Centre of Cardiology and Cardiovascular Surgery, University Hospital Eppendorf, Hamburg, Germany.
Am J Cardiol. 2010 Oct 1;106(7):1048-53. doi: 10.1016/j.amjcard.2010.05.038.
Mitral valve (MV) prolapse (MVP) has a high prevalence of 2% to 3% in the general population and thus constitutes the most common cause of severe nonischemic MV regurgitation (MVR). MVP is also common in persons with the Marfan syndrome. However, to date, a large-scale population-based cohort study using modern echocardiographic techniques has not been performed, and the frequency of MVP and the relation of MV dysfunction and age have not been investigated. Therefore, we conducted a population-based cohort study of 204 patients (108 males and 96 females, aged 31.2 ± 16.4 years) with classic Marfan syndrome. We performed echocardiographic follow-up of 174 patients for a mean of 4.4 ± 4.3 years. On the initial or subsequent echocardiographic scan, MVP was present in 82 patients (40%), severe MVR in 25 (12%), and MV endocarditis in 5 patients (2.5%). At 30 years of age, the Weibull cumulative distribution was 42.6% (95% confidence interval [CI] 36% to 50%) for MVP, 56.5% (95% CI 49.3% to 64%) for MVR of any degree, 6.7% (95% CI 3.9% to 11.3%) for severe MVR, and 0.92% (95% CI 0.21% to 3.91%) for MV endocarditis. The cumulative hazard for severe MVR and MV endocarditis was estimated to increase with age. MVP was associated with dural ectasia (p = 0.01), ectopia lentis (p = 0.02), and skeletal involvement (p <0.001). Severe MVR was related to tricuspid valve prolapse (p = 0.002) and to the sporadic form of the Marfan syndrome (p = 0.006). In conclusion, MVP was comparatively frequent in patients with the Marfan syndrome and carries an increased risk of progression to severe MVR and endocarditis, especially in older adults.
二尖瓣脱垂（MVP）在普通人群中的患病率为 2%至 3%，因此是严重非缺血性二尖瓣反流（MVR）最常见的原因。MVP 在马凡综合征患者中也很常见。然而，迄今为止，尚未使用现代超声心动图技术进行大规模的基于人群的队列研究，MVP 的频率以及二尖瓣功能障碍与年龄的关系尚未得到研究。因此，我们对 204 例（108 例男性，96 例女性，年龄 31.2±16.4 岁）具有典型马凡综合征的患者进行了基于人群的队列研究。我们对 174 例患者进行了平均 4.4±4.3 年的超声心动图随访。在初始或后续的超声心动图扫描中，82 例患者（40%）存在 MVP，25 例（12%）存在严重 MVR，5 例（2.5%）存在 MVP 感染性心内膜炎。在 30 岁时，MVP 的威布尔累积分布为 42.6%（95%置信区间 [CI] 36%至 50%），任何程度的 MVR 为 56.5%（95% CI 49.3%至 64%），严重 MVR 为 6.7%（95% CI 3.9%至 11.3%），MVP 感染性心内膜炎为 0.92%（95% CI 0.21%至 3.91%）。严重 MVR 和 MVP 感染性心内膜炎的累积风险估计随年龄增加而增加。MVP 与硬脊膜扩张（p=0.01）、晶状体异位（p=0.02）和骨骼受累（p<0.001）有关。严重 MVR 与三尖瓣脱垂（p=0.002）和马凡综合征的散发性形式（p=0.006）有关。总之，马凡综合征患者 MVP 较为常见，进展为严重 MVR 和感染性心内膜炎的风险增加，尤其是在老年人中。
