Bicuspid Aortic Valve Disease Research Articles

Repaired coarctation of the aorta does not affect four-dimensional flow metrics in bicuspid aortic valve disease.

The objective of this study was primarily to compare 4D flow magnetic resonance imaging (MRI) metrics in the ascending aorta (AA) of patients with right-left fusion type bicuspid aortic valve (RL-BAV) and repaired CoA to RL-BAV without CoA. Metrics of patients with RL-BAV were also compared to the matched group of patients with common tricuspid aortic valve (TAV). 11 patients with RL-BAV and CoA, 11 patients with RL-BAV without CoA, and 22 controls with TAV, were investigated. Peak velocity (cm/s), peak flow (ml/s), and flow displacement (FD, %) were analyzed at five pre-defined AA levels. In addition, regional wall shear stress (WSS, mN/m2), circumferential WSS (WSSc), and axial WSS (WSSa) at all levels were quantified in six sectors of the aortic circle. Averaged WSS values on each level (WSSavg, WSSc, avg and WSSa, avg) were calculated as well. Peak velocity at the proximal tubular AA was significantly lower in BAV and CoA group (p = 0.047) compared to BAV without CoA. In addition, the WSSa, avg was found to be higher for the BAV and CoA group at proximal AA respectively (p = 0.040). No other significant differences were found between these groups.BAV group's peak velocity was higher at every level (p < 0.001-0.004) compared to TAV group. FD was significantly higher for the BAV group at every level (p < 0.001) besides at the most distal level. All averaged WSS values were significantly higher in BAV patients in distal AA (p < 0.001-0.018). Repaired CoA does not relevantly alter 4D flow metrics in the AA of patients with RL-BAV. However, RL-BAV majorly alters flow dynamics in the AA when compared to patients with TAV. https://www.clinicaltrials.gov/study/NCT05065996, Unique Protocol ID 5063566.

Left ventricular strain–volume loops in bicuspid aortic valve disease: new insights in cardiomechanics

Abstract Aims By combining temporal changes in left ventricular (LV) global longitudinal strain (GLS) with LV volume, LV strain–volume loops can assess cardiac function across the cardiac cycle. This study compared LV strain–volume loops between bicuspid aortic valve (BAV) patients and controls, and investigated the loop’s prognostic value for clinical events. Methods and results From a prospective cohort of congenital heart disease patients, BAV patients were selected and compared with healthy volunteers, who were matched for age and sex at group level. GLS analysis from apical views was used to construct strain–volume loops. Associations with clinical events, i.e. a composite of all-cause mortality, heart failure, arrhythmias, and aortic valve replacement, were assessed by Cox regression. A total of 113 BAV patients were included (median age 32 years, 40% female). BAV patients demonstrated lower Sslope (0.21%/mL, [Q1–Q3: 0.17–0.28] vs. 0.27%/mL [0.24–0.34], P &amp;lt; 0.001) and ESslope (0.19%/mL [0.12–0.25] vs. 0.29%/mL [0.21–0.43], P &amp;lt; 0.001) compared with controls, but also greater uncoupling during early (0.48 ± 1.29 vs. 0.05 ± 1.21, P = 0.04) and late diastole (0.66 ± 1.02 vs. −0.07 ± 1.07, P &amp;lt; 0.001). Median follow-up duration was 9.9 [9.3–10.4] years. Peak aortic jet velocity (HR 1.22, P = 0.03), enlarged left atrium (HR 3.16, P = 0.003), E/e′ ratio (HR 1.17, P = 0.002), GLS (HR 1.16, P = 0.008), and ESslope (HR 0.66, P = 0.04) were associated with the occurrence of clinical events. Conclusion Greater uncoupling and lower systolic and diastolic slopes were observed in BAV patients compared with healthy controls, suggesting presence of altered LV cardiomechanics. Moreover, lower ESslope was associated with clinical events, highlighting the strain–volume loop’s potential as prognostic marker.

Thoracic aortic microcalcification activity in combined positron emission tomography and magnetic resonance imaging.

Non-invasive detection of pathological changes in thoracic aortic disease remains an unmet clinical need particularly for patients with congenital heart disease. Positron emission tomography combined with magnetic resonance imaging (PET-MRI) could provide a valuable low-radiation method of aortic surveillance in high-risk groups. Quantification of aortic microcalcification activity using sodium [18F]fluoride holds promise in the assessment of thoracic aortopathies. We sought to evaluate aortic sodium [18F]fluoride uptake in PET-MRI using three methods of attenuation correction compared to positron emission tomography computed tomography (PET-CT) in patients with bicuspid aortic valve, METHODS: Thirty asymptomatic patients under surveillance for bicuspid aortic valve disease underwent sodium [18F]fluoride PET-CT and PET-MRI of the ascending thoracic aorta during a single visit. PET-MRI data were reconstructed using three iterations of attenuation correction (Dixon, radial gradient recalled echo with two [RadialVIBE-2] or four [RadialVIBE-4] tissue segmentation). Images were qualitatively and quantitatively analysed for aortic sodium [18F]fluoride uptake on PET-CT and PET-MRI. Aortic sodium [18F]fluoride uptake on PET-MRI was visually comparable with PET-CT using each reconstruction and total aortic standardised uptake values on PET-CT strongly correlated with each PET-MRI attenuation correction method (Dixon R = 0.70; RadialVIBE-2 R = 0.63; RadialVIBE-4 R = 0.64; p < 0.001 for all). Breathing related artefact between soft tissue and lung were detected using Dixon and RadialVIBE-4 but not RadialVIBE-2 reconstructions, with the presence of this artefact adjacent to the atria leading to variations in blood pool activity estimates. Consequently, quantitative agreements between radiotracer activity on PET-CT and PET-MRI were most consistent with RadialVIBE-2. Ascending aortic microcalcification analysis in PET-MRI is feasible with comparable findings to PET-CT. RadialVIBE-2 tissue attenuation correction correlates best with the reference standard of PET-CT and is less susceptible to artefact. There remain challenges in segmenting tissue types in PET-MRI reconstructions, and improved attenuation correction methods are required.

Specific Multiomic Profiling in Aortic Stenosis in Bicuspid Aortic Valve Disease.

Bicuspid aortic valve (BAV) disease is associated with faster aortic valve degeneration and a high incidence of aortic stenosis (AS). In this study, we aimed to identify differences in the pathophysiology of AS between BAV and tricuspid aortic valve (TAV) patients in a multiomics study integrating metabolomics and transcriptomics as well as clinical data. Eighteen patients underwent aortic valve replacement due to severe aortic stenosis: 8 of them had a TAV, while 10 of them had a BAV. RNA sequencing (RNA-seq) and proton nuclear magnetic resonance spectroscopy (1H-NMR) were performed on these tissue samples to obtain the RNA profile and lipid and low-molecular-weight metabolites. These results combined with clinical data were posteriorly compared, and a multiomic profile specific to AS in BAV disease was obtained. H-NMR results showed that BAV patients with AS had different metabolic profiles than TAV patients. RNA-seq also showed differential RNA expression between the groups. Functional analysis helped connect this RNA pattern to mitochondrial dysfunction. Integration of RNA-seq, 1H-NMR and clinical data helped create a multiomic profile that suggested that mitochondrial dysfunction and oxidative stress are key players in the pathophysiology of AS in BAV disease. The pathophysiology of AS in BAV disease differs from patients with a TAV and has a specific RNA and metabolic profile. This profile was associated with mitochondrial dysfunction and increased oxidative stress.

MiR-130a expression is related to aortic dilation in bicuspid aortic valve children.

Bicuspid aortic valve disease (BAV) is present in 0.5-2% of the population and can promote aortic dilation, eventually leading to fatal consequences. Although some biomarkers have been proposed in adults, no studies have tested these candidates in children. We aimed to evaluate four miRNAs previously described to be related to BAV disease and aortic dilation in adults in a paediatric cohort. Eighty participants ≤17 years old (4-17; mean 12) were included. From the BAV group, 40% had a dilated aorta (z score >2). RT‒qPCR were performed in plasma samples to quantify miR-122, miR-130a, miR-486, and miR-718 using the delta-delta Ct method. Functional and enrichment analyses of miR-130a were also performed. miR-130a expression in plasma was found to be significantly lower in BAV patients with a dilated aorta versus nondilated patients (p = 0.008) and healthy TAV controls (p = 0.004). Furthermore, miR-130a expression in plasma was inversely correlated with ascending aorta (r = 0.318, p = 0.004) and aortic root z scores (r = 0.322; p = 0.004). Enrichment analysis showed that miR-130a target genes are related to the TGFβ signalling pathway. miR-130a expression in plasma is decreased in aortic-dilated BAV children compared to nondilated BAV children, helping differentiate low- to high-risk patients. miR-130a expression in plasma is related to aortic dilation in bicuspid aortic valve (BAV) children. To our knowledge, this is the first study that analyses miRNA patterns in bicuspid aortic valve children with aortic dilation. miR-130a expression in plasma could be a biomarker in order to help differentiate low-to high-risk BAV children, which is vitally important for advanced care planning.

Filamin A heart valve disease as a genetic cause of inherited bicuspid and tricuspid aortic valve disease

ObjectiveVariants in the FLNA gene have been associated with mitral valve dystrophy (MVD), and even polyvalvular disease has been reported. This study aimed to analyse the aortic valve and root...

Long-term outcome of bicuspid aortic valve disease.

Bicuspid aortic valve (BAV) is a common congenital condition that is frequently associated with aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic aneurysms, but specific outcome data are scarce. The present study sought to assess outcomes in a large cohort of consecutive patients with BAV. A total of 581 consecutive patients (median age 29 years, 157 female) with BAV were included in the study and followed prospectively in a heart valve clinic follow-up programme. The overall survival rate after 10 years was 94.5%. During follow-up, 158 patients developed an indication for surgery. Event-free survival rates were 97%, 94%, 87%, and 73% at 1, 2, 5, and 10 years, respectively. In the multivariable analysis, event rates were independently predicted by AS [subdistribution hazard ratio (SHR) 2.3 per degree of severity], AR (SHR 1.5 per degree of severity), baseline aortic dilatation ≥ 40 mm (SHR 1.9), and age (SHR 1.3) (P < 0.001). BAV disease is associated with a high rate of cardiac events, but state-of-the-art care results in good survival with low rates of infective endocarditis, aortic dissection, and sudden death. Incremental degrees of AS and regurgitation, the presence of aortic dilatation, and age are predictive of cardiac events.

Cooperative Response to Endocardial Notch Reveals Interaction With Hippo Pathway.

The endocardium is a crucial signaling center for cardiac valve development and maturation. Genetic analysis has identified several human endocardial genes whose inactivation leads to bicuspid aortic valve formation and calcific aortic valve disease, but knowledge is very limited about the role played in valve development and disease by noncoding endocardial regulatory regions and upstream factors. We manipulated Notch signaling in mouse embryonic endocardial cells by short-term and long-term coculture with OP9 stromal cells expressing Notch ligands and inhibition of Notch activity. We examined the transcriptional profile and chromatin accessibility landscape for each condition, integrated transcriptomic, transcription factor occupancy, chromatin accessibility, and proteomic datasets. We generated in vitro and in vivo models with CRISPR-Cas9-edited deletions of various noncoding regulatory elements and validated their regulatory potential. We identified primary and secondary transcriptional responses to Notch ligands in the mouse embryonic endocardium, and a NOTCH-dependent transcriptional signature in valve development and disease. By defining the changes in the chromatin accessibility landscape and integrating with the landscape in developing mouse endocardium and adult human valves, we identify potential noncoding regulatory elements, validated selected candidates, propose interacting cofactors, and define the timeframe of their regulatory activity. Additionally, we found cooperative transcriptional repression with Hippo pathway by inhibiting nuclear Yap (Yes-associated protein) activity in the endocardium during cardiac valve development. Sequential Notch-dependent transcriptional regulation in the embryonic endocardium involves multiple factors. Notch activates certain noncoding elements through these factors and simultaneously suppresses elements that could hinder cardiac valve development and homeostasis. Biorxviv: https://www.biorxiv.org/content/10.1101/2023.03.23.533882v1.full.

Transcatheter Aortic Valve Implantation for Bicuspid Aortic Valve Disease: Procedural Planning and Clinical Outcomes.

Bicuspid aortic valve (BAV) is the most common congenital heart disease, with a prevalence of 1-2% and occurring in >20% of octogenarians referred for aortic valve replacement. However, BAV patients have been systematically excluded from pivotal randomized trials. Since TAVI indications are moving toward low-risk patients, an increase in the number of BAV patients who undergo TAVI is expected. BAV represents a challenge due to its unique morphological features (raphe, extreme asymmetrical valve calcifications, cusp asymmetry and aortopathy) and the lack of consensus about the accurate sizing method. The role of multi-slice computed tomography (MSCT) in the planification of the TAVI procedure is well-established, being useful to define the optimal valve sizing and the implantation strategy. New-generation devices, more experience of the operators and better planification of the procedure have been associated with similar clinical outcomes in bicuspid and tricuspid patients undergoing TAVI.

Male-female differences in the natural history of ascending thoracic aortic aneurysms: insights from the dissexion study

Abstract Background The natural history of ascending thoracic aortic aneurysms (ATAAs) remains poorly understood, including potential male-female differences. Purpose Therefore, this study sought to evaluate the natural history and outcomes of patients with ATAAs, with an emphasis on aortic growth and male-female differences. Methods Adult patients who received diagnostic and/or follow-up imaging of an ATAA (≥40mm) in two academic centers between 2007-2021 were included. Aortic growth was modeled non-linearly through mixed-effects models using repeated measurements, including age and sex through interaction terms. Joint models were constructed to adjust for the effect of nonrandom dropout due to surgical intervention. Secondary outcomes were male-female differences in presentation, aneurysm characteristics, time to intervention, acute aortic syndromes and survival relative to the age- and sex-matched general Dutch population. Cox models were employed to evaluate predictors of intervention and survival. Results In total, 1,858 patients were included (31.6% female). Median clinical follow-up was 5.9 [IQR: 3.0-10.0] years. Median age was 65.4 years [53.4-71.7y] for females and 59.0 years [IQR: 49.3-68.0y] for males (p&amp;lt;0.001). Males had more bicuspid aortic valve (BAV) disease (male: 27.5%, female: 18.2%; p&amp;lt;0.001) and were more likely to have had a previous dissection or aneurysm in a major artery other than the thoracic aorta (male: 8.9%, female: 4.9%; p=0.004) before diagnosis. Maximum absolute aortic diameter at diagnosis did not differ between males (46.5mm) and females (45.0mm; p=0.388). Unadjusted 10-year survival was 76% (68%-78%) for females (relative survival: 68.8%) and 78% (75%-81%) for males (relative survival: 78.7%). For females, tubular ascending aortic growth was faster compared to males (p=0.005), particularly in the first 6 years after diagnosis. Contrarily, SoV growth was faster in males compared to females (p=0.002). Surgical intervention occurred more quickly after diagnosis in females (HR: 2.5 (1.2-5.2), corrected for BAV, diameter and age. Twenty-three type A dissections (TAD) occurred with an incidence rate ratio between females and males of 3.4 (95%CI:1.5-8.0, p=0.004). The last known aortic diameter before TAD was comparable between males (47.5mm [47.0-50.8]) and females (47.0mm [43.0-51.0]; p=0.416). Conclusions Ascending aortic aneurysms show a strikingly different growth pattern between males and females at the level of the SoV and tubular ascending aorta, suggesting that although females present at an older age, their aortic disease progresses faster and with more TAD compared to males, and warranting an even more careful follow-up. Absolute known aortic diameter before TAD was comparable between males and females. These insights can be used to fuel discussion about male-female specific follow-up and treatment recommendations in future clinical guidelines development.Ascending aortic growth (A) &amp; sinus (B)Freedom from intervention from diagnosis

Physical activity and sedentary behavior patterns in individuals with a bicuspid aortic valve compared to healthy controls

Abstract Background A bicuspid aortic valve (BAV) is the most common congenital heart disease and associated with a higher lifetime risk of cardiovascular morbidity and mortality. Management of cardiovascular risk factors, including habitual physical (in)activity, is therefore key. However, little is known about physical activity (PA) and sedentary behavior (SB) patterns in individuals with BAV compared to healthy controls while there is debate how PA may influence BAV related cardiovascular complications. Purpose To compare objectively measured PA and SB characteristics between individuals with BAV and healthy age- and sex-matched controls. Methods Adults with BAV from our outpatient clinic were approached to participate and matched with age and sex- matched controls from the general population. SB, moderate-to-vigorous PA (MVPA) and step count were objectively assessed for 24 h/day during 8 consecutive days using an accelerometer (activPAL3 micro®) attached to the subject’s thigh. Transthoracic echocardiograms of the BAV participants were used to determine the presence of valvular dysfunction and to measure aortic diameters. Aortic dilatation was defined as &amp;gt;40 mm at the sinus of Valsalva or in the ascending aorta, whereas valvular dysfunction was defined as moderate-to-severe aortic stenosis or regurgitation according to current ESC guidelines. Results We recruited 100 BAV individuals (45±16 years old; 59% male), of which n=12 had valve dysfunction, n=22 had aortic dilatation and n=20 had a combination of valve dysfunction and aortic dilatation. We also included 100 healthy age- and sex-matched controls. SB was comparable between the BAV and control group (9.3 [8.5-10.2] vs 9.3 [8.2-10.2] hrs/day, p=0.84), whereas time spent in MVPA (72 [59-89] vs 98 [75-116] min/day, p &amp;lt;0.001) and daily step count (4,826 [4,004-5,801] vs 6,252 [4,784-7,484] steps/day, p&amp;lt;0.001) were markedly lower in individuals with BAV (Figure 1). Furthermore, no significant differences in SB, MVPA or step-count were found across BAV disease severity subgroups (Figure 2). Conclusion Individuals with BAV are less physically active compared to age- and sex-matched healthy controls. These differences were independent of disease severity, indicating that the lower activity patterns were not due to functional or disease-related limitations.Figure 1.Figure 2.

Capturing heart valve development with Gene Ontology.

Introduction: The normal development of all heart valves requires highly coordinated signaling pathways and downstream mediators. While genomic variants can be responsible for congenital valve disease, environmental factors can also play a role. Later in life valve calcification is a leading cause of aortic valve stenosis, a progressive disease that may lead to heart failure. Current research into the causes of both congenital valve diseases and valve calcification is using a variety of high-throughput methodologies, including transcriptomics, proteomics and genomics. High quality genetic data from biological knowledge bases are essential to facilitate analyses and interpretation of these high-throughput datasets. The Gene Ontology (GO, http://geneontology.org/) is a major bioinformatics resource used to interpret these datasets, as it provides structured, computable knowledge describing the role of gene products across all organisms. The UCL Functional Gene Annotation team focuses on GO annotation of human gene products. Having identified that the GO annotations included in transcriptomic, proteomic and genomic data did not provide sufficient descriptive information about heart valve development, we initiated a focused project to address this issue. Methods: This project prioritized 138 proteins for GO annotation, which led to the curation of 100 peer-reviewed articles and the creation of 400 heart valve development-relevant GO annotations. Results: While the focus of this project was heart valve development, around 600 of the 1000 annotations created described the broader cellular role of these proteins, including those describing aortic valve morphogenesis, BMP signaling and endocardial cushion development. Our functional enrichment analysis of the 28 proteins known to have a role in bicuspid aortic valve disease confirmed that this annotation project has led to an improved interpretation of a heart valve genetic dataset. Discussion: To address the needs of the heart valve research community this project has provided GO annotations to describe the specific roles of key proteins involved in heart valve development. The breadth of GO annotations created by this project will benefit many of those seeking to interpret a wide range of cardiovascular genomic, transcriptomic, proteomic and metabolomic datasets.

A Case of Bicuspid Aorta with Stanford Type a Aortic Dissection

Bicuspid aortic valve disease (BAV) is a congenital disease that frequently produces complications during adulthood, and is considered not only a problem of valvulogenesis, but also a more complex genetic alteration that involves the development of the heart and the aorta, which entails a high morbidity and mortality in these patients. In 50% of adults with this pathology, non-valvular anomalies are observed, with dilation of the ascending aorta being the most common. Genes such as NOTCH1, UFDL1, ACTA2, eNOS, among others, have been identified as responsible for the appearance of aneurysms, which increases the risk in these patients of complications such as aortic dissection and its subsequent rupture. Painless aortic dissection may be seen in less than 5% of patients and may have an atypical clinical presentation. A case and review of the literature is presented in which we can observe the great complexity of this pathology, in the case of a patient with BAV, annulo-ectatic dilation of the aortic root and ascending aorta who developed heart failure, syncope and presented as a complication Stanford type A aortic dissection, requiring highly specialized surgical treatment.

Navigating the challenges of bicuspid aortic valve-aortopathy.

Bicuspid aortic valve (BAV) is a congenital heart defect that affects 0.5-2% of the general population with familial predominance. The modifications in hemodynamics and structure change at cellular level contribute to the dilation of aorta, resulting in bicuspid aortopathy, which can result in catastrophic aortic events. The American Heart Association recommends screening first-degree relatives of patients with bicuspid aortic valve and aortic root disease. BAV may or may not be associated with a syndrome, with the non-syndromic variety having a higher chance of predisposition to congenital and vascular abnormalities. Many genes have been implicated in the etiology of non-syndromic aortic aneurysm such as ACTA2, MYH11, FLNA, and SMAD3. Common diagnostic modalities include transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), multi system computer tomography (MSCT), and cardiac MRI. Medical management reduces the rate of disease progression and surgical management is indicated based on the diameter of the ascending aorta, which differs in American and European guidelines. Our article aims to explore the current understanding of the pathophysiology, clinical aspects, and surgical management of bicuspid aortic valve disease. Additionally, we have included a discussion on the management of this condition in special populations, such as athletes and pregnant women, who require distinct treatment recommendations.

Aortic Valve Dysfunction and Aortopathy Based on the Presence of Raphe in Patients with Bicuspid Aortic Valve Disease.

(1) Background: To identify the association between the presence or absence of a raphe and aortic valve dysfunction, as well as the presence of aortopathy in patients with a bicuspid aortic valve (BAV); (2) Methods: This retrospective study enrolled 312 participants (mean (SD) age, 52.7 (14.3) years; 227 men (72.8%)) with BAV. The BAVs were divided into those with the presence (raphe+) or absence (raphe-) of a raphe. Valvular function was classified as normal, aortic regurgitation (AR), or aortic stenosis (AS) using TTE. The pattern of BAV aortopathy was determined by the presence of dilatation at the sinus of Valsalva and the middle ascending aorta using CCT; (3) Results: BAVs with raphe+ had a higher prevalence of AR (148 (79.5%) vs. 48 (37.8%), p < 0.001), but a lower prevalence of AS (90 (48.6%) vs. 99 (78.0%), p < 0.001) compared with those with raphe-. The types of BAV aortopathy were significantly different (p = 0.021) according to those with BAV-raphe+ and BAV-raphe-; (4) Conclusions: The presence of a raphe was significantly associated with a higher prevalence of AR, but a lower prevalence of AS and combined dilatation of the aortic root and middle ascending aorta. The presence of a raphe was an independent determinant of AR.

Mid-Term Results of Aortic Valve Sparing Root Surgery Operations

Introduction: In this study, we retrospectively analyzed the mid-term outcomes of patients who underwent aortic valve-sparing root replacement (VSARR). Patients and Methods: Ninety-six patients operated on due to annuloaortic ectasia in our clinic between 2012 and 2016 were examined. Twenty-four patients who underwent VSARR were included in the study. They were evaluated using echocardiography and computed tomography in terms of mortality and reoperation according to their preoperative demographic characteristics, aortic regurgitation (AR), and annular dilatation levels. Results: The early mortality rate was 12.5% (n= 3) and was associated with emergency operation and total arch replacement. The mean follow-up was 33.00 ± 9.53 months. The early mortality rate was 12.5% (n= 3) and was associated with emergency surgery and total arch replacement (p= 0.035, p&lt; 0.05). The mean followup was 33.00 ± 9.53 months. None of the patients required reoperation. While postoperative AR was seen in one patient with Takayasu’s arteritis (n= 1, 4.16%), it was not observed in the patients with Marfan syndrome or bicuspid aortic valve disease. Postoperative AR and preoperative AR were related (p= 0.012, p&lt; 0.05), but preoperative annulus diameter was not (p= 0.296, p&gt; 0.05). There was no difference in valve durability between the use of Dacron and Valsalva grafts (p= 0.724, p&gt; 0.05). Conclusion: For patients with aortic root aneurysms, elective VSARR is a good surgical option. However, the presence of comorbidities is related to high mortality because it necessitates urgent and complicated surgery for patients with aortic dissection.

Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm

Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm

Clinical outcomes after aortic valve replacement with severe stenosis of trileaflet aortic valve and low valve calcium score

BackgroundSome patients have severe aortic valve stenosis (AS) despite a lower degree of aortic valve calcification (AVC). This study compared the clinical features and prognosis of patients undergoing aortic valve...

Early and Mid-Term Outcomes After Surgery for Bicuspid and Tricuspid Aortic Valve Disease: The Tasmanian Experience

This study aimed to compare the early and mid-term outcomes of patients with bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) disease who underwent aortic valve replacement (AVR) and/or aortic surgery at a tertiary, regional cardiothoracic unit.

The role of transforming growth factor beta in bicuspid aortic valve aortopathy

A bicuspid aortic valve (BAV) is the most prevalent congenital cardiac deformity, which is associated with an increased risk to develop a thoracic aortic aneurysm and/or an aortic dissection as compared to persons with a tricuspid aortic valve. Due to the high prevalence of a BAV in the general population and the associated life-long increased risk for adverse vascular events, BAV disease places a considerable burden on the public health. The aim of the present review is to discuss the role of transforming growth factor beta (TGF-β) signaling in the development of the vascular wall and on how this complex signaling pathway may be involved in thoracic aortic aneurysm formation in tricuspid and BAV patients.