Bicuspid aortic valve phenotype and aortic disease: a magnetic resonance study

Abstract Background Aortic stiffness has been shown to be abnormal in patients with bicuspid aortic valve (BAV), and is considered a component of the aortopathy associated with this condition. Progressive aortic stiffening associated with aging has been previously described in normal adults. However, it is not known if aging related aortic stiffening occurs at the same rate in BAV patients. We determined the longitudinal rate of decline in segmental distensibility in BAV patients using serial cardiovascular magnetic resonance (CMR) studies, and compared to previously published results from a group of patients with connective tissue disorders (CTD). Methods A retrospective review of CMR and clinical data on children and adults with BAV (n = 49, 73% male; 23 ± 11 years) with at least two CMRs (total 98 examinations) over a median follow-up of 4.1 years (range 1–9 years) was performed to measure aortic distensibility at the ascending (AAo) and descending aorta (DAo). Longitudinal changes in aortic stiffness were assessed using linear mixed-effects modeling. The comparison group of CTD patients had a similar age and gender profile (n = 50, 64% male; 20.6 ± 12 years). Results Compared to CTD patients, BAV patients had a more distensible AAo early in life but showed a steeper decline in distensibility on serial examinations [mean 10-year decline in AAo distensibility (× 10−3 mmHg−1) 2.4 in BAV vs 1.3 in CTD, p = 0.005]. In contrast, the DAo was more distensible in BAV patients throughout the age spectrum, and DAo distensibility declined with aging at a rate similar to CTD patients [mean 10 year decline in DAo distensibility (× 10−3 mmHg−1) 0.3 in BAV vs 0.4 in CTD, p = 0.58]. Conclusions On serial CMR measurements, AAo distensibility declined at significantly steeper rate in BAV patients compared to a comparison group with CTDs, while DAo distensibility declined at similar rates in both groups. These findings offer new mechanistic insights into the differing pathogenesis of the aortopathy seen in BAV and CTD patients.

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

Annals of Translational Medicine ◽

10.21037/atm.2017.04.26 ◽

2017 ◽

Vol 5 (16) ◽

pp. 333-333

Author(s):

Nicholas S. Burris ◽

Michael D. Hope

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Treatment Options ◽

Aortic Disease

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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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