Double aortic dissection in a patient with Marfan disease. A case report
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Abstract
Marfan syndrome is a dominant autosomal genetic disease of the connective tissue, leading to various complications. Cardiovascular complications are the main ones, with dilation of the aorta and aortic dissection which is the main cause of death. Double dissection with different sites of departure is scarcely reported by the literature. We reported the case of a 22-year’s-old young patient admitted for acute chest and abdominal pains, and cardiogenic shock. Investigations reported a double dissection of the aorta with a recent one of type A and an old one of type B. Ghent diagnostic criteria were used to allow the diagnosis of Marfan disease. Surgical management corrected successfully the type A dissection.
Early diagnostic and adequate management of aortic complications can reduce the high mortality rate of patients with Marfan syndrome.
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Hecht F, Beals RK. "New" syndrome of congenital contractural arachnodactyly originally described by Marfan in 1896. Pediatrics. 1972 Apr;49(4):574-9. PMID: 4552107.
Dietz HC, Cutting GR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, Puffenberger EG, Hamosh A, Nanthakumar EJ, Curristin SM, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991 Jul 25;352(6333):337-9. doi: 10.1038/352337a0. PMID: 1852208.
Dietz H, Francke U, Furthmayr H, Francomano C, De Paepe A, Devereux R, Ramirez F, Pyeritz R. The question of heterogeneity in Marfan syndrome. Nat Genet. 1995 Mar;9(3):228-31. doi: 10.1038/ng0395-228. PMID: 7773282.
Faivre L, Collod-Beroud G, Adès L, Arbustini E, Child A, Callewaert BL, Loeys B, Binquet C, Gautier E, Mayer K, Arslan-Kirchner M, Grasso M, Beroud C, Hamroun D, Bonithon-Kopp C, Plauchu H, Robinson PN, De Backer J, Coucke P, Francke U, Bouchot O, Wolf JE, Stheneur C, Hanna N, Detaint D, De Paepe A, Boileau C, Jondeau G. The new Ghent criteria for Marfan syndrome: what do they change? Clin Genet. 2012 May;81(5):433-42. doi: 10.1111/j.1399-0004.2011.01703.x. Epub 2011 Jun 2. PMID: 21564093.
Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, Hilhorst-Hofstee Y, Jondeau G, Faivre L, Milewicz DM, Pyeritz RE, Sponseller PD, Wordsworth P, De Paepe AM. The revised Ghent nosology for the Marfan syndrome. J Med Genet. 2010 Jul;47(7):476-85. doi: 10.1136/jmg.2009.072785. PMID: 20591885.
Higurashi M, Oda M, Iijima K, Iijima S, Takeshita T, Watanabe N, Yoneyama K. Livebirth prevalence and follow-up of malformation syndromes in 27,472 newborns. Brain Dev. 1990;12(6):770-3. doi: 10.1016/s0387-7604(12)80004-0. PMID: 2092586.
Grimes SJ, Acheson LS, Matthews AL, Wiesner GL. Clinical consult: Marfan syndrome. Prim Care. 2004 Sep;31(3):739-42, xii. doi: 10.1016/j.pop.2004.04.005. PMID: 15331256.
Chan YC, Ting CW, Ho P, Poon JT, Cheung GC, Cheng SW. Ten-year epidemiological review of in-hospital patients with Marfan syndrome. Ann Vasc Surg. 2008 Sep;22(5):608-12. doi: 10.1016/j.avsg.2008.04.005. Epub 2008 Jun 17. PMID: 18562163.
Nollen GJ, Groenink M, Tijssen JG, Van Der Wall EE, Mulder BJ. Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome. Eur Heart J. 2004 Jul;25(13):1146-52. doi: 10.1016/j.ehj.2004.04.033. PMID: 15231373.
Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968 Jul;23(4):338-9. doi: 10.1136/thx.23.4.338. PMID: 5664694; PMCID: PMC471799.
Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J, Eagle KA, Mehta RH, Nienaber CA, Pape LA; International Registry of Aortic Dissection (IRAD). Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). J Am Coll Cardiol. 2004 Feb 18;43(4):665-9. doi: 10.1016/j.jacc.2003.08.054. PMID: 14975480.
Grimes SJ, Acheson LS, Matthews AL, Wiesner GL. Clinical consult: Marfan syndrome. Prim Care. 2004 Sep;31(3):739-42, xii. doi: 10.1016/j.pop.2004.04.005. PMID: 15331256.
Silverman DI, Burton KJ, Gray J, Bosner MS, Kouchoukos NT, Roman MJ, Boxer M, Devereux RB, Tsipouras P. Life expectancy in the Marfan syndrome. Am J Cardiol. 1995 Jan 15;75(2):157-60. doi: 10.1016/s0002-9149(00)80066-1. PMID: 7810492.
Pyeritz RE. Marfan syndrome: 30 years of research equals 30 years of additional life expectancy. Heart. 2009 Mar;95(3):173-5. doi: 10.1136/hrt.2008.160515. Epub 2008 Nov 10. PMID: 19001001.
Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC); European Association for Cardio-Thoracic Surgery (EACTS), Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Barón-Esquivias G, Baumgartner H, Borger MA, Carrel TP, De Bonis M, Evangelista A, Falk V, Iung B, Lancellotti P, Pierard L, Price S, Schäfers HJ, Schuler G, Stepinska J, Swedberg K, Takkenberg J, Von Oppell UO, Windecker S, Zamorano JL, Zembala M. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J. 2012 Oct;33(19):2451-96. doi: 10.1093/eurheartj/ehs109. Epub 2012 Aug 24. PMID: 22922415.
David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992 Apr;103(4):617-21; discussion 622. PMID: 1532219.
Gao L, Zhou X, Zhang L, Wen D, Chang Q, Wu Y, Sun L, Hui R. Factors influencing prognosis in patients with marfan syndrome after aortic surgery. J Cardiothorac Vasc Anesth. 2011 Aug;25(4):625-31. doi: 10.1053/j.jvca.2010.11.019. Epub 2011 Jan 22. PMID: 21262573.
Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation. 2005 Mar 22;111(11):e150-7. doi: 10.1161/01.CIR.0000155243.70456.F4. PMID: 15781745.