Abstract 5770: Abnormal Aortic Root Elastic Properties in Family Members of Bicuspid Aortic Valve Subjects, despite Normal Aortic Valve Morphology.

Background: Abnormal aorta dilatation and elastic properties have been documented in subjects with bicuspid aortic valve (BAV). 10% of first degree relatives (FDRs) of probands with BAV inherit this valvular abnormality. There is no data regarding the elastic properties of aortic root in family members of BAV subjects. We evaluated the elastic properties in FDRs with normal tricuspid aortic valves. Methods: Systolic and diastolic aortic root dimensions were measured by 2D echocardiography in BAV probands (n=48) their FDRs (n = 49) and controls without structural heart disease matched by age and gender (n=44) at sinuses of Valsalva level. In addition, systolic (SBP) and diastolic blood pressure (DBP) were measured to calculate aortic distensibility, stiffness index and strain. Results: Five subjects (10%) in FDR group were found to have BAV, thus they were included in BAV group. There was no difference in age, gender, SBP and DBP in the three groups. History of hypertension was less prevalent in FDRs when compared to BAV and controls (16% vs 43% and 33%, p=0.03). All three parameters of aortic elastic properties including distensibility ×10 −3 mmHg (1.2±1.0 and 1.5±1.1 vs 2.3±1.5, p<0.001), stiffness index (56.5±76.4 and 29.1±32.5 vs 12.6±9.2, p<0.001) and strain (2.9±2.4and 3.6±2.5 vs 6.0±3.6, p<0.001) were significantly worse in BAV and FDRs compared to controls (Figure ). Conclusions: Aortic root elastic properties are abnormal in the family members of subjects with BAV despite normal tricuspid aortic valve morphology. This finding supports the hypothesis that there is a common genetic basis for BAV and abnormal elastic properties of aorta.

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ALTERATION OF ELASTIC PROPERTIES IN CONGENITAL/INHERITED AORTIC DISEASES: MRI STUDY IN MARFAN SYNDROME AND BICUSPID AORTIC VALVE

Journal of Mechanics in Medicine and Biology ◽

10.1142/s0219519406001807 ◽

2006 ◽

Vol 06 (01) ◽

pp. 47-53

Author(s):

LUIGI LOVATO ◽

PAOLO SBARZAGLIA ◽

SILVIA MAGI ◽

VINCENZO RUSSO ◽

ROMANO ZANNOLI ◽

...

Keyword(s):

Aortic Valve ◽

Marfan Syndrome ◽

Elastic Properties ◽

Bicuspid Aortic Valve ◽

Aortic Distensibility ◽

Control Group ◽

Aortic Dilation ◽

Aortic Diseases ◽

Magnetic Resonance Imaging Mri ◽

Mri Study

Marfan Syndrome and Bicuspid Aortic Valve (BAV) are characterized by the congenital/inherited alteration of the fibrillin proteins of aortic wall extracellular matrix, with higher incidence of aortic dissection/rupture even without aortic dilation. Therefore, early identification and treatment of aortic involvement could improve prognosis. The purpose of this study was to evaluate aortic elastic properties of BAV and Marfan Syndrome patients to identify an index of early aortic involvement. Methods: A Magnetic Resonance Imaging (MRI) study was performed on 38 BAV patients, 20 Marfan patients and 14 healthy volunteers as a control group. A high-resolution gradient-echo sequence was applied in the axial plane at the level of pulmonary artery bifurcation to evaluate aortic distensibility. Results: Aortic distensibility was significantly different among the three groups (ANOVA, p = 0.0001) and was sensibly reduced in BAV (0.0056 ± 0.0048 versus 0.025 ± 0.006 control group) and Marfan (0.0085 ± 0.006 versus 0.025 ± 0.006 control group). Intraobserver and interobserver reproducibility for diastolic measurements were 1.2% and 0.4% respectively and for systolic measurements, they were 1.1% and 0.3% respectively. Conclusion: In BAV or Marfan Syndrome patients, MRI evaluation of abnormal distensibility may provide a reliable index of early aortic involvement, even before aortic dilation occurs.

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Association of Bicuspid Aortic Valve Morphology and Aortic Root Dimensions: A Substudy of the Aortic Stenosis Progression Observation Measuring Effects of Rosuvastatin (ASTRONOMER) Study

Echocardiography ◽

10.1111/j.1540-8175.2009.00993.x ◽

2010 ◽

Vol 27 (2) ◽

pp. 174-179 ◽

Cited By ~ 21

Author(s):

Davinder S. Jassal ◽

Kapil M. Bhagirath ◽

James W. Tam ◽

Randall A. Sochowski ◽

Jean G. Dumesnil ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Stenosis ◽

Bicuspid Aortic Valve ◽

Aortic Root ◽

Valve Morphology

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P1601 Relationship between aortic distensibility and aortic regurgitation assessed by CMR in bicuspid valve patients

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.1019 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

L La Mura ◽

G Teixido-Tura ◽

A Guala ◽

A Ruiz-Munoz ◽

A Lopez Sainz ◽

...

Keyword(s):

Aortic Valve ◽

Aortic Regurgitation ◽

Bicuspid Aortic Valve ◽

Aortic Root ◽

Phase Contrast ◽

Aortic Distensibility ◽

Regurgitant Fraction ◽

Descending Aorta ◽

Bicuspid Valve ◽

The Impact

Abstract Funding Acknowledgements Spanish Ministry of Economy and Competitiveness RTC-2016-5152-1, ISCIII PI17/00381, La Marató de TV3 (20151330), Eur FP7/People 267128 and CIBERCV BACKGROUND The severity of aortic regurgitation can be evaluated with cardiac magnetic resonance (CMR) through calculation of regurgitant fraction (RF) in phase contrast sequences acquired at the aortic root (as close as possible to the aortic valve). However, the impact of aortic distensibility in this evaluation remains unkown. PURPOSE The aim of the study was to evaluate the relation between aortic distensibility and RF valve in bicuspid aortic valve patients. METHODS We enrolled bicuspid aortic valve patients without significant aortic stenosis (maximum velocity <2.5 m/s) and connective tissue disease. All patients underwent a CMR study with phase contrast sequences for evaluation of regurgitant fraction at the level of the aortic valve. Aortic regurgitation was considered as mild, moderate or severe depending on RF value (mild <15%; moderate 15-30%; severe >30%). Furthermore we used cine-sequences of aortic root, ascending and proximal descending aorta to estimate aortic diameters and distensibilities, using Art Fun software. Distensibility was calculated as (change in aortic area between systole and diastole/diastolic area)/brachial pulse pressure. RESULTS A total of 98 bicuspid aortic valve patients were included (30% female, 49.7 ± 14.5 years). 75 (76,5%) AR was mild, 17 (17,4%) moderate and 6 (6,1%) severe. RF valvewas significantly correlated with aortic root diameter (r= 0.430 y p < 0.001 )and aortic distensibility at the level of the ascending (r = 0.273 p =0.016) and descending aorta (r = 0.502 and p< 0.001). Aortic distensibility was positively correlated with RFvalve even after adjustment for aortic diameter ( p = 0.002 and p <0.001 respectively) . (Table) (IMG) CONCLUSIONS In our study, aortic regurgitation in bicuspid valve patients, evaluated by CMR using RF valve, is related to aortic distensibility. Thus, aortic distensibility should be included in the evaluation of aortic regurgitation by CMR as additional parameter. However, longitudinal studies are needed to evaluate the impact of including aortic distensibility in the evaluation of AR severity by CMR. AR SEVERITY MILD MODERATE SEVERE Descending aorta distensibility(mean ± std. deviation) 2693,68 ± 997,5 3285,8 ±1952,7 5042,99 ±2873,44 Correlation between AR severity (by RFvalve) and descending aorta distensibility Abstract P1601 Figure.

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Faculty Opinions recommendation of The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1128803.585884 ◽

2008 ◽

Author(s):

Bernard Iung

Keyword(s):

Aortic Valve ◽

Bicuspid Aortic Valve ◽

Aortic Root ◽

Phenotypic Classification

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Rapid ascending aorta stiffening in bicuspid aortic valve on serial cardiovascular magnetic resonance evaluation: comparison with connective tissue disorders

Journal of Cardiovascular Magnetic Resonance ◽

10.1186/s12968-021-00716-5 ◽

2021 ◽

Vol 23 (1) ◽

Author(s):

Alejandro Perez-Casares ◽

Audrey Dionne ◽

Kimberlee Gauvreau ◽

Ashwin Prakash

Keyword(s):

Cardiovascular Magnetic Resonance ◽

Magnetic Resonance ◽

Aortic Valve ◽

Connective Tissue ◽

Bicuspid Aortic Valve ◽

Aortic Stiffness ◽

Comparison Group ◽

Aortic Distensibility ◽

Connective Tissue Disorders ◽

Aortic Stiffening

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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