PDF(Pubmed)
Abstract：
Marfan syndrome (MFS) is a progressive connective tissue disease with a broad range of clinical manifestations. We sought to establish the spectrum of structural valvular abnormalities as cardiovascular involvement has been identified as the most life-threatening aspect of the syndrome. This was a systematic review with a meta-analysis of studies indexed in Medline from the inception of the database to November 7, 2022. Using the random-effects model, separate Forest and Galbraith plots were generated for each valvular abnormality assessed. Heterogeneity was assessed using the I2 statistics whilst funnel plots and Egger\'s test were used to assess for publication bias. From a total of 35 studies, a random-effects meta-analysis approximated the pooled summary estimates for the prevalence of cardiac valve abnormalities as mitral valve prolapse 65% (95% CI: 57%-73%); mitral valve regurgitation 40% (95% CI: 29%-51%); aortic valve regurgitation 40% (95% CI: 28%-53%); tricuspid valve prolapse 35% (95% CI: 15%-55%); and tricuspid valve regurgitation 43% (95% CI: 8%-78%). Only one study reported on the involvement of the pulmonary valve (pulmonary valve prolapse was estimated at 5.3% (95% CI: 1.9%-11.1%) in a cohort of 114 patients with MFS). We believe this study provides a description of the structural valvular disease spectrum and may help inform providers and patients in understanding the clinical history of MFS in the current treatment era with its increased life expectancy.
摘要：
马凡氏综合征（MFS）是一种进行性结缔组织疾病，临床表现广泛。我们试图建立结构性瓣膜异常的频谱，因为心血管受累已被确定为该综合征最危及生命的方面。这是一项系统评价，对Medline从数据库开始到2022年11月7日的研究进行了荟萃分析。使用随机效应模型，对于评估的每个瓣膜异常，分别生成Forest和Galbraith图。使用I2统计量评估异质性，同时使用漏斗图和Egger检验评估发表偏倚。在总共35项研究中，一项随机效应荟萃分析对心脏瓣膜异常患病率的汇总估计近似为二尖瓣脱垂65%(95%CI:57%-73%);二尖瓣关闭不全40%(95%CI:29%-51%);主动脉瓣关闭不全40%(95%CI:28%-53%);三尖瓣关闭不全35%(95%CI:15%-55%);只有一项研究报道了肺动脉瓣的受累（在114例MFS患者的队列中，肺动脉瓣脱垂估计为5.3％（95％CI：1.9％-11.1％））。我们相信这项研究提供了结构性瓣膜疾病谱的描述，并可能有助于告知提供者和患者了解当前治疗时代MFS的临床病史，并延长了预期寿命。
