不论性别或种族，流行率在世界各地类似[3]Grimes SJ, Acheson LS, Mathews AL, et al. Clinical consult: Marfan's syndrome. Prim Care. 2004 Sep;31(3):739-42, xii.http://www.ncbi.nlm.nih.gov/pubmed/15331256?tool=bestpractice.com 欧洲的估测发病率为3/10000。[4]Arslan-Kirchner M, Arbustini E, Boileau C, et al. Clinical utility gene card for: Marfan syndrome type 1 and related phenotypes [FBN1]. Eur J Hum Genet. 2010 Sep;18(9).http://www.nature.com/ejhg/journal/v18/n9/full/ejhg201042a.htmlhttp://www.ncbi.nlm.nih.gov/pubmed/20372188?tool=bestpractice.com 美国人口的发病率不确定，但估测为1/10000。[5]Gray JR, Bridges AB, Faed MJ, et al. Ascertainment and severity of Marfan syndrome in a Scottish population. J Med Genet. 1994 Jan;31(1):51-4.http://jmg.bmj.com/content/31/1/51.longhttp://www.ncbi.nlm.nih.gov/pubmed/8151638?tool=bestpractice.com 平均寿命可能只有32年，但由于早期接受手术，目前平均寿命接近一般人群。 一旦发生主动脉夹层，5年的生存率将减少到50%~70%。[6]Svensson LG, Crawford ES, Coselli JS, et al. Impact of cardiovascular operation on survival in the Marfan patient. Circulation. 1989 Sep;80(3 Pt 1):I233-42.http://www.ncbi.nlm.nih.gov/pubmed/2766531?tool=bestpractice.com[7]Svensson LG, Blackstone EH, Feng J, et al. Are Marfan syndrome and marfanoid patients distinguishable on long-term follow-up? Ann Thorac Surg. 2007 Mar;83(3):1067-74.http://www.ncbi.nlm.nih.gov/pubmed/17307461?tool=bestpractice.com
[Figure caption and citation for the preceding image starts]: 主动脉夹层对患者生存率的影响由LG Svensson, E Mendrinos, C Pournaras收集 [Citation ends].
