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Objective: Middle aortic syndrome (MAS) is extremely rare. The aim of this study was to review the treatment of this pathologic entity from a single institution.
Methods: Twenty-three patients who underwent operative intervention for MAS from January 1976 to December 2014 were retrospectively reviewed. Patient presentation, co-morbid conditions and operative approach were reviewed. The primary outcome was operative morbidity and mortality. Secondary outcomes included long term survival, patency and need for re-intervention.
Results: Of the 23 patients, 17 (74%) were female. The mean age was 21 years (range: 5-52 years). Clinical manifestations included: hypertension (n=23), claudication (n=18), abdominal pain (n=6) and dyspnea (n=3). Takayasu arteritis (n=8), neurofibromatosis type 1 (n=6) and tuberous sclerosis (n=2) were the most common etiologies. Aortic coarctations involved the supra-renal (n=4), intra-renal (n=17) and infra-renal (n=2) aorta. Aortic reconstruction included: descending thoracic to infrarenal aorta bypass (n=12), ascending to infrarenal aorta bypass (n=4), patch angioplasty (n=4), infrarenal aorta to bilateral iliac arteries bypass (n=2) and bypass to visceral vessels (n=1). Post-operative complications occurred in four patients (17%) including hemorrhage, abdominal compartment syndrome, delayed fascial closure, liver failure, ileus, acute kidney injury and tracheostomy. One patient (4%) expired related to the index operation after a prolonged hospitalization. Mean follow up was 129±133 months (range: 3-457months). Overall survival was 91% at fifteen years. All grafts were patent at follow up, however, seven patients (30%) required re-intervention due to graft stenosis and pseudoaneurysm formation.
Conclusion: Operative intervention for MAS is safe and offers excellent long term outcomes with a limited re-intervention rate.