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Takayasu’s arteritis and fibromuscular dysplasia as causes of acquired atypical coarctation of the aorta: retrospective analysis of seven cases

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Summary

Coarctation of the aorta usually occurs in the aortic isthmus; however, 2% of all coarctation is localized at atypical sites such as the aortic arch and the thoracic and/or abdominal aorta. Causal therapy involves vascular surgery, and during the procedure, biopsies of the involved vessels should be taken to establish the etiology. This study involved the retrospective analysis of clinical and histopathological findings for seven patients who underwent vascular surgical procedures (age range, 10–37 years; male/female ratio 3∶4). Histopathological analysis of specimens revealed two pathological processes taking place with different localization in the aortic wall as the cause of the atypical coarctation of the aorta: (1) fibrosis/scarification in the tunica media and adventitia compatible with chronic lesions of Takayasu’s arteritis (four cases); (2) fibromuscular dysplasia in the tunica media (three cases).

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Authors and Affiliations

  1. Unité d’Anatomie et de Cytologie Pathologiques, Hôpital Saint-Michel, 33, rue Olivier de Serres, F-75015, Paris, France

    Jan Janzen,  Phat N. Vuong & Katrin Rothenberger-Janzen

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  1. Jan Janzen
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  2. Phat N. Vuong
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  3. Katrin Rothenberger-Janzen
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This article is dedicated to Prof. K. Burger on his 65th birthday

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Janzen, J., Vuong, P.N. & Rothenberger-Janzen, K. Takayasu’s arteritis and fibromuscular dysplasia as causes of acquired atypical coarctation of the aorta: retrospective analysis of seven cases. Heart and Vessels 14, 277–282 (1999). https://doi.org/10.1007/BF03257239

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  • DOI: https://doi.org/10.1007/BF03257239

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