Baek Seung Min, Lee Yoon Seong, Song Mi Kyoung, Lee Sang Yun, Bae Eun Jung, Kim Gi Beom
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Seoul, Jongno-gu, 03080, South Korea.
Department of Radiology, Seoul National University Hospital, Seoul, South Korea.
Pediatr Cardiol. 2025 Feb;46(2):312-323. doi: 10.1007/s00246-023-03399-0. Epub 2024 Jan 29.
Midaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-defined diagnostic criteria in pediatric patients. This retrospective study aimed to aid in the diagnosis of MAS by employing computed tomography (CT) to measure the z-score of the aorta as well as to identify and understand its clinical features. CT images, echocardiography findings, and medical records of 17 patients diagnosed with MAS between 1997 and 2023 were reviewed, and z-scores were calculated. Aortic size on follow-up CT, blood pressure, and left ventricular function and hypertrophy at the last follow-up were analyzed, and possible prognostic factors were examined. Except for one patient, all individuals exhibited a z-score below - 2 at the level corresponding to stenosis. Left ventricular dysfunction occurred more frequently in patients aged < 5 years (p = 0.024). Patients with idiopathic MAS showed a better prognosis in terms of blood pressure and follow-up aortic size (p = 0.051 and 0.048, respectively). CT-measured aortic z-scores may be useful for the diagnosis and follow-up of MAS.
由于缺乏针对儿科患者的明确诊断标准，中段主动脉综合征（MAS）的诊断存在挑战。这项回顾性研究旨在通过使用计算机断层扫描（CT）测量主动脉的z评分以及识别和了解其临床特征，来辅助MAS的诊断。回顾了1997年至2023年间17例诊断为MAS患者的CT图像、超声心动图检查结果和病历，并计算z评分。分析了随访CT上的主动脉大小、血压以及最后一次随访时的左心室功能和肥厚情况，并检查了可能的预后因素。除1例患者外，所有个体在对应狭窄的水平上z评分均低于-2。左心室功能障碍在年龄<5岁的患者中更常见（p = 0.024）。特发性MAS患者在血压和随访主动脉大小方面预后较好（分别为p = 0.051和0.048）。CT测量的主动脉z评分可能有助于MAS的诊断和随访。
