Alice Benedetti ◽  
Alvise Del Monte ◽  
Maurizio Rubino ◽  
Daniela Mancuso

Abstract A 36-year-old woman at 31 weeks’ gestation presented with exertional dyspnoea and palpitations. She had a history of bicuspid aortic valve treated with surgical aortic valvotomy for severe stenosis, followed by ascending aorta replacement for type A acute aortic dissection and Bentall operation with a mechanical valve for severe aortic regurgitation. Eight years after the last surgery, magnetic resonance angiography showed aortic arch aneurysm (49 mm) with a small intimal flap. Thereafter, the patient was lost to follow-up until the current admission. She was hemodynamically stable on presentation and physical examination was unremarkable apart from a mechanical second heart sound. The electrocardiogram showed sinus rhythm with left bundle branch block (Panel A). Transthoracic echocardiography revealed severe left ventricular dilation (EDV 90 ml/m2) with mild dysfunction (EF 50%), normal prosthetic aortic valve function, and aortic arch dilation (50 mm) (Panel B and C). After a multidisciplinary evaluation, elective cesarean section was performed at 34 weeks’ gestation. A post-delivery aortic computed tomography angiography revealed aortic arch aneurysm (52 mm) with intimal flap and two pseudoaneurysms of the anterior aortic wall causing sternal erosion (Panel D, E, F and G). Subsequently, the patient underwent ascending aorta and aortic arch replacement by Frozen Elephant Trunk technique with a 24 x130 mm prosthesis between the aortic root and the descending aorta. The postoperative course was uneventful, and the patient was discharged to a cardiac rehabilitation centre.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Ribeyrolles ◽  
J L Monin ◽  
A Rohnean ◽  
C Diakov ◽  
C Caussin ◽  
...  

Abstract Background Mitral valve regurgitation (MR) is currently primarily assessed by a multiparametric approach with transthoracic echocardiography (TTE) that can be further completed by 2D Cardiac Magnetic Resonance (2D CMR) in case of doubt or poor acoustic window. TTE and 2D CMR have nevertheless imperfect agreement in terms of MR quantification. Time-resolved phase-contrast cardiac magnetic resonance imaging with flow-encoding in three spatial directions (4D Flow CMR) could help in MR quantification. Purpose Compare 4D Flow CMR quantification of MR with TTE using a multiparametric approach. Methods We conducted a monocentric, prospective study at the Institut Mutualiste Montsouris in Paris between November 2016 and 2017 including patients with chronic primitive MR. MR was evaluated with a multiparametric approach by two cardiologists with TTE and quantitatively by two radiologists with 4D Flow CMR. MR was classified as mild, moderate or severe and evaluated blindly with consensus in case of disagreement. 4D Flow CMR measurements consisted in quantifying MR regurgitant volume (RV) and MR regurgitant fraction (RF). 4D anterograde mitral flow was compared to left ventricular stroke volume using 2D-cine CMR. Results 33 patients were included. Inter-observer agreement was good in TTE (kappa= 0.75 95% CI [0.57- 0.92]) and excellent in 4D Flow CMR (ICC= 0.94 95% CI [0.79–0.95]). Agreement with TTE was excellent using optimized thresholds (Mild: RV≤20mL RF≤20%, Moderate: RV=21–39mL RF=21–36%, Severe: RV≥40mL RF≥37%): kappa= 0.93 95% CI [0.8–1] for RV and kappa= 0.90 95% CI [0.7–0.9] for RF. A validation cohort confirmed that the 4D flow thresholds as determined were accurate for MR grading. Agreement between 4D anterograde mitral flow and 2D-cine CMR left ventricular stroke volume was also excellent (ICC= 0.92 95% CI [0.85–0.96]). Conclusion 4D Flow CMR is a reliable tool for MR quantification. It provides direct quantitative evaluation of MR with low inter-observer variability. It may therefore be used as a gatekeeper before therapeutic decisions such as surgery.


Author(s):  
Eric J Keller ◽  
S C Malaisrie ◽  
Jane Kruse ◽  
Pim van Ooij ◽  
Edouard Semaan ◽  
...  

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