title: Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome.
abstract: BACKGROUND
  Bicuspid aortic valves are associated with valve dysfunction, ascending aortic aneurysm and dissection. Management of the ascending aorta at the time of aortic valve replacement (AVR) in these patients is controversial and has been extrapolated from experience with Marfan syndrome, despite the absence of comparative long-term outcome data.
  
  
  OBJECTIVE
  This study sought to assess whether the natural history of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease is substantially different from that seen in patients with Marfan syndrome.
  
  
  METHODS
  In this retrospective comparison, outcomes of 13,205 adults (2,079 with bicuspid aortic valves, 73 with Marfan syndrome, and 11,053 control patients with acquired aortic valve disease) who underwent primary AVR without replacement of the ascending aorta in New York State between 1995 and 2010 were compared. The median follow-up time was 6.6 years.
  
  
  RESULTS
  The long-term incidence of thoracic aortic dissection was significantly higher in patients with Marfan syndrome (5.5 ± 2.7%) compared with those with bicuspid valves (0.55 ± 0.21%) and control group patients (0.41 ± 0.08%, p < 0.001). Thoracic aortic aneurysms were significantly more likely to be diagnosed in late follow-up in patients with Marfan syndrome (10.8 ± 4.4%) compared with those with bicuspid valves (4.8 ± 0.8%) and control group patients (1.4 ± 0.2%) (p < 0.001). Patients with Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%) compared with those with bicuspid valves (2.5 ± 0.6%) and control group patients (0.50 ± 0.09%) (p < 0.001).
  
  
  CONCLUSIONS
  The much higher long-term rates of aortic complications after AVR observed in patients with Marfan syndrome compared with those with bicuspid aortic valves confirm that operative management of patients with bicuspid aortic valves should not be extrapolated from Marfan syndrome and support discrete treatment algorithms for these different clinical entities.
authors.affiliation: Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Shinobu
authors.initials: S
authors.lastname: Itagaki
authors.affiliation: Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York. Electronic address: joanna.chikwe@mountsinai.org.
authors.forename: Joanna P
authors.initials: JP
authors.lastname: Chikwe
authors.affiliation: Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Yuting P
authors.initials: YP
authors.lastname: Chiang
authors.affiliation: Department of Health Evidence and Policy, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Natalia N
authors.initials: NN
authors.lastname: Egorova
authors.affiliation: Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: David H
authors.initials: DH
authors.lastname: Adams
mesh_terms: D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000453:epidemiology* / Q000209:etiology; D017545:Aortic Aneurysm, Thoracic / Q000453:epidemiology* / Q000209:etiology; D001021:Aortic Valve / Q000002:abnormalities* / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D005260:Female; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000150:complications / Q000601:surgery*; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects*; D006801:Humans; D015994:Incidence; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D009518:New York / Q000453:epidemiology; D011183:Postoperative Complications / Q000453:epidemiology* / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors
keywords: Marfan syndrome; aortic dissection; ascending aorta; bicuspid aortic valve