SRS347 - Outcomes of aortic root surgery in adults with Loeys-Dietz syndrome: a systematic review and meta-analysis

Abstract Background Loeys–Dietz syndrome (LDS) is a rare aortopathy with early root dilatation, rapid progression, and dissection at small diameters. Prophylactic root replacement is recommended. Valve-sparing reimplantation (VSARR) avoids anticoagulation, but durability in LDS is uncertain. Objective To evaluate outcomes of root replacement in LDS adults and whether VSARR provides safe, durable results compared with valve-replacing surgery. Methods PRISMA-guided searches of MEDLINE, Embase, and Scopus (to September 2025) identified studies of genetically confirmed LDS. Marfan and other syndromes were excluded unless LDS data were separate. Interventions were VSARR (David/Yacoub) or valve-replacing procedures (composite graft, homograft, allograft). Primary outcomes were early mortality and survival; secondary outcomes were reoperation, regurgitation, valve-related complications, and distal events. Pooled proportions with 95% c.i.s were calculated using random-effects models; survival was extracted at fixed time points. Results Six single- and multicentre series (n = 171; cohort sizes 8–79; follow-up 2–10y) were included. In mixed VSARR/composite cohorts, operative mortality was 6.0% (95% c.i. 1.6–13.1), 5-year survival 91.1% (84.2–96.1), and 10-year survival 86.2% (76.7–93.5). Aortic reintervention occurred in 27.4% (7.0–54.8), with wide heterogeneity, largely in dissection cases. In VSARR-only series (n = 58), no perioperative deaths occurred; 5-year survival was 90.6% (80.0–97.5), valve-related reoperation 6.6% (2.0–13.7), and aortic reintervention 9.9% (2.6–21.1) at short-to-midterm follow-up. Conclusions Elective root replacement in LDS yields excellent survival. VSARR appears safe and durable with low valve reoperation. Distal aortic progression remains the key long-term risk. Larger comparative studies are needed to define VSARR versus valve-replacing outcomes.