Ascending aorta dilation in association with bicuspid aortic valve: A maturation defect of the aortic wall

AbstractThe characteristics of aortic valvular outflow jet affect aortopathy in the bicuspid aortic valve (BAV). This study aimed to elucidate the effects of BAV morphology on the aortic valvular outflow jets. Morphotype-specific valve-devising apparatuses were developed to create aortic valve models. A magnetic resonance imaging-compatible pulsatile flow circulation system was developed to quantify the outflow jet. The eccentricity and circulation values of the peak systolic jet were compared among tricuspid aortic valve (TAV), three asymmetric BAVs, and two symmetric BAVs. The results showed mean aortic flow and leakage did not differ among the five BAVs (six samples, each). Asymmetric BAVs demonstrated the eccentric outflow jets directed to the aortic wall facing the smaller leaflets. In the asymmetric BAV with the smaller leaflet facing the right-anterior, left-posterior, and left-anterior quadrants of the aorta, the outflow jets exclusively impinged on the outer curvature of the ascending aorta, proximal arch, and the supra-valvular aortic wall, respectively. Symmetric BAVs demonstrated mildly eccentric outflow jets that did not impinge on the aortic wall. The circulation values at peak systole increased in asymmetric BAVs. The bicuspid symmetry and the position of smaller leaflet were determinant factors of the characteristics of aortic valvular outflow jet.

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Collagen analysis in aneurysmal ascending aorta obtained from patients with bicuspid aortic valve disease compared with tricuspid aortic valve aneurysm

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-0034-1367284 ◽

2014 ◽

Vol 62 (S 01) ◽

Author(s):

A. Navarrete Santos ◽

P. Lochmann ◽

J.Y. Yan ◽

A. Simm ◽

R.-E. Silber ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Aortic Valve Disease ◽

Ascending Aorta ◽

Valve Disease ◽

Tricuspid Aortic Valve ◽

Collagen Analysis

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Beta-blocker therapy does not reduce ascending aorta wall shear stress in patients with bicuspid aortic valve

Journal of Cardiovascular Magnetic Resonance ◽

10.1186/1532-429x-17-s1-p399 ◽

2015 ◽

Vol 17 (S1) ◽

Cited By ~ 1

Author(s):

Bradley D Allen ◽

Michael Markl ◽

Alex J Barker ◽

Pim van Ooij ◽

James C Carr ◽

...

Keyword(s):

Shear Stress ◽

Aortic Valve ◽

Wall Shear Stress ◽

Bicuspid Aortic Valve ◽

Beta Blocker ◽

Ascending Aorta ◽

Blocker Therapy ◽

Beta Blocker Therapy ◽

Aorta Wall ◽

Wall Shear

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Elasticity of ascending aorta and left ventricular myocardial functions in children with bicuspid aortic valve

Echocardiography ◽

10.1111/echo.13664 ◽

2017 ◽

Vol 34 (11) ◽

pp. 1660-1666 ◽

Cited By ~ 2

Author(s):

Filiz Ekici ◽

Diyar Uslu ◽

Selen Bozkurt

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Left Ventricular ◽

Ascending Aorta

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Fate of mild-to-moderate bicuspid aortic valve disease untreated during ascending aorta replacement

10.22541/au.160901985.50675267/v1 ◽

2020 ◽

Author(s):

Alessandro Verzini ◽

Marta Bargagna ◽

Guido Ascione ◽

Alessandra Sala ◽

Davide Carino ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Ascending Aorta ◽

Term Survival ◽

Kaplan Meier ◽

Aortic Root Surgery ◽

Increased Risk ◽

Aorta Replacement ◽

Ascending Aorta Replacement

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and it is responsible for an increased risk of developing aortic valve and ascending aorta complications. In case of mild to moderate BAV disease in patients undergoing supracoronary ascending aorta replacement, it is unclear whether a concomitant aortic valve replacement should be performed. Methods: From June 2002 to January 2020, 75 patients with mild-to-moderate BAV regurgitation (± mild-to-moderate stenosis) who underwent isolated supracoronary ascending aorta replacement were retrospectively analyze. Clinical and echocardiographic follow-up was 100% complete (mean: 7.4±3.9 years, max 16.4). Kaplan Meier estimates were employed to analyze long-term survival. Cumulative incidence function for time to re-operation, recurrence of aortic regurgitation (AR)≥3+ and aortic stenosis (AS) greater than moderate, with death as competing risk, were computed. Results: There was no hospital mortality and no cardiac death occurred. Overall survival at 12 years was 97.4±2.5%, 95% CI [83.16-99.63]. At follow-up there were no cases of aortic root surgery whereas 3 patients underwent AV replacement. At 12 years the CIF of reoperation was 2.6±2.5%, 95% CI [0.20-11.53]. At follow up, AR 3+/4+ was present in 1 pt and AS greater than moderate in 3. At 12 years the CIF of AR>2+/4+ was 5.1±4.98% and of AS>moderate 6.9±3.8%. Conclusions: In our study mild to moderate regurgitation of a BAV did not significantly worse at least up to 10 years after isolated supracoronary ascending aorta replacement.

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Abstract 102: Granular Media Calcinosis in the Aortic Wall of Patients With Bicuspid and Tricuspid Aortic Valves

Arteriosclerosis Thrombosis and Vascular Biology ◽

10.1161/atvb.34.suppl_1.102 ◽

2014 ◽

Vol 34 (suppl_1) ◽

Author(s):

Sandy von Salisch ◽

Josephina Haunschild ◽

Martin Misfeld ◽

Michael A Borger ◽

Stefan Dhein ◽

...

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Granular Media ◽

Aortic Wall ◽

Aortic Disease ◽

Dissecting Aneurysm ◽

Media Thickness ◽

Significant Difference ◽

The Media ◽

Aneurysm Dissection

Background: Bicuspid aortic valve is the most frequent congenital cardiac abnormality and associated with proximal aortic disease (i.e. aneurysm, dissection or rupture). Granular media calcinosis(GMC)--suggested to increase stiffness and play a pathogenetic role in dissecting aneurysm--has not yet been quantified in BAV. Methods: Specimen of the proximal aortic wall from 76 patients--32 with tricuspid (TAV) and 44 with bicuspid aortic valve (BAV)--were obtained during surgery to quantify media thickness and GMC by von Kossa staining (panel C), comparing the convexity (Cvx) and concavity (Ccv) in BAV vs. TAV. Results: Interlamellar GMC affected the most central layers of the media and those adjacent to the outer adventitia with a doubling within both--the Cvx and Ccv--of pts with BAV compared to patients with TAV (13.3±9.6 vs. 6.6±7.4 and 12.8±10.8 vs. 6.4±7.1; p<0.05, panel A) was seen, but neither a difference in calcification between the Ccx and the Ccv side within the BAV nor the TAV group. No association between age and calcification grade , neither in the Cvx nor the Ccv (r=0.132, p=0.218 and 0.103, p=0.341) was seen. There was a significant difference in the total media thickness between BAV and TAV at the Cvx (867±162μm vs . 993±158μm; p<0.05) and the Ccv (1005 ± 236 vs 1223 ± 217μm; p<0.05, panel B). Independent of aortic valve morphology, the Cvx was thinner than the Ccv side (TAV: 993 ± 158 vs.1223 ± 217μm; p<0.001; BAV: 869 ± 162 vs.1005 ± 236μm; p<0.05, panel B). Conclusion: BAVs had significantly thinner media and twice as much GMC than their tricuspid peers possibly associated with the loosening of the bond between the elastic lamellae causing a decrease in elasticity possibly explaining a higher risk for dissection and rupture.

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

Journal of Clinical Medicine ◽

10.3390/jcm9040908 ◽

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.

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