Beyond hypertension: Clinical heterogeneity and cardiovascular outcomes in midaortic syndrome

INTRODUCTION: Midaortic syndrome (MAS) is a rare vascular disorder characterized by segmental or diffuse narrowing of the distal thoracic and/or abdominal aorta, frequently involving the renal and visceral arteries. We aimed to describe the clinical characteristics, cardiovascular findings, management strategies, and follow-up outcomes of patients with MAS treated at a tertiary referral center.
METHODS: This retrospective observational case series included all patients with radiologically confirmed MAS managed between 2010 and 2026. Demographic characteristics, clinical presentation, associated syndromic and cardiovascular abnormalities, electrocardiographic and echocardiographic findings, imaging studies, treatment strategies, and follow-up outcomes were reviewed.
RESULTS: Five patients were included. Hypertension was present in four patients at diagnosis, and the remaining patient developed hypertension during follow-up; all patients were managed medically. Three patients had associated genetic syndromes (Turner, Williams and Noonan syndromes), and four had concomitant congenital cardiovascular abnormalities. Three patients had undergone cardiac catheterization before the diagnosis, one of them with balloon angioplasty of coarctation of the aorta, whereas none required surgical reconstruction or bypass surgery for MAS. No deaths, heart transplantation, ventricular assist device implantation, or sustained arrhythmias occurred during follow-up. Notably, this cohort includes the long-term follow-up of a previously published patient with Turner syndrome and MAS from our institution, extending the available clinical data beyond the initial case report.
DISCUSSION AND CONCLUSION: MAS demonstrates substantial clinical heterogeneity and is frequently associated with congenital cardiovascular abnormalities and genetic syndromes. Early diagnosis, comprehensive vascular imaging, individualized management, and long-term follow-up may contribute to favorable cardiovascular outcomes.

Keywords: Midaortic syndrome, Hypertension, Pediatrics