title: Type A aortic dissection in Marfan syndrome: extent of initial surgery determines long-term outcome.
abstract: BACKGROUND
  Data on outcomes after Stanford type A aortic dissection in patients with Marfan syndrome are limited. We investigated the primary surgery and long-term results in patients with Marfan syndrome who suffered aortic dissection.
  
  
  RESULTS
  Among 1324 consecutive patients with aortic dissection type A, 74 with Marfan syndrome (58% men; median age, 37 years [first and third quartiles, 29 and 48 years]) underwent surgical repair (85% acute dissections; 68% DeBakey I; 55% composite valved graft, 30% supracoronary ascending replacement, 15% valve-sparing aortic root replacement; 12% total arch replacement; 3% in-hospital mortality) at 2 tertiary centers in the United States and Europe over the past 25 years. The rate of aortic reintervention with resternotomy was 24% (18 of 74) and of descending aorta (thoracic+abdominal) intervention was 30% (22 of 74) at a median follow-up of 8.4 years (first and third quartiles, 2.2 and 12.7 years). Freedom from need for aortic root reoperation in patients who underwent primarily a composite valved graft or valve-sparing aortic root replacement procedure was 95±3%, 88±5%, and 79±5% and in patients who underwent supracoronary ascending replacement was 83±9%, 60±13%, 20±16% at 5, 10, and 20 years. Secondary aortic arch surgery was necessary only in patients with initial hemi-arch replacement.
  
  
  CONCLUSIONS
  Emergency surgery for type A dissection in patients with Marfan syndrome is associated with low in-hospital mortality. Failure to extend the primary surgery to aortic root or arch repair leads to a highly complex clinical course. Aortic root replacement or repair is highly recommended because supracoronary ascending replacement is associated with a high need (>40%) for root reintervention.
authors.affiliation: Division of Cardiothoracic Surgery, Hospital of the University of Pennsylvania in Philadelphia (BR., J.E.B., E.B., N.D.D., R.K.M., W.Y.S., P.V.); and Department of Cardiovascular Surgery, Heart Center Freiburg University, Freiburg, Germany (B.R., F.B., M.S., F.A.K.).
authors.forename: Bartosz
authors.initials: B
authors.lastname: Rylski
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Bavaria
authors.forename: Friedhelm
authors.initials: F
authors.lastname: Beyersdorf
authors.forename: Emanuela
authors.initials: E
authors.lastname: Branchetti
authors.forename: Nimesh D
authors.initials: ND
authors.lastname: Desai
authors.forename: Rita K
authors.initials: RK
authors.lastname: Milewski
authors.forename: Wilson Y
authors.initials: WY
authors.lastname: Szeto
authors.forename: Prashanth
authors.initials: P
authors.lastname: Vallabhajosyula
authors.forename: Matthias
authors.initials: M
authors.lastname: Siepe
authors.forename: Fabian A
authors.initials: FA
authors.lastname: Kari
mesh_terms: D000328:Adult; D001013:Aorta, Thoracic / Q000601:surgery; D001014:Aortic Aneurysm / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D013504:Cardiovascular Surgical Procedures / Q000379:methods*; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D017052:Hospital Mortality; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D014481:United States
keywords: Marfan syndrome; aorta; cardiac imaging techniques; surgery