Middle Aortic Syndrome Treated by Implantation of an Advanta V12 Large Diameter Stent

Middle aortic syndrome (MAS) was coined by Sen et al.1 depicting diffuse hypoplasia or stenosis of the distal thoracic aorta and abdominal aorta. Most often MAS is idiopathic2; however, it may be associated with a variety of underlying diseases.2,3 Systemic hypertension, which is the most common feature of MAS, is usually found incidentally.2 Hypertensive encephalopathy was seen in 42% of patients with abdominal aortic coarctation, 45% of whom died before reaching 34 years of age.4 Thus, early recognition of hypertensive encephalopathy is crucial for patient survival. We report of a 12-year-old girl presenting with hypertensive encephalopathy as a harbinger of idiopathic MAS, which manifested as an isolated long-segment mid-thoracic aortic coarctation, and was successfully treated by endovascular implantation of an Advanta V12 Large Diameter Stent (AVLDS, Atrium, Hudson, NH, USA).