Background: We seek to report the long-term outcomes of the total arch replacement and frozen elephant trunk (TAR + FET) technique for type A aortic dissection (TAAD) following prior Bentall procedure in patients with Marfan syndrome (MFS). Methods: Between 2003 and 2015, we performed TAR + FET for 26 patients with MFS who developed TAAD following a prior Bentall procedure. Mean age at FET 36.9 +/- 9.7 years and 24 were males. TAAD was acute in 8 (30.8%, all new dissections from precious root aneurysm) and chronic in 18 (69.2%, 15 residual and 3 new). The interval from Bentall procedure to FET averaged 6.4 +/- 5.8 years, which was significantly longer in the acute group (10.3 +/- 6.3 vs. 4.6 +/- 4.9, P = 0.021). The early and long-term outcomes were compared between two groups and risk factors identified for late adverse events. Results: Operative mortality was 11.5% (3/26). Stroke, lower limb ischemia and reexploration for bleeding occurred in 1 patient each (3.8%). Follow-up was complete in 100% (23/23) at mean 5.1 +/- 2.3 years (range, 0.9-11.2 years). The maximal diameter (DMax) of distal aorta in the chronic group was significantly greater at the unstented descending aorta [DA, (56.4 +/- 15.5 vs. 35.6 +/- 12.2 mm, P = 0.006)] compared to acute patients. The false lumen was obliterated in 95.7% across the FET and 56.5% in the unstented DA. Distal aortic dilation occurred in 13 patients (11 chronic, 68.8%). Of those 11 patients, 4 underwent an open thoracoabdominal aortic repair and 3 died of distal aortic rupture. Late death occurred in 7 patients at mean 3.9 +/- 2.5 years. At 6 years, the incidence was 18% for death, 11% for distal aortic reoperation, and 71% for reoperation-free survival. Survival did not differ between two groups (75.0% vs. 71.3%, P = 0.851), while acute patients had significantly higher freedom from late rupture and reoperation at 6 years (100% vs. 61.9%, P = 0.046). Hypertension was the sole risk factor for distal aortic dilatation [hazard ratio (HR) = 7.271; 95% confidence interval (CI), 1.814-29.143; P = 0.005]. Risk factors for late adverse events were hypertension (HR = 6.712; 95% CI, 1.201-37.503; P = 0.030) and age < 35 years (HR = 6.760; 95% CI, 1.154-39.587; P = 0.034). Conclusions: The TAR and FET technique was feasible and efficacious for TAAD following previous Bentall procedure in patients with MFS. Early and late survival did not differ with acute and chronic dissections, while freedom from late rupture and reoperation is significantly higher in patients with acute TAAD. Patients with hypertension and aged < 35 years are at higher risk for late distal aortic dilation, reoperation and death.