Abstract
OBJECTIVE: To evaluate the remodeling of the distal aorta and outcomes after aortic surgery for type A aortic dissection (TAAD) in patients with Marfan syndrome and investigate whether morphologic characteristics of the dissection can predict negative remodeling. METHODS: Between 2013 and 2021, we performed total arch with a frozen elephant trunk for 325 patients with Marfan syndrome with DeBakey type I aortic dissection. Mean age was 47.13 ± 7.33 years, and 204 were men (63%). Follow-up was complete in 91.1% (296 out of 325) at a mean of 48.3 ± 13.1 months. Four-year incidence of death was 8.6% and reoperation rate was 10.4%. Negative remodeling was defined as an average growth rate >5 mm/year or >10% at any segment detected by computed tomography angiography. RESULTS: After surgery, negative remodeling occurred in 19.3% and 26.7% at TAAD follow-up at a mean of 13.6 and 38.3 months, respectively. There were 15.2% (12 out of 79) late deaths and 26.6% (21 out of 79) distal reoperations for those patients. The positive remodeling patients share a low rate of late death and distal reoperations of 6.5% (14 out of 217) and 7.8% (17 out of 217) (P < .01). Maximal aortic sizes before discharge for negative remodeling patients were 43.2, 35.1, and 32.5 mm, and growth rates were 4.5 ± 1.52, 3.1 ± 1.14, and 3.5 ± 1.33 mm/year at the level of diaphragm, celiac trunk, and renal artery respectively, which is larger and expands more quickly than the patients with positive remodeling (P < .01). Distal maximal aortic size (P < .01), number of entry tears (P = .03), and average entry tears size (P = .02) predicted rate of negative remodeling. CONCLUSIONS: Our results suggest that TAAD has a high rate of negative aortic remodeling in patients with Marfan syndrome. Distal maximal aortic size, number of entry tears, and average entry tears size were associated with the rate of negative aortic remodeling in patients with TAAD and Marfan syndrome.