Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management

作者：Porras Diego^*; Stein Deborah R; Ferguson Michael A; Chaudry Gulraiz; Alomari Ahmad; Vakili Khashayar; Fishman Steven J; Lock James E; Kim Heung B

来源：Pediatric Nephrology, 2013, 28(10): 2023-2033.

DOI：10.1007/s00467-013-2514-8

• Midaortic syndrome
• Hypertension
• Renal artery stenosis
• Atypical coarctation of the aorta
• Abdominal coarctation
• middle aortic syndrome

Midaortic syndrome is often associated with refractory hypertension. The aim of our study was to better understand the short- and medium-term outcomes in this patient population utilizing a multidisciplinary management approach.
We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years.
Fifty-three patients presented at a median age of 6.7 years (birth to 28.7 years). Thirty-five patients (66 %) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58 % at 1 year and 33 % at 5 years. Freedom from reintervention after a surgical procedure was longer: 83 % at 1 year and 72 % at 10 years. At the most recent follow-up, the majority of patients (69 %) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4-21.1) years. The median number of anti-hypertensive medications was 1 (0-5).
A multidisciplinary management strategy which couples comprehensive medical management with catheter-based and surgical interventions can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome.

• 出版日期2013-10