scholarly journals Applications of a Specialty Bicuspid Aortic Valve Program: Clinical Continuity and Translational Collaboration

2020 ◽  
Vol 9 (5) ◽  
pp. 1354
Author(s):  
Erin E. Crawford ◽  
Patrick M. McCarthy ◽  
S. Chris Malaisrie ◽  
Jyothy J. Puthumana ◽  
Joshua D. Robinson ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Biomarker Discovery ◽  
Elective Surgery ◽  
Prophylactic Surgery ◽  
Medical Patients ◽  
4D Flow ◽  
Clinical Registry ◽  
Tissue Samples ◽  
4D Flow Cmr

Bicuspid aortic valve (BAV) is a common congenital heart diagnosis and is associated with aortopathy. Current guidelines for aortic resection have been validated but are based on aortic diameter, which is insufficient to predict acute aortic events. Clinical and translational collaboration is necessary to identify biomarkers that can individualize the timing of prophylactic surgery for BAV aortopathy. We describe our multidisciplinary BAV program, including research protocols aimed at biomarker discovery and results from our longitudinal clinical registry. From 2012–2018, 887 patients enrolled in our clinical BAV registry with the option to undergo four dimensional flow cardiovascular magnetic resonance imaging (4D flow CMR) and donate serum plasma or tissue samples. Of 887 patients, 388 (44%) had an elective BAV-related procedure after initial presentation, while 499 (56%) continued with medical management. Of medical patients, 44 (9%) had elective surgery after 2.3 ± 1.4 years. Surgery patients’ biobank donations include 198 (46%) aorta, 374 (86%) aortic valve, and 314 (73%) plasma samples. The 4D flow CMR was completed for 215 (50%) surgery patients and 243 (49%) medical patients. Patients with BAV aortopathy can be safely followed by a multidisciplinary team to detect indications for surgery. Paired tissue and hemodynamic analysis holds opportunity for biomarker development in BAV aortopathy.

The length of the fusion between leaflets in bicuspid aortic valve is independently related to ascending aorta dilation and flow dynamics alterations assessed by 4D-flow CMR

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Guala ◽  
A Evangelista ◽  
L La Mura ◽  
G Teixido-Tura ◽  
L Dux-Santoy ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Statistical Significance ◽  
Ascending Aorta ◽  
Funding Source ◽  
Aortic Dilation ◽  
4D Flow ◽  
National Budget ◽  
4D Flow Cmr ◽  
Aortic Replacement

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<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).

Aortic flow patterns in bicuspid aortic valve disease visualised with 4D flow imaging

2012 ◽  
Vol 97 (Suppl 1) ◽  
pp. A129.3-A130
Author(s):  
MM Gedicke ◽  
A Pitcher ◽  
A Barker ◽  
J Bock ◽  
R Lorenz ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Flow Patterns ◽  
Aortic Flow ◽  
Valve Disease ◽  
Flow Imaging ◽  
4D Flow ◽  
4D Flow Imaging

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 ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Arch ◽  
Ascending Aorta ◽  
Aortic Dilation ◽  
4D Flow ◽  
4D Flow Cmr ◽  
Flow Displacement ◽  
Aortic Valve Leaflets ◽  
Partial Fusion ◽  
Cine Cmr

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

scholarly journals Bicuspid aortic valve patients show specific epigenetic tissue signature increasing extracellular matrix destruction

2019 ◽  
Vol 29 (6) ◽  
pp. 937-943 ◽  
Author(s):  
Josephina Haunschild ◽  
Isabel N Schellinger ◽  
Sarah J Barnard ◽  
Konstantin von Aspern ◽  
Piroze Davierwala ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Epigenetic Regulation ◽  
Tissue Inhibitor Of Metalloproteinase ◽  
Enzyme Linked Immunosorbent Assay ◽  
Aortic Tissue ◽  
Tissue Inhibitor ◽  
Tissue Samples ◽  
Increased Risk

Abstract OBJECTIVES Patients with a bicuspid aortic valve (BAV) have an increased risk for developing thoracic aortic aneurysm, which is characterized by the destruction of the elastic media of the aortic wall. Several important enzymes have been characterized to play key roles in extracellular matrix homeostasis, namely matrix metalloproteinases (MMPs). In this study, we investigated MMP-2 levels and their epigenetic regulation via the miR-29 family. METHODS Aortic tissue samples from 58 patients were collected during cardiac surgery, of which 30 presented with a BAV and 28 with a tricuspid aortic valve. Polymerase chain reaction, western blot analysis and immunohistochemistry were performed to analyse MMP-2. In addition, enzyme-linked immunosorbent assay measurements were carried out to investigate both MMP-2 and tissue inhibitor of metalloproteinase-2 levels. To examine the epigenetic regulation of aortic extracellular matrix homeostasis, we furthermore studied the expression levels of miR-29 via qRT-PCR. RESULTS Patients with a BAV were significantly younger at the time of surgery, presented significantly less frequently with arterial hypertension and displayed more often with an additional valvular disease. On a molecular level, we found that MMP-2 is increased on gene and protein level in BAV patients. Tissue inhibitor of metalloproteinase-2 levels do not differ between the groups. Interestingly, we also found that only miR-29A is significantly downregulated in BAVs. CONCLUSIONS Our findings highlight the importance of MMP-2 in the context of extracellular matrix destruction in BAV patients. We present new evidence that miR-29A is a crucial epigenetic regulator of these pathomechanistic processes and might hold promise for future translational research.

scholarly journals Hemodynamic Profiles Before and After Surgery in Bicuspid Aortic Valve Disease—A Systematic Review of the Literature

2021 ◽  
Vol 8 ◽  
Author(s):  
Daniel G. W. Cave ◽  
Hannah Panayiotou ◽  
Malenka M. Bissell
Keyword(s):  
Systematic Review ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Valve Disease ◽  
Flow Patterns ◽  
Valve Disease ◽  
Aortic Dilatation ◽  
4D Flow Mri ◽  
4D Flow ◽  
Flow Mri

Bicuspid aortic valve (BAV) disease presents a unique management challenge both pre- and post-operatively. 4D flow MRI offers multiple tools for the assessment of the thoracic aorta in aortic valve disease. In particular, its assessment of flow patterns and wall shear stress have led to new understandings around the mechanisms of aneurysm development in BAV disease. Novel parameters have now been developed that have the potential to predict pathological aortic dilatation and may help to risk stratify BAV patients in future. This systematic review analyses the current 4D flow MRI literature after aortic valve and/or ascending aortic replacement in bicuspid aortic valve disease. 4D flow MRI has also identified distinct challenges posed by this cohort at the time of valve replacement compared to standard management of tri-leaflet disorders, and may help tailor the type and timing of replacement. Eccentric pathological flow patterns seen after bioprosthetic valve implantation, but not with mechanical prostheses, might be an important future consideration in intervention planning. 4D flow MRI also has promising potential in supporting the development of artificial valve prostheses and aortic conduits with more physiological flow patterns.

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