Computed Tomography Measurement of the Aorta in Midaortic Syndrome in Children and Adolescents and Their Clinical Manifestations and Outcomes

Abstract

Background Midaortic syndrome (MAS) presents challenges in diagnosis due to the absence of well-defined diagnostic criteria, and its clinical features remain uncertain. In this retrospective study, we 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. Methods 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 at the last follow-up were analyzed, and possible prognostic factors were examined. Results With the exception of 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). Conclusions CT-measured aortic z-scores may be useful for the diagnosis and follow-up of MAS. A more conservative management plan should be considered for selective idiopathic patients.