Cor Vasa 2017, 59(2):e174-e176 | DOI: 10.1016/j.crvasa.2016.05.001

An unexpected complication. First descriptions of intercostal artery rupture in Ehlers-Danlos syndrome

Akram A. Youssef, Uwe Speiser, Karim Ibrahim, Christian Pfluecke, Felix M. Heidrich, Stephan Wiedemann, Ruth H. Strasser, Silvio Quick*
Technische Universität Dresden, Heart Center, University Hospital, Department of Internal Medicine and Cardiology, Dresden, Germany

A case of Ehlers-Danlos syndrome of vascular type with a history of mechanical valve prosthesis was admitted with dyspnea and chest pain. After exclusion a myocardial infarction, an angio-CT was performed and revealed a spontaneous rupture of an intercostal artery with subsequent hemothorax and compression atelectasis. The case was complicated by the ongoing anticoagulant therapy for the mechanical valve prosthesis with warfarin. After failure of conservative therapy, the case underwent surgical treatment. As shown in this case, mechanical valve prosthesis with necessary adequate anticoagulation, using long lasting warfarin derivates, could be an unneeded added risk for these patients.

Keywords: Anticoagulation; Aortic valve replacement; Ascending aorta aneurysm; Ehlers-Danlos syndrome; Intercostal artery rupture; Mechanical heart valve

Received: April 16, 2016; Accepted: May 4, 2016; Published: April 1, 2017  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Youssef AA, Speiser U, Ibrahim K, Pfluecke C, Heidrich FM, Wiedemann S, et al.. An unexpected complication. First descriptions of intercostal artery rupture in Ehlers-Danlos syndrome. Cor Vasa. 2017;59(2):e174-176. doi: 10.1016/j.crvasa.2016.05.001.
Download citation

References

  1. J.R. Mao, J. Bristow, The Ehlers-Danlos syndrome: on beyond collagens, The Journal of Clinical Investigation 107 (2001) 1063-1069. Go to original source... Go to PubMed...
  2. D.P. Germain, Ehlers-Danlos syndrome type IV, Orphanet Journal of Rare Diseases 2 (2007) 32. Go to original source... Go to PubMed...
  3. M. Pepin, U. Schwarze, A. Superti-Furga, P.H. Byers, Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type, New England Journal of Medicine 342 (2000) 673-680. Go to original source... Go to PubMed...
  4. D.R. Brinster, R.J. Rizzo, R.M. Bolman III, Ascending aortic aneurysms, in: L.H. Cohn (Ed.), Cardiac Surgery in the Adult, 3rd ed., McGraw-Hill, New York, NY, 2008, 1223.
  5. S.A. Jaffe, M.E. Geltman, E.G. Rodey, J. Uitto, Mitral valve prolapse: a consistent manifestation of type IV Ehlers-Danlos syndrome. The pathogenetic role of the abnormal production of type III collagen, Circulation 64 (1981) 121-125. Go to original source... Go to PubMed...




Cor et Vasa

You are accessing a site intended for medical professionals, not the lay public. The site may also contain information that is intended only for persons authorized to prescribe and dispense medicinal products for human use.

I therefore confirm that I am a healthcare professional under Act 40/1995 Coll. as amended by later regulations and that I have read the definition of a healthcare professional.