Marfan syndrome and aortic dissection in Bamako: a case report and literature review.
DOI:
https://doi.org/10.5281/zenodo.15515863Keywords:
Marfan syndrome, aortic dissection, BamakoAbstract
Acute aortic dissections account for 85 to 95% of acute aortic syndromes. This is a potentially fatal pathology of the aortic wall due to the spontaneous occurrence of an intimal lesion causing blood to escape from the arterial lumen to the media, leading to the formation of a false circulatory channel. We describe a dissection of the ascending aorta in a 30-year-old man with Marfan syndrome. It was complicated by severe aortic insufficiency with a PHT at 121 ms and severe renal failure with a GFR of 10 mL/min/m². A CMD with LVEF 34%, an infectious syndrome, and abnormal liver function tests with severe blood clots were detected. The patient received only medical care and died of congestive heart failure.
References
American College of Cardiology (ACA) guidelines 2010.
Kumar, A. Aortic Dissection. Critical Care Secrets, 2013, 3140, 204-211.
Hagan, P.G., Nienaber, C.A., Isselbacher, E.M., Bruckman, D., Karavite, D.J. et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.JAMA, 2000, 283 (7), 897-903.
Erbel, R., Alfonso, F., Boileau, C., Dirsch, O., Eber, B. et al. Task Force on Aortic Dissection, European Society of Cardiology. Diagnosis and management of aortic dissection. Eur Heart J, 2001, 22 (18), 1642-1681
Ramsey S. Elsayed, MD, Robbin G. Cohen, MD, MMM, Fernando Fleischman, MD, Michael E. Bowdish,MD. Acute Type A Aortic dissection. Cardiol Clin2017 ;http://dx.doi.org/10.1016/j.ccl.2017.03.004
Hachulla A-L, Ronot M, Noble S, et al. ECG-triggered high-pitch CT for simultaneousassessment of the aorta and coronary arteries. J Cardiovasc Comput Tomogr 2016;10(5):4077
Cohen RG, Hackmann AE, Fleischman F, et al. Type A Aortic Dissection: HowI Teach It. Ann Thorac Surg 2017;103(1):14–7.
Harris KM, Braverman AC, Eagle KA et al. Acute aortic intramural hematoma. Circulation. 2012;126(11 suppl 1):S91-S96.
Bonser RS, Ranasinghe AM, Loubani M, Evans JD, Thalji NMA, Bachet JE et al. Evidence, Lack of Evidence, Controversy, and Debate in the Provision and Performance of the Surgery of Acute Type A Aortic Dissection. J Am Coll Cardiol 2011;58:2455–74
Harris KM, Braverman AC, Eagle KA et al. Acute aortic intramural hematoma. Circulation. 2012;126(11 suppl 1):S91-S96.
Evangelista A, Mukherjee D, Mehta RH et al. Acute intramural hematoma of the aorta.Circulation.2005;111(8):1063-1070.
Tsai TT, Nienaber CA, Eagle KA. Acute aortic syndromes. Circulation.2005;112(24):3802-3813.
Daily PO, Trueblood HW, Stinson EB, Wuerflein RD, Shumway NE. Management ofacute aortic dissections. Ann Thorac Surg. 1970;10(3):237-247.
Debakey ME, Henly WS, Cooley DA, Morris GC Jr, Crawford ES, Beall AC Jr. Surgical management of dissecting aneurysms of the aorta. J Thorac Cardiovasc Surg. 1965;49:130-149.
ilacosta I, om nJA. Acute aortic syndrome. Heart. 2001;85 4 :365-368.
Mussa FF, Horton JD, Moridzadeh R, et al. Acute aortic dissection and intramural hematoma. JAMA 2016;316(7):754–810.
Cecconi M, Chirillo F, Costantini C, et al. The role of transthoracic echocardiography inthe diagnosis and management of acute typeA aortic syndrome. Am Heart J 2012;163(1):112–8. 31
S. G. Coelho et A. G. Almeida, « Síndrome de Marfan revisitada–da genética à clínica », Rev. Port.Cardiol., vol. 39, no 4, p. 215-226, avr. 2020, doi: 10.1016/j.repc.2019.09.008.
B. L. Loeys et al., « The revised Ghent nosology for the Marfan syndrome », J. Med. Genet., vol. 47, no 7, p.476-485, juill. 2010, doi: 10.1136/jmg.2009.072785.
J. A. N. Meester, A. Verstraeten, D. Schepers, M. Alaerts, L. Van Laer, et B. L. Loeys, «Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and LoeysDietz syndrome », Ann.Cardiothorac. Surg., vol. 6, no 6, p. 582-594, nov. 2017, doi: 10.21037/acs.2017.11.03.
Willoteaux S, Gaxotte V, Lions C, Mounier-Vehier C, Asseman P, Prat A,et al.Dissection aortique et syndrome de malperfusion Indications et techniques des traitementsendovasculaires. Médecine Thérapeutique Cardiol.2003May1;(2)77-84.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 IPHO-Journal of Advance Research in Medical & Health Science
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Author(s) and co-author(s) jointly and severally represent and warrant that the Article is original with the author(s) and does not infringe any copyright or violate any other right of any third parties and that the Article has not been published elsewhere. Author(s) agree to the terms that the IPHO Journal will have the full right to remove the published article on any misconduct found in the published article.
