Ex Vivo Biomechanics Cast Doubts on Current Diameter-Based Guidelines for Bicuspid Aortic Valve-Associated Proximal Aortic Disease

2020 ◽  
Author(s):  
J. Haunschild ◽  
S. Friebe ◽  
D. Petroff ◽  
M. Borger ◽  
S. Mayr ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ex Vivo ◽  
Aortic Disease

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

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.

Abstract 6147: A Tissue Stem Cell Niche Regulates Bicuspid Aortic Valve Disease

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Nalini M Rajamannan
Keyword(s):  
Stem Cell ◽  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Cross Talk ◽  
Aortic Valve Disease ◽  
Stem Cell Niche ◽  
Ex Vivo ◽  
Valve Disease ◽  
Aortic Valves ◽  
Cell Niche

Bicuspid aortic valve disease is the most common congenital cardiac malformation and indication for surgery for aortic valve patients. The age of onset for the development of stenosis is earlier in bicuspid aortic valves than tricuspid aortic valves. Understanding the cellular mechanisms of this valve lesion, will help to develop novel approaches towards slowing disease progression. This study hypothesizes that bicuspid aortic valve disease develops secondary to a tissue stem cell niche to activate a cross-talk mechanism which induces Notch1 cleavage and Lrp5 mediated bone formation specific to the bicuspid aortic valve. Human ex vivo bicuspid valves versus control aortic valves were tested for Notch1 expression by RTPCR, Western Blot and Immunohistochemistry. eNOS null bicuspid mice: control (n=20), cholesterol (n=20), cholesterol + Atorvastatin (n=20), were tested for the development of aortic stenosis by Visual Sonics Echo, Immunohistochemistry for Notch1, Wnt, Lrp5, Osteocalcin, PCNA and RTPCR for Notch1, Lrp5, Cbfa1, Osteocalcin. In vitro studies were performed to characterize Wnt secretion from aortic valve endothelial cells and gene expression for Notch1, Lp5 and osteocalcin from the valve myofibroblast cells. This study characterizes the secretion of Wnt3a (>300-fold, p<0.01) from aortic valve endothelium in the presence of abnormal nitric oxide regulation and lipids as measured by eNOS enzymatic activity and tissue nitrite levels. Osteoblastogenesis in the adjacent myofibroblast cell is activated via Notch1 cleavage(p<0.001) and upregulation of the Wnt3a/Lrp5 receptor. Human ex vivo valves express Notch1 cleavage as compared to normal valves from heart transplant(p<0.01). Cholesterol treated eNOS mice develop severe stenosis with cleavage of Notch1, increase in Lrp5, Wnt3a, cyclin, Cbfa1, and Osteopontin,(3-fold increase(p<0.01) which was not present in the controls and normalized in the statin treated valves. Targeting the Notch1/Wnt3a/Lrp5 pathway in bicuspid valvular calcification presents a novel approach towards treating this disease. The importance of this cross talk mechanism is demonstrated in three models of aortic valve disease and will have important clinical implications. This research has received full or partial funding support from the American Heart Association, AHA National Center.

scholarly journals Bicuspid aortic valve and aortic disease. The role of family screening

2013 ◽  
Vol 34 (suppl 1) ◽  
pp. P4757-P4757
Author(s):  
A. Carro ◽  
G. Teixido ◽  
M. Sanz ◽  
V. Galuppo ◽  
L. Galian ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Disease ◽  
Family Screening

scholarly journals MRI hemodynamic markers of progressive bicuspid aortic valve-related aortic disease

2013 ◽  
Vol 40 (1) ◽  
pp. 140-145 ◽  
Author(s):  
Michael D. Hope ◽  
Monica Sigovan ◽  
S. Jarrett Wrenn ◽  
David Saloner ◽  
Petter Dyverfeldt
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Aortic Disease

scholarly journals Aortic arch tortuosity, a novel biomarker for thoracic aortic disease, is increased in adults with bicuspid aortic valve

2019 ◽  
Vol 284 ◽  
pp. 84-89 ◽  
Author(s):  
Bader Aldeen Alhafez ◽  
Van Thi Thanh Truong ◽  
Daniel Ocazionez ◽  
Sahand Sohrabi ◽  
Harleen Sandhu ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Arch ◽  
Bicuspid Aortic Valve ◽  
Aortic Disease ◽  
Thoracic Aortic Disease

Circulating microRNAs vs. aortic diameter in bicuspid aortic valve aortopathy

2020 ◽  
pp. 021849232092723
Author(s):  
Shiho Naito ◽  
Johannes Petersen ◽  
Tatiana Sequeira-Gross ◽  
Tanja Zeller ◽  
Hermann Reichenspurner ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Risk Stratification ◽  
Bicuspid Aortic Valve ◽  
Treatment Guidelines ◽  
Aortic Disease ◽  
Current Evidence ◽  
Diagnostic Tools ◽  
Circulating Micrornas ◽  
Prediction Tools ◽  
Improve Risk Stratification

There is growing clinical need and interest to implement novel risk prediction tools in bicuspid aortic valve-associated proximal aortic disease, so-called bicuspid aortic valve aortopathy. Inherent limitations of the diameter-based risk stratification for adverse aortic events in bicuspid aortic valve aortopathy patients have recently been recognized. Therefore, alternative diagnostic tools and subsequent adjustments in the treatment guidelines are urgently needed. Herein, we summarize the current evidence on recent diagnostic developments to improve risk stratification in bicuspid aortic valve aortopathy, including circulating microRNAs as biomarkers to predict the progression of aortic disease.

scholarly journals Bicuspid aortic valve associated aortopathy: a genetic disease

2015 ◽  
Vol 4 (1) ◽  
Author(s):  
Gibran Minero ◽  
Simon C. Body
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Genetic Disease ◽  
Genetic Variant ◽  
Aortic Disease ◽  
Common Mechanism ◽  
Thoracic Aortic Disease ◽  
Strength Of Evidence ◽  
Flow Through ◽  
Hemodynamic Alteration

As the most common congenital heart defect, understanding the etiology and progression of aortopathy in bicuspid aortic valve (BAV) is imperative to management of patients with BAV. A reasonable hypothesis, based on the strength of evidence for both genetic and hemodynamic causes of BAV-associated thoracic aortic disease (TAD), is that BAV is caused by genetic variant(s) that also predispose to TAD by a common mechanism; presumably by cell-signaling resulting in an embryologic defect that causes BAV and a postnatal risk of TAD that is accentuated by hemodynamic stress of abnormal flow through the BAV valve. Clinical heterogeneity seen in BAVassociated TAD is likely due to individual genetic variation and the severity of hemodynamic alteration.

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