Overview
Middle aortic syndrome (MAS) is characterized by severe narrowing of the distal thoracic and/or abdominal aorta, predominantly observed in childhood, often with idiopathic etiology and associated extra-aortic vascular involvement 1.Diagnosis
Clinical Presentation: Symptoms may include hypertension, abdominal or back pain, and signs of organ ischemia depending on affected arteries 1.
Imaging Studies: Essential for diagnosis; typically includes computed tomography angiography (CTA) and magnetic resonance angiography (MRA) to assess aortic narrowing and extra-aortic involvement 1.
Vascular Phenotype: Common involvement includes renal (70%), superior mesenteric (30%), and celiac (22%) arteries, with rare inferior mesenteric artery involvement 1.Management
Surgical Interventions: Majority of cases (72%) may require endovascular or open surgical interventions to relieve aortic stenosis 1.
Medical Management: Focuses on controlling hypertension and managing complications; specific drug classes not detailed in provided abstracts 1.
Supportive Care: Includes close monitoring of organ function, particularly renal and gastrointestinal systems, due to potential ischemia 1.Special Populations
Pediatrics: MAS predominantly affects children, with idiopathic cases being most frequent (64%) 1.
Comorbidities: Often associated with Mendelian disorders (15%) and inflammatory diseases (17%), impacting management strategies 1.Key Recommendations
Imaging with CTA or MRA is crucial for diagnosing MAS and assessing extra-aortic involvement (Evidence: Moderate 1).
Endovascular or surgical interventions are often necessary for managing significant aortic stenosis in MAS (Evidence: Moderate 1).
Close monitoring of renal and superior mesenteric artery function is essential due to high involvement rates (Evidence: Expert opinion 1).References
1 Rumman RK, Nickel C, Matsuda-Abedini M, Lorenzo AJ, Langlois V, Radhakrishnan S et al.. Disease Beyond the Arch: A Systematic Review of Middle Aortic Syndrome in Childhood. American journal of hypertension 2015. link