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Aortic root growth in men and women with the Marfan's syndrome
Lilian J Meijboom1, Janneke Timmermans, Aeiko H Zwinderman
1Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
Abstract:
The leading cause of premature death in patients with Marfan's syndrome (MS) is type A aortic dissection or rupture due to progressive aortic root dilation. The aim of this study was to analyze aortic root growth in 113 men and 108 women with MS. All patients were prospectively followed with serial echocardiograms of the native aortic root. At baseline, women had on average a 5-mm smaller aortic root diameter adjusted for age than men. Average aortic root growth was 0.42 mm/year (SE 0.05) in men and 0.38 mm/year (SE 0.04) in women. On the basis of aortic root growth rates, the men and women could be divided into 2 normally distributed subgroups: fast and slow growers. Approximately 1 in 7 men (1.5 mm/year, SE 0.5) and approximately 1 in 9 women (1.8 mm/year, SE 0.3) had fast-growing aortic root diameters. Significantly more type A dissections (25% vs 4%, p <0.001) were observed in fast growers than in slow growers; this was found in men and women. Type A dissections were observed in 4 men and 9 women. By reducing the cut-off value by 5 mm for elective aortic root replacement in women, type A dissections could have been prevented in 3 women. In conclusion, guidelines should take gender differences into account, and therefore, the investigators propose reducing the threshold for elective aortic root replacement in women with MS by 5 mm.
Insights
Men and women with Marfan syndrome (MS) show different aortic root growth rates. Fast-growing aortic roots in MS patients are linked to increased dissection risk, suggesting gender-specific screening and treatment thresholds.
Area of Science:
- Cardiology
- Genetics
- Vascular Surgery
Background:
- Marfan syndrome (MS) is a genetic disorder affecting connective tissue.
- Aortic root dilation and subsequent type A aortic dissection or rupture are leading causes of premature death in MS patients.
- Current guidelines for aortic root replacement do not fully account for gender-specific differences in aortic growth.
Purpose of the Study:
- To analyze and compare aortic root growth patterns in men and women with Marfan syndrome.
- To identify subgroups of fast and slow aortic root growers within the MS population.
- To evaluate the association between aortic root growth rates and the incidence of type A aortic dissection.
Main Methods:
- Prospective follow-up of 113 men and 108 women with Marfan syndrome.
- Serial echocardiograms to measure native aortic root diameter.
- Statistical analysis to determine average growth rates and identify fast/slow growers based on gender.
Main Results:
- Women had a smaller baseline aortic root diameter (adjusted for age) compared to men.
- Average aortic root growth was 0.42 mm/year in men and 0.38 mm/year in women.
- Fast-growing aortic roots (approx. 1 in 7 men, 1 in 9 women) were associated with a significantly higher incidence of type A dissections (25% vs 4% in slow growers).
Conclusions:
- Gender differences in aortic root growth exist in Marfan syndrome.
- Aortic root growth rate is a significant predictor of type A dissection risk.
- The study proposes reducing the threshold for elective aortic root replacement in women with MS by 5 mm to prevent dissections.
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