title: Acute aortic dissection determines the fate of initially untreated aortic segments in Marfan syndrome.
abstract: BACKGROUND
  The aim of the current study was to investigate incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome.
  
  
  RESULTS
  Retrospective analysis of 86 consecutive Marfan syndrome patients fulfilling Ghent criteria that underwent 136 aortic surgeries and were followed at this institution in the past 15 years. Mean follow-up was 8.8±6.8 y. Thirty-day, 6-month, 1-year, and overall mortality was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Ninety-two percent of patients initially presented with aortic root, ascending aortic or arch lesions, whereas 8% presented with descending aortic or thoraco-abdominal lesions. Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurismal disease in 64%. Secondary complete arch replacement had to be performed in only 6% of patients without AAD, but in 36% with AAD (P=0.0005). In patients without AAD, 11% required surgery on primarily nontreated aortic segments (5 of 6 patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; P=0.0002).
  
  
  CONCLUSIONS
  The need for surgery in primarily nontreated aortic segments is precipitated by an initial presentation with AAD. Early elective surgery is associated with low mortality and reintervention rates. Type B dissection in patients with Marfan syndrome is associated with a high need for extensive aortic repair, even if the dissection is being considered uncomplicated by conventional criteria.
authors.affiliation: Department of Cardiovascular Surgery, University Hospital Berne, 3010 Berne, Switzerland.
authors.forename: Florian S
authors.initials: FS
authors.lastname: Schoenhoff
authors.forename: Silvan
authors.initials: S
authors.lastname: Jungi
authors.forename: Martin
authors.initials: M
authors.lastname: Czerny
authors.forename: Eva
authors.initials: E
authors.lastname: Roost
authors.forename: David
authors.initials: D
authors.lastname: Reineke
authors.forename: Gabor
authors.initials: G
authors.lastname: Matyas
authors.forename: Beat
authors.initials: B
authors.lastname: Steinmann
authors.forename: Juerg
authors.initials: J
authors.lastname: Schmidli
authors.forename: Alexander
authors.initials: A
authors.lastname: Kadner
authors.forename: Thierry
authors.initials: T
authors.lastname: Carrel
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D001011:Aorta / Q000473:pathology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000601:surgery*; D001021:Aortic Valve / Q000473:pathology; D019917:Blood Vessel Prosthesis Implantation / Q000706:statistics & numerical data*; D002311:Cardiomyopathy, Dilated / Q000209:etiology; D002423:Cause of Death; D002648:Child; D018450:Disease Progression; D018572:Disease-Free Survival; D017558:Elective Surgical Procedures / Q000706:statistics & numerical data; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000473:pathology; D008875:Middle Aged; D011183:Postoperative Complications / Q000453:epidemiology / Q000601:surgery; D012086:Reoperation / Q000706:statistics & numerical data; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult