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Simultaneous Sessions: Congenital Heart Disease
Aortic Dilatation is Associated with Aortic Valve Dysfunction in Patients with Bicuspid Aortic Valve Byron K Yip, Colleen Clennon, Jeremy Collins, Paul WM Fedak, Robert O Bonow, Alex J Barker, Adin-Cristian Andrei, S. Chris Malaisrie MD Monday, April 27, 2015 Simultaneous Sessions: Congenital Heart Disease AATS 95th Annual Meeting Seattle, WA
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Disclosures Adin-Cristian Andrei, PhD: Consultant, AtriCure
S. Chris Malaisrie, MD: Consultant/Speaker: Edwards Lifesciences, Baxter, Abiomed, Bolton None for other authors
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Background Congenital versus acquired?
Bicuspid aortic valve (BAV) prevalence of 0.5-2%1 Associated with earlier and more frequent development of:2 Aortic stenosis (AS) Aortic regurgitation (AR) Thoracic aortic aneurysm Aortic dilatation most common vascular abnormality in BAV3 Pathophysiology of BAV aortopathy still not well understood Congenital versus acquired? 1 Siu SC, Silversides CK. Bicuspid aortic valve disease. Journal of the American College of Cardiology. 2010;55: 2 Wassmuth R, von Knobelsdorff-Brenkenhoff F, Gruettner H, Utz W, Schulz-Menger J. Cardiac magnetic resonance imaging of congenital bicuspid aortic valves and associated aortic pathologies in adults. European heart journal cardiovascular Imaging. 2014;15:673-9 3 Cecconi M, Manfrin M, Moraca A, Zanoli R, Colonna PL, Bettuzzi MG, et al. Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction. The American journal of cardiology. 2005;95:292-4.
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Objective: To determine the association of ascending aortic dilatation with aortic valve dysfunction in a cohort of patients with BAV 4
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Materials and Methods Retrospective medical chart review
Study period: October 2003 – November 2013 Inclusion criteria Age 18-85 Known or incidentally diagnosed BAV Cardiac magnetic resonance (CMR) imaging & transthoracic echo (TTE) No prior history of intervention involving the AV or aorta No concomitant genetic syndromes involving the aorta Imaging data collected CMR: Max ascending aortic diameters (AAoD) from aortic root, tubular ascending aorta, and proximal aortic arch TTE: Severity grading for AV dysfunction AS: None/trace, mild, moderate, severe AR: None/trace, mild, moderate, moderate-severe, severe CMR to diagnose BAV TEE to determine valve dysfunction 5
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Results n=373 BAV patients Mean age: 47 ± 13 years Gender: 69% male 6
730 patients total 440 had both CMR and TEE for analysis Excluded: 31 CoA, 34 with previous AV surgery, 2 MFS/Turners, 20 inadequate CMR 6
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Results Control Group: Patients with no AS and no AR
Study Group: Patients with any severity of AS or AR Subset analysis: 1. Patients with AS 2. Patients with AR
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Results n=373 BAV patients Mean age: 47 ± 13 years Gender: 69% male
Mean height and weight: 174 cm and 81 kg 730 patients total 440 had both CMR and TEE for analysis Excluded: 31 CoA, 34 with previous AV surgery, 2 MFS/Turners, 20 inadequate CMR 8
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Results Location of dilatation = root (46%), asc (53%), arch (1%)
Mean diameters: root (4.1cm), asc (4.2cm), arch (3.9cm) Max diameter in Study vs Control: 4.0 cm vs 4.2 cm 730 patients total 440 had both CMR and TEE for analysis Excluded: 31 CoA, 34 with previous AV surgery, 2 MFS/Turners, 20 inadequate CMR 9
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AAoD: No AS/AR vs. AS/AR
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AAoD: No AR vs. AR
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AAoD: AR severity
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Aortic root: AR severity
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Gender-stratified analyses
AS: Significant difference in AAoD between No AS vs. AS only in women (p=0.03) AR: No significant differences with or without AR in men or women AS/AR: No significant differences with or without AS/AR in men or women
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Discussion Present study findings:
Significant association between ascending aortic dimensions and presence of AV dysfunction in BAV patients Stratified by segment, aortic root dilatation strongly associated with AR No significant association between maximal AAoD and presence or severity of AS, except in women GREEN: No AF YELLOW: TrAF RED: Not treated AF Keep consistent throughout the presentation 15
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Discussion Previous findings on BAV-associated aortic dilatation and AV dysfunction have varied Most studies have used echo as imaging modality of reference Associative findings Non-associative findings GREEN: No AF YELLOW: TrAF RED: Not treated AF Keep consistent throughout the presentation 16
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Discussion (cont’d) Recent studies using CMR as imaging modality of reference CMR provides accurate and detailed imaging of BAV and aorta, especially when echo is indeterminate (Malaisrie et al., 2012) Recent 4-D MRI study findings Altered wall shear stress (WSS) is exerted on ascending aorta and influenced by different BAV fusion patterns (Barker et al., 2012) Altered hemodynamics in ascending aorta associated with expression of BAV aortopathy (Mahadevia et al., 2014) GREEN: No AF YELLOW: TrAF RED: Not treated AF Keep consistent throughout the presentation 17
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4D Flow MRI BAV with RN fusion (no AS)
Mahadevia R, Barker AJ, Schnell S, Entezari P, Kansal P, Fedak PW, Malaisrie SC, McCarthy P, Collins J, Carr J, Markl M. Bicuspid aortic cusp fusion morphology alters aortic three-dimensional outflow patterns, wall shear stress, and expression of aortopathy. Circulation. 2014;129:
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Study Limitations Single tertiary referral center
Imaging guideline changes over 10-year study period (e.g. timing of first CMR, extent of clinical data collected) Reliance on CMR without surgical and pathological ID of BAV Retrospective study design reason for initial presentation for medical care time between initial BAV dx and referral for CMR Intra- and inter-observer variability in imaging evaluations Non-standardized imaging equipment and techniques GREEN: No AF YELLOW: TrAF RED: Not treated AF Keep consistent throughout the presentation 19
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Conclusions Aortic dilatation is associated with presence of AV dysfunction in BAV patients Presence and severity of AR is associated with aortic root diameters Presence of AS is associated with aortic dilatation in women only Hemodynamically-significant valve dysfunction contributes to progression of aortic dilatation in BAV patients
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