title: Long-term implications of emergency versus elective proximal aortic surgery in patients with Marfan syndrome in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium Registry.
abstract: OBJECTIVE
  Patients with Marfan syndrome with aortic root aneurysms undergo elective aortic root replacement to avoid the life-threatening outcomes of aortic dissection and emergency repair. The long-term implications of failed aortic surveillance leading to acute dissection and emergency repair are poorly defined. We compared the long-term clinical courses of patients with Marfan syndrome who survive emergency versus elective proximal aortic surgery.
  
  
  METHODS
  The Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Registry is a National Institutes of Health-funded multicenter database and biorepository that enrolls patients with genetically triggered thoracic aortic aneurysms. Of the 635 patients with Marfan syndrome enrolled as of March 2011, 194 had undergone proximal aortic replacement. Patients were grouped according to emergency (n = 47) or elective (n = 147) status at the time of surgery.
  
  
  RESULTS
  Patients in the emergency group were more likely to have incomplete proximal aortic resection; 83% of emergency procedures included aortic root replacement, compared with 95% of elective procedures. At long-term follow-up (mean, >6 years), the emergency group had a higher incidence of chronic dissection of the distal aorta and significantly larger diameters in distal aortic segments than elective patients. Patients in the emergency group had undergone more operations (1.31 vs 1.11 procedures/patient; P = .01) and had lower activity scores on a health-related quality of life survey.
  
  
  CONCLUSIONS
  For patients with Marfan syndrome, failed aortic surveillance and consequent emergency dissection repair have important long-term implications with regard to the status of the distal aorta, need for multiple procedures, and quality of life. These findings emphasize the importance of aortic surveillance and timely elective aortic root aneurysm repair for patients with Marfan syndrome.
authors: Division of Cardiothoracic Surgery, Oregon Health & Science University, Portland, OR 97239, USA. songh@ohsu.edu
authors: Howard K
authors: HK
authors: Song
authors: Mark
authors: M
authors: Kindem
authors: Joseph E
authors: JE
authors: Bavaria
authors: Harry C
authors: HC
authors: Dietz
authors: Dianna M
authors: DM
authors: Milewicz
authors: Richard B
authors: RB
authors: Devereux
authors: Kim A
authors: KA
authors: Eagle
authors: Cheryl L
authors: CL
authors: Maslen
authors: Barbara L
authors: BL
authors: Kroner
authors: Reed E
authors: RE
authors: Pyeritz
authors: Kathryn W
authors: KW
authors: Holmes
authors: Jonathan W
authors: JW
authors: Weinsaft
authors: Victor
authors: V
authors: Menashe
authors: William
authors: W
authors: Ravekes
authors: Scott A
authors: SA
authors: LeMaire
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D017558:Elective Surgical Procedures; D004630:Emergencies; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D008875:Middle Aged; D011788:Quality of Life; D012042:Registries; D012086:Reoperation; D018570:Risk Assessment; D012307:Risk Factors; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D055815:Young Adult