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Background <p>Marfan Syndrome (MFS) and Ehlers–Danlos Syndrome (EDS) are hereditary connective tissue disorders with significant clinical variability, predominantly affecting cardiovascular, musculoskeletal, and vascular systems. Surgical interventions play a pivotal role in managing life-threatening complications such as aortic dissections in MFS and vascular ruptures in EDS. However, the long-term effectiveness of surgical strategies remains debated, necessitating a comprehensive evaluation of their outcomes to guide clinical decision-making.</p> Methodology <p>A systematic review adhering to PRISMA guidelines was undertaken. A thorough search was conducted across PubMed, Scopus, and Web of Science, utilizing targeted keywords to identify studies focused on surgical interventions in Marfan syndrome (MFS) and Ehlers–Danlos syndrome (EDS). Data were extracted on survival rates, reoperation frequencies, and complication profiles. Meta-analysis was conducted where appropriate, and subgroup analyses compared outcomes of specific surgical techniques, such as the Bentall and David procedures. Bias mitigation strategies included sensitivity analyses and subgroup evaluations.</p> Results <p>The David procedure demonstrated outstanding long-term efficacy, achieving a 100% survival rate at 20&#xa0;years with minimal complications or reoperations. The Bentall procedure also showed high durability with a 94% survival rate over 20&#xa0;years. General surgical approaches yielded lower survival and reoperation-free rates, particularly in aortic dissection cases. Meta-analysis highlighted superior outcomes with valve-sparing techniques over composite valve grafting, reducing complications such as thromboembolism and hemorrhage. Additional findings included improved musculoskeletal outcomes in MFS patients undergoing scoliosis correction, with a significant reduction in spinal curvature.</p> Conclusion <p>Specialized surgical interventions significantly improve long-term survival and reduce complications in MFS and EDS patients. Valve-sparing procedures, such as the David technique, represent gold standards for optimizing outcomes. Tailored surgical strategies, combined with advancements in imaging and structured follow-up protocols, are crucial for enhancing patient care. Future research should focus on multicenter trials and integrating genetic and molecular profiling to further refine treatment paradigms.</p>

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Efficacy of Vascular Surgical Interventions in Marfan Syndrome and Ehlers–Danlos Syndrome: A Review of Long-Term Patient Outcomes

  • 期刊论文

  • 发表日期：

    2025年7月4日

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  • K. R. Arunvignesh,
  • V. Ananthanarayanan,
  • Asish Mathew Joseph,
  • Musunuri Manoj Kumar,
  • Aditi Saxena,
  • Vikram S. Amberkar

摘要

Background

Marfan Syndrome (MFS) and Ehlers–Danlos Syndrome (EDS) are hereditary connective tissue disorders with significant clinical variability, predominantly affecting cardiovascular, musculoskeletal, and vascular systems. Surgical interventions play a pivotal role in managing life-threatening complications such as aortic dissections in MFS and vascular ruptures in EDS. However, the long-term effectiveness of surgical strategies remains debated, necessitating a comprehensive evaluation of their outcomes to guide clinical decision-making.

Methodology

A systematic review adhering to PRISMA guidelines was undertaken. A thorough search was conducted across PubMed, Scopus, and Web of Science, utilizing targeted keywords to identify studies focused on surgical interventions in Marfan syndrome (MFS) and Ehlers–Danlos syndrome (EDS). Data were extracted on survival rates, reoperation frequencies, and complication profiles. Meta-analysis was conducted where appropriate, and subgroup analyses compared outcomes of specific surgical techniques, such as the Bentall and David procedures. Bias mitigation strategies included sensitivity analyses and subgroup evaluations.

Results

The David procedure demonstrated outstanding long-term efficacy, achieving a 100% survival rate at 20 years with minimal complications or reoperations. The Bentall procedure also showed high durability with a 94% survival rate over 20 years. General surgical approaches yielded lower survival and reoperation-free rates, particularly in aortic dissection cases. Meta-analysis highlighted superior outcomes with valve-sparing techniques over composite valve grafting, reducing complications such as thromboembolism and hemorrhage. Additional findings included improved musculoskeletal outcomes in MFS patients undergoing scoliosis correction, with a significant reduction in spinal curvature.

Conclusion

Specialized surgical interventions significantly improve long-term survival and reduce complications in MFS and EDS patients. Valve-sparing procedures, such as the David technique, represent gold standards for optimizing outcomes. Tailored surgical strategies, combined with advancements in imaging and structured follow-up protocols, are crucial for enhancing patient care. Future research should focus on multicenter trials and integrating genetic and molecular profiling to further refine treatment paradigms.