Abstract： Objective To evaluate surgical outcomes of patients with Marfan syndrome （MFS） complicated by type A aortic dissection （AAD） during follow-up. Methods We retrospectively reviewed clinical data of 44 patients with MFS complicated by AAD who were admitted to Wuhan Asia Heart Hospital from January 2006 to January 2012. There were 31 male patients and 13 female patients with their age of 12-54 （33.0±9.8） years. Twenty-three patients underwent Bentall procedure at different time after the onset of AAD， while the other 21 patients received conservative treatment in stead of surgery because of economical or other reasons. COX regression with time-varying covariates was performed to analyze related factors， using primary end point， primary end point＋secondary end point as the outcome variables respectively， to compare postoperative outcomes and quality of life between the surgical treatment patients and conservative treatment patients. Results Postoperatively 1 patient died of multiple organ failure， and the other 22 patients survived the surgery. All the 43 patients were followed up from 1 to 75 months. The 3-year survival rate of the 22 surgical treatment patients was 95.7%， and they all had a good quality of life during follow-up. The survival condition and quality of life of the 21 conservative treatment patients was poor， and 13 patients （61.9%） died with the 3-year survival rate of only 31.7%. The main causes of their death included acute cardiac tamponade， aortic dissection rupture， acute myocardial infarction andcardiogenic shock. COX regression with time-varying covariates showed that the treatment outcomes of the surgical treatment patients were statistically different from those of the conservative treatment patients after modifying the influence caused by different operating time （OR of T_COV_ =0.088， P=0.028） . The risk of death of surgical treatment was only 8.8% of that of conservative treatment. Conclusion The prognosis of patients with MFS complicated by AAD is very poor. Therefore， all these patient， both in acute stage and chronic stage， should undergo surgical treatment as early as possible. The short-term and follow-up outcomes of surgical treatment are satisfactory.