Related Experiment Video
Updated: Feb 18, 2026
Novel and Innovative Hybrid Technique for Type A Aortic Dissection
Published on: March 28, 2025
Advances in Marfan Syndrome Care: The Limits of Type B Dissection
Maria Tchitchinadze1, Olivier Milleron2, Ludivine Eliahou1
1Centre de référence pour le syndrome de Marfan et apparentés, maladies aortiques rares, Service de cardiologie, Hopital Bichat, Paris, France.
Background:
To evaluate the progress of care for patients with Marfan syndrome with FBN1 pathogenic variant carriers over the past 30 years.
Methods:
Patients who visited the center were entered into a prospective registry. Aortic events (surgeries and dissections) and deaths are reported in 10-year periods (1995-2004, 2005-2014, and 2015-2023).
Results:
A total of 1898 Marfan syndrome with FBN1 pathogenic variant carrier patients were included, median age 24.90 (interquartile range, 10.83-39.07) years at their first visit, 51% were female. Age at first visit decreased by 6 years over the 3 periods. The incidence of aortic root surgery before the first visit tended to increase (38 of 516 [7.4%] vs 77 of 903 [8.5%] vs 41 of 479 [8.6%]), and valve-sparing surgery increased at the expense of Bentall procedures. Aortic dissections occurrence decreased (7.8% vs 6.1% vs 4.8%, P < .001). This decrease was limited to type A aortic dissections (6.0% vs 4.7% vs 2.7%, P < .001), which mainly occurred before the first visit (87%). In contrast, the incidence of type B aortic dissections did not change (1.7% vs 1.4% vs 2.1%), with 52% occurring after the first visit. Lastly, survival according to the year of first visit significantly increased (P < .001), with the percentage of patients who survived for 75 years increasing from 52.4%, to 63.0%, and 79.4%.
Conclusions:
The incidence of type A aortic dissections decreased, valve-sparing surgery is becoming the standard, and survival improved. However, current care does not prevent type B dissections. These results are important for patients' information, and focusing research on remaining issues.
Insights
Care for Marfan syndrome (MFS) patients has improved, with decreased type A aortic dissections and increased survival. However, type B dissections remain a challenge, requiring further research for MFS patients.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Medical Research
Background:
- Marfan syndrome (MFS) is a genetic disorder affecting connective tissue.
- FBN1 pathogenic variants are a primary cause of MFS.
- Evaluating long-term care progress is crucial for MFS management.
Purpose of the Study:
- To assess the evolution of Marfan syndrome patient care over three decades.
- To analyze trends in aortic events and survival rates.
- To identify areas for future research in MFS management.
Main Methods:
- Prospective registry of Marfan syndrome patients.
- Analysis of aortic surgeries and dissections across three 10-year periods (1995-2023).
- Evaluation of survival rates based on the year of first patient visit.
Main Results:
- A decrease in type A aortic dissections and an increase in valve-sparing surgeries were observed.
- Survival rates significantly improved over the study period.
- Type B aortic dissections incidence remained unchanged, with a notable proportion occurring post-initial visit.
Conclusions:
- Marfan syndrome care has advanced, marked by reduced type A dissections and enhanced survival.
- Valve-sparing surgery is increasingly preferred over traditional procedures.
- Current management strategies do not prevent type B aortic dissections, highlighting a critical area for future research.
Related Concept Videos
01:26Aneurysm III: Interprofessional Care
01:30Aneurysm I: Introduction
01:26Mitral Stenosis III: Medical Management
01:25Aortic Regurgitation III: Medical Management