scholarly journals (PEARS implantation in a patient with ascending aortic dilation after bicuspidalization of aortic valve)

Cor et Vasa ◽  
2021 ◽  
Vol 63 (5) ◽  
pp. 597-601
Author(s):  
Albert Stehlík ◽  
Petr Fila ◽  
Daniela Žáková ◽  
Petr Němec
Keyword(s):  
Aortic Valve ◽  
Aortic Dilation

High prevalence of ascending aortic dilation in adults with repaired coarctation of the aorta

2021 ◽  
pp. 1-6
Author(s):  
Daniel Rinnström ◽  
Mikael Dellborg ◽  
Ulf Thilén ◽  
Peder Sörensson ◽  
Niels-Erik Nielsen ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Aortic Valve ◽  
Aortic Valve Disease ◽  
Congenital Heart ◽  
Coarctation Of The Aorta ◽  
Valve Disease ◽  
Aortic Dilation ◽  
Factors Associated ◽  
High Prevalence ◽  
Life Threatening

Abstract Background: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients. Methods and results: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]). Conclusions: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

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.

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 New fibrillin gene mutation - possible cause of ascending aortic dilation in patients with aortic valve disease: Preliminary results

2009 ◽  
Vol 18 (02) ◽  
pp. 99-102
Author(s):  
Ján Dudra ◽  
Jaroslav Lindner ◽  
Ivan Vaněk ◽  
Jana Šimova ◽  
Ivan Mazura ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Gene Mutation ◽  
Aortic Valve Disease ◽  
Valve Disease ◽  
Aortic Dilation ◽  
Preliminary Results ◽  
Possible Cause

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

scholarly journals Effect of Aortic Valve Disease on 3D Hemodynamics in Patients With Aortic Dilation and Trileaflet Aortic Valve Morphology

2019 ◽  
Vol 51 (2) ◽  
pp. 481-491 ◽  
Author(s):  
Kenichiro Suwa ◽  
Ozair Abdul Rahman ◽  
Emilie Bollache ◽  
Michael J. Rose ◽  
Amir Ali Rahsepar ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Disease ◽  
Valve Disease ◽  
Aortic Dilation ◽  
Valve Morphology

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.

Sign in / Sign up

Export Citation Format

Share Document