To evaluate the long-term impact of frozen elephant trunk (FET) on the distal aorta of patients with Marfan syndrome (MFS) sustaining type I dissection confined to the thoracic aorta (above diaphragmatic hiatus). Between 2003 and 2016, 42 MFS (Ghent/revised Ghent criteria) patients (age 33.3±8.9 years; 27 men, 64.3%) with type I dissection above diaphragmatic hiatus involving the arch (22 acute, 52.4%) underwent total arch replacement and FET. Dissection extended distally to mid-descending aorta in 32 (76%) and to above diaphragmatic hiatus in 10 (24%). Operative mortality was 4.8% (2/42). Follow-up was 100% at 6.3±3.0 years. Maximal aortic sizes (DMaxs) at mid-descending aorta, diaphragmatic hiatus, renal arteries and largest segment of abdominal aorta were 22.8, 21.1, 19.1 and 19.9 mm preoperatively and 23.1, 22.0, 19.8 and 22.4 mm on latest CTA. Dilation and complete remodeling of distal aorta occurred in 10.0% (4/40) and 90% (36/40), respectively. There were 1 late death and 3 distal reoperations. Preoperative abdominal aortic DMax was predictive of distal dilatation (mm) (hazard ratio, HR=1.78, p=0.021) and reoperation (≥ versus <25 mm) (HR=12.88, p=0.037). At 10 years, freedom from dilation, reoperation and death were 69.8%, 78.1% and 90.0%, respectively. At 8 years, the rates of death, reoperation and reoperation-free survival were 10%, 11% and 79%, respectively. The FET technique has a positive remodeling impact on type I dissection confined to thoracic aorta in MFS patients. This study adds evidence supporting the safety and durability of this extended approach for aortic dissection in Marfan syndrome. Copyright © 2019. Published by Elsevier Inc.