title: Characteristics and long-term outcomes of contemporary patients with bicuspid aortic valves.
abstract: BACKGROUND
  We sought to study the impact of surgical intervention on long-term outcomes in bicuspid aortic valve (BAV) patients who develop aortic valvular complications and/or have a concomitant aortopathy.
  
  
  METHODS
  We studied 1890 consecutive patients with BAV (age 50 ± 14 years; 75% men), evaluated in the time period 2003 to 2007. Clinical and imaging data were recorded. The endpoint was a composite of death or type A aortic dissection.
  
  
  RESULTS
  The mean left ventricular ejection fraction was 55% ± 8%; 31% had New York Heart Association class ≥ III aortic regurgitation, and 17% had mean aortic valve (AV) gradient ≥30 mm Hg. Dilated (≥4 cm) aortic root and ascending aorta were observed in 35% and 42% of patients, respectively. At 8.1 ± 2 years, 918 (49%) patients underwent surgery (883 had AV replacement and/or repair (± combination procedures), 471 with ascending aortic grafting (30 had isolated aortic grafting), and 171 (9%) events (169 deaths and 2 dissections) occurred; 0.4% was the 30-day mortality). On multivariable Cox survival analysis, increasing age (hazard ratio [HR] 1.06, 95% confidence interval [CI] [1.05-1.07]), increasing aortic root size (HR 1.32, 95% CI [1.07-1.65]), decreasing LV ejection fraction (HR 1.04, 95% CI [1.03-1.05]), and hyperlipidemia (HR 1.51, 95% CI [1.20-1.89]) had higher associated events, and surgery related to BAV (time-dependent covariate) (HR 0.44, 95% CI [0.31-0.70]) was associated with significantly lower events (all P < .01). Addition of surgery to BAV risk score (a composite of age, ≥moderate-severe aortic stenosis or regurgitation) and aortic root size further improved risk stratification (the C-statistic increased from 0.65 to 0.73; P = .01).
  
  
  CONCLUSIONS
  Patients with BAV have a high prevalence of AV dysfunction and concomitant aortopathy. Undergoing surgery (AV replacement and/or repair ± AAR) was associated with a significantly lower incidence of death and/or dissection.
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Ahmad
authors.initials: A
authors.lastname: Masri
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Vidyasagar
authors.initials: V
authors.lastname: Kalahasti
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Saqer
authors.initials: S
authors.lastname: Alkharabsheh
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Lars G
authors.initials: LG
authors.lastname: Svensson
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Joseph F
authors.initials: JF
authors.lastname: Sabik
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Eric E
authors.initials: EE
authors.lastname: Roselli
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Donald
authors.initials: D
authors.lastname: Hammer
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Douglas R
authors.initials: DR
authors.lastname: Johnston
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Patrick
authors.initials: P
authors.lastname: Collier
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: L Leonardo
authors.initials: LL
authors.lastname: Rodriguez
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
authors.forename: Brian P
authors.initials: BP
authors.lastname: Griffin
authors.affiliation: Valve Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address: desaim2@ccf.org.
authors.forename: Milind Y
authors.initials: MY
authors.lastname: Desai
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D001807:Blood Vessel Prosthesis; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006350:Heart Valve Prosthesis; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D061330:Multidetector Computed Tomography; D009820:Ohio / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013997:Time Factors
keywords: and outcomes; aortopathy; bicuspid aortic valve; surgery