Mitral valve prolapse affects 59.9% of Marfan patients and is linked to faster aortic root growth (2.9 vs 1.3 mm/yr, p=0.001) and earlier aortic events (33 vs 40 years, p=0.001).
Does the presence of mitral valve prolapse worsen cardiovascular outcomes and aortic disease progression in patients with Marfan syndrome?
In patients with Marfan syndrome, mitral valve prolapse is highly prevalent and associated with faster aortic growth and earlier aortic events, suggesting a more aggressive disease course despite similar overall event rates.
Absolute Event Rate: 0% vs 0%
Abstract Background Mitral valve prolapse (MVP) is the second most common cardiovascular manifestation in Marfan syndrome (MFS), following aortic root dilation. While frequently observed, its impact on aortic disease progression, left ventricular function, and major cardiovascular events remains unclear. Purpose To assess the prevalence of MVP in patients with MFS and investigate its association with cardiovascular outcomes. Methods Consecutive patients were enrolled retrospectively among those diagnosed with MFS fulfilling the Revised Ghent Criteria, with a confirmed pathogenic FBN1 variant, across three European tertiary referral Centers from 2004 to 2024. MVP diagnosis was based on echocardiographic or cardiac magnetic resonance (CMR) imaging. Outcomes of interest included death, aortic events (aortic dissection or prophylactic aortic surgery) and arrhythmic events (defined as ventricular fibrillation, sustained ventricular tachycardia, or intracardiac defibrillator implantation). Results 298 MFS patients were included, of which 180 (59.9%) had MVP. Patients with MVP were younger (p 0.001) and had higher Ghent scores (p = 0.02). At last follow-up, the MVP group exhibited larger aortic root diameters (42.0 ± 5.7 mm vs. 39.7 ± 5.9 mm, p = 0.009), larger ventricular end-diastolic volumes (155 ± 42 mL vs. 147 ± 29 mL, p 0.001) and lower ejection fraction (59% ± 6% vs. 63% ± 6%, p = 0.005). Over a median follow-up of 10 years a total of 11 death, 136 aortic events, and 21 arrhythmic events occurred. No significant differences were found in mortality, aortic events, or arrhythmic events between the two groups (respectively p=0.1, p=0.2 and p=0.5). However, MVP patients exhibited a significantly higher aortic root growth rate (2.9 ± 3.5 mm vs. 1.3 ± 2.6 mm per year, p = 0.001) and experienced aortic events at a younger age (33 ± 15 years vs. 40 ± 16 years, p = 0.001). Conclusion In our population MVP is highly prevalent in MFS and linked to greater aortic and ventricular remodelling. While it does not increase mortality or aortic and arrhythmic events, MVP is associated with faster aortic growth and earlier aortic events suggesting a more aggressive disease course. MVP may serve as a marker of severe MFS, warranting further research to refine risk stratification and management strategies.
Bela' et al. (Sat,) reported a other. Mitral valve prolapse affects 59.9% of Marfan patients and is linked to faster aortic root growth (2.9 vs 1.3 mm/yr, p=0.001) and earlier aortic events (33 vs 40 years, p=0.001).