Pediatric Nephrology

, Volume 28, Issue 10, pp 2023–2033 | Cite as

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

  • Diego Porras
  • Deborah R. Stein
  • Michael A. Ferguson
  • Gulraiz Chaudry
  • Ahmad Alomari
  • Khashayar Vakili
  • Steven J. Fishman
  • James E. Lock
  • Heung B. Kim
Original Article



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.


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


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Copyright information

© IPNA 2013

Authors and Affiliations

  • Diego Porras
    • 1
    • 5
  • Deborah R. Stein
    • 2
  • Michael A. Ferguson
    • 2
  • Gulraiz Chaudry
    • 3
  • Ahmad Alomari
    • 3
  • Khashayar Vakili
    • 4
  • Steven J. Fishman
    • 4
  • James E. Lock
    • 1
  • Heung B. Kim
    • 4
  1. 1.Department of CardiologyBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  2. 2.Division of NephrologyBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  3. 3.Division of Interventional RadiologyBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  4. 4.Department of SurgeryBoston Children’s Hospital and Harvard Medical SchoolBostonUSA
  5. 5.Department of CardiologyBoston Children’s HospitalBostonUSA

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