Middle aortic syndrome

Middle aortic syndrome (MAS), also known as midaortic syndrome, midaortic dysplastic syndrome, or abdominal aortic coarctation, is a rare vascular condition characterized by segmental narrowing (stenosis) of the abdominal or distal thoracic aorta. This narrowing often involves the renal arteries, mesenteric arteries, and other major branches of the abdominal aorta. The condition leads to severe renovascular hypertension, which is frequently refractory to medical therapy and can present in childhood. Patients may experience headaches, claudication of the lower extremities, abdominal angina, failure to thrive, and discrepant blood pressures between the upper and lower limbs. In severe cases, end-organ damage including left ventricular hypertrophy, renal insufficiency, and mesenteric ischemia can occur. Middle aortic syndrome can be congenital or acquired. Congenital forms may occur in isolation or in association with other conditions such as neurofibromatosis type 1, Williams syndrome, Alagille syndrome, fibromuscular dysplasia, or Takayasu arteritis. Acquired forms are most commonly linked to inflammatory arteritis. The exact etiology of the idiopathic congenital form remains unclear, though developmental abnormalities of the aorta during embryogenesis are suspected. Treatment depends on the severity of the stenosis and the patient's clinical status. Medical management with antihypertensive medications is often the first-line approach but is frequently insufficient to control blood pressure. Interventional procedures including percutaneous transluminal angioplasty with or without stenting may be employed. Surgical reconstruction, including aortic bypass grafting, patch aortoplasty, or renal artery revascularization, is often necessary in severe or progressive cases. Early diagnosis and management are critical to prevent complications such as heart failure, stroke, and renal failure. Outcomes have improved significantly with advances in surgical and endovascular techniques, though long-term follow-up is essential due to the risk of restenosis and ongoing vascular complications.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Middle aortic syndrome