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

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.