scholarly journals Aortic stenosis exacerbates flow aberrations related to the bicuspid aortic valve fusion pattern and the aortopathy phenotype

2018 ◽  
Vol 55 (3) ◽  
pp. 534-542 ◽  
Author(s):  
Yan Shan ◽  
Jun Li ◽  
Yongshi Wang ◽  
Boting Wu ◽  
Alex J Barker ◽  
...  

Abstract OBJECTIVES A bicuspid aortic valve (BAV) is characterized by variable phenotypic manifestations, as well as longitudinal evolution of valve dysfunction and ascending aorta dilatation. The present study investigated the impact of severe aortic stenosis (AS) on the flow patterns and wall shear stress (WSS) distribution in BAV patients with right–left (RL) and right-non-coronary (RN) cusp fusion types, and the study aimed to reveal whether aortic dysfunction could further alter intrinsic aortic haemodynamic aberrations generated by abnormal BAV cusp fusion patterns. METHODS Four-dimensional flow magnetic resonance imaging was performed in 120 BAV subjects and 20 tricuspid aortic valve controls. BAV patients were evenly categorized into 4 cohorts, including RL and RN BAV with no more than mild aortic dysfunction as well as RL and RN BAV-AS with isolated severe AS. RESULTS BAV subjects exhibited eccentric outflow jets resulting in regional WSS elevation at the right-anterior position of the ascending aorta in the RL group and the right-posterior location in the RN group (P < 0.005). The presence of severe AS resulted in accelerated outflow jets and more prominent flow and WSS eccentricity (P < 0.005) by marked helical (P = 0.014) and vortical flow formation (P < 0.005), as well as increased prevalence of tubular and transverse arch dilatation. The changes to the flow jet in BAV-AS subjects blurred the differences in peak flow velocity and WSS distribution between RL and RN BAV. Differences in the phenotypes of aortopathy were associated with changes in functional haemodynamic parameters such as flow displacement and WSS eccentricity. CONCLUSIONS Severe AS markedly exacerbated aortic flow aberrations in BAV patients and masked the existing distinct flow features deriving from RL and RN fusion types. Longitudinal studies are needed to investigate the evolution of ascending aortic dilatation relative to the interaction between intrinsic cusp fusion types and acquired severe valve dysfunction.

Heart ◽  
2017 ◽  
Vol 104 (7) ◽  
pp. 566-573 ◽  
Author(s):  
Arturo Evangelista ◽  
Pastora Gallego ◽  
Francisco Calvo-Iglesias ◽  
Javier Bermejo ◽  
Juan Robledo-Carmona ◽  
...  

ObjectiveBicuspid aortic valve (BAV) is associated with early valvular dysfunction and proximal aorta dilation with high heterogeneity. This study aimed to assess the determinants of these complications.MethodsEight hundred and fifty-two consecutive adults diagnosed of BAV referred from cardiac outpatient clinics to eight echocardiographic laboratories of tertiary hospitals were prospectively recruited. Exclusion criteria were aortic coarctation, other congenital disorders or intervention. BAV morphotype, significant valve dysfunction and aorta dilation (≥2 Z-score) at sinuses and ascending aorta were established.ResultsThree BAV morphotypes were identified: right–left coronary cusp fusion (RL) in 72.9%, right–non-coronary (RN) in 24.1% and left–non-coronary (LN) in 3.0%. BAV without raphe was observed in 18.3%. Multivariate analysis showed aortic regurgitation (23%) to be related to male sex (OR: 2.80, p<0.0001) and valve prolapse (OR: 5.16, p<0.0001), and aortic stenosis (22%) to BAV-RN (OR: 2.09, p<0.001), the presence of raphe (OR: 2.75, p<0.001), age (OR: 1.03; p<0.001), dyslipidaemia (OR: 1.77, p<0.01) and smoking (OR: 1.63, p<0.05). Ascending aorta was dilated in 76% without differences among morphotypes and associated with significant valvular dysfunction. By contrast, aortic root was dilated in 34% and related to male sex and aortic regurgitation but was less frequent in aortic stenosis and BAV-RN.ConclusionsNormofunctional valves are more prevalent in BAV without raphe. Aortic stenosis is more frequent in BAV-RN and associated with some cardiovascular risk factors, whereas aortic regurgitation (AR) is associated with male sex and sigmoid prolapse. Although ascending aorta is the most commonly dilated segment, aortic root dilation is present in one-third of patients and associated with AR. Remarkably, BAV-RL increases the risk for dilation of the proximal aorta, whereas BAV-RN spares this area.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Kaoru Hattori ◽  
Natsuki Nakama ◽  
Jumpei Takada ◽  
Gohki Nishimura ◽  
Ryo Moriwaki ◽  
...  

AbstractThe characteristics of aortic valvular outflow jet affect aortopathy in the bicuspid aortic valve (BAV). This study aimed to elucidate the effects of BAV morphology on the aortic valvular outflow jets. Morphotype-specific valve-devising apparatuses were developed to create aortic valve models. A magnetic resonance imaging-compatible pulsatile flow circulation system was developed to quantify the outflow jet. The eccentricity and circulation values of the peak systolic jet were compared among tricuspid aortic valve (TAV), three asymmetric BAVs, and two symmetric BAVs. The results showed mean aortic flow and leakage did not differ among the five BAVs (six samples, each). Asymmetric BAVs demonstrated the eccentric outflow jets directed to the aortic wall facing the smaller leaflets. In the asymmetric BAV with the smaller leaflet facing the right-anterior, left-posterior, and left-anterior quadrants of the aorta, the outflow jets exclusively impinged on the outer curvature of the ascending aorta, proximal arch, and the supra-valvular aortic wall, respectively. Symmetric BAVs demonstrated mildly eccentric outflow jets that did not impinge on the aortic wall. The circulation values at peak systole increased in asymmetric BAVs. The bicuspid symmetry and the position of smaller leaflet were determinant factors of the characteristics of aortic valvular outflow jet.


Author(s):  
Patrizia Carità ◽  
Massimo Verdecchia ◽  
Elisabetta Mancini ◽  
Giovanni Ferro ◽  
Marco Guglielmo ◽  
...  

<p align="JUSTIFY"><span style="color: #000000;"><span style="font-family: 'Times New Roman', serif;"><span style="font-size: medium;"><span lang="en-GB">The bicuspid aortic valve</span></span></span></span><span style="font-family: 'Times New Roman', serif;"><span style="font-size: medium;"><span lang="en-GB"> is one of the most common forms of congenital heart defect. It is a complex entity, in which</span></span></span><span style="color: #000000;"><span style="font-family: 'Times New Roman', serif;"><span style="font-size: medium;"><span lang="en-GB"> involvement of the thoracic aorta forms part of the disease spectrum</span></span></span></span><span style="font-family: 'Times New Roman', serif;"><span style="font-size: medium;"><span lang="en-GB">.</span></span></span><span style="color: #000000;"><span style="font-family: 'Times New Roman', serif;"><span style="font-size: medium;"><span lang="en-GB"> The ascending aorta is the most common site involved in this process.</span></span></span></span><span style="color: #000000;"><span style="font-family: 'Times New Roman', serif;"><span style="font-size: medium;"><span lang="en-GB"> We report the extremely rare case of a patient with true BAV associated with a diffuse aortopathy, involving the aneurysm of the non coronary sinus, that suddenly rupted into the right atrium and required urgent cardiac surgery, and the aneurismal dilation of a (non aberrant) left subclavian artery, resembling a Kommerell’s diverticulum. </span></span></span></span></p>


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 .


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.


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