Segmental aortic stiffness in patients with bicuspid aortic valve compared with first-degree relatives

Heart ◽  
2018 ◽  
Vol 105 (2) ◽  
pp. 130-136 ◽  
Author(s):  
Guillaume Goudot ◽  
Tristan Mirault ◽  
Aude Rossi ◽  
Samuel Zarka ◽  
Juliette Albuisson ◽  
...  

AimsTo compare the stiffness index in patients with bicuspid aortic valve (BAV) with first-degree relatives at each segment of the thoracic ascending aorta and to compare segmental analysis of aortic stiffness in association with BAV morphotype and function.Methods219 patients with BAV and 148 first-degree relatives (without BAV) were consecutively included at a reference centre for BAV. Ultrasound assessment of aortic and carotid stiffness was based on the variation of the segmental arterial diameters during the cardiac cycle and on blood pressure.ResultsWithout adjustment, the ascending aorta of patients with BAV seemed stiffer at each segment compared with controls (stiffness index at the sinus of Valsalva: 17.0±10.9 vs 8.9±6.1, p<0.001; tubular aorta: 20.4±31.3 vs 12.7±4.8, p=0.04). However, after adjustment on aortic diameter and age, only the sinus of Valsalva remained stiffer (p<0.001), whereas the tubular aorta no longer differed (p=0.610). In patients with BAV, aortic diameters were not influenced by the valve morphotype, except for the arch, which was more dilated in the case of 1- Non coronary sinus-Right subtype of BAV : 36.1 vs 27.6 mm, p<0.001. Aortic regurgitation was associated with an increase in aortic diameters at the sinus of Valsalva (p<0.001) and the tubular aortic levels (p=0.04).ConclusionStiffness increase at the sinus of Valsalva level is independent of aortic dilatation in patients with BAV, contrary to the classic relationship between stiffness and dilatation found on the other segments. The relationship between stiffness and clinical impact needs to be assessed at each aortic segment.

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.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
L Cozijnsen ◽  
R.L Braam ◽  
M Bakker-De Boo ◽  
A.M Otten ◽  
J.G Post ◽  
...  

Abstract Background Bicuspid aortic valve (BAV) may frequently lead to aortic dilatation with risk of aortic dissection. In patients with BAV both familial clustering and aortic dilatation in first-degree relatives (FDR) without BAV has been demonstrated. Based on these findings the ESC Aortic Guidelines recommend to consider screening of FDR, while the ACC/AHA Guidelines on Valvular Heart Diseases consider screening of FDR only if the index patient has associated aortopathy. Currently, no data about the effectiveness of screening is available. Purpose To investigate the yield of screening FDR of patients with isolated BAV and to explore subgroups with FDR of patients who had needed surgery or of patients with aortic dilatation. We hypothesized that aortic dilatation (&gt;40mm) in the index patient is not a risk factor for BAV in FDR. Methods From 2012, patients with BAV visiting the outpatient clinic of a teaching hospital, received information advising cardiac screening of FDR. FDR of patients with isolated BAV who were referred, were included. From the 10 index patients from other hospitals, information was retrieved. [Fig.1] Results Referred were FDR from 118 index patients (mean age 60 years, standard deviation [SD] 14, range 15–90 years, 82 males [70%]). Of all index patients 63 (53%) had undergone aortic valve replacement, including concomitant ascending aorta replacement in 25 (21%). In the non-operated index patients, 31 (26%) had dilatation (&gt;40mm) of sinus of Valsalva and/or tubular ascending aorta. Screened were 257 FDR (median 2 per index patient) comprising 20 parents (8%), 103 siblings (40%) and 134 offspring (52%). Mean age of FDR was 48 years (SD16, range 4–83 years) and 89 subjects (42%) were male. The diagnostic imaging modality was echocardiography in 240 cases (93%) and MRI in 17 cases. Ten FDR had an already known BAV and were not included in the screening. Among the 257 FDR, we diagnosed 12 new BAV (4.7%, 95% confidence interval [CI]2.9–8.0%) (mean age 44 years, 50% male). Additionally, we diagnosed 23 new isolated aorta dilatations (8.9%; 95% CI 6.0–13%) at level of sinus of Valsalva and/or tubular ascending aorta (mean age 57 years, 18 [78%] were male) [Fig. 1]. Among them, 11 had hypertension. FDR (n=147) of index patients with BAV and previous aortic valve surgery (n=63), had a risk ratio (RR) of 2.25 (95% CI 0.62–8.10) of having a BAV. FDR (n=126) of index patients with BAV and repaired or unrepaired aortic dilatation (n=56) had RR 0.35 (95% CI 0.10–1.25) of having a BAV. Conclusions Screening FDR of patients with isolated BAV resulted in a reasonable yield of 14% new cases with BAV or isolated aortic dilatation. The RR of the subgroup with aorta dilatation did not justify the limitation of the FDR as suggested in the ACC/AHA Guidelines. Figure 1. Flowchart of screening and result Funding Acknowledgement Type of funding source: None


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Vandana Sachdev ◽  
Lea Ann Matura ◽  
Stanislav Sidenko ◽  
Vincent Ho ◽  
Andrew Arai ◽  
...  

Women with Turner syndrome (TS) have an increased risk of congenital cardiovascular defects. Previous studies have reported a 10 –20% prevalence rate of bicuspid aortic valves and there are increasing reports of a vasculopathy that predisposes patients to aortic dilatation and dissection. This prospective study aimed to characterize aortic valve and aortic root structure in unselected asymptomatic individuals with TS. A total of 253 females aged 7– 67 years with karyotype proven TS were examined. Transthoracic echocardiography revealed a normal tricuspid aortic valve (TAV) in 162, a ‘probable TAV’ in 8 subjects, a bicuspid aortic valve (BAV) in 65 and ‘probable BAV’ in 3 subjects. The aortic valve could not be visualized by echocardiography in 15/253 or 6%. Magnetic resonance imaging (MRI) revealed valve structure in 11/12 of the probable cases (all confirmatory of the ‘probable’ diagnosis) and 12/15 of the non-visualized cases (8 BAV and 4 TAV), so only 3/253 subjects could not be visualized by either modality. The aortic valve was bicuspid in 76 of the 250 adequately imaged subjects (30%). Peak aortic valve flow was higher in BAV subjects (1.72±0.07 vs. 1.90v0.03 m/sec, P=0.0002), with one case of significant aortic stenosis. Among subjects with a BAV, aortic regurgitation was moderate or greater in ∼15%. Aortic diameters at the annulus, sinuses of Valsalva, sinotubular junction and ascending aorta were all significantly greater in the BAV group. Thirty patients in the BAV group (12%) had aortic root diameters that were outside of the 95% normal confidence limits based on Roman nomograms. Ascending aortic diameters by echo and MRI were highly correlated (r=0.77). In summary, echocardiography supplemented with MRI reveals an extraordinarily high prevalence of abnormal aortic valves in asymptomatic subjects with TS. The abnormal valve structure is associated with higher peak flows, evidence of clinically significant valvular dysfunction, and widening of the ascending aorta in a significant number of patients. All girls and women with TS should have careful echocardiographic evaluation upon diagnosis to identify the one in three asymptomatic individuals with an abnormal valve requiring monitoring for aortic root dilatation and valvular dysfunction.


2020 ◽  
pp. 021849232092873
Author(s):  
Dimitrios C Iliopoulos ◽  
Dimitrios P Sokolis

Bicuspid aortic valve is the most common congenital cardiovascular defect, often associated with proximal aortic dilatation, and the ideal management strategy is debated. The inconsistency in previous and present guideline recommendations emphasizes the insufficiency of the maximal diameter as the sole criterion for prophylactic repair. Our ability to guide clinical decisions may improve through an understanding of the mechanical properties of ascending thoracic aortic aneurysms in bicuspid compared to tricuspid aortic valve patients and non-aneurysmal aortas, because dissection and rupture are aortic wall mechanical failures. Such an understanding of the mechanical properties has been attempted by several authors, and this article addresses whether there is a controversy in the accumulated knowledge. The available mechanical studies are briefly reviewed, discussing factors such as age, sex, and the region of mechanical examination that may be responsible for the lack of unanimity in the reported findings. The rationale for acquiring layer-specific properties is presented along with the main results from our recent study. No mechanical vulnerability of ascending thoracic aortic aneurysms was evidenced in bicuspid aortic valve patients, corroborating present conservative guidelines concerning the management of bicuspid aortopathy. Weakening and additional vulnerability was evidenced in aged patients and those with coexisting valve pathology, aortic root dilatation, hypertension, and hyperlipidemia. Discussion of these results from age- and sex-matched subjects, accounting for the region- and layer-specific aortic heterogeneity, in relation to intact wall results and histologic confirmation, helps to reconcile previous findings and affords a universal interpretation of ascending aorta mechanics in bicuspid aortopathy.


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 .


2017 ◽  
Vol 25 (3) ◽  
pp. 192-198
Author(s):  
Pablo Straneo ◽  
Gabriel Parma ◽  
Natalia Lluberas ◽  
Alvaro Marichal ◽  
Gerardo Soca ◽  
...  

Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m−2, p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = −0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.


2020 ◽  
Author(s):  
Tie Zheng ◽  
Shuai Zhu ◽  
Shijie Lu ◽  
Jiafu Ou ◽  
Jun-Ming Zhu

Abstract Background: The bicuspid aortic valve is one of the common congenital heart anomalies in adults. Although many studies have proved the coincidence between bicuspid aortic valve and the occurrence of ascending aortic dilation, seldom study has focused on the hemodynamic environments after the dilation already formed. Four numerical models of bicuspid aortic valve were constructed in this study, based on medical images, with different ascending aortic dilation levels. The diameters of ascending aortic are 3.5cm, 4.0cm, 4.5cm and 5.0cm, respectively; while, the size and the geometry of other parts are fixed. Then hemodynamics in these models was simulated numerically and the flow patterns and loading distributions were investigated. Aim of this study is to investigate the hemodynamic environment characteristics in the ascending aorta after dilation formed for the bicuspid aortic valve (BAV) patients. Results: Hemodynamics environments in the dilated ascending aorta were simulated, with different level of dilation. As the diameter increases, the blood flow becomes more disturbing. The wall shear stress at the ascending aortic decreases while oscillatory shear index increases with the increase of diameter. The pressure at ascending aortic increases as the diameter increases. Moreover, all these hemodynamic parameters described above are asymmetrically distributed with the increase of ascending aortic diameter and more parts of aorta would be affected with the increasing ascending aorta diametersConclusions: The study revealed that the ascending aortic dilation levels can significantly influence the magnificent and distribution of the dynamics. There are altered flow patterns, pressure difference, WSS and OSI distribution features in bicuspid aortic valve patients with valvular dilation. As the extent of aortic dilatation increases, more parts of aorta like aortic arch should be paid more attention to when an individual is referred for surgery


2021 ◽  
Vol 21 (1) ◽  
pp. 96-104
Author(s):  
Burak Acar ◽  
Cagrı Yayla ◽  
Murat Gul ◽  
Mustafa Karanfil ◽  
Sefa Unal ◽  
...  

Background: The importance of monocyte count-to-HDL-cholesterol ratio (MHR) in cardio- vascular diseases has been shown in various studies. Ascending aortic dilatation (AAD) is a common complication in the patients with bicuspid aortic valve. In this study, we aimed to investigate the relationship between MHR and the presence of aortic dilatation in the pa- tients with bicuspid aortic valve. Methods: The study population included totally 347 patients with bicuspid aortic valve.169 patients with aortic dilatation (ascending aorta diameter ≥ 4.0 cm) and 178 patients with no aortic dilatation. Echocardiographic and laboratory measure- ment was done and compared between groups. Results: The mean age of the participants was 44.7 ± 15.4 years and average ascending aorta diameter was 3.2 ± 0.3 cm in dilatation negative group and 4.4 ± 0.4 cm in positive group. MHR was significantly increased in in patients with aortic dilatation. MHR and uric acid level was independently associated with the presence of aortic dilatation in the patients with bicuspid aortic valve. Conclusion: We found a significant relationship between MHR and aortic dilatation in the patients with bicuspid aortic valve. Keywords: Bicuspid aorta; aorta aneurysm; monocyte HDL ratio; inflammation.


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