CURRENT PRACTICE IN MARFANS-SYNDROME AND ANNULOAORTIC ECTASIA - AORTIC ROOT REPLACEMENT WITH A COMPOSITE GRAFT OVER A 20-YEAR PERIOD

Background: From October 1973 to December 1995, 251 patients (204 male , 47 female) aged from 10 to 75 years (mean: 46.6 +/- 15) underwent an ascending aortic replacement with a composite graft for: dystrophic a neurysm (AN), 168 cases (66.9%); chronic dissection (CD), 36 cases (14 %); and type A acute dissection (AD), 48 cases (19.1%). Fifty-one pati ents (20.3%) suffered from Marfan's disease (25 AN, 17 AD, 9 CD). Thir ty-seven patients (14.7%) had undergone a previous cardiac or aortic o peration. The ascending aortic replacement was extended to the transve rse arch in 31 patients (12.3%). A mechanical valve was used in 233 pa tients (92.8%). The classic ''Bentall'' technique was used in 87 patie nts (34.6%), the ''button'' technique in 121 patients (48.2%), the ''C abrol'' technique in 26 patients (10.3%) and a ''mixed'' technique in 17 patients (6.2%). Results: The hospital mortality accounts for 7.2% (18 out of 251) (AN: 4 out of 68, 2.3%, CD: 4 out of 36, 11.1%, AD: 9 out of 48, 18.7%). When emergencies are considered, the hospital morta lity is 12 out of 54 (22.2%) versus 6 out of 197 (3%) in elective proc edures. The predictors of hospital death were emergency, AD (p < 0.03) and arch replacement (p < 0.02). Mean follow up is 38 +/- 15 months ( 4 -262). The overall long term survival rate is (Kaplan Meier): 92 +/- 6% at one year, 77.9 +/- 9% at 5 years, 67.7 +/- 12% at 10 years, and 61.3 +/- 15% at 12 years. The 10-year survival rate is significantly higher in patients with AN (93 +/- 6%) than in patients with AD (61.6 +/- 17%) (p < 0.01). The late survival rate is also significantly high er after the ''button'' (93.8 +/- 5%) or Bentall's reimplantation (88. 7 +/- 6%, 83.8 +/- 9%, and 76.6 +/- 12%) than after the ''Cabrol'' pro cedure (80 +/- 18%, 63 +/- 21% and 58 +/- 35%) at 1, 5, and 8 years, r espectively. Conclusion: Ascending aortic replacement with a composite graft is a safe procedure, especially when performed electively in pa tients with dystrophic aneurysm or Marfan's disease. The technique of coronary reimplantation has a significant influence of the long-term r esults, with the reimplantation of choice being the ''button'' techniq ue. The ''Cabrol'' technique must be used when the ''button'' or the ' 'Bentall'' reimplantation is not feasible.