title: Nonsyndromic Thoracic Aortic Aneurysm and Dissection: Outcomes With Marfan Syndrome Versus Bicuspid Aortic Valve Aneurysm.
abstract: BACKGROUND
  Genetic aortopathy (GA) underlies thoracic aortic aneurysms (TAA) in younger adults. Comparative survival and predictors of outcomes in nonsyndromic TAA (NS-TAA) are incompletely defined compared to Marfan syndrome (MFS) and bicuspid aortic valve (BAV).
  
  
  OBJECTIVE
  The study sought to compare survival and clinical outcomes for individuals with NS-TAA, MFS, and BAV.
  
  
  METHODS
  From 1988 to 2014, all patients presenting with GA 16 to 60 years of age were enrolled in a prospective study of clinical outcomes. Risk factors for death and aortic dissection were identified by Cox proportional hazards modeling and a mortality risk score developed.
  
  
  RESULTS
  Diagnosis of GA was made for 760 patients (age 36.9 ± 13.6 years, 26.8% female; NS-TAA, n = 311; MFS, n = 221; BAV, n = 228). MFS patients were younger than NS-TAA and BAV. Presentation with aortic dissection was more common for NS-TAA than MFS or BAV. The 687 patients surviving >30 days after presentation were followed for a median of 7 years. Calculated 10-year mortality was 7.8% for NS-TAA, 8.7% for MFS, and 3.5% for BAV (NS-TAA and MFS vs. BAV p <0.05). Factors associated with all-cause mortality were MFS (p = 0.04), age at presentation, and family history of dissection.
  
  
  CONCLUSIONS
  Clinical outcomes for MFS and NS-TAA are similar but worse than BAV. Independent predictors of mortality, including family history of aortic dissection and age, can be included in an Aortopathy Mortality Risk Score to predict survival. Management of NS-TAA, including surgical intervention, should be similar to that of MFS.
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia.
authors: Andrew G
authors: AG
authors: Sherrah
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia.
authors: Sarah
authors: S
authors: Andvik
authors: Sydney Medical School, University of Sydney, Sydney, Australia.
authors: Denise
authors: D
authors: van der Linde
authors: NHMRC Clinical Trials Centre, University of Sydney, Sydney, Australia.
authors: Lucy
authors: L
authors: Davies
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia; Department of Cardiothoracic Surgery, Royal Prince Alfred Hospital, Sydney, Australia.
authors: Paul G
authors: PG
authors: Bannon
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia.
authors: Ratnasari
authors: R
authors: Padang
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia; Department of Cardiothoracic Surgery, Royal Prince Alfred Hospital, Sydney, Australia.
authors: Michael P
authors: MP
authors: Vallely
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia; Department of Cardiothoracic Surgery, Royal Prince Alfred Hospital, Sydney, Australia.
authors: Michael K
authors: MK
authors: Wilson
authors: Sydney Medical School, University of Sydney, Sydney, Australia; NHMRC Clinical Trials Centre, University of Sydney, Sydney, Australia.
authors: Anthony C
authors: AC
authors: Keech
authors: The Baird Institute for Applied Heart and Lung Surgical Research, and Sydney Medical School, University of Sydney, Sydney, Australia. Electronic address: richmond.jeremy@sydney.edu.au.
authors: Richmond W
authors: RW
authors: Jeremy
mesh_terms: D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000150:complications* / Q000401:mortality / Q000601:surgery; D017545:Aortic Aneurysm, Thoracic / Q000150:complications* / Q000401:mortality; D001021:Aortic Valve / Q000002:abnormalities* / Q000473:pathology; D001315:Australia / Q000453:epidemiology; D000082882:Bicuspid Aortic Valve Disease; D004108:Dilatation, Pathologic; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality / Q000601:surgery; D008875:Middle Aged; D011446:Prospective Studies; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D014656:Vascular Surgical Procedures / Q000379:methods; D055815:Young Adult
keywords: aortic surgery; genetic aortopathy; mortality; risk