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Middle Aortic Syndrome: Long-Term Surgical Outcomes
Young Erben, MD, Peter Gloviczki, MD, Gustavo S. Oderich, MD, Audra A. Duncan, MD, Manju Kalra, MD, Mark D. Fleming, MD, Randall R. De Martino, MD, Thomas C. Bower, MD.
Mayo Clinic, Rochester, MN, USA.

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.

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