J Thorac Dis. 2026 May 31;18(5):520. doi: 10.21037/jtd-2026-1-0366. Epub 2026 May 27.
ABSTRACT
BACKGROUND: Marfan syndrome (MFS) is a heritable connective tissue disorder associated with a heightened risk of acute type A aortic dissection (ATAAD). The long-term impact of MFS on outcomes following total arch replacement with frozen elephant trunk (TAR with FET) remains poorly characterized. This study aimed to evaluate this impact in a large cohort from a single high-volume aortic center.
METHODS: Patients with ATAAD who underwent TAR with FET at Fuwai Hospital (2010-2018) were categorized into MFS and non-MFS groups. Long-term follow-up was complete for all surviving patients, with a median follow-up of 6.08 years (terminating in December 2023). Clinical characteristics and long-term follow-up outcomes were analyzed, with primary endpoints including survival, reoperation rates, and functional status. Multivariable logistic regression and competing risks Cox regression models were employed to identify independent predictors of outcomes.
RESULTS: Among 1,086 patients with a mean age of 46.6 years (104 MFS, 982 non-MFS), Marfan patients were significantly younger (36.1 vs. 47.7 years, P<0.001) with higher rates of previous cardiovascular surgery (11.5% vs. 2.2%, P<0.001) and root replacement (76.0% vs. 22.7%, P<0.001). Overall operative mortality was 7.4% (80/1,086) with no significant between-group difference. At median follow-up of 6.08 years, 10-year survival was 80.6% and comparable between groups (P=0.22). Over 90% of survivors maintained complete self-care ability. Among 67 total reoperations, 25 occurred in Marfan patients. MFS remained an independent risk factor for reoperation (hazard ratio 2.06, 95% confidence interval: 1.18-3.59, P=0.01).
CONCLUSIONS: While MFS does not compromise long-term survival in ATAAD patients undergoing TAR with FET, significantly elevated reoperation rates necessitate specialized long-term surveillance.
PMID:42306665 | PMC:PMC13266832 | DOI:10.21037/jtd-2026-1-0366