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Aortic Dilatation is Associated withAortic Valve Dysfunction in Patientswith Bicuspid Aortic ValveByron K Yip, Colleen Clennon, Jeremy Collins, Paul WM Fedak, Robert O Bonow, Alex J Barker, Adin-Cristian Andrei, S. Chris Malaisrie MDMonday, April 27, 2015Simultaneous Sessions: Congenital Heart DiseaseAATS 95th Annual MeetingSeattle, WA DisclosuresAdin-Cristian Andrei, PhD: Consultant, AtriCure

S. Chris Malaisrie, MD: Consultant/Speaker: Edwards Lifesciences, Baxter, Abiomed, Bolton

None for other authorsBackgroundBicuspid aortic valve (BAV) prevalence of 0.5-2%1

Associated with earlier and more frequent development of:2Aortic stenosis (AS)Aortic regurgitation (AR)Thoracic aortic aneurysm

Aortic dilatation most common vascular abnormality in BAV3

Pathophysiology of BAV aortopathy still not well understoodCongenital versus acquired?

1 Siu SC, Silversides CK. Bicuspid aortic valve disease. Journal of the American College of Cardiology. 2010;55:2789-800. 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;15:673-93 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.Objective:To determine the association of ascending aortic dilatation with aortic valve dysfunction in a cohort of patients with BAV4Materials and Methods5Retrospective medical chart reviewStudy period: October 2003 November 2013Inclusion criteriaAge 18-85Known or incidentally diagnosed BAVCardiac magnetic resonance (CMR) imaging & transthoracic echo (TTE)No prior history of intervention involving the AV or aortaNo concomitant genetic syndromes involving the aortaImaging data collectedCMR: Max ascending aortic diameters (AAoD) from aortic root, tubular ascending aorta, and proximal aortic archTTE: Severity grading for AV dysfunctionAS: None/trace, mild, moderate, severeAR: None/trace, mild, moderate, moderate-severe, severeCMR to diagnose BAVTEE to determine valve dysfunctionResults6n=373 BAV patientsMean age: 47 13 yearsGender: 69% male730 patients total440 had both CMR and TEE for analysisExcluded: 31 CoA, 34 with previous AV surgery, 2 MFS/Turners, 20 inadequate CMRResults

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

Results8n=373 BAV patients

Mean age: 47 13 yearsGender: 69% maleMean height and weight: 174 cm and 81 kg

730 patients total440 had both CMR and TEE for analysisExcluded: 31 CoA, 34 with previous AV surgery, 2 MFS/Turners, 20 inadequate CMRResults9Location 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 total440 had both CMR and TEE for analysisExcluded: 31 CoA, 34 with previous AV surgery, 2 MFS/Turners, 20 inadequate CMRAAoD: No AS/AR vs. AS/AR

AAoD: No AR vs. AR

AAoD: AR severity

Aortic root: AR severity

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 womenDiscussion15Present 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 womenGREEN: No AFYELLOW: TrAFRED: Not treated AF

Keep consistent throughout the presentationDiscussion16Previous findings on BAV-associated aortic dilatation and AV dysfunction have varied

Most studies have used echo as imaging modality of referenceAssociative findings

Non-associative findings

GREEN: No AFYELLOW: TrAFRED: Not treated AF

Keep consistent throughout the presentationDiscussion (contd)17Recent 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 findingsAltered 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 AFYELLOW: TrAFRED: Not treated AF

Keep consistent throughout the presentation

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;129:673-682

Study Limitations19Single tertiary referral centerImaging 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 BAVRetrospective study designreason for initial presentation for medical caretime between initial BAV dx and referral for CMRIntra- and inter-observer variability in imaging evaluationsNon-standardized imaging equipment and techniquesGREEN: No AFYELLOW: TrAFRED: Not treated AF

Keep consistent throughout the presentationConclusionsAortic 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