Life expectancy of a patient with Marfan syndrome is approximately the same as the general population with a timely diagnosis and multidisciplinary management.[3]Tinkle BT, Lacro RV, Burke LW, et al. Health supervision for children and adolescents with Marfan syndrome. Pediatrics. 2023 Apr 1;151(4):e2023061450.
http://www.ncbi.nlm.nih.gov/pubmed/36938616?tool=bestpractice.com
The most important factors in treatment of Marfan syndrome are the diagnosis of the condition, careful long-term follow-up for aortic expansion, referral for surgery when the aortic root area/body height ratio reaches 10 or aortic root diameter in the sinus of Valsalva reaches 4.5 to 5.0 cm, and emergency surgery for acute dissection. Long-term survival is excellent with beta-blocker control and surgery when indicated. Acute dissection results in a reduced survival even if successfully treated.[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
However, early diagnosis followed by modern medical and surgical management has definitely improved quality and length of life.[84]Pyeritz RE. Marfan syndrome: 30 years of research equals 30 years of additional life expectancy. Heart. 2009 Mar;95(3):173-5.
http://www.ncbi.nlm.nih.gov/pubmed/19001001?tool=bestpractice.com
Aortic surgery during pregnancy has increased the chance of preservation of lives of both mother and baby.[85]Yates MT, Soppa G, Smelt J, et al. Perioperative management and outcomes of aortic surgery during pregnancy. J Thorac Cardiovasc Surg. 2015 Feb;149(2):607-10.
http://www.ncbi.nlm.nih.gov/pubmed/25524653?tool=bestpractice.com
[86]Sakaguchi M, Kitahara H, Seto T, et al. Surgery for acute type A aortic dissection in pregnant patients with Marfan syndrome. Eur J Cardiothorac Surg. 2005 Aug;28(2):280-3; discussion 283-5.
https://www.doi.org/10.1016/j.ejcts.2005.02.047
http://www.ncbi.nlm.nih.gov/pubmed/15939618?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].