Marfan Syndrome versus Loeys-Dietz Syndrome after Aortic Surgery: A Systematic Review and Meta-analysis of Reconstructed Time-to-Event Data.

Abstract

Objective: Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS) are hereditary connective tissue diseases, both characterized by aortic root and vessel involvement with an increased risk of aneurysms and dissections. Despite advances in surgical techniques and improved postoperative follow-up, the impact of surgery on long-term outcomes in these patient groups is still unknown.

Methods: We performed a systematic review and a meta-analysis to compare the postoperative long-term events. The main outcome was long-term survival. Additional outcomes were freedom from dissection and reoperation. After reconstruction of time-to-event data for the individual survival analysis, pooled Kaplan-Meier curves for the endpoints were generated.

Results: A total of 1,794 studies were identified, of which 5 were included in the analysis, encompassing 1,005 patients (MFS: 778; LDS: 227). With MFS as the reference group, there was no significant difference in long-term survival between both groups, based on age (hazard ratio [HR]: 0.919; 95% confidence interval [CI]: 0.58-1.45; p=0.717) or postoperative follow-up (HR: 0.997; 95% CI: 0.45-2.19; p=0.995). However, LDS was associated with higher risk of dissection (HR: 0.331; 95% CI: 0.21-0.53; p<0.005) and reoperation (HR: 0.603; 95% CI: 0.40-0.90; p=0.014), reflecting unadjusted comparisons reconstructed from Kaplan-Meier curves.

Conclusion: Although long-term survival is similar between MFS and LDS patients, those with LDS face a significantly higher risk of aortic dissection and reoperation. This elevated risk may be partly explained by the intrinsic genotypic heterogeneity of the LDS population. However, our pooled analysis could not fully adjust for this heterogeneity, underscoring the need for vigilant lifelong imaging surveillance and individualized surgical thresholds in LDS.