scholarly journals Data on the circulating levels of endothelial microparticles are elevated in patients with bicuspid aortic valve and are related to aortic dilation

Data in Brief ◽  
2016 ◽  
Vol 8 ◽  
pp. 666-669
Author(s):  
Josep M. Alegret ◽  
Neus Martínez-Micaelo ◽  
Gerard Aragonès ◽  
Raúl Beltrán-Debón
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Dilation ◽  
Endothelial Microparticles ◽  
Circulating Levels

scholarly journals The Development of the Ascending Aortic Wall in Tricuspid and Bicuspid Aortic Valve: A Process from Maturation to Degeneration

2020 ◽  
Vol 9 (4) ◽  
pp. 908
Author(s):  
Nimrat Grewal ◽  
Adriana C. Gittenberger-de Groot ◽  
Jan von der Thusen ◽  
Lambertus J. Wisse ◽  
Margot M. Bartelings ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Young Child ◽  
Bicuspid Aortic Valve ◽  
Aortic Wall ◽  
Age Groups ◽  
Intimal Thickening ◽  
Aortic Dilation ◽  
Future Studies ◽  
Increased Risk ◽  
Two Phases

Background: Patients with a bicuspid aortic valve (BAV) have an increased risk for aortic dilation and dissection. In this study, we provide a histological stratification of the developing aorta in the tricuspid aortic valve (TAV) and the BAV populations as a reference for future studies on aortopathy and related syndromes. Methods: Non-dilated TAV and BAV ascending aortic wall samples were collected, including 60 TAV (embryonic–70 years) and 32 BAV specimens (fetal–72 years, categorized in eight age groups. Results: In TAV, intimal development starts in the neonatal phase. After birth, the thickness of the medial layer increases significantly by increase of elastic lamellae up to and including the “young child” phase stabilizing afterwards. The BAV shows already prenatal intimal thickening becoming significantly thinner after birth subsequently stabilizing. In BAV, increase in elastic lamellae is seen between the young child and the adolescent phases, stabilizing afterwards. Conclusions: Vascular development in TAV is described in three phases: maturation, stabilization, and degeneration. For BAV, the development can be described in two phases: maturation (already prenatally) and degeneration. After birth, the development of the aorta is characterized by degeneration, leading to weakening of the ascending aortic wall and increasing the risk of aortopathy.

scholarly journals Influence of Aortic Dilation on the Regional Aortic Stiffness of Bicuspid Aortic Valve Assessed by 4-Dimensional Flow Cardiac Magnetic Resonance

2019 ◽  
Vol 12 (6) ◽  
pp. 1020-1029 ◽  
Author(s):  
Andrea Guala ◽  
Jose Rodriguez-Palomares ◽  
Lydia Dux-Santoy ◽  
Gisela Teixido-Tura ◽  
Giuliana Maldonado ◽  
...  
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Stiffness ◽  
Aortic Dilation ◽  
Dimensional Flow

ALTERATION OF ELASTIC PROPERTIES IN CONGENITAL/INHERITED AORTIC DISEASES: MRI STUDY IN MARFAN SYNDROME AND BICUSPID AORTIC VALVE

2006 ◽  
Vol 06 (01) ◽  
pp. 47-53
Author(s):  
LUIGI LOVATO ◽  
PAOLO SBARZAGLIA ◽  
SILVIA MAGI ◽  
VINCENZO RUSSO ◽  
ROMANO ZANNOLI ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Marfan Syndrome ◽  
Elastic Properties ◽  
Bicuspid Aortic Valve ◽  
Aortic Distensibility ◽  
Control Group ◽  
Aortic Dilation ◽  
Aortic Diseases ◽  
Magnetic Resonance Imaging Mri ◽  
Mri Study

Marfan Syndrome and Bicuspid Aortic Valve (BAV) are characterized by the congenital/inherited alteration of the fibrillin proteins of aortic wall extracellular matrix, with higher incidence of aortic dissection/rupture even without aortic dilation. Therefore, early identification and treatment of aortic involvement could improve prognosis. The purpose of this study was to evaluate aortic elastic properties of BAV and Marfan Syndrome patients to identify an index of early aortic involvement. Methods: A Magnetic Resonance Imaging (MRI) study was performed on 38 BAV patients, 20 Marfan patients and 14 healthy volunteers as a control group. A high-resolution gradient-echo sequence was applied in the axial plane at the level of pulmonary artery bifurcation to evaluate aortic distensibility. Results: Aortic distensibility was significantly different among the three groups (ANOVA, p = 0.0001) and was sensibly reduced in BAV (0.0056 ± 0.0048 versus 0.025 ± 0.006 control group) and Marfan (0.0085 ± 0.006 versus 0.025 ± 0.006 control group). Intraobserver and interobserver reproducibility for diastolic measurements were 1.2% and 0.4% respectively and for systolic measurements, they were 1.1% and 0.3% respectively. Conclusion: In BAV or Marfan Syndrome patients, MRI evaluation of abnormal distensibility may provide a reliable index of early aortic involvement, even before aortic dilation occurs.

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 The bicuspid aortic valve and its relation to aortic dilation

Clinics ◽  
2010 ◽  
Vol 65 (5) ◽  
Author(s):  
Shi-Min Yuan ◽  
Hua Jing ◽  
Jacob Lavee
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Dilation

Bicuspid aortic valve disease and competitive sports: key considerations and challenges

2019 ◽  
pp. 233-238
Author(s):  
Benjamin S. Wessler ◽  
Natesa G. Pandian
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Regurgitation ◽  
Bicuspid Aortic Valve ◽  
Aortic Dilation ◽  
Valvular Stenosis ◽  
Valvular Function ◽  
A Minor ◽  
Valve Lesion

Bicuspid aortic valve (BAV) is a common congenital disorder. It could simply be a minor anatomic abnormality or be associated with progressive aortic stenosis, aortic regurgitation, and aortic dilation. If an athlete is recognized to have a BAV, questions arise with regard to whether they can pursue their selected sports, particularly elite athletic activity, and what type of follow-up examinations are necessary and how often should be done. Valvular disorders such as the degree of aortic stenosis and aortic regurgitation, aortic size, and coexisting disorders are also influencing factors. The absence of robust controlled studies, which are difficult to perform, make decision-making difficult, although recommendations by expert panels provide some guidance. The general consensus is that athletes with BAV with normal valvular function and no aortic dilation can participate in all athletic activities. Those with mild aortic dilation should undergo annual screening, some more frequently than others. Those with moderate or severe valvular stenosis or regurgitation should be managed based on the haemodynamic impact of the valve lesion. Athletes with coexisting lesions or syndromes should be evaluated comprehensively. The overall recommendation to an individual athlete should incorporate many factors and employ a multidisciplinary approach.

scholarly journals Familial clustering of bicuspid aortic valve and its relationship with aortic dilation in first-degree relatives

Heart ◽  
2018 ◽  
pp. heartjnl-2018-313802 ◽  
Author(s):  
Laura Galian-Gay ◽  
Amelia Carro Hevia ◽  
Gisela Teixido-Turà ◽  
José Rodríguez Palomares ◽  
Laura Gutiérrez-Moreno ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Prevalence Rate ◽  
Valvular Regurgitation ◽  
Aortic Dilation ◽  
Tertiary Referral ◽  
Aortic Valves ◽  
First Degree Relatives ◽  
Sequential Screening ◽  
Familial Clustering

ObjectiveBicuspid aortic valve (BAV) is the most common congenital heart disease. This study aimed to determine the prevalence rate of BAV in first-degree relatives (FDR) and the inheritance pattern according to different morphotypes and aortic dilation.MethodsBAV probands were consecutively studied at eight tertiary referral centres. After sequential screening, FDR were included in the study. The BAV morphotype, aortic dilation and aortic phenotype were assessed by transthoracic echocardiography.ResultsSeven hundred and twenty-four FDR of 256 BAV probands agreed to undergo family screening. The prevalence of BAV was 6.4% in FDR (9.2% in men, 3.5% in women, p=0.002). Aortic dilation was diagnosed in 9.6% of FRD with tricuspid aortic valves (TAV), with a root phenotype in 2.7% and tubular in 6.9% and more frequently in the presence of arterial hypertension (OR 4.48; CI 95% 2.51 to 7.99; p=0.0001) and valvular regurgitation (OR 5.87, CI 95% 1.37 to 25.16; p=0.025). The heritability (h2) of BAV was highly significant (0.47; p=0.002); however, no concordance was observed among valve morphotypes. Aortic dilation heritability was not significant.ConclusionsThe BAV prevalence rate in FDR was low (6.4%) but aortic dilation was observed in 9.6% of FDR with TAV. The heritability of BAV was high without concordance in valve morphotypes, and aortic dilation heritability was not observed. Patients with BAV should be made aware of its familial pattern.

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