Back to 2015 Annual Symposium Program

Objectives: The Middle Aortic Syndrome (MAS) is a rare pathology typified by segmental narrowing of the abdominal or distal descending thoracic aorta with branch involvement. Patients often present as children with severe hypertension, claudication, and or postprandial abdominal pain. We present our experience with surgical treatment for MAS.
Methods: Patients with MAS who had surgical reconstruction at our institution were identified. Symptomatology, timing of operation, comorbid conditions, type of reconstruction, resolution or improvement of hypertension, and secondary interventions were all assessed.
Results: There were eight patient with MAS who had surgical reconstruction with followup ranging from 9 months to 10 years. All patients presented with hypertension; two patients having claudication, two postprandial pain, and one who presented with stroke. The mean age at diagnosis was 6 with a mean age at repair of 10. Comorbid conditions included neurofibromatosis (3), Moya Moya (2), and Takayasu’s arteritis (1). Reconstructions included renal patch angioplasty (2), renal reimplantation (1), renal artery bypass (5) with aortic reconstruction (3) and mesenteric revascularization (2). There were no deaths and no patient required dialysis. All patients were on antihypertensive medications preoperatively (mean 3) with better control postoperatively (mean 1.8 ) with one patient remaining off all medications. Mesenteric and aortic reconstruction were undertaken only when patients were symptomatic with resolution postoperatively. No asymptomatic patient with mesenteric involvement has developed symptoms during follow-up. Two patients required secondary interventions (renal artery angioplasty).
Conclusions: Surgical reconstruction for patients with MAS is safe and durable. Despite renal revascularization, most patients continue to require antihypertensive medications postoperatively, but fewer medications overall. Mesenteric revascularization can be reserved for symptomatic patients at presentation. Asymptomatic mesenteric involvement in MAS can be safely treated conservatively.