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Clinical Investigation| Volume 365, ISSUE 5, P420-428, May 2023

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A single-center cohort of mid-aortic syndrome among adults in China: Etiology, presentation and imaging features

  • Author Footnotes
    # Xu Meng and Jinhong Xue contributed equally to this work.
    Xu Meng
    Footnotes
    # Xu Meng and Jinhong Xue contributed equally to this work.
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Author Footnotes
    # Xu Meng and Jinhong Xue contributed equally to this work.
    Jinhong Xue
    Footnotes
    # Xu Meng and Jinhong Xue contributed equally to this work.
    Affiliations
    Department of Cardiology, The Fifth Central Hospital of Tianjin, Tianjin, China
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  • Jun Cai
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Huimin Zhang
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Wenjun Ma
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Haiying Wu
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Xianliang Zhou
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Ying Lou
    Correspondence
    Corresponding authors. Ying Lou, MD and Linping Wang, MD
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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  • Linping Wang
    Correspondence
    Corresponding authors. Ying Lou, MD and Linping Wang, MD
    Affiliations
    Department of Cardiology, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
    Search for articles by this author
  • Author Footnotes
    # Xu Meng and Jinhong Xue contributed equally to this work.

      Abstract

      Background

      Mid-aortic syndrome (MAS), characterized by segmental stricture of the distal thoracic and abdominal aorta, is a heterogeneous clinical syndrome with multiple etiologies.

      Methods

      We retrospectively analyzed 143 consecutive patients (99 females and 44 males, mean age 40.93 ± 15.31 years) with MAS seen from January 1, 2010 to January 1, 2019.

      Results

      Takayasu arteritis (76.9%, 110/143) and atherosclerosis (19.6%, 28/143) were the most-common causes. There were also one patient with Behçet's disease and one with congenital MAS in the cohort. Hypertension was the most-common manifestation. Constitutional symptoms were mainly seen in Takayasu arteritis, and neurological, gastrointestinal and vascular symptoms were common in both Takayasu arteritis and atherosclerosis. The infrarenal segment was the most-commonly involved in atherosclerosis (89.3%, 25/28), whereas lesions were more distributed in Takayasu arteritis. The mean length of involved segments was longer (43.45 ± 23.64 mm vs. 30.68 ± 12.66 mm; P = 0.018) and the degree of stenosis was lower (80.20 ± 13.36% vs. 87.50 ± 13.95%, P = 0.004) in Takayasu arteritis than atherosclerosis. The most-common concurrently involved branch was the renal artery, followed by the celiac trunk and mesenteric arteries, in both Takayasu arteritis (51.8%, 32.7% and 27.3%, respectively) and atherosclerosis (53.6%, 25.0% and 17.9%, respectively). Concurrent artery involvement and coexisting lesions were absent in MAS caused by congenial coarctation of the abdominal aorta and Behçet's disease.

      Conclusions

      Takayasu arteritis and atherosclerosis were the most-common causes of MAS among these adults. Imaging tests provided evidence of involved segments and luminal and mural changes, aiding conclusive diagnoses and etiological differentiation of MAS.
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