the natural history of the bicuspid aortic valve and ...€¦ · the natural history of the...
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The natural history of the
bicuspid aortic valve and
bicuspid aortopathy
Paul W.M. Fedak, MD PhD FRCSC FAHA
Associate Professor, University of Calgary
Libin Cardiovascular Institute of Alberta
Adjunct Associate Professor, Northwestern University
Director of Translational Research,
Martha and Richard Melman Bicuspid Aortic Valve Program
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No Disclosures
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Clinical Problem
• Burden of surgery for BAV patients in the USA exceeds 1 billion dollars per year
• Frequency of aortic interventions has doubled over the past decade
• There is limited scientific evidence for the timing and extent of these prophylactic interventions
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Uncertainty of Clinical Guidelines for BAV Aortopathy
Hardikar AA, Marwick TH. Int J Cardiol 2015;199:150-153.
1998 2014
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Genetics
Hemodynamics
VS
Surgical approaches are aggressive for genetic aortopathy
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- Common in young asymptomatic patients
- Seen with “normal” BAV function (No AS/AR)
- Progression of dilatation after AVR
- Some similarities to known genetic aortopathies
Inherited Aortopathy?
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Beroukhim … Yetman, AJC 2006
Aortic Dilation: BAV versus TAV in Children
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AO
RTIC
MED
IA
Tricuspid AV (TAV) Bicuspid AV (BAV)
Vascular Matrix Disruption in BAV Disease
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Circ Res 2001; 88(1): 37
Fibrillin-1 Deficient Mouse:Matrix Disruption in Aortic Media
Normal Mouse Fbn-1 Deficient Mouse
Disrupted MatrixAortic Aneurysm
Normal MatrixNo Aortic Dilation
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JTCVS 1999; 118: 588
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Molecular Mechanisms of BAV Aortopathy
Fedak et al. Circulation 2002
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• 52% believed genetics, 2% believed valve hemodynamics
• 61% believed in resection at 45mm at time of AVR
• 33% performed resection at <45mm at time of AVR
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Perspectives and attitudes on the
etiology (inherited aortopathy versus
acquired from hemodynamic stress)
rather than validated scientific and
clinical evidence appeared to influence
the surgical treatment of BAV
aortopathy
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Is progression of BAV aortopathy
predictable for individual patients?
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Variable Patterns of BAV Aortopathy
ROOT
MID ASCPROX ARCH
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Variable BAV Cusp Morphology
Sabet et al, Mayo Clin Proc, 1994;74:14-26
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Mahadevia et al. Circ 2014: 673-82. / Verma & Siu. NEJM 2014: 1920-9.
RL fusion RN fusion
Valve Fusion and Flow Pattern
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Risk of Aneurysm Formation and Aortic Dissection After Definite Bicuspid Aortic Valve Diagnosis
Michelena, H. I. et al. JAMA 2011;306:1104-1112
Copyright restrictions may apply.
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Can valve-mediated
hemodynamic stress be
assessed in the ascending
aorta of patients with BAV
disease?
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• Res. ~ 2mm isotropic, TRes ~ 40ms • TAcq ~ 8 - 15min
4D Flow MRI Methods
Buonocore MH. Magn Reson Med 1998;40:210-226Napel S et al. J Magn Reson Imaging 1992;2:143-153Markl M, et al. J Magn Reson Imaging 2007; 25:824-831
4D (3D volume + time); flow (3-dir. velocity encoding) MRI
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Barker et al. Circ Cardiovasc Imaging 2012: 457-66.
Tricuspid AV (TAV)
(No valve dysfunction)
Bicuspid AV (BAV)
(No valve dysfunction)
BAV without dysfunction has
significant flow eccentricity
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23Barker AJ, Markl M, et al. Circulation: Cardiovascular Imaging 2012. 5(4): p. 457-66.
Flow eccentricity creates “shear”
on inner surface of aortic wall
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Wall Shear Stress (WSS)• Hemodynamic WSS is the tangential force exerted on the
endothelium due to a blood velocity gradient (dV/dr) and blood viscosity (μ)
• The drag force exerted on a vessel wall
WSS
Axial WSS
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Individual Patient: Detect regions with abnormal WSS
BAV patient
3D WSS 3D Ao geometry
WSS [Pa]
Normal WSS
Healthy Atlas
WSS heat map
WSS WSSNormal WSS
WSS WSSNormal WSS
WSS WSSNormal WSS
±95% CI
Registration & interpolation
van Ooij et al, ABME 2015. 43(6): p. 1385-1397
WSS [Pa]
WSS Atlases and Heatmaps
FLIRT
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Mahadevia et al. Circ 2014: 673-82. / Verma & Siu. NEJM 2014: 1920-9.
RL fusion RN fusion
Does local tissue tissue remodeling follow
patterns of valve-mediated hemodynamics?
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Valve-mediated hemodynamics
predict local tissue remodeling
Guzzardi, Barker, Fedak et al, JACC 2015
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Key Points
• BAV aortopathy with progressive dilation of ascending aorta is common
• Patterns and rates of progression are highly variable between patients
• Risk of fatal aortic events is low
• Valve-mediated hemodynamics may play an important role in progression
• Patient-specific resection strategies are needed to avoid unnecessary surgery