title: Marfan syndrome is associated with recurrent dissection of the dissected aorta.
abstract: BACKGROUND
  Recurrent dissection of a previously dissected aortic segment has been reported as a rare, late complication in single case reports. The infrequency of this event makes informed risk assessment in an individual patient challenging.
  
  
  METHODS
  To investigate this issue we examined the database of the Massachusetts General Hospital Thoracic Aortic Center between January 1, 2003 and December 31, 2012. A retrospective review was performed to identify patients with both (1) an acute aortic dissection after a prior aortic dissection and (2) evidence of a new dissection within a previously dissected aortic segment creating a triple lumen aorta. Data were reviewed to identify factors predisposing to dissection of a previously dissected aortic segment.
  
  
  RESULTS
  Over a 10-year period we identified 5 cases of aortic dissection within a previously dissected aortic segment presenting as a new acute aortic syndrome. On average, the recurrent dissection occurred 1 decade after the first aortic dissection (mean = 9.8 ± 1.9 years). Patients identified in this series were significantly younger at first dissection and more likely to carry the diagnosis of Marfan syndrome. Aortic aneurysm diameter was quantified before and after the new dissection event and demonstrated a marked increase in aneurysmal size (mean increase = 1.6 ± 0.3 cm).
  
  
  CONCLUSIONS
  We conclude that medial degeneration, as seen in the Marfan aorta, represents a predisposing factor for recurrent dissection of the dissected aorta. Our data indicate that double aortic dissections cause significant arterial destabilization and a low threshold for surgical intervention is appropriate.
authors.affiliation: Cardiac Arrhythmia Service, MGH Heart Center, Massachusetts General Hospital, Boston, Massachusetts; Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Leon M
authors.initials: LM
authors.lastname: Ptaszek
authors.affiliation: MGH Cardiovascular Research Center, Massachusetts General Hospital, Boston, Massachusetts; MGH Thoracic Aortic Center, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Kibeom
authors.initials: K
authors.lastname: Kim
authors.affiliation: Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts; MGH Thoracic Aortic Center, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Amy E
authors.initials: AE
authors.lastname: Spooner
authors.affiliation: Division of Cardiac Surgery, MGH Heart Center, Massachusetts General Hospital, Boston, Massachusetts; MGH Thoracic Aortic Center, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Thomas E
authors.initials: TE
authors.lastname: MacGillivray
authors.affiliation: Division of Vascular and Endovascular Surgery, Massachusetts General Hospital, Boston, Massachusetts; MGH Thoracic Aortic Center, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Richard P
authors.initials: RP
authors.lastname: Cambria
authors.affiliation: Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts; MGH Cardiovascular Research Center, Massachusetts General Hospital, Boston, Massachusetts; MGH Thoracic Aortic Center, Massachusetts General Hospital, Boston, Massachusetts; Pediatric Cardiology Division, Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Mark E
authors.initials: ME
authors.lastname: Lindsay
authors.affiliation: Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts; MGH Thoracic Aortic Center, Massachusetts General Hospital, Boston, Massachusetts. Electronic address: eisselbacher@mgh.harvard.edu.
authors.forename: Eric M
authors.initials: EM
authors.lastname: Isselbacher
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000784:Aortic Dissection / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008404:Massachusetts; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors