Prevalence is thought to be similar throughout the world and regardless of sex or ethnicity.[5]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
Men and women are affected equally, however men tend to be more severely affected in the cardiovascular system. The incidence in the US population is not accurately known but is estimated to be 1 in 10,000.[6]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.long
http://www.ncbi.nlm.nih.gov/pubmed/8151638?tool=bestpractice.com
The incidence in the European population is estimated to be 3 in 10,000.[7]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.html
http://www.ncbi.nlm.nih.gov/pubmed/20372188?tool=bestpractice.com
The average life expectancy used to be only 32 years but due to early surgery, and general increase in life expectancy, is now 72 years and approaching that of the general population.[8]Silverman DI, Burton KJ, Gray J, et al. Life expectancy in the Marfan syndrome. Am J Cardiol. 1995 Jan 15;75(2):157-60.
http://www.ncbi.nlm.nih.gov/pubmed/7810492?tool=bestpractice.com
Once aortic dissection occurs, survival is considerably reduced to between 50% and 70% at 5 years.[9]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
[10]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]: Impact of aortic dissection on patient survivalFrom the collection of LG Svensson, E Mendrinos, C Pournaras [Citation ends].