Sama Carlson, Fongwen Noah T, Chobufo Muchi Ditah, Ajibade Ademola, Roberts Melissa, Greathouse Mark, Ngonge Anthony Lyonga, Adekolu Ayowumi, Hamirani Yasmin S
Internal Medicine, West Virginia University School of Medicine, Morgantown, USA.
Public Health Sciences, Africa Centre for Disease Control and Prevention (Africa CDC), Addis Ababa, ETH.
Cureus. 2024 Feb 13;16(2):e54141. doi: 10.7759/cureus.54141. eCollection 2024 Feb.
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 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.
马凡综合征（MFS）是一种进行性结缔组织疾病，临床表现广泛。由于心血管受累已被确定为该综合征最危及生命的方面，我们试图确定结构性瓣膜异常的范围。这是一项系统评价，并对从数据库建立到2022年11月7日在Medline上索引的研究进行荟萃分析。使用随机效应模型，为每个评估的瓣膜异常生成单独的森林图和加尔布雷斯图。使用统计量评估异质性，同时使用漏斗图和埃格检验评估发表偏倚。在总共35项研究中，随机效应荟萃分析近似得出心脏瓣膜异常患病率的汇总估计值，二尖瓣脱垂为65%（95%CI：57%-73%）；二尖瓣反流为40%（95%CI：29%-51%）；主动脉瓣反流为40%（95%CI：28%-53%）；三尖瓣脱垂为35%（95%CI：15%-55%）；三尖瓣反流为43%（95%CI：8%-78%）。只有一项研究报告了肺动脉瓣受累情况（在一组114例马凡综合征患者中，肺动脉瓣脱垂估计为5.3%（95%CI：1.9%-11.1%））。我们认为这项研究描述了结构性瓣膜疾病谱，可能有助于告知医疗服务提供者和患者，在当前预期寿命增加的治疗时代，了解马凡综合征的临床病史。
