1981. Aortic dissection and congestive heart failure due to aortic and mitral valvular anomalies accounted for over 90% of the known causes of death. Robins PR, Moe JH, Winter RB. 1258926-overview Thorac Cardiovasc Surg. 143A (24):3137-9. [Medline]. [Full Text]. Diseases & Conditions, 2002 [Medline]. [Medline].

Marfan syndrome-diagnosis and management. … Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. 2012 May.

What is the best treatment for my condition. McKusick VA. [61], Aortic surgery during pregnancy has increased the chance of preservation of lives of both mother and baby. Patients with Marfan syndrome also need routine eye care for optical correction of refractive errors, periodic monitoring of intraocular pressure, and anterior segment examination with a slit-lamp to assess the eye for lens location and opacities. Specific attention and more frequent monitoring (once every trimester) is required in pregnant women or women planning pregnancy.

Improving clinical recognition of Marfan syndrome.

1994 May 12. 946315-overview 47 (7):476-85. 2010 Mar.

Dietz HC, Loeys B, Carta L, Ramirez F. Recent progress towards a molecular understanding of Marfan syndrome.

Information provided on this site is for informational purposes only; it is not intended as a substitute for advice from your own medical team. Svensson LG, Crawford ES, Coselli JS, et al. 1986 Dec. 103(6 pt 1):2499-509. Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome. [Medline]. 286(15):804-8. 2007 Nov 29. Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug ReferenceDisclosure: Received salary from Medscape for employment. 2009 Mar;95(3):173-5. J Bone Joint Surg Am. These patients should also receive genetic counseling about their 50% risk of passing on their condition to their children. Mariucci EM, Lovato L, Rosati M, Palena LM, Bonvicini M, Fattori R. Dilation of peripheral vessels in Marfan syndrome: importance of thoracoabdominal MR angiography. 79:684-733. Clin Genet. The information on this site is not to be used for diagnosing or treating any health concerns you may have - please contact your physician or health care professional for all your medical needs. [, Thornhill AR, Handyside AH, Ottolini C, et al. Help Clin Genet. 1994 Sep. 9 (5):482-9. Scripting". After acute dissection or surgical repair of chronic dissection, an initial checkup should be scheduled every 3 months, then every 1 to 2 years, and include echo and MRI/CT scan of the thorax. Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, et al. Before the advent of pharmacologic and surgical therapy for aortic root and valvular disease, the life expectancy for patients with MFS was about two thirds that of the healthy population. The cardiovascular aspects of Marfan's syndrome: a heritable disorder of connective tissue. This page cannot be displayed because your browser has been 9(2):93-8. Prashanth Inna, MBBS, MS, DNB is a member of the following medical societies: Medical Council of India, Indian Orthopedic Association, National Academy of Medical Sciences (India)Disclosure: Nothing to disclose.

[Medline]. 1972 Apr 13.

Adult with Marfan syndrome. Patients are instructed to seek immediate ocular examination if they develop ophthalmologic symptoms such as the perception of floaters (myodesopsia), flashing lights (photopsia), glare, or visual field defect. [Medline]. Marfan-Syndrom: Krankheitsverlauf und Prognose Die Übertragungswahrscheinlichkeit der Mutation von einem Elternteil auf ein Kind liegt bei 50 Prozent.

[Medline]. 7 (5):266-76. 1989 Sep;80(3 Pt 1):I233-42.

Send feedback FDA reporting form, 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 cm to 5.0 cm, and emergency surgery for acute dissection. Nature.

Available at http://www.marfan.org/dx/revised-ghent-nosology. Eur J Vasc Endovasc Surg. The prognosis of Marfan syndrome largely depends on the severity of the complications previously mentioned and the degree of progressive aortic dilation which can lead to death at a young age.

Nat Rev Cardiol. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly93d3cubWVkc2NhcGUuY29tL2Fuc3dlcnMvMTI1ODkyNi0xMDgzNTkvd2hhdC1pcy10aGUtcHJvZ25vc2lzLW9mLW1hcmZhbi1zeW5kcm9tZS1tZnM=. Charles T Mehlman, DO, MPH is a member of the following medical societies: American Academy of Pediatrics, American Fracture Association, Scoliosis Research Society, Pediatric Orthopaedic Society of North America, American Medical Association, American Orthopaedic Foot and Ankle Society, American Osteopathic Association, Arthroscopy Association of North America, North American Spine Society, Ohio State Medical AssociationDisclosure: Nothing to disclose. This may include: netball, basketball, tennis, golf, baseball, football, swinging an axe or spade, weightlifting, and suddenly lifting something heavy. Are Marfan syndrome and marfanoid patients distinguishable on long-term follow-up? However, the life expectancy for patients with Marfan syndrome has improved over time, presumably due to improved detection and intervention, including surgical procedures and the use of beta-blockers. configured to disable JavaScript, also known as "Active

2008 Feb. 135 (2):389-94. 7(9):e44639. A newly recognized syndrome of Marfanoid habitus; long face; hypotelorism; long, thin nose; long, thin hands and feet; and a specific pattern of language and learning disabilities. [Medline]. Shores J, Berger KR, Murphy EA, Pyeritz RE. and What is the prognosis of Marfan syndrome (MFS)?

2010 Jul. [28] Gjolaj JP, Sponseller PD, Shah SA, Newton PO, Flynn JM, Neubauer PR, et al. Heart. 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 cm to 5.0 cm, and emergency surgery for acute dissection. Annu Rev Med.

N Engl J Med. 2007 Mar;83(3):1067-74. [Full Text]. Patients with an aortic diameter <4.5 cm or aortic root area/body height ratio <10, treated with beta-blockers or verapamil, have a repeat echo and CT of thorax after 3 to 6 months, and then the trend is noted at yearly intervals. J Assist Reprod Genet. 2007 Dec 15. Procedures, 2002 139C(1):4-9. J Card Surg. 58(2):76-80.

Ammash NM, Sundt TM, Connolly HM. 5th ed. 2010 May.

Complications associated with surgical repair of syndromic scoliosis. [Medline]. [Medline]. Multi-scale biomechanical remodeling in aging and genetic mutant murine mitral valve leaflets: insights into marfan syndrome. 2015. This website also contains material copyrighted by 3rd parties. http://www.nhlbi.nih.gov/health/dci/Diseases/mar/mar_diagnosis.html, http://www.marfan.org/dx/revised-ghent-nosology, National Academy of Medical Sciences (India), Pediatric Orthopaedic Society of North America, American Orthopaedic Foot and Ankle Society. Share cases and questions with Physicians on Medscape consult. Copyright © 2014 Health Grades Inc. All rights reserved.

Patient longevity now approaches that of persons without MFS, although cardiovascular compromise is still the most common cause of patient death, likely due to sudden death in the previously undiagnosed patient and a new diagnosis in those whose disease process has progressed beyond the scope of medical or surgical cure.

Circulation. 77(6):867-76. Spinal deformities in Marfan syndrome. 2016 Jan;77(1):38-41.

Please confirm that you would like to log out of Medscape. 1975 Apr.

Cañadas V, Vilacosta I, Bruna I, Fuster V. Marfan syndrome.

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