Marfan Syndrome

"Marfan Syndrome" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity.

MeSH information

Definition | Details | More General Concepts | Related Concepts | More Specific Concepts

An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome is associated with mutations in the gene encoding fibrillin, a major element of extracellular microfibrils of connective tissue.

Descriptor ID	D008382
MeSH Number(s)	C05.116.099.674 C14.240.400.725 C14.280.400.725 C16.131.077.550 C16.131.240.400.720 C16.320.540 C17.300.500
Concept/Terms	Marfan Syndrome Marfan Syndrome Syndrome, Marfan Marfan Syndrome, Type I Marfan's Syndrome Marfans Syndrome Syndrome, Marfan's

Below are MeSH descriptors whose meaning is more general than "Marfan Syndrome".

Diseases [C]
Musculoskeletal Diseases [C05]
Bone Diseases [C05.116]
Bone Diseases, Developmental [C05.116.099]
Marfan Syndrome [C05.116.099.674]
Cardiovascular Diseases [C14]
Cardiovascular Abnormalities [C14.240]
Heart Defects, Congenital [C14.240.400]
Marfan Syndrome [C14.240.400.725]
Heart Diseases [C14.280]
Heart Defects, Congenital [C14.280.400]
Marfan Syndrome [C14.280.400.725]
Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
Congenital Abnormalities [C16.131]
Abnormalities, Multiple [C16.131.077]
Marfan Syndrome [C16.131.077.550]
Cardiovascular Abnormalities [C16.131.240]
Heart Defects, Congenital [C16.131.240.400]
Marfan Syndrome [C16.131.240.400.720]
Genetic Diseases, Inborn [C16.320]
Marfan Syndrome [C16.320.540]
Skin and Connective Tissue Diseases [C17]
Connective Tissue Diseases [C17.300]
Marfan Syndrome [C17.300.500]

Below are MeSH descriptors whose meaning is related to "Marfan Syndrome".

Below are MeSH descriptors whose meaning is more specific than "Marfan Syndrome".

publications

Timeline | Most Recent

This graph shows the total number of publications written about "Marfan Syndrome" by people in this website by year, and whether "Marfan Syndrome" was a major or minor topic of these publications.

Bar chart showing 30 publications over 12 distinct years, with a maximum of 7 publications in 2009

To see the data from this visualization as text, click here.

Year	Major Topic	Minor Topic	Total
2002	1	0	1
2006	2	1	3
2007	1	0	1
2008	2	0	2
2009	3	4	7
2011	1	1	2
2012	2	2	4
2014	3	0	3
2016	0	1	1
2017	0	2	2
2018	2	0	2
2021	2	0	2

Below are the most recent publications written about "Marfan Syndrome" by people in Profiles.

Burke CR, Bavaria J. The personalized external aortic root support procedure: interesting niche or ready for prime time? Heart. 2021 11; 107(22):1768-1769.

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Bellaire LL, Zhang C, Smith JT, Heflin JA, Klatt J, Roye D, Sponseller P, Samdani A, Fedorak GT. Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure. J Pediatr Orthop. 2021 Mar 01; 41(3):e204-e210.

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Rossin EJ, VanderVeen DK, Yonekawa Y. Bilateral Immediate Sequential Vitrectomy and Lensectomy for Bilateral Lens Dislocation in Severe Neonatal Marfan Syndrome. Ophthalmic Surg Lasers Imaging Retina. 2018 10 01; 49(10):e151-e153.

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Kirpekar MK, Silvay G, Augoustides JG, Castillo JG, Leong R, Sibbald R, Bainbridge D, Bishop CE, Fernando RJ, Morris B. The Adolescent Minor and Urgent Aortic Surgery: Challenges and Solutions With Capacity and Competence in Informed Consent. J Cardiothorac Vasc Anesth. 2019 Apr; 33(4):1139-1145.

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Newell K, Smith W, Ghoshhajra B, Isselbacher E, Lin A, Lindsay ME. Cervical artery dissection expands the cardiovascular phenotype in FBN1-related Weill-Marchesani syndrome. Am J Med Genet A. 2017 Sep; 173(9):2551-2556.

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Javadikasgari H, Roselli EE, Aftab M, Suri RM, Desai MY, Khosravi M, Cikach F, Isabella M, Idrees JJ, Raza S, Tappuni B, Griffin BP, Svensson LG, Gillinov AM. Combined aortic root replacement and mitral valve surgery: The quest to preserve both valves. J Thorac Cardiovasc Surg. 2017 05; 153(5):1023-1030.e1.

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Weinsaft JW, Devereux RB, Preiss LR, Feher A, Roman MJ, Basson CT, Geevarghese A, Ravekes W, Dietz HC, Holmes K, Habashi J, Pyeritz RE, Bavaria J, Milewski K, LeMaire SA, Morris S, Milewicz DM, Prakash S, Maslen C, Song HK, Silberbach GM, Shohet RV, McDonnell N, Hendershot T, Eagle KA, Asch FM. Aortic Dissection in Patients With Genetically Mediated Aneurysms: Incidence and Predictors in the GenTAC Registry. J Am Coll Cardiol. 2016 06 14; 67(23):2744-2754.

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Helder MR, Schaff HV, Dearani JA, Li Z, Stulak JM, Suri RM, Connolly HM. Management of mitral regurgitation in Marfan syndrome: Outcomes of valve repair versus replacement and comparison with myxomatous mitral valve disease. J Thorac Cardiovasc Surg. 2014 Sep; 148(3):1020-4; discussion 1024.

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Song HK, Preiss LR, Maslen CL, Kroner B, Devereux RB, Roman MJ, Holmes KW, Tolunay HE, Desvigne-Nickens P, Asch FM, Milewski RK, Bavaria J, LeMaire SA. Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions. J Heart Valve Dis. 2014 May; 23(3):292-8.

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Rylski B, Bavaria JE, Beyersdorf F, Branchetti E, Desai ND, Milewski RK, Szeto WY, Vallabhajosyula P, Siepe M, Kari FA. Type A aortic dissection in Marfan syndrome: extent of initial surgery determines long-term outcome. Circulation. 2014 Apr 01; 129(13):1381-6.

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