Association Between Bicuspid Aortic Valve Phenotype and Patterns of Valvular Dysfunction and Bicuspid Aortopathy

Kang, Joon‐Won; Song, Hae-Geun; Yang, Dong Hyun; Baek, Seunghee; Kim, Dae–Hee; Song, Jong–Min; Kang, Duk–Hyun; Lim, Tae‐Hwan; Song, Jae‐Kwan

doi:10.1016/j.jcmg.2012.11.007

Cited by 200 publications

(188 citation statements)

References 22 publications

Supporting

Mentioning

162

Contrasting

Unclassified

Order By: Relevance

“…The low WSS variability (IQR) across a large cohort of nonstenotic BAV patients (n=113 RL‐BAV; n=23 RN‐BAV) suggests that BAV‐mediated changes in WSS are consistent across nonstenotic patients and associated with the cusp fusion phenotype. Interestingly, these regions of increased WSS corresponded with known differences in aortopathy expression for RL‐BAV and RN‐BAV: Dilatation of the tubular AAo accompanied by aortic‐root dilatation has been associated with RL‐BAV, whereas more‐distal dilatation of the tubular AAo extending to the proximal arch with sparing of the aortic root has been associated with RN‐BAV 27. These findings therefore support the notion that increased WSS may influence outward vessel remodeling in the presence of BAV aortopathy 28.…”

Section: Discussionmentioning

confidence: 70%

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Ooij

Markl

Collins

et al. 2017

JAHA

133

104

View full text Add to dashboard Cite

BackgroundWall shear stress (WSS) is a stimulus for vessel wall remodeling. Differences in ascending aorta (AAo) hemodynamics have been reported between bicuspid aortic valve (BAV) and tricuspid aortic valve patients with aortic dilatation, but the confounding impact of aortic valve stenosis (AS) is unknown.Methods and ResultsFive hundred seventy‐one subjects underwent 4‐dimensional flow magnetic resonance imaging in the thoracic aorta (210 right‐left BAV cusp fusions, 60 right‐noncoronary BAV cusp fusions, 245 tricuspid aortic valve patients with aortic dilatation, and 56 healthy controls). There were 166 of 515 (32%) patients with AS. WSS atlases were created to quantify group‐specific WSS patterns in the AAo as a function of AS severity. In BAV patients without AS, the different cusp fusion phenotypes resulted in distinct differences in eccentric WSS elevation: right‐left BAV patients exhibited increased WSS by 9% to 34% (P<0.001) at the aortic root and along the entire outer curvature of the AAo whereas right‐noncoronary BAV patients showed 30% WSS increase (P<0.001) at the distal portion of the AAo. WSS in tricuspid aortic valve patients with aortic dilatation patients with no AS was significantly reduced by 21% to 33% (P<0.01) in 4 of 6 AAo regions. In all patient groups, mild, moderate, and severe AS resulted in a marked increase in regional WSS (P<0.001). Moderate‐to‐severe AS further increased WSS magnitude and variability in the AAo. Differences between valve phenotypes were no longer apparent.Conclusions AS significantly alters aortic hemodynamics and WSS independent of aortic valve phenotype and over‐rides previously described flow patterns associated with BAV and tricuspid aortic valve with aortic dilatation. Severity of AS must be considered when investigating valve‐mediated aortopathy.

show abstract

Section: Discussionmentioning

confidence: 70%

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Ooij

Markl

Collins

et al. 2017

JAHA

133

104

View full text Add to dashboard Cite

show abstract

“…Data suggests that the majority of patients with BAV eventually develop aortic stenosis, and that dilation of the tubular ascending aorta is more than twice as common as isolated root dilation (8,9). Given the marked thickening, calcification and restriction of leaflet motion seen with BAV stenosis, the valve repair discussed by Schneider at al.…”

mentioning

confidence: 99%

Evolving treatment options for valve and aortic disease with bicuspid aortic valve

Burris

Hope

2017

Ann. Transl. Med.

View full text Add to dashboard Cite

“…2). A heterogeneous pattern of aortic dilatation develops in an affected person, probably reflecting heterogeneity in molecular, rheologic, and clinical features [2].In this issue, Lee et al [3] suggest that aortic remodeling in patients with bicuspid aortic valve-associated thoracic aneurysms is sex-dependent. They studied the structural remodeling in patients with bicuspid aortic valves and compared molecular alterations in men and women by investigating aortic aneurysmal tissue samples.…”

mentioning

confidence: 99%

“…2). A heterogeneous pattern of aortic dilatation develops in an affected person, probably reflecting heterogeneity in molecular, rheologic, and clinical features [2].…”

mentioning

confidence: 99%

“…Aortopathy occurs in over half the patients. The 10 levels used in aortic dimension measurements (A-J) with representative MDCT images of bicuspid aortopathy phenotypes are shown (published in Kang et al [2] and permission provided by courtesy of Elsevier Publisher). a Scheme of the 10 levels used in aortic dimension measurements (A-J) with b representative MDCT images of bicuspid aortopathy phenotypes is shown.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Aortas reveal the myth of male privilege

Luft

2014

J Mol Med

View full text Add to dashboard Cite

The commonest congenital heart defect in adults is the bicuspid aortic valve. It affects 1.3 % of the population worldwide and is responsible for more deaths and complications than all the other congenital heart defects combined (Fig. 1). Fusion of aortic valve leaflets occurs most commonly (≈80 %) between the right coronary and left coronary leaflets (RL), which are the anterior leaflets of the aortic valve. Fusion also occurs between the right coronary and noncoronary leaflets (RN; ≈17 %), and least commonly between the noncoronary and left coronary leaflets (LN; ≈2 %). Verma and Siu recently provided an overview of the basic principles, recent advances, and recommendations for the treatment of adults with bicuspid aortopathy [1]. Aortic stenosis and insufficiency are the hallmarks of a bicuspid aortic valve; however, dilatation of any or all proximal aortic segments extending from the aortic root to the aortic arch, namely, bicuspid aortopathy, is also present in at least half of affected persons (Fig. 2). A heterogeneous pattern of aortic dilatation develops in an affected person, probably reflecting heterogeneity in molecular, rheologic, and clinical features [2].In this issue, Lee et al. [3] suggest that aortic remodeling in patients with bicuspid aortic valve-associated thoracic aneurysms is sex-dependent. They studied the structural remodeling in patients with bicuspid aortic valves and compared molecular alterations in men and women by investigating aortic aneurysmal tissue samples. Women had smaller aortic aneurysms than men, but the size difference disappeared when the data were normalized for body surface area. Elastin fibers were disorganized and reduced in men and women. However, only men exhibited disarrayed collagen fibers and reduced protein. The mRNA levels were similar in both groups, suggesting post-translational degradation of proteins, more prominent in men compared to women. The enzymatic elastase activity was elevated in both men and women, while the membrane-anchored collagenase membrane type 1 matrix metalloprotease (MT1-MMP) was more increased in men than in women. Furthermore, matrix metalloprotease (MMP)-8 and MMP-13 were lower, while MMP-2 was higher in women than men. Tissue-inhibitor of metalloprotease (TIMP)-3 and TIMP-4 were similarly decreased in both sexes, while TIMP-2 was increased in women. The vascular smooth muscle cell density in the media of dilated aortas from men corresponded to an increase in caspase-3 cleavage, compared to women. The authors suggest a sex-dependent MMP/TIMP axis, including collagen remodeling and vascular smooth muscle cell survival, could protect women from aortic rupture.Lee et al. [3] claim that sex-related effects have not been explored in thoracic aortic aneurysms associated with bicuspid aortic valve disease. Predictors of bicuspid aortopathy progression include older age, male sex, elevated systolic blood pressure, coexisting aortic valve stenosis or regurgitation, and morphologic features of the valve, namely, right or left cusp fusion [4]....

show abstract

Association Between Bicuspid Aortic Valve Phenotype and Patterns of Valvular Dysfunction and Bicuspid Aortopathy

Abstract: The patterns of valvular dysfunction and bicuspid aortopathy differed significantly between the 2 BAV phenotypes, suggesting the possibility of etiologically different entities.

Cited by 200 publications

References 22 publications

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4‐Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects

Evolving treatment options for valve and aortic disease with bicuspid aortic valve

Aortas reveal the myth of male privilege

Contact Info

Product

Resources

About