title: Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy.
abstract: Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality.
  
  
  
  To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality).
  
  
  
  In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study.
  
  
  
  Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction.
  
  
  
  Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection.
  
  
  
  Of the 2118 patients (mean [SD] age, 47 [18] years; 1525 [72.0%] male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 [33.1%] vs 57 [24.1%], P < .001) and aortic stenosis (728 [38.7%] vs 51 [21.5%], P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 [19.9%] at 1 year, 393 [21.4%] at 2 years, and 447 [24.4%] at 5 years) vs patients without raphe (30 [14.0%] at 1 year, 32 [15.0%] at 2 years, and 40 [18.0%] at 5 years) (P = .02). In addition, the all-cause mortality event rates were significantly higher among patients with BAV with raphe (77 [5.1%] at 1 year, 87 [6.2%] at 2 years, and 110 [9.5%] at 5 years) vs patients without raphe (2 [1.8%] at 1 year, 3 [3.0%] at 2 years, and 5 [4.4%] at 5 years) (P = .03). However, on multivariable analysis, the presence of raphe was not significantly associated with all-cause mortality.
  
  
  
  In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands2Department of Cardiology, National University Heart Center, National University Health System, Singapore.
authors.forename: William K F
authors.initials: WK
authors.lastname: Kong
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Victoria
authors.initials: V
authors.lastname: Delgado
authors.affiliation: Department of Cardiology, National University Heart Center, National University Health System, Singapore3Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors.forename: Kian Keong
authors.initials: KK
authors.lastname: Poh
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Madelien V
authors.initials: MV
authors.lastname: Regeer
authors.affiliation: Department of Cardiology, Princess Alexandra Hospital, The University of Queensland, Queensland, Australia.
authors.forename: Arnold C T
authors.initials: AC
authors.lastname: Ng
authors.affiliation: Department of Cardiology, Princess Alexandra Hospital, The University of Queensland, Queensland, Australia.
authors.forename: Louise
authors.initials: L
authors.lastname: McCormack
authors.affiliation: Department of Cardiology, National University Heart Center, National University Health System, Singapore3Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors.forename: Tiong Cheng
authors.initials: TC
authors.lastname: Yeo
authors.affiliation: Division of Cardiology, University of Alberta, Mazankowski Alberta Heart Institute, Alberta, Canada.
authors.forename: Miriam
authors.initials: M
authors.lastname: Shanks
authors.affiliation: Division of Cardiology, University of Alberta, Mazankowski Alberta Heart Institute, Alberta, Canada.
authors.forename: Sarah
authors.initials: S
authors.lastname: Parent
authors.affiliation: University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Institute of Cardiovascular Diseases "Prof Dr C. C. Iliescu," Bucharest, Romania.
authors.forename: Roxana
authors.initials: R
authors.lastname: Enache
authors.affiliation: University of Medicine and Pharmacy "Carol Davila"-Euroecolab, Institute of Cardiovascular Diseases "Prof Dr C. C. Iliescu," Bucharest, Romania.
authors.forename: Bogdan A
authors.initials: BA
authors.lastname: Popescu
authors.affiliation: Department of Cardiology, National University Heart Center, National University Health System, Singapore7Department of Cardiology, Khoo Teck Puat Hospital, Singapore.
authors.forename: Michael
authors.initials: M
authors.lastname: Liang
authors.affiliation: Department of Cardiology, National University Heart Center, National University Health System, Singapore3Yong Loo Lin School of Medicine, National University of Singapore, Singapore.
authors.forename: James W
authors.initials: JW
authors.lastname: Yip
authors.affiliation: Department of Cardiology, Princess Alexandra Hospital, The University of Queensland, Queensland, Australia.
authors.forename: Lawrence C W
authors.initials: LC
authors.lastname: Ma
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands8Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece.
authors.forename: Vasileios
authors.initials: V
authors.lastname: Kamperidis
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Philippe J
authors.initials: PJ
authors.lastname: van Rosendael
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Enno T
authors.initials: ET
authors.lastname: van der Velde
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Nina
authors.initials: N
authors.lastname: Ajmone Marsan
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Jeroen J
authors.initials: JJ
authors.lastname: Bax
mesh_terms: D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D002423:Cause of Death / Q000639:trends; D004452:Echocardiography; D005260:Female; D014943:Global Health; D006349:Heart Valve Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D019918:Heart Valve Prosthesis Implantation / Q000639:trends*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends