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

BACKGROUND: 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.

METHODS: We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years.

RESULTS: 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).

CONCLUSIONS: 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.