scholarly journals Bicuspid Aortic Valve Disease and Ascending Aortic Aneurysms: Gaps in Knowledge

2012 ◽  
Vol 2012 ◽  
pp. 1-16 ◽  
Author(s):  
Katie L. Losenno ◽  
Robert L. Goodman ◽  
Michael W. A. Chu

The bicuspid aortic valve is the most common congenital cardiac anomaly in developed nations. The abnormal bicuspid morphology of the aortic valve results in valvular dysfunction and subsequent hemodynamic derangements. However, the clinical presentation of bicuspid aortic valve disease remains quite heterogeneous with patients presenting from infancy to late adulthood with variable degrees of valvular stenosis and insufficiency and associated abnormalities including aortic coarctation, hypoplastic left heart structures, and ascending aortic dilatation. Emerging evidence suggests that the heterogeneous presentation of bicuspid aortic valve phenotypes may be a more complex matter related to congenital, genetic, and/or connective tissue abnormalities. Optimal management of patients with BAV disease and associated ascending aortic aneurysms often requires a thoughtful approach, carefully assessing various risk factors of the aortic valve and the aorta and discerning individual indications for ongoing surveillance, medical management, and operative intervention. We review current concepts of anatomic classification, pathophysiology, natural history, and clinical management of bicuspid aortic valve disease with associated ascending aortic aneurysms.

2016 ◽  
Vol 122 (5) ◽  
pp. 289-294 ◽  
Author(s):  
Alexander Navarrete Santos ◽  
Junfeng Yan ◽  
Peter Lochmann ◽  
Heike Pfeil ◽  
Michael Petersen ◽  
...  

2021 ◽  
Vol 8 ◽  
Author(s):  
Daniel G. W. Cave ◽  
Hannah Panayiotou ◽  
Malenka M. Bissell

Bicuspid aortic valve (BAV) disease presents a unique management challenge both pre- and post-operatively. 4D flow MRI offers multiple tools for the assessment of the thoracic aorta in aortic valve disease. In particular, its assessment of flow patterns and wall shear stress have led to new understandings around the mechanisms of aneurysm development in BAV disease. Novel parameters have now been developed that have the potential to predict pathological aortic dilatation and may help to risk stratify BAV patients in future. This systematic review analyses the current 4D flow MRI literature after aortic valve and/or ascending aortic replacement in bicuspid aortic valve disease. 4D flow MRI has also identified distinct challenges posed by this cohort at the time of valve replacement compared to standard management of tri-leaflet disorders, and may help tailor the type and timing of replacement. Eccentric pathological flow patterns seen after bioprosthetic valve implantation, but not with mechanical prostheses, might be an important future consideration in intervention planning. 4D flow MRI also has promising potential in supporting the development of artificial valve prostheses and aortic conduits with more physiological flow patterns.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Ayyaz Ali ◽  
Amit Patel ◽  
Darren Freed ◽  
Yasir Abu-Omar ◽  
Ahmad Y Sheikh ◽  
...  

Objectives A bicuspid aortic valve may be associated with an aortopathy, this may lead to progressive aortic dilatation over time. It is uncertain whether the ascending aorta should be replaced prophylactically during AVR in these patients. We analyzed change in ascending aortic diameter following AVR, to determine whether a clinically important aortic pathology exists in patients with bicuspid aortic valve disease. Methods Demographic, operative and clinical data were obtained retrospectively through casenote review. AVR was performed using a homograft or porcine stentless valve using the subcoronary implantation technique. Patients were grouped according to whether their native aortic valve was identified as tricuspid (TC) or bicuspid (BC) at operation. Serial transthoracic echocardiograms were analyzed to measure pre-operative and post-operative ascending aortic diameter. Results 217 patients underwent AVR between 1 st January 1991 and 1 st January 2001. Ninety patients had a bicuspid aortic valve, in the remaining 127 the valve was tricuspid. The bicuspid group was younger ( BC 62yr +/− 15, TC 71yr +/− 12 yrs; p < 0.001). Follow-up echocardiography was performed 6.0 +/− 4.3 years post-operatively. Pre-operative ascending aortic diameter was similar (BC 3.2 +/− 0.5, TC 3.2 +/− 0.5 cm; p = 0.56) There was no difference in the increase in ascending aortic diameter over follow-up (BC 0.1 +/− 0.5, TC 0.0 +/− 0.5 cm; p = 0.34) Conclusion The clinical importance of “bicuspid aortopathy” in an older age group appears to be minimal. Additional aortic procedures designed to protect against progressive aortic aneurysmal disease in this setting are not justified.


2012 ◽  
Vol 111 (suppl_1) ◽  
Author(s):  
Ling Sun ◽  
Santanu Chandra ◽  
Philippe Sucosky

INTRODUCTION: The bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly and is frequently associated with calcific aortic valve disease (CAVD). Although CAVD also develops in the normal tricuspid aortic valve (TAV), its progression in the BAV is more rapid. While the accelerated calcification of BAV leaflets has been linked to genetic and atherogenic predispositions, hemodynamic abnormalities are increasingly pointed as potential pathogenic contributors. HYPOTHESIS: Supported by our previous work, which demonstrated the sensitivity of valve leaflets to the surrounding blood flow and associated wall-shear stress (WSS), we hypothesize that the abnormal WSS experienced by BAV leaflets contribute to CAVD development by promoting valvular inflammation, remodeling and osteogenic differentiation. OBJECTIVE: This study aims at comparing ex vivo the effects of TAV and BAV leaflet WSS on valvular pathogenesis. METHODS: The native, side-specific WSS experienced by TAV and type-I (i.e., fused and non-coronary) BAV leaflets were obtained computationally using fluid-structure interaction simulations. Fresh porcine leaflets were subjected for 48 hours to each of the three WSS conditions using a novel double-sided shear stress bioreactor. Tissue response was characterized via Western blot and immunohistochemistry in terms of markers of endothelial activation (VCAM-1, ICAM-1), paracrine expression (BMP-4), TGF-β/Wnt signaling pathways (TGF-β1, β-catenin), extracellular matrix remodeling (cathepsin L, MMP-2, MMP-9) and osteogenic differentiation (α-SMA, osteocalcin). RESULTS: No significant differences in VCAM-1 and ICAM-1 expressions were detected between tissue exposed to TAV and BAV WSS. While the native WSS experienced by the TAV and non-coronary BAV leaflets maintained tissue homeostasis, tissue exposure to the fused BAV leaflet WSS resulted in a significant pathological response marked by the upregulations of BMP-4, β-catenin, MMP-2 and osteocalcin expressions. CONCLUSION: This study demonstrates the pathological nature of the native BAV hemodynamics and confirms the higher susceptibility of the fused BAV leaflet to calcify. The results provide new insights into the hemodynamic theory of BAV calcification.


2007 ◽  
Vol 134 (2) ◽  
pp. 290-296 ◽  
Author(s):  
Stephen H. McKellar ◽  
David J. Tester ◽  
Marineh Yagubyan ◽  
Ramanath Majumdar ◽  
Michael J. Ackerman ◽  
...  

Heart ◽  
2014 ◽  
Vol 100 (Suppl 3) ◽  
pp. A56.1-A56 ◽  
Author(s):  
Margaret Loudon ◽  
Sacha Bull ◽  
Jubin Joseph ◽  
Jane Francis ◽  
Oliver Rider ◽  
...  

2012 ◽  
Vol 65 (11) ◽  
pp. 977-981
Author(s):  
Amelia Carro ◽  
Gisela Teixido-Tura ◽  
Arturo Evangelista

2010 ◽  
Vol 89 (6) ◽  
pp. 1918-1924 ◽  
Author(s):  
Rakan I. Nazer ◽  
Abdelsalam M. Elhenawy ◽  
Shafie S. Fazel ◽  
Luis E. Garrido-Olivares ◽  
Susan Armstrong ◽  
...  

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