Objective: The use of the frozen elephant trunk technique for type A aortic dissection in Marfan syndrome is limited by the lack of imaging evidence for long-term aortic remodeling. We seek to evaluate the changes of the distal aorta and late outcomes after frozen elephant trunk and total arch replacement for type A aortic dissection in patients with Marfan syndrome. Methods: Between 2003 and 2015, we performed frozen elephant trunk thorn total arch replacement for 172 patients with Marfan syndrome suffering from type A aortic dissection (94 acute; 78 chronic). Mean age was 34.6 +/- 9.3 years, and 121 were male (70.3%). Early mortality was 8.1% (14/172), and follow-up was complete in 98.7% (156/158) at a mean of 6.2 +/- 3.3 years. Aortic dilatation was defined as a maximal diameter of greater than 50 mm or an average growth rate of greater than 5 mm/year at any segment detected by computed tomographic angiography. Temporal changes in the false and true lumens and maximal aortic size were analyzed with linear mixed modeling. Results: After surgery, false lumen obliteration occurred in 86%, 39%, 26%, and 21% at the frozen elephant trunk, unstented descending aorta, diaphragm, and renal artery, respectively. The true lumen expanded significantly over time at all segments (P < .001), whereas the false lumen shrank at the frozen elephant trunk (P < .001) and was stable at distal levels (P > .05). Maximal aortic size was stable at the frozen elephant trunk and renal artery (P > .05), but grew at the descending aorta (P = .001) and diaphragm (P < .001). Respective maximal aortic sizes before discharge were 40.2 mm, 32.1 mm, 31.6 mm, and 26.9 mm, and growth rate was 0.4 mm/year, 2.8 mm/year, 3.6 mm/year, and 2.6 mm/year. By the latest follow-up, distal maximal aortic size was stable in 63.5% (99/156), and complete remodeling down to the mid-descending aorta occurred in 28.8% (45/156). There were 22 late deaths and 23 distal reoperations. Eight-year incidence of death was 15%, reoperation rate was 20%, and event-free survival was 65%. Preoperative distal maximal aortic size (mm) predicted dilatation (hazard ratio, 1.11; P < .001) and reoperation (hazard ratio, 1.07; P < .001). A patent false lumen in the descending aorta predicted dilatation (hazard ratio, 3.88; P < .001), reoperation (hazard ratio, 3.36; P = .014), and late death (hazard ratio, 3.31; P = .045). Conclusions: The frozen elephant trunk technique can expand the true lumen across the aorta, decrease or stabilize the false lumen, and stabilize the distal aorta in patients with Marfan syndrome with type A aortic dissection, thereby inducing favorable remodeling in the distal aorta. This study adds long-term clinical and radiologic evidence supporting the use of the frozen elephant trunk technique for type A dissection in Marfan syndrome.