Middle Aortic Syndrome: Long-Term Surgical Outcomes

Abstract

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. Twenty-three patients who underwent operative intervention for MAS from January 1976 to December 2014 were retrospectively reviewed. Patient presentation, comorbid conditions, and operative approach were reviewed. The primary outcome was operative morbidity and mortality. Secondary outcomes included long-term survival, patency, and need for reintervention. 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 causes. Aortic coarctations involved the suprarenal (n = 4), intrarenal (n = 17), and infrarenal (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). Postoperative 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%) died in relation to the index operation after a prolonged hospitalization. Mean follow-up was 129 ± 133 months (range, 3-457 months). Overall survival was 91% at 15 years. All grafts were patent at follow-up; however, seven patients (30%) required reintervention because of graft stenosis and pseudoaneurysm formation. Operative intervention for MAS is safe and offers excellent long-term outcomes with a limited reintervention rate.