P3373Predictors of dilatation of ascending aorta in patients with bicuspid aortic valve, a longitudinal multicenter study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I Dentamaro ◽  
A Sao-Aviles ◽  
G Teixido ◽  
L Galian ◽  
L Gutierrez ◽  
...  
Keyword(s):  
Risk Factors ◽  
Aortic Valve ◽  
Aortic Stenosis ◽  
Bicuspid Aortic Valve ◽  
Multicenter Study ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Annual Growth ◽  
Aortic Dilation ◽  
Male Sex

Abstract Introduction The bicuspid aortic valve (BAV) is frequently associated to dilation of the ascending aorta. Some cross-sectional studies have related the aortic dilation with morphotype and valvular dysfunction. The aim of this longitudinal multicenter study was to analyze the progression of the aortic dilation and to identify its predictors. Methods We included 459 patients (mean age 52±17; 325 men 70.8%) with BAV, without aortic coarctation. The BAV morphotype, significant valvular dysfunction and dilation of the aortic root and ascending aorta were established by echocardiography. The patients were followed annually, with an average of 7.5±3.2 years. Results 77% of the patients had BAV with a fusion between left and right cusps, 21% between right and non coronary cusps and 2% between left and non coronary cusps, with a raphe in 77% of these patients. Risk factors included: 35% hypertension, 20% smoking, 5% diabetes and 18% dyslipidemia. The baseline study showed a maximum root diameter of 36±6.2 mm and ascending aorta of 39±8.1 mm. In 7% the aortic root was>45 mm, while in 32% the ascending aorta>45 mm. There was no valvular dysfunction in 17% of patients, while the 8% had significant aortic stenosis and 35% significant aortic regurgitation. The annual growth of the aortic root was 0.33±0.2 mm and for the ascending aorta was 0.38±0.3 mm. At the end of follow-up, 16% of the patients had a root>45 mm and 41% an ascending aorta>45 mm. The annual progression of aortic diameters was not related to valvular morphotype, valvular dysfunction or cardiovascular risk factors. The univariate analysis showed a significant relationship between the annual growth of the aortic root and arterial hypertension (p=0.028) and the annual growth of the ascending aorta with the male sex (p=0.019), smoking (p=0.046) and significant (moderate or severe) aortic stenosis (p=0.013). Diabetes mellitus and the presence of raphe were found to be slightly protective (p=0.049 and p=0.031, respectively). In the multivariate analysis, only the male sex and significant aortic stenosis were independent predictors of dilation of the ascending aorta. Conclusions In patients with bicuspid aortic valve, the progression of the dilation of the aortic root is related to hypertension and the growth of the ascending aorta with the male sex and the presence of significant aortic stenosis. Both bicuspid valve morphotype, basal aortic diameter or age were not related to the progression of aortic dilation.

scholarly journals Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease

Heart ◽  
2017 ◽  
Vol 104 (7) ◽  
pp. 566-573 ◽  
Author(s):  
Arturo Evangelista ◽  
Pastora Gallego ◽  
Francisco Calvo-Iglesias ◽  
Javier Bermejo ◽  
Juan Robledo-Carmona ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Clinical Predictors ◽  
Valve Dysfunction ◽  
Tertiary Hospitals ◽  
Male Sex

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.

scholarly journals Predictors of Ascending Aorta Enlargement and Valvular Dysfunction Progression in Patients with Bicuspid Aortic Valve

2021 ◽  
Vol 10 (22) ◽  
pp. 5264
Author(s):  
Angela Lopez ◽  
Ilaria Dentamaro ◽  
Laura Galian ◽  
Francisco Calvo ◽  
Josep M. Alegret ◽  
...  
Keyword(s):  
Risk Factors ◽  
Growth Rate ◽  
Cardiovascular Risk ◽  
Aortic Valve ◽  
Cardiovascular Risk Factors ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Ascending Aorta ◽  
Valve Dysfunction

Bicuspid aortic valve (BAV) patients are at high risk of developing progressive aortic valve dysfunction and ascending aorta dilation. However, the progression of the disease is not well defined. We aimed to assess mid-long-term aorta dilation and valve dysfunction progression and their predictors. Patients were referred from cardiac outpatient clinics to the echocardiographic laboratories of 10 tertiary hospitals and followed clinically and by echocardiography for >5 years. Seven hundred and eighteen patients with BAV (median age 47.8 years [IQR 33–62], 69.2% male) were recruited. BAV without raphe was observed in 11.3%. After a median follow-up of 7.2 years [IQR5–8], mean aortic root growth rate was 0.23 ± 0.15 mm/year. On multivariate analysis, rapid aortic root dilation (>0.35 mm/year) was associated with male sex, hypertension, presence of raphe and aortic regurgitation. Annual ascending aorta growth rate was 0.43 ± 0.32 mm/year. Rapid ascending aorta dilation was related only to hypertension. Variables associated with aortic stenosis and regurgitation progression, adjusted by follow-up time, were presence of raphe, hypertension and dyslipidemia and basal valvular dysfunction, respectively. Intrinsic BAV characteristics and cardiovascular risk factors were associated with aorta dilation and valvular dysfunction progression, taking into account the inherent limitations of our study-design. Strict and early control of cardiovascular risk factors is mandatory in BAV patients.

scholarly journals Is raphe only an innocent bystander finding or a predictor of complications in bicuspid aortic valve patients? Experience of Italian REBECCA registry

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Bellino ◽  
R Citro ◽  
S La Carrubba ◽  
I Fabiani ◽  
P Faggiano ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Wide Spectrum ◽  
Smoking Habit ◽  
Ascending Aorta ◽  
Innocent Bystander ◽  
Type B ◽  
Type 3

Abstract Background Bicuspid aortic valve (BAV) is the most common congenital heart disease, affecting 0.5%–2% of the general population. It is associated with valvular dysfunction (aortic stenosis and/or regurgitation, endocarditis) but also with a wide spectrum of aortopathy with unpredictable clinical presentations. The role of the raphe is still controversial. Methods The REgistro della Valvola Aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging is a retrospective/prospective, multicenter, observational registry, with definitive diagnosis of BAV. Anamnestic, demographic, clinical, and instrumental data are collected into dedicated software at first evaluation and during follow-up. Aortopathy was defined as: annulus ≥14 mm/m2; root ≥20 mm/m2; sino-tubular junction (STJ) ≥16 mm/m2; ascending aorta (AA) ≥17 mm/m2; it was classified in: type A, dilation of the ascending aorta; type B, dilation of the aortic root and the ascending aorta; and type C, isolated dilation of the aortic root. Patients were divided in two groups; those with raphe and those without. Results At December 2019, 800 patients with BAV (Male, 73.3%; M:F ratio 2.9; Mean Age at diagnosis 44±23 years) have been included in the registry. Prevalence of hypertension was 29%, diabetes mellitus 3%, smoking-habit 8%. We reported a majority (42.7%, 342) of patients with type 1, followed by type 2 (10.2%, 82) and type 3 (3.7%, 30). No gender differences were observed according to BAV phenotypes, while male gender was associated to higher prevalence of aortic valve regurgitation (p=0.0003). Moreover, in patients with raphe, aortic stenosis (49.8% vs 38.4%; p=0.014) and aortopathy (57.4% vs 46.1%; p=0.034) were significantly prevalent (see Table 1). Of note no difference about aortic regurgitation (62.8% vs 48.7%; p=0.064) between two groups were detected. At univariable logistic regression analysis the presence of raphe was significantly associated with aortopathy [OR: 1.57; 95% CI: 1.02–1.42; p=0.037] especially with Type B Aortopathy [OR: 0.55: 95% CI: 0.33–0.93: p=0.02]. Conclusion Preliminary data from Italian Multicenter REBECCA registry highlight that, in patients with BAV, the raphe is not an innocent bystander but a risk factor for aortic stenosis and type B aortopathy. Funding Acknowledgement Type of funding source: None

scholarly journals Evaluation of relationship between bicuspid aortic valve phenotype with valve dysfunction and associated aortopathy

2018 ◽  
Vol 10 (4) ◽  
pp. 236-242
Author(s):  
Mehrnoush Toufan Tabrizi ◽  
Roghaieh Rahimi Asl ◽  
Soheyla Nazarnia ◽  
Leili Pourafkari
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Risk Stratification ◽  
Bicuspid Aortic Valve ◽  
Aortic Insufficiency ◽  
P Value ◽  
Aortic Dilation ◽  
Valve Dysfunction ◽  
Northwest Of Iran ◽  
Male Sex

Introduction: Morphology of bicuspid aortic valve (BAV) may have implication in the associated pathologies including aortic stenosis (AS), aortic insufficiency (AI) and aortic dilation. The aim of this study is to investigate the frequency and patterns of valvular dysfunction and aortopathy associated with different phenotypes of BAV in a referral center in northwest of Iran. Methods: In this prospective study patients who presented to our echocardiography lab between January 2014 and December 2015 and were diagnosed with BAV were assessed. Frequency of various BAV phenotypes and their association with valvular dysfunction and aortopathy was evaluated. A P value less than 0.05 was considered statistically significant. Results: The average age of the study patients was 40±16 years, with predominance of male sex (72%). Patients with anteroposteriorly located BAV (BAV-AP) phenotype constituted majority of our cases with prevalence of 62.7%, while 37.3% of cases had right-left (BAV-RL) located valves. BAV-RL patients when compared to BAV-AP patients had higher frequencies of dilated aortic arch (25% vs. 4.3%, P < 0.001), AS (56.3% vs. 31.4%, P < 0.001), mass or vegetation on aortic valve (14.3 vs. 6.4%, P = 0.023) and lower frequencies of dilated aortic root (42.9% vs. 57.4%, P = 0.01), aortic insufficiency (68.8% vs. 79.8%, P = 0.034) and co-arctation of aorta (3.6% vs. 11.7%, P = 0.01). Conclusion: There seems to be a relationship between various BAV phenotypes, and frequency and pattern of aortic valve dysfunction and aortopathy. These findings suggest that examining leaflet morphology in BAV might help in risk stratification of these patients.

scholarly journals The Role of ECG-Gated CT in Patients with Bicuspid Aortic Valve Replacement: New Perspectives in Short- and Long-Term Followup

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Massimiliano Sperandio ◽  
Chiara Arganini ◽  
Alessio Bindi ◽  
Armando Fusco ◽  
Carlo Olevano ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Root Diameter ◽  
Ascending Aorta ◽  
Short And Long Term ◽  
The Stability

The aim of our study was to compare the results of the TTE (transthoracic echocardiography) with the results obtained by the ECG-gated 64 slices CT during the followup of patients with bicuspid aortic valve (BAV), after aortic valve replacement; in particular we evaluated the aortic root and the ascending aorta looking for a new algorithm in the followup of these patients. From January 1999 to December 2009 our attention was focused on 67 patients with isolated surgical substitution of aortic valve; after dismissal they were strictly observed. During the period between May and September 2010, these patients underwent their last evaluation, and clinical exams, ECG, TTE, and an ECG-gated-MDCT were performed. At followup TTE results showed an aortic root of 36.7±4 mm and an ascending aorta of 39.6±4.8 mm. ECG- gated CT showed an aortic root of 37.9±5.5 mm and an ascending aorta of 43.1±5.2. The comparison between preoperative and postoperative TTE shows a significant long-term dilatation of the ascending aorta while the aortic root diameter seems to be stable. ECG-gated CT confirms the stability of the aortic root diameter (38.2±5.3 mm versus 37.9±5.5  mm; <0.0001) and the increasing diameter value of the ascending aorta (40.2±3.9 mm versus 43.1±5.2 mm; P=0.0156). Due to the different findings between CT and TTE studies, ECG-gated CT should no longer be considered as a complementary exam in the followup of patients with BAV, but as a fundamental role since it is a real necessity.

Progressive dilation of the diameter of the aortic root in adults with a bicuspid aortic valve

2003 ◽  
Vol 13 (6) ◽  
pp. 526-531 ◽  
Author(s):  
Annie Dore ◽  
Marie-Claude Brochu ◽  
Jean-François Baril ◽  
Marie-Claude Guertin ◽  
Lise-Andrée Mercier
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Left Ventricular Outflow Tract ◽  
Normal Population ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Ascending Aorta ◽  
Rate Of Progression ◽  
Left Ventricular Outflow

Background: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve. Methods: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction. Results: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation. Conclusion: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.

scholarly journals Disturbed nitric oxide signalling gives rise to congenital bicuspid aortic valve and aortopathy

2020 ◽  
Vol 13 (9) ◽  
pp. dmm044990
Author(s):  
Joshua C. Peterson ◽  
Lambertus J. Wisse ◽  
Valerie Wirokromo ◽  
Tessa van Herwaarden ◽  
Anke M. Smits ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Neural Crest Cell ◽  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Aortic Dilation ◽  
Elastic Fibres ◽  
Increased Risk

ABSTRACTPatients with a congenital bicuspid aortic valve (BAV), a valve with two instead of three aortic leaflets, have an increased risk of developing thoracic aneurysms and aortic dissection. The mechanisms underlying BAV-associated aortopathy are poorly understood. This study examined BAV-associated aortopathy in Nos3−/− mice, a model with congenital BAV formation. A combination of histological examination and in vivo ultrasound imaging was used to investigate aortic dilation and dissections in Nos3−/− mice. Moreover, cell lineage analysis and single-cell RNA sequencing were used to observe the molecular anomalies within vascular smooth muscle cells (VSMCs) of Nos3−/− mice. Spontaneous aortic dissections were found in ascending aortas located at the sinotubular junction in ∼13% of Nos3−/− mice. Moreover, Nos3−/− mice were prone to developing aortic dilations in the proximal and distal ascending aorta during early adulthood. Lower volumes of elastic fibres were found within vessel walls of the ascending aortas of Nos3−/− mice, as well as incomplete coverage of the aortic inner media by neural crest cell (NCC)-derived VSMCs. VSMCs of Nos3−/− mice showed downregulation of 15 genes, of which seven were associated with aortic aneurysms and dissections in the human population. Elastin mRNA was most markedly downregulated, followed by fibulin-5 expression, both primary components of elastic fibres. This study demonstrates that, in addition to congenital BAV formation, disrupted endothelial-mediated nitric oxide (NO) signalling in Nos3−/− mice also causes aortic dilation and dissection, as a consequence of inhibited elastic fibre formation in VSMCs within the ascending aorta.

scholarly journals Effect of bicuspid aortic valve phenotype on progression of aortic stenosis

2020 ◽  
Vol 21 (7) ◽  
pp. 727-734 ◽  
Author(s):  
Mylène Shen ◽  
Lionel Tastet ◽  
Romain Capoulade ◽  
Marie Arsenault ◽  
Élisabeth Bédard ◽  
...  
Keyword(s):  
Risk Factors ◽  
Aortic Valve ◽  
Aortic Stenosis ◽  
Bicuspid Aortic Valve ◽  
Density Ratio ◽  
Progression Rate ◽  
Aortic Valve Calcification ◽  
Unique Identifier ◽  
Clinical Trial Registration ◽  
Valve Calcification

Abstract Aims To compare the progression of aortic stenosis (AS) in patients with bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV). Methods and results One hundred and forty-one patients with mild-to-moderate AS, recruited prospectively in the PROGRESSA study, were included in this sub-analysis. Baseline clinical, Doppler echocardiography and multidetector computed tomography characteristics were compared between BAV (n = 32) and TAV (n = 109) patients. The 2-year haemodynamic [i.e. peak aortic jet velocity (Vpeak) and mean transvalvular gradient (MG)] and anatomic [i.e. aortic valve calcification density (AVCd) and aortic valve calcification density ratio (AVCd ratio)] progression of AS were compared between the two valve phenotypes. The 2-year progression rate of Vpeak was: 16 (−0 to 40) vs. 17 (3–35) cm/s, P = 0.95; of MG was: 1.8 (−0.7 to 5.8) vs. 2.6 (0.4–4.8) mmHg, P = 0.56; of AVCd was 32 (2–109) vs. 52 (25–85) AU/cm2, P = 0.15; and of AVCd ratio was: 0.08 (0.01–0.23) vs. 0.12 (0.06–0.18), P = 0.16 in patients with BAV vs. TAV. In univariable analyses, BAV was not associated with AS progression (all, P ≥ 0.26). However, with further adjustment for age, AS baseline severity, and several risk factors (i.e. sex, history of hypertension, creatinine level, diabetes, metabolic syndrome), BAV was independently associated with faster haemodynamic (Vpeak: β = 0.31, P = 0.02) and anatomic (AVCd: β = 0.26, P = 0.03 and AVCd ratio: β = 0.26, P = 0.03) progression of AS. Conclusion In patients with mild-to-moderate AS, patients with BAV have faster haemodynamic and anatomic progression of AS when compared to TAV patients with similar age and risk profile. This study highlights the importance and necessity to closely monitor patients with BAV and to adequately control and treat their risk factors. Clinical trial registration https://clinicaltrials.gov Unique identifier: NCT01679431.

Serial evaluation of the thoracic aorta in patients with a bicuspid aortic valve: A magnetic resonance angiography study

VASA ◽  
2010 ◽  
Vol 39 (2) ◽  
pp. 140-144 ◽  
Author(s):  
Tutarel ◽  
Meyer ◽  
Lotz ◽  
Westhoff-Bleck
Keyword(s):  
Aortic Valve ◽  
Thoracic Aorta ◽  
Bicuspid Aortic Valve ◽  
Aortic Root ◽  
Innominate Artery ◽  
Ascending Aorta ◽  
Current Data ◽  
Descending Aorta ◽  
Valve Function ◽  
A Prospective Study

Background: Bicuspid aortic valve (BAV) is associated with an arteriopathy leading to a progressive dilatation of the aortic root. Recent studies have shown that the whole thoracic aorta is affected by this arteriopathy. Longitudinal data regarding the progression of this arteriopathy in the whole thoracic aorta has not been reported before. Patients and methods: In this retrospective study 40 patients (mean age 28.5 ± 9.1 years) had 2 MR-angiographies (mean interval 37.1 ± 15.2 months). In 23 patients the aortic valve was regurgitant, in 1 stenotic, in 4 combined aortic stenosis / regurgitation was found, while in 12 the valve function was normal. Aortic diameters were measured at 6 different, standardized anatomical points. The influence of demographic and clinical parameters was assessed. Results: A significant increase of the diameter was observed at the aortic root (35.4 ± 5.6 mm → 39.1 ± 6.5 mm, p < 0.001), the ascending aorta (37.3 ± 8.0 mm → 39.5 ± 8.5 mm, p = 0.001), proximal to the innominate artery (29.4 ± 6.1 mm → 31.6 ± 6.8 mm, p = 0.008), and the descending aorta (20.2 ± 2.4 mm → 21.6 ± 4.2 mm, p = 0.03). There was no significant increase proximal (24.0 ± 5.7 mm → 24.6 ± 5.3 mm, p = 0.44) and distal to the left subclavian artery (21.4 ± 4.6 mm → 21.9 ± 4.5 mm, p = 0.19). These observations were independent of the presence of arterial hypertension, a previous operation, gender, and functional status of the aortic valve. Conclusions: The progressive dilatation of the aortic root and ascending aorta that can be observed in patients with BAV was not found in the more distal parts of the thoracic aorta with the exception of the descending aorta in this study. If the dilatation of the descending aorta bears any clinical significance can't be answered with the current data. A prospective study should be performed to confirm these results.

Sign in / Sign up

Export Citation Format

Share Document