Janzen J, Vuong P N, Rothenberger-Janzen K
Unité d'Anatomie et de Cytologie Pathologiques, Hôpital Saint-Michel, Paris, France.
Heart Vessels. 1999;14(6):277-82. doi: 10.1007/BF03257239.
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).
主动脉缩窄通常发生在主动脉峡部；然而，所有主动脉缩窄病例中有2%位于非典型部位，如主动脉弓以及胸主动脉和/或腹主动脉。病因治疗需要进行血管手术，在此过程中，应采集受累血管的活检样本以确定病因。本研究对7例接受血管外科手术的患者（年龄范围10 - 37岁；男女比例为3:4）的临床和组织病理学结果进行了回顾性分析。标本的组织病理学分析显示，主动脉壁不同部位发生了两种病理过程，这是主动脉非典型缩窄的原因：（1）中膜和外膜的纤维化/瘢痕形成，符合高安动脉炎的慢性病变（4例）；（2）中膜纤维肌发育不良（3例）。
