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Comprehensive 4-stage categorization of bicuspid aortic valve leaflet morphology by cardiac MRI in 386 patients

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Abstract

Bicuspid aortic valve (BAV) disease is heterogeneous and related to valve dysfunction and aortopathy. Appropriate follow up and surveillance of patients with BAV may depend on correct phenotypic categorization. There are multiple classification schemes, however a need exists to comprehensively capture commissure fusion, leaflet asymmetry, and valve orifice orientation. Our aim was to develop a BAV classification scheme for use at MRI to ascertain the frequency of different phenotypes and the consistency of BAV classification. The BAV classification scheme builds on the Sievers surgical BAV classification, adding valve orifice orientation, partial leaflet fusion and leaflet asymmetry. A single observer successfully applied this classification to 386 of 398 Cardiac MRI studies. Repeatability of categorization was ascertained with intraobserver and interobserver kappa scores. Sensitivity and specificity of MRI findings was determined from operative reports, where available. Fusion of the right and left leaflets accounted for over half of all cases. Partial leaflet fusion was seen in 46% of patients. Good interobserver agreement was seen for orientation of the valve opening (κ = 0.90), type (κ = 0.72) and presence of partial fusion (κ = 0.83, p < 0.0001). Retrospective review of operative notes showed sensitivity and specificity for orientation (90, 93%) and for Sievers type (73, 87%). The proposed BAV classification schema was assessed by MRI for its reliability to classify valve morphology in addition to illustrating the wide heterogeneity of leaflet size, orifice orientation, and commissural fusion. The classification may be helpful in further understanding the relationship between valve morphology, flow derangement and aortopathy.

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Abbreviations

BAV:

Bicuspid aortic valve

MRI:

Magnetic resonance imaging

PC:

Phase contrast

bSSFP:

Balanced steady-state free precession

GRAPPA:

Generalized autocalibrating partially parallel acquisition

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Acknowledgements

Research reported in this publication was supported by the National Heart, Lung and Blood Institute of the National Institutes of Health under Award Number K25HL119608.

Funding

NIH NHLBI grants R01HL115828 and NIH K25HL119608. Additional support by the Martha and Richard Melman Family Bicuspid Bicuspid Aortic Valve Program at Northwestern’s Bluhm Cardiovascular Institute.

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Authors and Affiliations

  1. Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA

    I. G. Murphy, J. Collins, A. Powell, M. Markl, J. C. Carr & A. J. Barker

  2. Department Biomedical Engineering, McCormick School of Engineering, Northwestern University, Chicago, IL, USA

    M. Markl

  3. Division of Cardiac Surgery, Martha and Richard Melman Family Bicuspid Aortic Valve Program at Northwestern Medicine’s Bluhm Cardiovascular Institute, Northwestern Memorial Hospital, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    P. McCarthy & S. C. Malaisrie

Authors
  1. I. G. Murphy
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  2. J. Collins
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  3. A. Powell
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  4. M. Markl
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  5. P. McCarthy
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  6. S. C. Malaisrie
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  7. J. C. Carr
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  8. A. J. Barker
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Correspondence to A. J. Barker.

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There is no conflict of interest, financial or otherwise, for any of the authors of this study.

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Murphy, I.G., Collins, J., Powell, A. et al. Comprehensive 4-stage categorization of bicuspid aortic valve leaflet morphology by cardiac MRI in 386 patients. Int J Cardiovasc Imaging 33, 1213–1221 (2017). https://doi.org/10.1007/s10554-017-1107-1

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  • DOI: https://doi.org/10.1007/s10554-017-1107-1

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