title: Comprehensive 4-stage categorization of bicuspid aortic valve leaflet morphology by cardiac MRI in 386 patients.
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.
authors.affiliation: Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA.
authors.forename: I G
authors.initials: IG
authors.lastname: Murphy
authors.affiliation: Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA.
authors.forename: J
authors.initials: J
authors.lastname: Collins
authors.affiliation: Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA.
authors.forename: A
authors.identifier: http://orcid.org/0000-0003-2480-7207
authors.initials: A
authors.lastname: Powell
authors.affiliation: Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA.|Department Biomedical Engineering, McCormick School of Engineering, Northwestern University, Chicago, IL, USA.
authors.forename: M
authors.initials: M
authors.lastname: Markl
authors.affiliation: 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.
authors.forename: P
authors.initials: P
authors.lastname: McCarthy
authors.affiliation: 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.
authors.forename: S C
authors.initials: SC
authors.lastname: Malaisrie
authors.affiliation: Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA.
authors.forename: J C
authors.initials: JC
authors.lastname: Carr
authors.affiliation: Department of Radiology, Feinberg School of Medicine, Northwestern University, 737 N. Michigan Ave, Suite 1600, Chicago, IL, 60611, USA. alex.barker@northwestern.edu.
authors.forename: A J
authors.initials: AJ
authors.lastname: Barker
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D000082882:Bicuspid Aortic Valve Disease; D055424:Cardiac-Gated Imaging Techniques; D004562:Electrocardiography; D005240:Feasibility Studies; D005260:Female; D006349:Heart Valve Diseases / Q000145:classification / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006801:Humans; D018810:Magnetic Resonance Angiography; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D010641:Phenotype; D011237:Predictive Value of Tests; D015203:Reproducibility of Results; D009626:Terminology as Topic
keywords: Aortopathy; Bicuspid aortic valve; MRI; Valve disease