Journal of Chest Surgery

  • 제47권6호
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  • Pages.536-540
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  • 2014
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  • 2765-1606(pISSN)
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  • 2765-1614(eISSN)
대한심장혈관흉부외과학회 (The Korean Society for Thoracic and Cardiovascular Surgery)
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Multiple Aortic Operations in Loeys-Dietz Syndrome: Report of 2 Cases

  • Na, Kwon Joong (Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine) ;
  • Park, Kay-Hyun (Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine)
  • 투고 : 2014.05.08
  • 심사 : 2014.09.03
  • 발행 : 2014.12.05
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초록

Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often misdiagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology. However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making. We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

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참고문헌

  1. Woo EY, Mcgarvey M, Jackson BM, Bavaria JE, Fairman RM, Pochettino A. Spinal cord ischemia may be reduced via a novel technique of intercostal artery revascularization during open thoracoabdominal aneurysm repair. J Vasc Surg 2007;46:421-6. https://doi.org/10.1016/j.jvs.2007.04.048
  2. Loeys BL, Schwarze U, Holm T, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med 2006;355:788-98. https://doi.org/10.1056/NEJMoa055695
  3. Aalberts JJ, van den Berg MP, Bergman JE, et al. The many faces of aggressive aortic pathology: Loeys-Dietz syndrome. Neth Heart J 2008;16:299-304. https://doi.org/10.1007/BF03086168
  4. Williams JA, Loeys BL, Nwakanma LU, et al. Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease. Ann Thorac Surg 2007;83:S757-63. https://doi.org/10.1016/j.athoracsur.2006.10.091
  5. Kim HW, Lee TY, Moon DH, Choo SJ, Chung CH, Lee JW. Ascending aortic rupture in a young woman with Loeys-Dietz syndrome: the first case report in Korea. Korean J Thorac Cardiovasc Surg 2009;42:639-44.
  6. Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010;47:476-85. https://doi.org/10.1136/jmg.2009.072785