Mid-aortic Syndrome: A Rare Cause of Juvenile Hypertension

A 49-year-old woman with a history of juvenile hypertension since 4 years of age was referred to our hospital. She had previously experienced a subarachnoid hemorrhage at 18 years of age. Her blood pressure (BP) had been wellcontrolled (under 130/90 mmHg) with antihypertensive treatment. Contrast-enhanced computed tomography showed a narrowing of the interrenal abdominal aorta with ostial stenosis of the right renal artery (Picture A: frontal view; Picture B: frontal view excluding the arc of Riolan; Picture C: posterior view). Collateral circulation had developed through the arc of Riolan (arrow heads). She had no history of either any systemic symptoms or elevated inflammatory markers. Therefore, a diagnosis of congenital mid-aortic syndrome (MAS) was made. MAS is characterized by the segmental narrowing of the descending thoracic and/or abdominal aorta (1), which is one of the important causes of juvenile hypertension. MAS patients with well-controlled BP have the potential to achieve a good prognosis without the need for aortic reconstruction by invasive intervention.