Marfan Syndrome versus Loeys-Dietz Syndrome after Aortic Surgery: A Systematic Review and Meta-analysis of Reconstructed Time-to-Event Data.
7 affiliations
- 1Friedrich-Schiller-University Jena, Department of Cardiothoracic Surgery, Germany, Jena.
- 2College of Health Sciences, Obafemi Awolowo University, Medical School, Nigeria, Ile-ife.
- 3University College London, Department of Cardiovascular Science, United Kingdom, London.
- 4MaterDei Hospital, Department of Thoracic Surgery, Bahia, Brazil, Salvador.
- 5West German Heart and Vascular Center, University Hospital Essen, Department of Cardiology and Vascular Medicine, Germany, Essen.
- 6UPMC Heart and Vascular Institute, University of Pittsburgh Medical Center, Department of Cardiothoracic Surgery, Pennsylvania, United States, Pittsburgh.
- 7Jena University Hospital, Universitatsklinikum Jena, Department of Cardiothoracic Surgery, Germany, Jena.
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.
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BibTeX
@article{fischer2026marfan,
title = {{Marfan Syndrome versus Loeys-Dietz Syndrome after Aortic Surgery: A Systematic Review and Meta-analysis of Reconstructed Time-to-Event Data.}},
author = {Johannes Fischer and Steve Wehner and Ayodeji Arobo and Abdullah Almehandi and Felipe S Passos and Christian Eitel and Hristo Kirov and Xander Jacquemyn and Ibrahim Sultan and Torsten Doenst and Tulio Caldonazo},
journal = {Aorta (Stamford, Conn.)},
year = {2026},
month = oct,
doi = {10.1055/a-2965-6400},
url = {https://europepmc.org/article/MED/42849511},
note = {CC BY},
}
RIS
TY - JOUR
TI - Marfan Syndrome versus Loeys-Dietz Syndrome after Aortic Surgery: A Systematic Review and Meta-analysis of Reconstructed Time-to-Event Data.
AU - Johannes Fischer
AU - Steve Wehner
AU - Ayodeji Arobo
AU - Abdullah Almehandi
AU - Felipe S Passos
AU - Christian Eitel
AU - Hristo Kirov
AU - Xander Jacquemyn
AU - Ibrahim Sultan
AU - Torsten Doenst
AU - Tulio Caldonazo
PY - 2026
DA - 2026/10/08
JO - Aorta (Stamford, Conn.)
DO - 10.1055/a-2965-6400
UR - https://europepmc.org/article/MED/42849511
AB - 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.
SN - 2325-4637
ER -