治疗马方综合征的最重要因素是诊断出本病，对主动脉的扩张进行仔细地长期随访，当主动脉根部面积/身高比值达到 10，或者主动脉窦处主动脉根直径达到 4.5-5.0 cm 时可考虑手术治疗，急诊手术用于治疗急性主动脉夹层。应用 β 受体阻滞剂进行控制，并在适当时机进行手术治疗的情况下，远期生存情况良好。即使急性夹层通过手术得以成功治疗，也会导致生存率下降。[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 然而，早期诊断随后进行现代内外科综合管理确实提高了患者的生活质量和寿命。[60]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
妊娠期主动脉手术提高了母婴生存几率。[61]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
[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].
