title: The extent of the raphe in bicuspid aortic valves is associated with aortic regurgitation and aortic root dilatation.
abstract: BACKGROUND
  The clinical course of bicuspid aortic valves (BAVs) is variable. Data on predictors of aortopathy and valvular dysfunction mainly focus on valve morphology.
  
  
  OBJECTIVE
  To determine whether the presence and extent of the raphe (fusion site of valve leaflets) is associated with the degree of aortopathy and valvular dysfunction in patients with isolated BAV and associated aortic coarctation (CoA).
  
  
  METHODS
  Valve morphology and aortic dimensions of 255 BAV patients were evaluated retrospectively by echocardiography.
  
  
  RESULTS
  BAVs with a complete raphe had a significantly higher prevalence of valve dysfunction (especially aortic regurgitation) than BAVs with incomplete raphes (82.9 vs. 66.7 %, p = 0.01). Type 1A BAVs (fusion of right and left coronary leaflets) and complete raphe had larger aortic sinus diameters compared with the rest of the population (37.74 vs. 36.01, p = 0.031). Patients with CoA and type 1A BAV had significantly less valve regurgitation (13.6 vs. 55.8 %, p < 0.001) and smaller diameters of the ascending aorta (33.7 vs. 37.8 mm, p < 0.001) and aortic arch (25.8 vs. 30.2 mm, p < 0.001) than patients with isolated BAV.
  
  
  CONCLUSIONS
  Type 1A BAV with complete raphe is associated with more aortic regurgitation and root dilatation. The majority of CoA patients have incomplete raphes, associated with smaller aortic root diameters and less valve regurgitation.
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
authors.forename: W M C
authors.initials: WM
authors.lastname: Koenraadt
authors.affiliation: Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, The Netherlands.|Department of Anatomy & Embryology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The Netherlands.
authors.forename: N
authors.initials: N
authors.lastname: Grewal
authors.affiliation: Department of Anatomy & Embryology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The Netherlands.
authors.forename: O Y
authors.initials: OY
authors.lastname: Gaidoukevitch
authors.affiliation: Department of Anatomy & Embryology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The Netherlands.
authors.forename: M C
authors.initials: MC
authors.lastname: DeRuiter
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.|Department of Anatomy & Embryology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The Netherlands.
authors.forename: A C
authors.initials: AC
authors.lastname: Gittenberger-de Groot
authors.affiliation: Department of Anatomy & Embryology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The Netherlands.
authors.forename: M M
authors.initials: MM
authors.lastname: Bartelings
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
authors.forename: E R
authors.initials: ER
authors.lastname: Holman
authors.affiliation: Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: R J M
authors.initials: RJ
authors.lastname: Klautz
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: Schalij
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands. m.r.m.jongbloed@lumc.nl.|Department of Anatomy & Embryology, Leiden University Medical Center, PO Box 9600, 2300 RC, Leiden, The Netherlands. m.r.m.jongbloed@lumc.nl.
authors.forename: M R M
authors.initials: MR
authors.lastname: Jongbloed
keywords: Aortic diseases; Echocardiography; Heart defects, Congenital; Valvular heart disease